paranóia

Desmoid tumor or aggressive fibromatosis is extremely rare, with less than five cases per million inhabitants [3]. In extra-abdominal areas, the shoulder girdle is the commonest location, frequently seen in adolescent and young adults, as in our patient [1–3]. Pain, and symptoms of nerve or vascular compression occur when the lesion becomes voluminous. As in our case, the lesion is usually poorly circumscribed, infiltrating adjacent bone and muscles, leading to great difficulties in determining the exact boundaries, even, during operation [1,2]. Because desmoid tumors have been produced in rats with estrogen, and would have a faster rate of growth in fertile women, an hormonal influence has been suspected [3]. However, until today, few responses have been reported under hormonal therapy [2]. Prognosis factors lack to predict the clinical course of patients. Resection is the best option but recurrences are numerous, especially when it is incomplete [1–4], usually within 2 years of operation, with a rate ranging from 25 to 68%. Adequate margin of resection cannot be always easily obtained, due to the need of preserving vital or important structures, and due to the lack of encapsulation makes often difficult for the surgeon to distinguish clearly between tumor and scar tissue [1,2]. Furthermore, spontaneous regression or no recurrence following incomplete resection, as in our case, may occur, with an incidence rate of 20% in one study [4]. Complementary radiotherapy was reported as effective in preventing recurrence in 10 patients with narrow margins [6]. Because in axillary area, radiotherapy may lead to lymphoedema, we did not administer radiotherapy in your young patient, who remained free of recurrence 13 years later. When the tumor recurs and/or cannot be excised, a forearm amputation must be considered or high doses of radiotherapy and possibly chemotherapy, although outcome under this condition is not well known. Finally, the desmoid tumor is an aggressive fibromatosis that remains a very interesting but poorly understood neoplasm.

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