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EXTRA-ABDOMINAL DESMOID TUMOURS

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					                                                            EXTRA-ABDOMINAL                                                                                 DESMOID                                                TUMOURS

                                                                                                             A.        McDOUGALL.                             G.       McGARRITY

                                                    From               the       Division                of        Orthopaedic                            Surgery.                  Victoria                   Infirmary,                          Glasgo                 ii’


                   Desmoid     tumours     are not common       but have a distinct    resemblance        to fibrosarcomata.                                                                                                                                                   Their                      clinical
            appearance       and progress      should    be recognised   since failure     to distinguish       them from                                                                                                                                                 sarcomata                          could
            result   in extensive      and unnecessarily      mutilating   operations.
                         Three             cases           of extra-abdominal                                      desmoid                  tumours                   are          reported.                        Two             of the                patients                     had          tumours
            arising   from multicentric    foci in the same limb. The disappearance                                                                                                                ofthe               tumours    in two patients   after the
            menopause,     and the variation    in the tumours   during the menstrual                                                                                                              cycle              in the third patient,    add weight   to
            the      theories                   about           endocrine                    control.


           The           term            desmoid                    tumour               is used                  to        describe                 a         of adjacent                          bones.   Histologically                                            they resemble     cellular
curious            neoplastic                     proliferation                          of fibrous                     tissue             with                fibromata,                         the individual       cells                                      being    uniform   in size and
some             of both a benign
             features                   fibroma    and a malignant                                                                                             shape                ; the absence                            of mitotic                     figures                   and         giant             cells         helps
fibrosarcoma    (Robbins      1962).   It was first mentioned       by                                                                                         to distinguish                              desmoid                            tumours                           from               fibrosarcomata
Dr J. McFarlane      of Glasgow      in 1 832 when he recorded                                                                                                 (Figs.   1 and                            2).
the histories   of two patients        with organising      tumours                                                                                                           We           report                  three            cases            of extra-abdominal                                                 desmoid
between             the muscular     layers     of the abdominal       wall. The                                                                              tumours;     two  of them        featured                                                                  multicentric                            foci in a
desmoid              tumour    found    in the abdominal         wall of some                                                                                 single   limb,   a characteristic                                                                     first     noted                           by Barber,
women              who have borne        children     is well recognised     and                                                                              Galasko     and Woods      (1973).
described   in textbooks   of surgery    and pathology.                                                                                  It has
been stated   that the majority    of desmoid    tumours                                                                                  occur                                                                                    CASE             REPORTS

in women,                 and           their       frequency                    after          pregnancy                      has been                        Case         1. A woman                         aged          twenty-five                    years          was         referred               to our           clinic             in
attributed                     to       the         violent                   muscular                   contractions                             of           1 949          on      account                 of a swelling                  of her              right         foot.         It consisted                   of a hard
                                                                                                                                                               mass           involving                 the        forefoot,              palpable                both          on the             dorsum               and       on the
parturition.                        Desmoid                    tumours                   are          said             to      be          most
                                                                                                                                                               sole      and          fixed            to the         metatarsals.                   The           patient             could            not      say        how            long
common                in the third                          to fifth   decades                                but they  may                                    the       swelling                 had          been            present,               but          for         several              weeks              before               her
present            at any age and                         in either  sex. Apart                               from the more                                    attendance                      it had         increased               rapidly              in size            and       she        had        been          unable                to
common                   abdominal                        tumours,                     histologically                          identical                       put       on         her        shoe.
tumours             occur               in other               parts          of the body,                    particularly                        in                      Radiographs                          showed                inyolvement                         of      the         metatarsals                      (Fig.          3).
                                                                                                                                                               Because                of the            history            and        radiographic                        appearance.                      the       question                 of
the shoulder      girdle   and arm,                                               in the thigh                              and in the
                                                                                                                                                               malignancy                       was           discussed               and           biopsy               undertaken.                       The           Pathology
buttock     where     they   present                                              considerable                               diagnostic                        Department                        reported               an     “   active”           fibroma                  but      did        not      consider              it to be
problems.                                                                                                                                                      malignant.                      As much               of the          tumour               mass           as possible                 was        excised,               but         it
        The          features      of the                       desmoid      tumour      are                                 that it is a                      was       recognised                     that         excision             was        far         from          complete.                  The        patient                had
proliferation               of fibroblasts;                         it is locally   invasive                                    but does                       informed                   us at the                time         of her          admission                      that         she     was          three          months
                                                                                                                                                               pregnant.                   Physical                examination                      was          normal                in all other                  respects.                 as
not      metastasise;                      it does             not       arise          from          muscle                but         rather
                                                                                                                                                               were           the         biochemical                     investigations.
from        the      musculo-aponeurotic                                          structures                  of the              body;              it                   A recurrence                          of the             growth            was          excised              one          year         later         and          this
is, properly                   speaking,                  a tumour                    of the supporting                                 tissues                patient              had        no further                 trouble            until          1 953,         when             a lump            appeared                     over
of muscles.                The           presenting                    sign           is a firm,          rapidly                 growing                      the       right            adbominal                   wall.          At      this          time          she          was         again         three           months

