CD34-Negative Malignant Renal Solitary Fibrous Tumor: Case Report and Diagnostic Insights

TL;DR

A rare case of a malignant kidney tumor that lacks a common marker (CD34) is reported, highlighting the importance of accurate diagnosis for proper treatment.

Contribution

This case report adds to the limited knowledge of CD34-negative malignant renal solitary fibrous tumors and their diagnostic challenges.

Findings

The tumor was CD34-negative but positive for STAT6, vimentin, and Bcl-2.

NAB2::STAT6 gene fusion was confirmed using fluorescence in situ hybridization.

The case underscores the need to consider SFT in the differential diagnosis of unusual renal tumors, even when CD34 is negative.

Abstract

Solitary fibrous tumors (SFTs) are rare fibroblastic neoplasms with diverse biological behaviors and widespread distribution. Primary renal SFTs are uncommon, and their malignant variants, especially those that are CD34 negative, are even rarer. This study presents a case of malignant renal SFT in a 57-year-old female, focusing on its immunomorphological features. On gross examination, the tumor's large size (11.5 cm) was remarkable. Microscopic analysis showed high cellularity, diffuse sheets of moderately pleomorphic ovoid cells, prominent staghorn vessels, tumor cell necrosis, and a high mitotic count. Immunohistochemistry revealed strong positivity for STAT6, vimentin, and Bcl-2 and, notably, negativity for CD34. The presence of the NAB2::STAT6 gene fusion was confirmed through fluorescence in situ hybridization. This case emphasizes the need to consider SFT in the differential diagnosis of unusual renal tumors, even when CD34 is negative. The infrequency, morphological diversity, and resemblance to other tumors make diagnosing renal SFTs challenging. Accurate identification and classification as benign or malignant are crucial for proper clinical management and prognosis.

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