Fibromatosis of the upper limb Two cases of fibromatosis of the upper limb are presented, neither of which could be placed in a specific category as described in the literature. Both patients were black, and both had a history of previous trauma to the region, which has been regarded as an initiating factor. Emphasis is placed on conservative treatment in order to maintain hand function as long as possible.

Hilton Becker, F.R.C.S., and Laurence Chait, F.R.C.S., Johannesburg, South Africa

T he fibromatoses are poorly understood conditions

with regard to their etiology, pathology, and management. Pathologically related conditions may occur in different parts of the body and have been classified into specific types. Lesions, however, are still encountered which, although related histologically to the fibromatoses, fail to meet the requirements that would place them in a specific category. We present two unusual cases of fibromatosis occurring in the upper limbs of adult black patients.

Case reports Case 1. A 38-year-old male clerk developed a rapidly growing tumor in the region of the thenar eminence of his left hand over a period of 10 months. Ten years previously he had sustained a fracture of the first metacarpal of this hand when he caught it in a rolling mill. On examination of the left hand. there was a large mass occupying and protruding from the thenar space (Fig. I. A). This felt hard and appeared to be attached to deeper structures. Sensation distally was normal and tendon function was intact, but movements were limited by the size of the tumor. Radiological examination revealed no bony abnormality, except for the previous fracture site (Fig. I, B). A presumptive diagnosis of fibrosarcoma was made. but a biopsy indicated that the condition was fibromatosis. At operation the tumor was found to have a smooth. lobulated surface and to have displaced the surrounding normal tissues (Fig. I, C). It was removed easily, except for one area of attachment to the fibrous flexor sheath of the index finger from which it appeared to arise. The pathological report described a bosselated fibrous mass measuring 10.5 x 7 x 5.5 cm, which appeared to be encapsulated. Histological examiFrom the Department of Plastic and Reconstructive Surgery, University of the Witwatersrand Medical School, Johannesburg, South Africa. Received for publication Sept. 2, 1978. Reprint requests: H. Becker, F.R.C.S., Department of Plastic Surgery, Medical College of Virginia, P.O. Box 154, MeV Station, Richmond, VA 23298.

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nation revealed the presence of fibrous proliferation. The collagen bundles were thickened and homogeneous. No sarcomatous change was noted. Nine months after operation there was no evidence of recurrence and the patient had full use of his hand. Case 2. A 19-year-old woman presented with a very large area of swelling involving the right forearm (Fig. 2A). Six years prior to admission, a hematoma, which was the result of trauma, was evacuated from the medial aspect of the distal forearm. Three years later a tumor was noted in this region. It was excised together with the distal end of the ulna. Histological diagnosis at this stage was a fibrosarcoma of lowgrade malignancy. Examination of the hand 3 years after operation showed completely normal sensory and motor function distal to the tumor. The roentgenographic findings showed no evidence of bony erosion (Fig. 2B). At operation a large bosselated tumor extended around the whole forearm and had displaced but not invaded the surrounding tissues (Fig. 2C). The tumor was removed through volar and dorsal incision. It was dissected off the radius and the remaining proximal end of the ulna to which it appeared to be attached. Full function returned 3 weeks after operation, and 7 months later there was as yet no evidence of recurrence (Fig.2D). Pathological findings showed a tumor measuring 17 x 12 x 10 cm. Histological features were similar to those in case I (Fig. 2E). Electron microscopy in both cases revealed tumor cells with the ultrastructural features of fibroblasts which are actively synthesizing collagen.

Discussion The generic term "fibromatosis" was proposed by Stout! for a group of related conditions having in common the following features: (I) proliferation of welldifferentiated fibroblasts; (2) infiltrative pattern of growth; (3) presence of a variable (but usually abundant) amount of collagen between proliferating cells; (4) lack of cytological features of malignancy and scanty or absent mitotic activity; (5) aggressive clinical behavior characterized by local recurrences but without the capacity to produce distance metastases.

0363-5023/79/030264+06$00.60/0 © 1979 American Society for Surgery of the Hand

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Fig. 1. A, Preoperative photograph showing biopsy sites case I. B, Roentgenogram showing soft-tissue mass occupying the first web space and an old fracture site of first metacarpal. C, An intraoperative photograph showing the size of the tumor.

Grossly the lesions often are large, firm, and whitish, with an ill-defined outline and an irregulary whorled surface. They often arise in a muscular fascia. Microscopically the fibroblastic nature of the cells is usually quite obvious; however in some actively proliferating types, the plump nuclei with blunted ends can closely resemble those of smooth-muscle cells. In these instances the use of electron microscopy can be decisive in differential diagnosis. Some pathologists add the adjective "aggressive" to some forms of fibromatosis to emphasize the biological

behavior. However, Ackerman and Rosai 2 regard all fibromatoses as being potentially aggressive. There is very little correlation between the cellularity and other microscopic features and their biological behavior. A number of fibromatoses have received special names. The World Health Organization classification3 is presented in Table I. However, a number of fibrous tumors are encountered which do not appear here, e.g., juvenile aponeurotic fibroma, 4 recurring digital fibrous tumor of childhood,5 desmoplastic fibroma,6 fascial fibromatosis with osseous involvement. 7 Our two pa-

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266 Becker and Chait

Fig. 2A. Preoperative photograph of case 2.