swelling.            Pain is not                          a marked                      feature           unless                  there              is        pregnant                   so she         was         referred             to the           Gynaecology                        Department                       where               a
                                                                                                                                                              classical              abdominal                       desmoid               tumour                 was         removed.
pressure            on surrounding                          structures.                      The         overlying                   skin            is
                                                                                                                                                                          In 1955                a rapidly              growing              lump,               about          the size            of a closed                 fist and
freely   movable      but the                                   tumour                 is fixed            to the involved                                    causing               discomfort                  when           she        sat down,                appeared                  in the upper                   posterior
muscles.    A histologically                                      similar              tumour             is found  in bone                                    third of her right thigh.                                     It had          invaded    the hamstring                                     muscles              but was
(Jaffe        1958).                                                                                                                                           mobile   in the transverse                                     plane,          and its removal      did                             not     present              a great

           Microscopically                               the         lesions              are         grey-white,                          firm,               problem                    though               the        bellies            of       the           involved                  muscles                  had            to      be
                                                                                                                                                               sacrificed.                 The         histological                  findings              were          as before,                but        the      pathologist
non-encapsulated     and poorly      demarcated,           varying   in
                                                                                                                                                               felt      that             we     had          to      consider               the          possibility                  of     fibrosarcoma.                           After
size from 1 to 20 centimetres.     The tumours          are firm and                                                                                          discussion,                      however,               it was         agreed               that      the        tumour              was        a desmoid                     and
rubbery,   invading muscles    and muscle      bundles;       in two of                                                                                        that       a further                extensive                   operation                   was       not         indicated.
our      cases           the         tumour              had         invaded                 the      trabecular                        spaces                            In         1961          a lump                 appeared                   in      the         right          thigh             proximal                to         the

A.     McDougall.                   F.R.C.S.,            Consultant              Orthopaedic                  Surgeon               .     Victoria          Infirmary.                Glasgow                   G42           9TY.           Scotland.

G.     McGarrity,               F.R.C.S.,           Senior             Orthopaedic                   Registrar                      J
Requests           for     reprints             should         be      sent      to     Mr      G.     McGarrity.


VOL.       61-B,     No. 3. AUGUST                         1979                                                                                                                                                                                                                                                                             373
374                                                                                                                                    A.         McDOUGALL,                                                                      G.                    MCGARRITY

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      Photomicrograph                                                    showing      the                  main       histological   features       of a desmoid     tumour.     The   picture      is that                                                                                                                                                                                                                                   of a fibroma                                              with
      benign-looking                                             cells      in a fibrous                   matrix.       The absence     of mitotic    figures   and giant cells helps    to distinguish                                                                                                                                                                                                                                     these tumours                                             from
                                                                                                                   fibrosarcomata.       (Haematoxylin         and eosin,    x 125.)




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                                                                                                                                                                                                    Fig.                          2

      Photomicrograph                                                  showing  the interface                               between     desmoid     tumour    and                                                                                                   muscle.   The close                                                           apposition                                 between                                             the            tissues                       is seen
                                                                        with an inflammatory                                   cell exudate     at the interface.                                                                                                     (Haematoxylin                                                               and eosin,                                 x 125.)


