Superficial CD34-positive fibroblastic tumor (SCPFT) is an extremely rare neoplasm with an indolent behavior. It was incorporated for the first time in the fifth edition of the World Health Organization (WHO) classification in the rarely metastasizing category of soft tissue tumors with an intermediate biological potential. It is a challenging clinical entity coupled with a pathological dilemma due to moderate to marked pleomorphism in tumor cells mimicking various sarcomas. Histologically, these tumors appear to be malignant at the outset but atypical mitosis or necrosis are rarely seen. These cells show diffuse positivity for CD34 and are focally positive for Pan-cytokeratin which further aids in diagnosis. Some of the SCPFT harbor PRDM10 rearrangement; however, the diagnosis does not require molecular testing. We are discussing a series of five cases of SCPFT, highlighting the diagnostic histological and immune-histochemical features of this enigmatic entity. We pen down the series as an accurate histopathological diagnosis of SCPFT is undoubtedly a key to prevent its misdiagnosis as a malignant tumor with drastically different clinical implications.
Keywords: CD34-positive fibroblastic; PRDM10; Sarcoma; World Health Organization.
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