Superficial CD34-Positive Fibroblastic Tumor: A Newly Recognized Entity With Overlapping Features of PRDM10-Rearranged Soft Tissue Tumors

浅表CD34阳性成纤维细胞肿瘤:一种新发现的实体,具有与PRDM10重排软组织肿瘤重叠的特征

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Abstract

Superficial CD34-positive fibroblastic tumor (SCPFT) is a recently identified, infrequent, low-grade mesenchymal neoplasm, first identified in 2014. Although it is relatively new to the field, SCPFT has been gaining prominence in surgical pathology practice because of its distinctive features. As of now, there are limited reported cases of SCPFT, with fewer than 100 instances documented in scientific literature. This distinctive blend of rarity and intriguing variability in presentation emphasizes the significance of identifying and understanding this uncommon entity, facilitating precise diagnosis and optimal management. In this article, we aimed to present a notable case of SCPFT in a male in his 20s who presented with a distinct subcutaneous mass measuring 2.4 × 1.8 cm at the medial aspect of the knee joint. The patient reported no significant medical history or trauma to the affected area. MRI of the knee showed a well-defined 2.4 × 1.8 cm subcutaneous mass with no definite communication with the underlying ligament or meniscus. The histopathological examination revealed spindle cell neoplasm arranged in intersecting fascicles, accompanied by arborizing blood vessels. Neoplastic spindle cells exhibited marked nuclear pleomorphism, and abundant and eosinophilic cytoplasm, with focal areas of granular, glassy, and lipidized cytoplasm. Nuclear pseudo inclusions and a few mitotic figures (1-2 per high-power field) were noted. Inflammatory infiltrates were identified within the neoplasm, comprising eosinophils and lymphocytes, highlighting an immune response within the tumor microenvironment. The surgical margin exhibited involvement with the tumor infiltrates, with the neoplastic cells extending into the adjacent fat tissue. This finding indicates local tumor spread and potential challenges in achieving complete resection. Immunohistochemical staining showed positive staining for CD34, corroborating the diagnosis of a CD34-positive fibroblastic tumor. Focal positive staining for pan-CK was noted. Staining for CD31, smooth muscle actin (SMA), desmin, S100, and anaplastic lymphoma kinase (ALK) was negative, supporting the diagnosis. The Ki67 proliferation index was low.

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