                                                                                                                                                                                                                                                                                                                                    THE            JOURNAL                                       OF BONE                                                 AND                       JOINT                   SURGERY
                                                                                                                            EXTRA-ABDOMINAL                                         DESMOID                     TUMOURS                                                                                                                                                           375




previous               tumour.                It was           fixed             deeply             to        the      ischium                   and          produced               Case         2      A woman                        aged           thirty-seven                           on         first         attendance                           presented                     at
symptoms                 from          pressure              on the              sciatic          nerve.             At operation                       the       tumour             the        Orthopaedic                             Clinic             in         1971                  with           intermittent                              backache                       and
was        more            extensive                 than          had          been             apparent.                  It had               infiltrated               the       left-sided                 sciatica              of several                   years           duration.                        She         had           also          noted             a firm
semitendinosus                         and          semimembranosus,                                     extended                    under              the       gluteus            swelling                  on          the          posteromedial                                  aspect                    of          the          thigh                which                was
maximus                involving               it and         the         pyriformis,                    enveloping                       the      sciatic          nerve,           occasionally                       painful.
which            was       dissected                free      with          great            difficulty.               The          greater               part        of the                    Examination                             revealed                 a firm                 swelling                      about              the         size         of a large
involved               muscles               had      to be sacrificed                            in its removal.                          The          pathologist                  apple            lying           between                  the         hamstrings                          and            the           adductor                      muscles.                  The
who        studied             the      specimen                  said          that         he would                 regard              it as a low-grade                          swelling                was           smooth                  and          rounded                      and           attached                       proximally                         to         the
fibrosarcoma;                        once          again          after         discussion                    the     section               was         accepted               as    muscle              origins                 around                 the          ischiopubic                           ramus;                    it was                 not         painful.
that       of a desmoid                       tumour.                                                                                                                                Radiographs                           of         the          lumbar                  spine               and               pelvis                 revealed                      no          bony
                                                                                                                                                                                     abnormality,                       and           the      history               of backache                           and             sciatica             was            not      thought
                                                                                                                                                                                     to be related                     to this           swelling                in the           thigh.                Routine                    physical                 examination
                                                                                                                                                                                     and        laboratory                      tests          were           negative.
                                                                                                                                                                                                Exploration                        and           excision                of the               tumour                   was          undertaken                          in April
                                                                                                                                                                                     1 97 1 . A firm                    tumour                 was encountered                                     beneath                    the semimembranosus
                                                                                                                                                                                     and       adductor                    magnus;                   the        latter           muscle                   was              infiltrated                    by strands                      of
                                                                                                                                                                                     tumour                 tissue            particularly                      near             its         origin.              The              tumour                   was            densely
                                                                                                                                                                                     adherent                  to the           sciatic            nerve           at the              upper              border                of the               adductors.                     The
                                                                                                                                                                                     bulk        of the              tumour                 mass           was        excised                  but         it was               realised                  that         excision
                                                                                                                                                                                     was        incomplete.                        Histological                            examination                                of      the         specimen                         showed
                                                                                                                                                                                     what         was          thought                 to be a well-differentiated                                                    fibrosarcoma;                               on further
                                                                                                                                                                                     study            the           diagnosis                  was         revised                     to      that            of          a musculo-aponeurotic
                                                                                                                                                                                     fibromatosis.                          The             pathologist                        added                    that               while              the         tumour                    was
                                                                                                                                                                                     unlikely               to metastasise                           it was           likely                to recur                  locally                 and         to spread,                     an
                                                                                                                                                                                     opinion                which             events               proved              to be                correct.
                                                                                                                                                                                                Between                    June              1 972         and          April                1 974            the           patient                 underwent                       five
                                                                                                                                                                                     further            explorations                         of the           posterior                      aspect              of the             thigh             for       excision                  of
                                                                                                                                                                                     the       recurrent                    tumour.                  On         each             occasion                      a marked                         feature                 was             the
                                                                                                                                                                                     involvement                        of        the        sciatic             nerve                 and          after              the          third             operation                         the
                                                                                                                                                                                     patient             developed                          a foot              drop              which                  has           persisted.                         At         the          sixth
                                                                                                                                                                                     operation                  the        tumour              was         found              to have                 entered                  the        pelvis               through                  the
                                                                                                                                                                                     greater             sciatic             notch             in company                         with             the         sciatic              nerve.                At         this         point
                                                                                                                                                                                     the      opinion                ofa        radiotherapist                           was           sought,                 to determine                           the         possibility
