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Figure A
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Figure B
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Figure C
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Figure D
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Figure E
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The etiology of an enlarging, painless mass over the shoulder in a patient with familial adenomatous polyposis (FAP) is most consistent with an extra-abdominal desmoid tumor, with histologic findings including well-differentiated fibroblasts, uniform spindle cells with elongated nuclei and occasional mitoses (Figure B). Extra-abdominal desmoid tumors are the most locally invasive of all benign soft tissue tumors. They are most often found in female patients between the ages of 15 and 40 and often occur in the shoulder, chest-wall/back, and thigh regions. Patients with familial adenomatous polyposis (FAP) have a 10,000 times increased risk of developing these tumors. Treatment includes nonoperative management with chemotherapy (tamoxifen) for inoperable lesions, and wide surgical resection with external beam radiation therapy. El-Haddad et al. recently reviewed their institution’s experience with 54 patients undergoing surgical and/or radiation therapy for aggressive fibromatosis. The author noted comparable results between patients presenting with tumor recurrence and those with initial presentation with regard to local tumor control following treatment. Hosalkar et al. reviewed musculoskeletal desmoid tumors. In this Level V review, the authors noted that despite the benign nature of desmoid tumors, multidisciplinary methods of treatment are needed, including surgery, chemotherapy, hormonal therapy, and/or radiation therapy is needed. Incorrect Responses: 1 - This pathology is consistent with Neurofibroma, showing fibroblasts with mixed Schwann cells, mast cells, and lymphocytes; with elongated, wavy nuclei (Figure A) 3 - This pathology is consistent with Pigmented Villonodular Synovitis, with mononuclear stromal cells, pigmented foam cells, hemosiderin stained multinucleated giant cells, with multiple mitotic figures (Figure C) 4 - This pathology is consistent with Ewing's Sarcoma, with monotonous small round blue cells with prominent nuclei and minimal cytoplasm (Figure D) 5 - This pathology is consistent with Non-Ossifying Fibroma, with fibroblastic spindle cells in whirled/storiform pattern, lipophages, giant cells, hemosiderin pigmentation (Figure E)
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