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					                                                                                                                                                                                                                                                APRIL,             1976

                       CAVITATING                                                  LUNG     NODULES                                                                AND   PNEUMO-
                        THORAX                                       IN            CHILDREN       WITH                                                              METASTATIC
                                                                                    WILMS’     TUMOR*
                                          By     E.     GEORGE                 KASSNER,                         M.D.,t   HAROLD                             S. GOLDMAN,                                      M.D.4
                                                                             and ALBERTO                           ELGUEZABAL,                               M.D.
                                                                                      BRONX             AND          BROOKLYN,           NEW          YORK


              Lung  metastases       evolved       into                                large thin-walled     Cysts in two children          with Wilms’
          tumor.   Histologic       examination                                        of one lesion     suggested       that   invasion      of a small
          bronchus     or bronchiole,          leading                                    to ball-valve    obstruction,       was responsible.        One
          patient  also experienced          recurrent                                    pneumothorax,      probably      on the same basis.
              The formation      of thin-walled                                        cysts   has not previously       been observed        with meta-
          static              Wilms’                   tumor.             Pneumothorax         is a rare  complication        of metastatic       Wilms’

 C AVITATION               of pulmonary       metastases                                                                            A second                  course                of radiation                             therapy                 was ad-
       is very rare in children.         This phenome-                                                                        ministered                    to         a small                  field              that              encompassed
non     has   been     reported      in three     children                                                                    the metastasis                           in the left lung                                      (Fig.          iA).            After
                                                                                                                              750       rads          of a planned                           tumor                 dose              of     2,250                rads
with metastatic          Wilms’    tumor.3’5     We have
                                                                                                                              had        been          delivered,                       a cavity                    was              noted                within
observed      two children        with    Wilms’     tumor
                                                                                                                              the      metastasis                      (Fig.            iB).         This               cavity              continued
in   whom       pulmonary        metastases       evolved
                                                                                                                              to      enlarge               and         the          heart              was              displaced                         to      the
into             large              thin-walled                    cysts.            One           of       these
                                                                                                                              right           (Fig.           i,       C and                   D).           The               patient                     had            a
patients                  had recurrent          episodes     of pneumo-                                                      cough            and          was          easily                fatigued                       but          he        was           not
thorax,                  a complication          that has rarely       been                                                   in       acute                distress.                      Radiation                                therapy                       was
associated                   with     metastatic         Wilms’    tumor.4                                                    stopped              after              1,050           rads           and            lingulectomy                                  was
                                                                                                                              performed,                      16       months                   after              the          appearance                              of
                                            REPORT           OF       CASES
                                                                                                                              the metastatic     deposit   in the left lung and 25
          CASE            I.  (Jewish                      Hospital                  and             Medical                  months    after nephrectomy.
Center                 of Brooklyn.)                      A.R. had                a right           nephrec-                      The resected     lung specimen     consisted      of a
tomy               for         Wilms’              tumor             at     two          and        one-half                  collapsed    empty    sac, which measured        4 cm    in
years              Tumor
                  of       was present
                             age.            in the renal                                                                     diameter,                     partially                      surrounded                                  by             a         solid
vein.    Lung metastases     were not detected         in the                                                                 tumor  which                          measured                    5X3.5X2                             cm.             Where                it
initial chest roentgenogram.         A course of actino-                                                                      did not abut                         the tumor                    mass,                   the         cyst            wall          was
mycin      D was begun     and 3,000      rads    were     de-                                                                1-2      mm            thick.
livered     to the tumor      bed using        Co-6o     tele-                                                                      Microscopically                                  the          tumor                       was               a     typical
therapy.      Six months       after   nephrectomy             a                                                             Wilms’                tumor;                     the          sarcomatous                                     component
pulmonary       metastatic   nodule    was seen in the                                                                        predominated                             and           there              were                 a few              scattered
right            lower              lobe.       Alternating                 courses            of        actino-              epithelial               areas.             Tumor                   completely                              surrounded
mycin              D and                 vincristine             were           administered                    and           the      cyst           and          extended                     either                  to      the         lining                epi-
300    rads were                          delivered           to a small     field encom-                                     thelium,            if present                    (Fig.              2,        ii      and             B),       or to               the
passing        the                        nodule.            Sixteen      months       after                                  lumen             in areas                      where                the              lining                 epithelium
nephrectomy                               another           small    pulmonary        meta-                                   was        absent.                 In      most              areas                  the          cyst             was             lined
static     deposit                         appeared           in the lingula       and 300                                   with         respiratory                          epithelium.                               There                  were              foci
rads             were           delivered                to this            lesion.          After            nine           of       squamous                     metaplasia                           or         flattened                        cuboidal
months                  without                change          the        nodule           in the            right           epithelium.   Where  lining epithelium       was ab-
lung             began              to      enlarge          and          was       excised              with          a     sent the cyst wall was composed      of a thin layer
cuff        of tumor-free                         lung.                                                                      of        tumor                covered                     externally                             by           subpleural