Fig. 2B. Roentgenogram showing large soft-tissue mass.

tients had several features similar to some types but did not fall into any specific category. Both cases followed trauma and occurred in black patients, there was no invasion of surrounding tissues, the tumor was easily enucleated except from its point of attachment, and the histological and electron microscopic findings were similar.

In view of the fact that black people have a propensity to keloid formation following skin trauma, it would seem possible that scarring of deeper connective tissue could have been an initiating factor in the development of the fibromatosis in our two patients. It is emphasized that the management of these tumors in the upper limb should be conservative .

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Fig. 2C. An intraoperative photograph . Note the smooth surface of the tumor.

Fig. 2D. Histological section showing collagenized fibrous tissue. The numerous fibroblasts have plump nuclei, and the collagen bundles are thickened and homogeneous. There was no evidence of sarcomatous change.

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268 Becker and Chait

Fig. 2E. An electron microscopic view showing tumor cells with the ultrastructural features of fibroblasts which are actively synthesizing collagen.

Table I. Classification of fibromatosis I. Palmar fibromatosis

2. Plantar fibromatosis 3. Keloid

4. Cicatricial fibromatosis 5. Irradiation fibromatosis 6. Nodular fascitis (pseudosarcomatous fibromatosis) 7. Fibromatosis or aggressive fibromatosis (extra-abdominal desmoid) 8. Juvenile aponeurotic fibroma (calcifying fibroma)

9. Abdominal fibromatosis (abdominal desmoid) 10. Nasopharyngeal fibroma 11. Congenital generalized fibromatosis 12. Penile fibromatosis (Peyrones disease) 13. Fibromatosis colli

Benign nodular infiltrating fibrous lesion of variable cellularity from palmar aponeuroses. May cause contracture of the fingers (Dupuytren's contracture). Multiple lesions involving feet and hands occasionally observed. Lesions may grow to such a size that they form a tumor, but not always in association with Dupuytren's contracture. Such tumors have been called the fibroma or pseudotumour of Dupuytren's contracture. They may be associated with other fibromatoses. The same as in palmar fibromatosis. Superficial nodular parvicellular fibrous growth characterized by well-defined broad bands of homogenous acidophilic collagen. Lesions usually following some form of skin injury and usually found in black people. These lesions, unlike hypertrophic scars, tend to recur. Nonmetastasizing progressive overgrowth of fibrous tissue arising in association with a scar. Benign, infiltrating. and often aggressive growth. Bizarre cells present. Difficult to differentiate from rare postirradiation fibrosarcoma. Benign and probably reactive fibroblastic growth, extends as solitary nodule from superficial into subcutaneous fat and muscle. May be confused with sarcoma. Most common in upper extremity, trunk, and neck region of young adults. Nonmetastasizing tumor-like fibroblastic growth of unknown pathogenesis involving voluntary muscles as well as aponeurotic and fascial structures. Strong tendency to local recurrence. Most common in shoulder, thigh, and buttocks of young adults. Rare, infiltrating, fibrous growths affecting chiefly muscles and subcutaneous fat of volar aspect of hands. Characteristically, tumor is associated with irregular foci of calcification and chondroid metaplasia. Frequent local occurrences. Locally aggressive infiltrating tumor-like fibroblastic growth of unknown pathogenesis arising from aponeurotic structures of the abdominal musculature. Occurs most commonly in women during pregnancy. Locally invasive growth of nasopharynx. May regress spontaneously. Extremely rare. Occurs prior to birth or during first year of life. Can be fatal. Dense infiltrating fibrous growths affecting fascial structures of the penis. Benign fibrous growths arising in sternocleidomastoid muscle. Contracture may give rise to torticollis.

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Where possible they should be removed by local incision without sacrificing vital structures. If the tumor recurs, repeated excisions should be carried out.

Conclusion Although many of the fibromatoses have been classified, lesions still appear that do not fill the requirements of a specific category. Two such cases are presented. Common histories suggest that trauma might be a factor, especially in the black race groups in whom keloid formation following trauma is seen so frequentIy. As function often can be preserved after local resection, a conservative approach to these tumors in the initial stage of management is emphasized.

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REFERENCES I. Stout AP: Juvenile fibromatosis. Cancer 7:953-78, 1954 2. Ackerman LV, Rosai J: Surgical pathology, ed 5. St. Louis, 1974, The CV Mosby Co 3. World Health Organization: Histological typing of soft tissu.e tumours, ill International histological classification of tumours, No.3, Geneva, 1969 4. Specht EE, Staheli LT: Juvenile aponeurotic fibroma. J HAND SURG 2:256-7, 1977 5. Poppen NK, Niebauer JJ: Recurring digital fibrous tumour of children. J HAND SURG 2:253-5, 1977 6. Schenkar DL, Kleinert HE: Desmoplastic fibroma of the Hand. Plast Reconstr Surg 59: 128-33, 1977 7. Arlen M, Koven L, Frieder M: Juvenile fibromatosis of the forearm with osseous involvement. J Bone Joint Surg [Am] 51:591-5, 1969

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Fibromatosis of the upper limb.

Fibromatosis of the upper limb Two cases of fibromatosis of the upper limb are presented, neither of which could be placed in a specific category as d...
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