                                                                                                                                                                                     of treatment                      since            reports            indicated                        that        the         tumour                   became                  quiescent
                                                                                                                                                                                     after        the          onset            of the             menopause                           (Musgrove                             and         McDonald                            1948).
                                                                                                                                                                                     Endometrial                        curettings                   were          normal                    so an induction                                 of the            menopause
                                                                                                                                                                                     was       carried                out         by        irradiation                       when             the         patient                   was            aged             forty-one
                                                                                                                                                                                     years.
                                                                                                                                                                                                In the                four         years             since            the         treatment                           there              has          been             no         local
                                                                                                                                                                                     recurrence                      in the            thigh           and         the         patient                  has           been              in good                 health,                 the
                                                                                                                                                                                     only        remaining                       feature               being           the         foot             drop.
                                                                                                                                                                                     Case 3. A girl aged                                sixteen     years                     came to the Orthopaedic                                                    Department
                                                                                                                                                                                     in December    1 974                               complaining                           of a swelling above   the                                              inner aspect                         of
                                                                                                                                                                                     the       right           elbow.              She         had          noticed                    the         swelling                   over            a period                     of nine
                                                                                                                                                                                     months,                but        it had            recently               become                      larger             and           slightly                painful.                 There
                                                                                                                                                                                     was       a diffuse,               firm           swelling               extending                       from             the          medial              epicondyle                        for         a
                                                                                                                                                                                     distance               of five             centimetres                      up the                 arm;             it was              firmly             adherent                      to the
                                                                                                                                                                                     underlying                     structures.                    Radiology                      revealed                       erosion                 of the                humerus                    at
                                                                                 Fig.        3
                                                                                                                                                                                     the       site      of the               tumour                 (Fig.           4).       Physical                    examination                               was          otherwise
                                 Case      I. Radiograph         of the right      foot    in
                                                                                                                                                                                     normal.             At operation                          a firm           fibrous                 tumour                   was          found             arising               from              the
                                  1 978 showing         the changes    in the metatar-
                                 sal    and     phalanges       of the    second        ray.                                                                                         common                    flexor            origin,             infiltrating                      the         muscles,                    the           joint          capsule                 and
                                 Similar     changes       had been present      in 1949.                                                                                            the       underlying                        bone;             the          ulnar            nerve                  was            in      the            substance                      of         the
                                                                                                                                                                                     growth             and           great           difficulty                was           experienced                              in dissecting                           it free.            The
          Towards                the        end      of 1 963              there           was       recurrence                     of the             last      growth,             pathologist                      who          examined                     the           sections                   said          that             the         possibility                    of a
which            was        removed                   with          considerable                         difficulty;                  the          pathological                      low-grade                      fibrosarcoma                           had           to       be          considered,                               but          he        obtained                       a
findings            were         identical                 to those               in the            previous                 report.                                                 second             opinion                 and         it was         agreed                that          it was               fibromatosis.                              The          patient
          In I 966           this patient                   reported                  once        more          with         a tumour                   mass        low        in    returned                  to      work              but         was           seen            in         August                       1 976              with          a local                 and
the      thigh         extending                  across       the         popliteal                fossa           into      the         calf      muscles,               the       extensive                  recurrence                       of the              tumour;                       movements                            of      the         elbow                 were
bulk       of which              interfered                 with          the      function              of the             knee.           A large              tumour              restricted                 and          the         joint           was         fixed              in flexion.                          Radiography                              revealed
was      excised            with           difficulty,              the         operation                entailing                  the      removal               of the            increased                  erosion                 of       the        lower              shaft               of      the              humerus                    (Fig.            5).        The
biceps           tendon,             part      of gastrocnemius                               and        soleus;             this         resulted             in a foot             tumour              was           excised                with         great              difficulty;                      again               the         ulnar              nerve             was
drop       which           was       controlled                by a caliper.                      During              the     next          two         years          there         completely                      enveloped                     and          spared                 only             by tedious                       dissection.
was       no      recurrence                  of the          tumour                   and        an improvement                                 in strength                   of               Professor                     Mackenzie                         of         the              Department                              of         Histopathology,
the      leg      enabled              her         to discard                   the      caliper.                                                                                    Westminster                           Medical                   School,                   saw             a        section                    of         the         tumour                    and
          Since          the         onset           of     the       menopause                          in         1968        there             has          been         no      diagnosed                       musculo-aponeurotic                                          fibromatosis;                                his        report                concluded,
recurrence                  of       the          tumours.                 At          present                the      only               evidence                of       the      “There               is no doubt                        this       will        recur           and             will        continue                       to do so until                            the
multiple            tumours                 is the         extensive                  scarring            of her            many             operations                  and        lesion             has           been          completely                         excised.”                         His            prediction                         was          correct:
weakness                of dorsiflexion                       of her              foot.                                                                                              within            three          months                 there           was         evidence                    of return                      of the            growth                 on the



