Atypical fibroxanthoma: Case series of 16 patients

Pathology Quiz Case 3: Diagnosis

“…3 It is typically asymptomatic but may result in ulceration or hemorrhage. 3,6,7 Risk factors for AFX may include immunodeficiency and sun exposure. 3 AFX represents a small proportion of head and neck sarcomas, which collectively have an incidence of 3.0 to 4.5 per 100 000.…”

“…The differential diagnosis also includes malignant melanoma, Merkel cell carcinoma, and metastasis from a primary tumor, as well as other softtissue sarcomas such as malignant fibrous histiocytoma (MFH) and dermatofibrosarcoma protuberans. [6][7][8] The histogenesis of AFX is controversial; some consider it to be within the spectrum of soft-tissue sarcomas. [6][7][8] The tumor cells are arranged in a haphazard or somewhat fascicular pattern and have elongated, irregular, hyperchromatic nuclei.…”

“…7 Unlike MFH, AFX is less often found on the extremities and does not infiltrate into skeletal muscle. 7 Unlike MFH, AFX is less often found on the extremities and does not infiltrate into skeletal muscle.…”

“…Treatment options include wide local excision with 0.6to 2.0-cm margins 2,7 or Mohs microhistographic surgery, which has been reported to provide a lower recurrence rate, a cosmetically superior outcome, and confirmation of tumorfree margins. The local recurrence rate after treatment has been reported to be 0% to 7% with Mohs surgery 3,7 and up to 12% with wide local excision.…”

“…The local recurrence rate after treatment has been reported to be 0% to 7% with Mohs surgery 3,7 and up to 12% with wide local excision. 2,4,7,10 There have been at least 21 reported cases of metastases in the literature, including extension of disease to regional lymph nodes, skin, liver, and lungs. 2,4,7,10 There have been at least 21 reported cases of metastases in the literature, including extension of disease to regional lymph nodes, skin, liver, and lungs.…”