An 84-year-old man presents with a lesion on his scalp, which he first noted several months earlier. The growth is painless but has recently begun to bleed spontaneously. He gives a history of ample lifetime sun exposure as well as basal and squamous cell carcinomas and solar keratoses. Examination reveals a 1.8-cm erythematous nodule. Cervical and submandibular lymph nodes are nonpalpable.
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Atypical fibroxanthoma (also termed pleomorphic sarcoma) is an uncommon cutaneous neoplasm that arises within actinically damaged skin.1 The lesion is polypoid or nodular in appearance, pink to erythematous in color, rapidly growing, and prone to both bleeding and ulceration.2 Patients often have a history of chronic sun exposure and nonmelanoma skin cancer. Diagnosis of atypical fibroxanthoma is confirmed by histology, which reveals a nonencapsulated dermal tumor comprised of spindle-shaped pleomorphic cells.3
Although surgical excision is usually curative, a subset of tumors is aggressive and marked by recurrence and/or metastatic spread.4 Mohs micrographic surgery, which allows for complete margin evaluation, is considered the treatment of choice.5
Stephen Schleicher, MD, is an associate professor of medicine at the Commonwealth Medical College in Scranton, Pennsylvania, and an adjunct assistant professor of dermatology at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia. He practices dermatology in Hazleton, Pennsylvania.
- Beer TW, Drury P, Heenan PJ. Atypical fibroxanthoma: a histological and immunohistochemical review of 171 cases. Am J Dermatopathol. 2010;32:533-540.
- Miller K, Goodlad JR, Brenn T. Pleomorphic dermal sarcoma: adverse histologic features predict aggressive behavior and allow distinction from atypical fibroxanthoma. Am J Surg Pathol. 2012;36:1317-1326.
- Mirza B, Weedon D. Atypical fibroxanthoma: a clinicopathological study of 89 cases. Australas J Dermatol. 2005;46:235-238.
- Cooper JZ, Newman SR, Scott GA. Brown MD. Metastasizing atypical fibroxanthoma (cutaneous malignant histiocytoma): report of five cases. Dermatol Surg. 2005;31:221-225.
- Iorizzo LJ 3rd, Brown MD. Atypical fibroxanthoma: a review of the literature. Dermatol Surg. 2011;37:146-157.