VOL.        61-B,          No.        3. AUGUST                      1979
376                                                                                                                                     A.      McDOUGALL,                           G.   McGARRITY


 inner           aspect           of        the      elbow.                   It was         decided               to       temporise                  and       to    our
                                                                                                                                                                                      known         but        have          been        reported         in the           literature           with
 gratification                  the          tumour               began              to     diminish               in      size.
                                                                                                                                                                                     conflicting            theories            about     their          aetiology     and              the     best
           In       April              1977.            however.                    a second                 tumour                   appeared               over       the
 deltoid           region             on the            same            arm         and     grew            rapidly,              involving            the      deltoid              form      of       treatment               (Musgrove                and     McDonald                     1948;
 and       part           of the            triceps.              It was            fixed          deeply             to        the      humerus,               though                Strode     1954;   Ramsey     1955;   Hart,                             Morgan            and Acker-
 there        was         no radiological                             evidence              of involvement                            of the      bone.         By this               man     1960;    Enzinger    and Shiraki                                1967).           When    these
 time       the       tumour                 on      the         inner           side       of the           arm         had          resolved            and       could             lesions     do    occur   extra-abdominally,                                          they    have     a
 not       be palpated                      (Fig.          6).        The        two        tumours                were            discrete         and         had       no
                                                                                                                                                                                      predilection        for the muscles                           of the shoulder         girdle   and
 connection.                    ln         view         of the               behaviour                of the            first         tumour           a policy             of
 waiting            was         adopted,                  but          in January                  1978         the         patient             complained                  of        upper      arm, the buttock        and                         the thigh.       In all previous
  pain      in the            deltoid               region.                  A red         fluctuant               area           in the         middle             of the            reports      except    one, the purely                          extra-abdominal          desmoid




                                                                                Fig. 4                                                                                           Fig. 5                                                      Fig. 6

                                           Case    3. Figure    4-Radiograph                                                    of the right        elbow    on first presentation      in December          1974.      There     are                            erosive
                                           changes     on the medial       epicondyle                                              and supracondylar           ridge of the humerus.        Figure   5-The         right elbow        in                          March
                                           I 976. The erosive       changes       have                                          greatly     enlarged,     and the tumour-bone         interface    is much      more irregular.                                   Figure
                                           6-The       right elbow     in September                                                  I 977. At the time of this radiograph             the tumour       at the medial          aspect                              of the
                                           elbow     had diminished         and was                                             impalpable,           and improvement        was seen     in the radiological          appearance                                  of the
                                                                                                                                                            humerus.


