title: Metastatic atypical fibroxanthoma: a series of 11 cases including with minimal and no subcutaneous involvement.
abstract: Atypical fibroxanthoma (AFX) is a dermal mesenchymal neoplasm arising in sun-damaged skin, primarily of the head and neck region of older men. Conservative excision cures most. However, varying degrees of subcutaneous involvement can lead to a more aggressive course and rare metastases. Thus, AFX involving the subcutis are termed pleomorphic dermal sarcomas or other monikers by some to recognize the more threatening natural history. We reviewed cases of "metastatic AFX" from our institution and from the files of a consultative dermatopathology practice. Nine of 152 patients with AFX were identified at a single institution (2000-2011). Two additional patients were identified from the files of a consultative practice. Clinical, radiological, and pathological features were reviewed and cases with histologically verified metastases identified. Median age was 67 (range, 45-91) years, all male, and involving the head and neck region. Two cases had no documented involvement of the subcutis, and 2 cases had only superficial subcutis involvement. Median time to metastases was 13 (range, 8-49) months. Three patients developed solitary regional lymph node metastases while 8 had widespread metastases. Five patients developed local recurrence within 8 months, and all 5 developed widespread metastasis. With median follow-up of 26 (range, 10-145) months, 6 died of disease (median, 19 months; range, 10-35 months), 4 were alive and well, and 1 was alive with disease. AFX has very rare metastatic potential, even those without or with minimal subcutis involvement, and can lead to mortality. Most metastasis and local recurrence occurred within 1 year of presentation. Solitary regional metastases were associated with better outcomes than those with multiple distant metastases. Patients with repeated local recurrences portended more aggressive disease including development of distant metastases.
authors.affiliation: *Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX; †Department of Pathology, Lahey Clinic, Burlington, MA; and ‡Dermatopathology Consultations.com, Needham Heights, MA.
authors.forename: Wei-Lien
authors.initials: WL
authors.lastname: Wang
authors.forename: Carlos
authors.initials: C
authors.lastname: Torres-Cabala
authors.forename: Jonathan L
authors.initials: JL
authors.lastname: Curry
authors.forename: Doina
authors.initials: D
authors.lastname: Ivan
authors.forename: Michael
authors.initials: M
authors.lastname: McLemore
authors.forename: Michael
authors.initials: M
authors.lastname: Tetzlaff
authors.forename: Artur
authors.initials: A
authors.lastname: Zembowicz
authors.forename: Victor G
authors.initials: VG
authors.lastname: Prieto
authors.forename: Alexander J
authors.initials: AJ
authors.lastname: Lazar
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D006258:Head and Neck Neoplasms / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis / Q000473:pathology*; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*