      *   From         the     Departments              of Radiology            of the     State        University         of New      York,       Downstate                  Medical           Center,t                and     the       Albert           Einstein
Medical           Center,           Bronx,      New      York4        and       the Department               of Pathology           of The      Jewish        Hospital              and      Medical              Center            of Brooklyn.

VOL.     126,    No.                                                    Cavitating           Lung         Nodules                                                                                   729

L                                                                                                                                                                    ___

FIG.    I. Case        ,.   (A) March              7. Twenty degree   right     anterior      oblique       proiection         of the chest    shows   a metastatic
    nodule    in the lingula.           (B) April i8. A cavity            is visible     within     the nodule.          (The changes       on the right-basilar
    parenchymal           infiltrate,       pleural     thickening      and elevation           of the hemidiaphragm-are                    the residua     of previ-
    ous surgery.)         (C and D) May              26. A large     thin-walled         cyst with several             ‘septa”      has developed      at the site of
    the nodule.        The heart          is displaced        to the right.      On the lateral          projection          the solid portion      of the tumor      is
    visible   anteriorly.          Lingulectomy           was performed          the following         day.

fibrous  tissue and pleura.                            Tumor   did not pene-                    ably         related           to      radiation           therapy                 were        promi-
trate the pleura.   Cartilage                            was not seen in the                    nence          of      the         endothelial             cells           of     some         vessels
cyst     wall.                                                                                  and squamous                        nietaplasia            in a few bronchi.
   Necrosis,     bizarre     nuclei   or other    radiation                                             Subsequently                     tumor            recurred                 in        the     left
changes      were not present       in the tumor.        There                                  lung,         mediastinuni,                      pleura        and               abdomen.               He
was no evidence        of acute radiation    pneumonitis,                                       died        at five          and     one-half         years          of age,            38 months
vasculitis         or       fibrosis          in      the     surrounding             lung      after        nephrectomv.                    Permission                    for     autopsy          was
parenchyma.                 The        only        features      that       were     prob-      not       obtained.
730                                                      E. G. Kassner,                       H. S. Goldman               and A. Elguezabal                                         APRIL,     1976

                                                                                                               four months        after     nephrectomy               he was re-
                                                                                                               admitted      with a four day history               of right chest
                                                                                                               pain. A chest roentgenogram                 showed       a pneumo-
                                                                                                               thorax    on the right and metastatic                   deposits      in
                                                                                                               both lungs (Fig. 3A). A chest tube was inserted
                                                                                                               and actinomycin         D and vincristine             were begun.
                                                                                                               A tumor       dose of I ,600 rads was delivered                      by
                                                                                                               moving     strip technique          (eight      fractions      in ten
                                                                                                               days)    to opposing         fields     that      included        both
                                                                                                               entire   lungs.   Cavitation          of several         metastatic
                                                                                       .    7-:’’’             deposits    was observed         about       three weeks after
                                                                                                               the completion       of radiation          therapy        (Fig. 3B).
                                                                                                               Some evolved       into thin-walled             cysts (Fig. 3C).
                                                                                                                   He was re-admitted            several       times for treat-
                                                                                                               ment       of    recurrent          right-sided           pneumothorax.
                                                                                                               Several             in the right lung reached
                                                                                                                               cysts                            enor-
                                                                                                               mous     size (Fig.    3D). He died at home at six
                                                                                                               years and five months of age, 38 months after
                            I                                                                                  nephrectomy.        Autopsy   was not performed.

