Atrophic dermatofibrosarcoma protuberans with diffuse eosinophilic infiltrate

Abstract

Atrophic dermatofibrosarcoma protuberans (atrophic DFSP) is a variant of dermatofibrosarcoma protuberans (DFSP), and is clinically characterized by depressed lesions. We report a patient with a typical atrophic DFSP lesion with marked eosinophilic infiltration. The patient was a 55-year-old woman with a dark-red, depressed lesion in the epigastric region. Histopathological examination of the lesion showed proliferation of fibroblast-like cells in a storiform pattern in the dermis and subcutaneous tissue. Immunohistochemical staining of tumor cells was positive for CD34. The lesion was histopathologically typical of DFSP, but no elevated lesion was clinically observed. Thus, a diagnosis of atrophic DFSP was made. Moreover, this tumor tissue exhibited marked eosinophilic infiltration. To our knowledge, they are no reports of eosinophilic infiltration in DFSP tissue. Therefore, this seems to be an extremely rare case of DFSP.

Atrophic Dermatofibrosarcoma Protuberans with Eosinophilic Infiltration

“…Tissue eosinophilia in this lesion is a highly unusual finding. Eosinophilia is a frequently encountered finding in malignant neoplasms that is considered a favorable host response to malignant tissue [ 10 ]. Eosinophils have an anti-tumorigenic (e.g., TNF-α, granzyme, cationic proteins, and IL-18) and protumorigenic role in different cancers, depending on the location.…”

“…The atrophic variant was first described by Lambert et al in 1985 [ 8 , 9 ]. Eosinophilic infiltration of atrophic DFSP has been reported in only one case to date [ 10 ]. We report another case of atrophic DFSP with eosinophilic infiltration in a young female patient.…”

Congenital Atrophic Dermatofibrosarcoma Protuberans in a 7‐Month‐Old Boy Treated with Mohs Micrographic Surgery

“…The genetic mutation responsible for more than 90% of DFSP cases has been described as a fusion between the collagen type 1-alpha-1 gene on chromosome 17 and the platelet-derived growth factor beta-chain gene (PDGFB) on chromosome 22 via either the creation of a supernumerary ring chromosome derived from t (17;22) or a reciprocal translocation t(17;22)(q22;q13.1) (7,29,(42)(43)(44)(45)(46)(47)(48). This genetic alteration results in deregulation of PDGFB chain expression and thus over-activation of the platelet-derived growth factor receptor protein tyrosine kinase (PDGFR) leading to autocrine-stimulated malignant cell growth.…”

“…Diagnosing the infrequent atrophic variant of DFSP can be difficult, as it presents as a depressed or sclerotic plaque mimicking other atrophic dermatosis (25,26). To date, there have been only 36 reported cases of atrophic DFSP (described as atrophic or morpheaform) (27–33). Of all reported patients with atrophic DFSP, 58% were women and had an average age at diagnosis of 31 years, slightly younger than those with all variants of DFSP.…”