A 77-year-old Hispanic woman is referred for evaluation and treatment of a lesion on her left cheek. Through an interpreter she states that the growth has been present for about 2 months and has bled on several occasions when inadvertently scratched. She is a former smoker and is receiving treatment for arthritis and elevated cholesterol.
Upon physical examination a firm erythematous nodule 1.4 cm in diameter that is fixed to underlying tissue is observed. Marked wrinkling and furrows along with numerous comedones were noted on both cheeks and her forehead. Submandibular lymph nodes were nonpalpable.
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Atypical fibroxanthoma, also known as pleomorphic sarcoma,1 was first named in 1961 by the American dermatopathologist Elson Helwig who subsequently published a 140 case series describing clinical and pathology findings.2
Lesions typically arise on actinically damaged skin on the head and neck.1 Men and women are both affected and lesions usually appear in elderly individuals (≥ 65 years of age).1 Lesions are polypoid or nodular in appearance and pink to erythematous in color. They grow rapidly and are prone to bleeding and ulceration.
Diagnosis of atypical fibroxanthoma is confirmed by histology, which reveals a nonencapsulated dermal tumor comprised of large spindle-shaped and anaplastic cells. Immunohistochemistry is necessary to exclude other possible tumors.3 Red structureless areas are noted on dermoscopy, but findings are nonspecific.4
A subset of these tumors are aggressive and may metastasize.5 Therefore, surgical excision with wide margins or Mohs micrographic surgery are the treatments of choice.6,7
1. 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(9):1317-1326.
2. López L, Vélez R. Atypical fibroxanthoma. Arch Pathol Lab Med. 2016;140(4):376-379.
3. Sarac E, Yuksel M, Turkmen IC, Ozdemir M. Case for diagnosis. Atypical fibroxanthoma. An Bras Dermatol. 2019;94(2):239-241.
4. Moscarella E, Piana S, Specchio F, et al. Dermoscopy features of atypical fibroxanthoma: a multicenter study of the International Dermoscopy Society. Australas J Dermatol. 2018;59(4):309-314.
5. Cooper JZ, Newman SR, Scott GA, Brown MD. Metastasizing atypical fibroxanthoma (cutaneous malignant histiocytoma): report of five cases. Dermatol Surg. 2005;31(2):221-225.
6. Soleymani T, Aasi SZ, Novoa R, Hollmig ST. Atypical fibroxanthoma and pleomorphic dermal sarcoma: updates on classification and management. Dermatol Clin. 2019;37(3):253-259.
7. Tolkachjov SN, Kelley BF, Alahdab F, Erwin PJ, Brewer JD. Atypical fibroxanthoma: systematic review and meta-analysis of treatment with Mohs micrographic surgery or excision. J Am Acad Dermatol. 2018;79(5):929-934.e6.