                                                                                             -i                     Necrosis

                                                                                                                                       due to inadequate                     blood       supply
                                                                                                               -often            cited      as the major              cause        of cavita-
                                                                                                               tion in lung metastases2-rarely                                    if ever ac-
                                                                                                               counts           for cavitation               in small            nodules          or
                                                                                                               cavities          in which         the wall is extremely                       thin
                                                                                                               ( 1-2 mm).6 Breakdown                              of tumor              usually
                                                                                                               results        in cavities         with thick,             irregular         walls
                 ‘.                   ,/-_                                                                     which          do not           enlarge          rapidly           nor      reach
                                                                                                               great        size.2 Tumor               necrosis           does not ade-
                            Bt                                                                                 quately             explain         the      rapid           formation             of
                                                                                                               large thin-walled                  cysts       that occurred               in our
FIG.        2. Case              I.     (A)      Photomicrograph                      of     the     excised
       metastatic           nodule,      showing        an area      in which       the                        patients.
       cyst abuts          the main        tumor      mass. Tumor          is visible                              Anderson               and Pierce’             described             six pa-
      just       beneath         the lining        epithelium         of the cyst.                             patients           with       carcinomas             of the bronchus
       Two       types      of epithelium          are present        in this field:
                                                                                                               which          had       the roentgenographic                           appear-
       respiratory           epithelium        on the right         and flattened
       cuboidal        epithelium        on theleft.        (H & E, 300X        mag-
                                                                                                               ance of thin-walled                     cysts.      Grossly,           the cyst
       nification.)          (B) High        power.      The cyst lining          con-                         walls were smooth,                    gray,       and shiny.            Inflam-
       sists of respiratory               epithelium.         Tumor      is present                            mation,            if present,            was       minimal.              Micro-
      just      beneath        the epithelium.            (H & E, 750X          mag-                           scopically,              the       cyst        walls          consisted            of
                                                                                                               fibrous         tissue      and squamous                 cell carcinoma.
                                                                                                               The       lining        consisted          of malignant                 cells or
       CASE           II.        (Albert                Einstein            Medical            Center.)        squamous                 metaplastic               epithelium,                with
M.C.           had              a left         nephrectomy                    for locally             inva-    malignant              tumor       just      beneath            the surface.
sive       Wilms’                 tumor            at three              years        of age.        There
                                                                                                               In some instances,                    parts        of the cavity              wall
was no evidence   of pulmonary                                                          metastases.
                                                                                                               were formed                of compressed                lung. Occasion-
Actinomycin  D was administered                                                       and, because
                                                                                                               ally there           was continuity                between           tumor         in
the       tumor                 had          ruptured              during         surgery,            3,300
rads          were               delivered                 to      the       entire           abdomen          the cavity             and the nodule                 of growth             in an
using          Co-6o      teletherapy:                             the right               kidney     was      adj acent           bronchus.
shielded            after                                 rads.       Several              subsequent              Anderson                and        Pierce          attributed                the
courses               of actinomycin                            D were           given.       Twenty-          morphology                of these tumors                 to a ball-valve
 VOL.       126,     No.                                                            Cavitating                      Lung        Nodules                                                                                    73’

