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Indian Journal of Surgery, Vol. 67, No. 5, September-October, 2005, pp. 273-275 Case Reports Infantile fibromatosis of scrotum M. G. Sailukar, T. B. Yuvraja, M. R. Ramadwar1, P. A. Kurkure2, H. B. Tongaonkar3 Surgical Oncology, 1Pathology, 2Paediatric Oncology and 3Genitourinary Oncology, Tata Memorial Hospital, Dr Ernest Borges Marg, Parel, Mumbai, India Code Number: is05085 Abstract Infantile fibromatosis is a benign but locally aggressive tumour, the tumour presenting as asymptomatic, firm and solid mass. The most frequent types are the infantile fibromatosis (head, neck, shoulder, upper arm or thigh), extra-abdominal fibromatosis (chest wall, back and thigh) and fibromatosis colli (neck), but only rarely has it been reported to involve the external genitalia or scrotum. We report a case of infantile fibromatosis of scrotum in a 1-year-old child. Keywords: Fibromatosis, genitalia, scrotum, soft tissue tumours Fibromatosis is a broad group of fibrous tissue proliferation of similar microscopic appearance whose behaviour is intermediate between that of benign fibrous lesions and fibrosarcoma. They are locally aggressive tumours with high recurrent potential, but they do not metastasize.[1] Infantile fibromatosis represents a childhood counterpart of musculoaponeurotic fibromatosis commonly involving skeletal muscles of head and neck, shoulder, upper arm and thigh.[2] Involvement of scrotal sac by fibromatosis in children has rarely been reported. Extensive literature search shows that previously two such cases have been reported.[3] We discuss a case of infantile fibromatosis of the scrotum, which was managed surgically. The important issues to be considered are the differential diagnosis in view of the site and the adequacy of excision in view of the propensity of these lesions to recur. Case report A 1-year-old male child presented with painless, slowly growing left-sided scrotal mass of 3-month duration. On examination a 6 x 4 cm, firm, nontender, bosselated mass was present in left scrotal cavity not extending above in the inguinal canal [Figure - 1]. The scrotal skin was thinned out over the tumour mass. The left testis could be palpated inferior to the mass as a separate structure but was adherent to the mass. The right testis and scrotum were normal. There was no palpable lymphadenopathy. The tumour markers,a-fetoprotein and b-HCG were within normal limits. The CT scan of abdomen, pelvis and scrotum reveal a heterogeneously enhancing mass in the left scrotum measuring 4.5 x 4.5 x 6.0 cm in size. Right testis and bilateral spermatic cords were normal. No abdominal lymphadenopathy was noted. The chest X-ray was within normal limits. Left inguinal orchiectomy with wide excision of mass was done along with overlying scrotal skin, which was stretched and thinned out. The surgical specimen consisted of an enlarged scrotal sac containing a firm, white tumour filling the entire sac [Figure - 2]. Though the mass was adherent to the testis, the spermatic cord was encased by the mass. As removal of mass was not possible without vascular compromise to the testis and to achieve complete excision with negative margins, en-bloc excision of tumour along with testis was done. Histopathology showed features of fibromatosis. The tumour was composed of a proliferation of uniform, bland spindle cells within a collegenous stroma [Figure - 3]. No mitoses or necrosis was present. This lesion was infiltrating the scrotal skin along with dartos muscle and spermatic cord. Immunohistochemistry showed tumour cells to be positive for calponin while they were negative for SMA, desmin and S-100. Testis with its tunics and appendages was free of tumour. The cutaneous resection margin was free of the lesion. Discussion Infantile fibromatosis, originally described by Stout in 1954.[4] It seems to be genetic in origin although the mode of transmission is not clear.[5] Fibromatosis, either in adults or in children is a broad group of fibrous proliferation whose behaviour is intermediate between benign fibrous lesions and fibrosarcoma. These are usually widely infiltrative, ill-circumscribed locally aggressive tumours with a high local recurrent potential. However, they do not metastasize. The childhood counterpart of typical musculoaponeurotic fibromatosis is very similar to the adult lesions with respect to histology and behaviour. An exception may be a diffuse, cellular fibromatosis more commonly seen in infants. This form can be histologically mistaken for fibrosarcoma.[6] In addition to the typical forms of musculoaponeurotic fibromatosis, several specific types are known in children who differ in their clinical presentations. In 1981, Chung and Enzinger reviewed their cases of infancy and childhood fibromatosis and categorized them using the Armed Forces Institute of Pathology classification: fibrous hamartoma of infancy, infantile digital fibromatosis, fibromatosis colli, infantile myofibromatosis, infantile (desmoid-type) fibromatosis, hyaline fibromatosis, gingival fibromatosis and calcifying aponeurotic fibromatosis.[7] Infantile fibromatosis affects muscles of the head and neck, shoulder, upper arm and thigh. The progression is unpredictable. Wide local excision with free margins can provide cure. However, due to the ill-circumscribed, infiltrative nature of proliferation, achieving free margins may be difficult.[8] Local recurrences occur usually within 18 months after surgery. Imaging modalities, especially MRI are useful to determine the extent of lesion and follow up. Unresectable fibromatosis needs multidisciplinary approach. Nonaggressive therapy with tamoxifen and diclofenec may be useful, most probably through their anti-angiogenic properties. In aggressive disease cytotoxic chemotherapy is needed. Weekly administration of vinblastine and methotrexate seems to be safe and effective in this children.[9] External genitalia are very rarely involved by fibromatosis. This 1-year-old male child with a scrotal sac tumour posed a serious differential diagnosis of paratesticular rhabdomyosarcoma and infantile fibrosarcoma. The therapeutic issues involved were those of chemotherapy and extent of surgery. Histopathology showed bland spindle cell proliferation without mitoses and necrosis ruling out the diagnosis of embryonal rhabdomyosarcoma or fibrosarcoma. Immunohistochemistry was negative for muscle markers supporting the above diagnosis. We were successful in achieving free skin margins. The child is disease free 8 months after surgery. However, it is essential to follow up this child since late recurrences are known in spite of clear margins. References
Copyright 2005 - Indian Journal of Surgery The following images related to this document are available:Photo images[is05085f2.jpg] [is05085f3.jpg] [is05085f1.jpg] |
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