  tumour             was        explored                   and          found             to be a haematoma,                                 probably            due        to       tumours             have   been                  unicentric          in origin             with          direct
  injury         at work.               A large                section           ofthe           tumourwasexcised,                                part of which                      spread;           the exception                   was the         report    by           Barber           et a!.
  was      sent         for     histological                      examination                         and      part         for        hormonal               assay.         It
                                                                                                                                                                                     (1973)     of two cases in which            extra-abdominal             desmoid
  had      been           noted            that        pain           was       a variable              feature,                and      when          questioned
  the      patient             stated              that          her         pain         was      worse            premenstrually;                           she      also
                                                                                                                                                                                     tumours      arose   from     multicentric         foci in a single         limb.
  mentioned                   that         her      periods                  were       irregular.             Both             the cytoplasm                   and       the               The     three    cases      described          in this      report       are
  nucleus            from            the      tumour                  cell     contained                a significant                   level      of oestrogen                      considered       to be of interest         on three      grounds.        First,   in
  receptor,               and          a trial            of     tamoxifen                   was        recommended,                             10 milligrams                       two of the cases          the tumour          arose     from      multicentric
  three         times          daily.            This          drug           was       administered                     for       two        months            without
                                                                                                                                                                                     origins        in a single                limb,       as described               by      Barber           et a!.
  any       obvious               effect            on          the          tumour.             As     the        patient               was      a young              and
  premenopausal                             girl       it was            decided                on ethical                 grounds              to discontinue
                                                                                                                                                                                      ( 1 973).   Secondly,    in two of the reported                                   cases there              was
  the      drug           in the            absence               of clinical                   improvement.                                                                          involvement        of bone    which  increased                                  the problems                 of
                                                                                                                                                                                     diagnosis            and          treatment.             Thirdly,          the        course         of      the
                                                                      DISCUSSION                                                                                                     tumours            has       led us            to    suggest     treatment                  which         is at
  Desmoid       tumours                                     are unique                             in their                clinical    manifesta-                                    variance           with       Barber’s              recommendations.
  tion.    They     are                                   so locally                            invasive                    that    they     appear                                            These           three          cases        differed           greatly           from          those
  malignant        but                                    metastatic                              spread                    does      not     occur.                                 described        by Barber        et a!. (1973);           our patients      were
  Complete     surgical     excision   is usually  impossible         due to                                                                                                         female,     theirs     were male.      There       was nothing       to suggest
  involvement      of important      nerves    and blood     vessels,      and                                                                                                       that injury       was an aetiological          factor.     In two of the cases
  local recurrence       is common.                                                                                                                                                  the tumour         regressed     after the onset           of the menopause,
          The desmoid         tumour     of the abdominal             wall     is                                                                                                    this being      therapeutically         induced        in one patient     at the
  familiar.   Extra-abdominal           desmoids     are not so well                                                                                                                 age of forty-one.             In the youngest            of our patients       the


                                                                                                                                                                                                                       THE    JOURNAL           OF     BONE     AND        JOINT        SURGERY
                                                                                                                 EXTRA-ABDOMINAL                                    DESMOID                  TUMOURS                                                                                                                377