mechanism              :* Growth              of tumor            into a small                                         cavitation.             At thoracotomy,                   the pleura         was
 bronchus            caused          partial          obstruction                and                                   carefully            inspected.            When           the anesthetic
led to cystic                distention            of distal             air sacs.                                     bag was pumped,                      gas escaped             from a small
 Subsequent               growth         of tumor              into the cyst                                           hole        in the pleura                 overlying            the cavity.
 formed        a secondary                  lining         layer        of tumor                                       Both          the      cavity         and        the pleural             defect
 cells.    Rupture             of the lining                 of tumor          cells                                   were lined with tumor.
 by increases               in intnacavitary                     pressure         ac-                                       Lodmell            and Capps9                 adapted         the “air-
counted          for the portions                     of the cyst               that                                   block”           theory        of the Macklins                   to explain
were       lined          by respiratory                     epithelium             or                                 cases        in which            pleural        invasion          by tumor
compressed               lung.                                                                                         was         not       present.           They           suggested           that
     The      resected            metastasis                in our patient                                             peripheral            tumor        nodules          sometimes           caused
with       Wilms’           tumor        (Case          i)     was morpho-                                             partial         bronchiolar              obstruction.            Intercom-
logically        similar          to these         cases of bronchial                                                  munication                 between            alveoli         of adjacent
carcinoma.                A ball-valve                 mechanism,                 ne-                                  lobules         usually         prevented             the development
suiting        from          growth           of tumor               into     small                                    of excessive             local pressures,                but in some          pa-
bronchi          or bronchioles,                    satisfactorily                ex-                                  tients         strategically-placed                      tumor        nodules
plains      the initial           cavitation             and subsequent                                                produced               a ball-valve                effect.       Relatively
evolution           into thin-walled                   cysts          that    char-                                    small         changes            in intra-alveolar                 pressure,
actenized         the metastatic                  deposits             in both of                                      e.g., with coughing                     and sneezing,              could       re-
our patients.                Although             both         children          had                                   sult       in dissection                 of air along               vascular
received           radiation              therapy               and        chemo-                                      sheaths            and,       in some             instances,          lead       to
therapy,          we believe               that      these          changes         in                                 pneumomediastinum                            and         pneumothonax.
the metastatic                  deposits          were         the result           of                                 Lodmell             and Capps              cited        the presence             of
tumor        growth           rather         than      the consequence                                                 interstitial          emphysema               and subpleural              blebs
of therapy.                                                                                                            in one of their patients                        as evidence            in sup-
     No single             explanation              satisfactorily                ac-                                  port of this concept.
counts        for all instances                    of pneumothorax                                                          Regression             of tumor            and radiation              ther-
that have been associated                            with lung metas-                                                  apv do not appear                        to predispose               patients
tases.4”#{176}” Most               are probably                    due to the                                          with        lung        metastases                      to     pneumothorax.8”2
formation               of      malignant                  bronchopleural                                              Our        patient            (Case          II)        had          his       first      episode
fistulas.”        This mechanism                      was documented                                                   of pneumothorax                            before              radiation                  therapy
in one of the rare instances                               in which          pneu-                                     to the lung was                          begun.
mothorax             and cavitation                 were observed                   in                                 E. George Kassner, M.D.
the same            patient.          Kew7 described                       a teen-                                     Department         of Radiology
ager with            a solitary            subpleural               metastasis                                         State   University      of New                     York
of osteogenic                   sarcoma              that           underwent                                          Downstate    Medical  Center
                                                                                                                       450 Clarkson   Avenue
    *   Dodd       and     Boyle’       have      proposed          that     certain       properties         of       Box    45
squamous          epithelium’          account        for the thin-walled                cavitary        form          Brooklyn,            New      York         11203
of bronchial           carcinoma          and for thin-walled                cystic       metastases           of
squamous           cell     carcinoma.          As the growing                 tumor         breaks       into
alveoli,      malignant          squamous          epithelium        is likely        to come        in con-
tact with air; the process                 ofcornification          is facilitated           and respira-                  1.   ANDERSON,            H. J., and                PiERCE,            J. W. Carcinoma
tory      motion        assists     in stripping           the keratinized              surface        layers
                                                                                                                                   of bronchus       presenting                        as     thin-walled            cysts.
from cavity          walls.      Meanwhile,          growth       progresses          peripherally,           so
that      the size of the cavity                 increases       while      its wall maintains                  a                  Thorax,     1954,  9!, 100-105.
relatively        constant          thickness.         In some         instances,           a ball-valve                   2.   CHAUDHURI,                 M.     R.       Cavitary                  pulmonary             me-
mechanism-manifest                     on serial       roentgenograms               as an alternately
                                                                                                                                   tastases.         Thorax,           1970,        25,     375-38        I.
smooth       and crenated            appearance          of the external            wall-may            assist
in the growth            of thin-walled cystic neoplasms.                                                                  3.   COUSSEMENT,            A. M.,             and GOODING,      C. A. Cavi-
   Dodd    and            Boyle   suggested  that  inherent                       properties            of   the                   tating       pulmonary                 metastatic  disease   in chil-
normal   primary tissue might account                              for thin-walled  cystic me-
                                                                                                                                   dren.       AM.    J.     ROENTGENOL.,                      RAD.            IHERAPY        &
tastases that occasionally    occur with                           other tumors.   We are not
                                                                                                                                   NUCLEAR            MED.,        1973,            117,     833-839.
aware of any property      of embryonal                            tissue that would    explain
this phenomenon   in metastatic    Wilms’                           tumor.                                                 4.   D’ANGlo,          G. J., and              IANNACCONE,                    G. Spontane-
732                                                E.      G.      Kassnen,             H.        S. Goldman              and A. Elguezabal                                         APRIL,       1976

