Granular cell atypical fibroxanthoma: case report and review of the literature

Abstract

We present a case of granular cell atypical fibroxanthoma of the scalp. The neoplasm occurred as a tender nodule on the frontal scalp of an 82-year-old Caucasian man. Histology demonstrated a diffuse proliferation of atypical epithelioid cells with abundant granular cytoplasm. Many of the cells had large irregular nuclei and atypical mitotic figures were present. Immunohistochemical stains were positive for CD68 and procollagen 1 and negative for cytokeratin, HMB-45 and S-100 protein. The granular cell phenotype has been observed in other cutaneous neoplasms including granular cell tumors, dermatofibromas, dermatofibrosarcoma protuberans, fibrous papules, basal cell carcinomas, leiomyosarcomas, angiosarcomas and primitive polypoid granular cell tumors. We discuss the differential diagnosis and review the previously reported cases of this rare variant of atypical fibroxanthoma.

Atypical Fibroxanthoma: A Histological and Immunohistochemical Review of 171 Cases

“…Clear cell and granular cell tumors were uncommon (3 cases of each), but may lead to contemplation of alternative diagnoses to those traditionally considered with AFX. 36,37 Similarly, the rare occurrence of widespread myxoid change in AFX (seen in only 1 patient in this series) demands consideration of a different spectrum of lesions to those typically entertained. The myxoid pattern of AFX has only recently been described in the literature in a series of 4 cases.…”

Congenital Granular Cell Tumor of the Arm

“…11 Melanoma or a benign melanocytic lesion can demonstrate a junctional component or some nesting of cells and would have immunoreactivity for S100 protein. [7][8][9] The granular cell variant of fibrous papule is restricted to the central face and is less cellular than other variants, containing only focal wispy histiocytes. 12 Cutaneous involvement in CGCT, as in our patient, is extremely rare, and to the best of our knowledge, only 2 cases have been reported in the literature.…”

“…3,5 Granular changes because of the accumulation of lysosomes can be observed in a variety of neoplasms, including leiomyoma, leiomyosarcoma, dermatofibroma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, benign melanocytic lesion, melanoma, fibrous papule, angiosarcoma, basal cell carcinoma, metastatic tumors, and primitive nonneural GCTs (so-called primitive polypoid GCTs). [5][6][7][8][9][10][11] Primitive polypoid GCT was first described by LeBoit et al 6 in 1991. Subsequently, Lazar and Fletcher 7 reported a series of similar tumors that they named "primitive nonneural granular cell tumor."…”

“…7,8 The histomorphological appearance and absence of actin, desmin, and/or caldesmon immunoreactivity effectively exclude leiomyoma and leiomyosarcoma. 8,9 Basal cell carcinomas with granular cell change are usually of the nodular type with an immunohistochemical profile demonstrating reactivity with cytokeratins. 10 Granular cell atypical fibroxanthoma usually presents as a rapidly enlarging dome-shaped nodule and typically located on sun-exposed areas of elderly patients.…”

A case of primitive non-neural granular cell tumor presenting as a single painless bleeding nodule

“…In fact, granular changes due to lysosome accumulation can be observed in a variety of neoplasms, including conventional granular-cell tumor, melanocytic neoplasms, smooth muscle neoplasms, dermatofibromas, epithelioid cell histiocytomas, dermatofibrosarcoma protuberans, fibrous papules, basal cell carcinomas, atypical fibroxanthomas, angiosarcomas, malignant fibrohistiocytomas, perineuromas and metastatic carcinomas [10,11]. This granular cell change usually involves only part of the lesion, allowing differentiation by conventional morphological and immunohistochemical criteria [3].…”