behaviour                             of her              tumours                 fluctuated                    during              her         mens-                  wide              local               excision.                  Seventeen                        tumours,                         however,
trual cycle,   and her periods      were   irregular.                                                                              These     facts                     recurred                      after           local            excision                    and            required                       further
tend to substantiate     the theories    that there                                                                              is endocrine                          operations,        one patient  having                                             four          more operations.     All
influence    on the course   of this condition,                                                                                as previously                           these     patients    were alive and                                              free          of recurrence     no less
noted                by Strode                        (   I 954)            and         Dahn,             Jonsson                  and        Lundh                    than            ten       years            after         initial         diagnosis,                     and           these           authors
(1963).                                                                                                                                                                concluded                         that           while           the           chances                    of        survival                 were
               There                   have            been              no      previous                 reports                 of      involve-                     excellent   the prognosis      in regard    to recurrence                                                                         was poor.
ment               of bone                   by a desmoid                         tumour                 as occurred                      in two           of                 While radical    amputation       will completely                                                                          ablate the
our cases.                            The        term desmoplastic                                   fibroma                    was        used by                     tumour                 in most              cases,          a less than                   radical              amputation                     may
Jaffe   (1                     958)              to describe       an                              intra-osseous                            fibrous                    leave             some             of it behind                     in the                stump                if the             tumour                is
tumour,                        distinct             from             fibrosarcoma,                         and            various            benign                    multicentric         in origin.     Also,     in one of our patients         the
fibrous                   lesions               of bone.                   The         term         desmoplastic                           fibroma                     tumour       entered       the pelvis      via the greater   sciatic    notch;
was used because                                           of histological                         similarity                    to the more                           we feel that          even     hindquarter        amputation      would      not
familiar desmoid                                          tumour      of the                      abdominal                       wall. These                          have              totally                 ablated                the           tumour                     in         such            a       case.
tumours,     however,     differ from the cases recorded                                                                                     here in                   Accordingly                           we         suggest            the          following                     procedure                     if an
that they remain       intra-osseous     and cause    bone                                                                                   expan-                    extra-abdominal                                  desmoid                 tumour                  is suspected.
sion. unlike     the destruction     from outside   caused                                                                                    in our                          Biopsy     should    be carried  out to verify the diagnosis
patients;    also, the desmoplastic                                                           fibroma    is intra-osseous                                              and distinguish          the tumour     from     fibrosarcoma.      If it
in its origin     and course.      Further                                                        cases of desmoplastic                                                grows    to such a size as to interfere      with function     then the
fibroma      have    been   described                                                         by Dahlin       and Hoover                                               tumour       should      be excised.    We agree          with Ramsey
(1964),                        Godinho,                      Chiconelli                      and         Lemos                   (1967)             and                (   1 955)             that        incomplete        local   excision                                       is preferable        to
Sugiura                        (1976).                                                                                                                                 sacrificing                      major    peripheral       vascular                                       and neural      struc-
               The             course             of the tumours                             in our             patients                incline            us          tures,            or sacrificing                      an extremity                         unless              there          is a risk             to
to make                         different    recommendations                                                to those                      made    by                   life.
Barber                    ci     a!. in I 973, although                                       we         must stress                      the basic                                  The         place             of hormones                          in the                treatment                    of these
differences                            of sex in the groups                                  reported.                    We        agree          with                tumours                  remains                   unclear.              In our                third           case,         a girl          aged
Strode                          (1954)                     that               desmoid                     tumours                         affecting                    sixteen,                study             of the            tumour               by techniques                              devised                for
musculo-aponeurotic                                                  structures                   outside                 the      abdominal                           breast                cancer               did        show          oestrogen                          receptor                   sites.           An
wall continue                                  to pose                    problems    in interpretation                                              and               anti-oestrogen                      agent,   tamoxifen,       was prescribed,        but
management.                                   Musgrove                      and McDonald      (1 948)                                     said      that               administration                     was not prolonged         in view of the lack of
many                surgeons                     have              not realised                   that         only        a radical               local               response                  of the tumour        and the age of the patient.
excision                        will          ablate               the        lesion.              Enzinger                     and          Shiraki                                  Why        multicentric     lesions    should    confine   themselves
(   1 967)                 reviewed                  thirty                 cases     of tumours     affecting                                       the               to one limb                   is not known.                                It is interesting                            that fibrous
upper                    arm and                  shoulder                    girdle,    with a follow-up                                          of at               dysplasia                  of bone    often                              affects    only     one                         half of the
least              ten           years           after              initial           diagnosis.                  They             found            that               skeletal                system     and that                             it is histologically                              a develop-
thirteen                       patients                were           cured             by the             initial              treatment                  of          mental                 overgrowth                      of fibrous                    tissue            in bone.

             We    wish to thank      Mr T. H. Norton,     Consultant     Orthopaedic            Surgeon.       for permission       to study one                                                                                       of his cases.                 We also acknowledge                             with
thanks           the assistance     of Mr S. Saunders     and staff,   Medical      Illustration          Department;         Dr W. G. S. Spilg,                                                                                         Consultant                   Pathologist. and Mr                           A. W.
Kerr.          Senior    Chief   Scientific Officer.   Pathology      Department;           and Miss         M. Mortimer,        Secretary.




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