FIG.       3. Case           u.    (A)      September              21.     Metastatic             deposits    are    present        in   the hilar       lymph     nodes     and in the mid-
      zones        of both         lungs.        A small          pneumothorax                is present         right.
                                                                                                              on the        (B) November         3. The                    right lung has re-
      expanded.              Nodules            in both         lungs       have undergone       cystic change.        (C) November          20. There                    is a large pneumo-
       thorax         on     the right;
                                     a portion                           of the right    lower     lobe is adherent         to the parietal       pleura.                    Numerous         thin-
      walled      cysts are present      in both                           lungs.   (D) May      17 (five  weeks      before    death).      Numerous                      giant    thin-walled
      cysts have almost         totally    replaced                           the right lung and there is a marked               mediastinal       shift                 to the left. Several
      thin-walled        cysts are visible      on                        the left. There     is a small   pneumothorax            on the left. Solid                        masses     of tumor
      are present       in the mediastinum                               and in both lungs.

                ous        pneumothorax                    as    complication                of      pul-       6. DODD,    G. D., and BOYLE, J. J. Excavating         pul-
                monary            metastases              in malignant             tumors.           AM.             monary      metastases.       AM. J. ROENTGENOL.,
                J. ROENTGENOL.,                     RAD.         THERAPY            &   NUCLEAR                      RAD.    IHERAPY         & NUCLEAR   MED.,  1961,   85,
                MED.,        1961,        86,    I092-I         102.                                                     277-293.
 .       DECK,             F. W., and           SHERMAN,       R. S. Excavation                          of     7.    KEw,      M.        C.     Cavitating         pulmonary       metastasis
                metastatic               nodules      in lung:    roentgenographic                                      associated               with     spontaneous         pneumothorax.
                considerations.                Radiology,      1959, 72, 30-34.                                         Lancet,          1966,      86, 57 1-574.
VOL.    126,   No.   4                                Cavitating           Lung     Nodules                                                                               733

 8. LAuclus,        J. F., BRODOVSKY,       H. S., and HOWE,                  II.   SPITTLE,           M. F., HEAL,   J.,                         HARMER,          C., and
          C. D. Spontaneous pneumothorax                and pneu-                      WHITE,           W. F. Association                             of spontaneous
          momediastinum       as complications        of sarcoma.                      pneumothorax                      with         pulmonary           metastases             in
          7. Thoracic     & Cardiovasc.        Surg.,    1972,    6,                   bone          tumors        of      children.          C/in.     Radiol.,         1968,
 9.    LODMELL,      E. A., and      CAPPS,     S. C. Spontaneous
                                                                              12.   STOLZENBERG,                   J. G., and                     CLEMENTS,             J. P.
          pneumothorax       associated           with      metastatic
                                                                                       Bilateral              simultaneous                 pneumothorax                during
          sarcomas. Radiology,       1949,52,        88-93.
10.    SHERMAN,    R. S., and BRANT,            E. E. X-ray study                      radiation              therapy           for      metastatic        disease  from
          of spontaneous pneumothorax                 due   to cancer                  osteogenic               sarcoma:              case report        and review     of
          metastases to lungs. Dis.               Chest,    1953,    26,               literature.             Radiol.          C/in.       Biol.,     ‘970,       39, 437-
         328-337.                                                                      442.