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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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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.
REFERENCES
Barber, H. M., Galasko, C. S. B., and Woods, C. G. (I 973) Multicentric extra-abdominal desmoid tumours.JournalofBone andJoint Surgery.
55-B, 858-863.
Dahlin, D. C., and Hoover, N. W. (1964) Desmoplastic fibroma of bone. Journal ofthe American Medical Association, 188, 685-687.
Dahn, I., Jonsson, N., and Lundh, G. (1 963) Desmoid tumours. Acta Chirurgica Scandinavica, 126, 305-314.
Enzinger, F. M., and Shiraki, M. (1967) Musculo-aponeurotic fibromatosis of the shoulder girdle (extra-abdominal desmoid). Cancer,
20, 1131-1140.
Godinho, F. S., Chiconelli, J. R., and Lemos, C. (1967) Desmoplastic fibroma of bone. Journal ofBone and Joint Surgery, 49-B, 560-56 1.
Hart, R. T. N., Morgen, H. C., and Ackerman, L. V. (1960) Principles in the management ofextra-abdominal desmoids. Cancer, 13, 825-836.
JafTe, H. L. ( 1 958) Desmoplastic fibroma and fibrosarcoma. In Tumors and Tumorous Conditions of the Bones and Joints, pp. 298-303.
London: Henry Kimpton.
McFarlane, J. (1 832) Clinical Reports of the Surgical Practice of the Glasgow Royal Infirmary. pp. 63-66. Glasgow: D. Robertson.
Musgrove, j. E., and McDonald, J. R. (1948) Extra-abdominal desmoid tumors. Archives ofPathology, 45, 5 13-540.
Ramsey, R. H. ( 1 955) The pathology. diagnosis, and treatment of extra-abdominal desmoid tumors. Journal of Bone and Joint Surgery,
37-A, 1012-1018.
Robbins, S. L. ( I 962) Text-book of Pathology wit/i Clinical Application. p. 1 06 1 . Philadelphia and London: W. B. Saunders Co.
Strode, J. E. (1954) Desmoid tumors particularly as related to their surgical removal. Annals ofSurgerv, 139, 335-340.
Sugiura, I. ( I 976) Desmoplastic fibroma. Journal of Bone and Joint Surgery. 58-A, 1 26- 1 30.
VOL. 61-B, No. 3. AUGUST 1979
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