Recurrent Cutaneous Angiosarcoma of the Scalp With Aberrant Expression of S100: A Case Report

Abstract

Angiosarcoma (AS) is a malignant mesenchymal neoplasm of endothelial origin with a predominantly lymphatic immunophenotype, which accounts for less than 1% of all sarcomas. Cutaneous AS of the scalp is associated with high rates of local recurrence and a poor prognosis. Histologically, poorly differentiated AS often comprises solid epithelioid cells, although rare variants involving spindle cells have been reported; diagnosis requires immunohistochemical analysis using vascular cell markers. We report on a cutaneous spindle-cell AS of the scalp in a female patient; key features included spontaneous regression after biopsy, local recurrence 2 years later, and aberrant nuclear staining for S100 protein in an area of the tumor not expressing CD34 or D2-40. Tumor cells exhibited positivity for vascular markers CD31, CD34, D2-40, ERG and FLI-1 and were negative for myoid markers (αSMA and desmin), epithelial (EMA and cytokeratin AE1/AE3) and melanocyte markers (HMB45 and melan-A). Cutaneous spindle-cell AS of the scalp is a rare variant with a poor prognosis. Diagnosis of spindle-cell AS was confirmed by immunohistochemical analysis using CD31, CD34, ERG, FLI-1, podoplanin (D2-40), and claudin-5. Although a number of authors have noted aberrant expression of cytokeratins, CD30, CD117 and neuroendocrine markers (synaptophysin and chromogranin A) in AS, intense positive nuclear staining for S100 protein in neoplastic cells has not hitherto been observed. This article reports on a spindle-cell AS of the scalp notable for aberrant expression of S100, spontaneous regression and recurrence 2 years later at the same site and displaying identical histological and immunohistochemical features.

Primary Undifferentiated/Dedifferentiated Cutaneous Melanomas—A Review on Histological, Immunohistochemical, and Molecular Features with Emphasis on Prognosis and Treatment

“…A total of 7 patients died of the disease on average 16.1 months after the diagnosis (median, 8; range 2-60) while 9 patients showed signs of progression (recurrence, nodal, and/or distant metastasis) on average 16.3 months after diagnosis (median, 12; range 3-42) and 7 patients showed no sign of progression after a mean follow up period of 20.7 months (median, 24; range 6-34). The deceased patients had a mean age of 70.5 (median, 72; range [64][65][66][67][68][69][70][71][72][73][74][75][76] and the maleto-female ratio was 3:4. The patients with progressive disease had a mean age of 62.2 (median 70 range 1-96) and the male-to-female ratio was 2:1.…”

“…Another similar case was noted by Macías-García et al, who described a cutaneous angiosarcoma with areas of S100 positivity and CD34 and D2-40 negativity. Extensive immunohistochemical analysis of the whole specimen rendered the final diagnosis [ 65 ].…”