Predominantly epithelial-type synovial sarcoma with overwhelming neuroendocrine differentiation: a potential diagnostic pitfall.

Ying Chen, Ning Zhou, Deyu Guo, Xiaodong Wang, Xin He, Yujuan Xu

Diagnostic pathology 2022 Jul 11

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Synovial sarcoma is an uncommon soft tissue tumor of soft tissue, characterized by a specific SS18 rearrangement. It generally manifests as a lesion composed of monomorphic spindle cells and sometimes shows variable epithelial differentiation. Epithelial-type synovial sarcoma is rare, and synovial sarcoma with overwhelming neuroendocrine differentiation has not been reported previously. Here, we present a case of a young man with an epithelial-type synovial sarcoma of the right leg that showed an overwhelming neuroendocrine differentiation. The diagnosis was confirmed by the detection of targeted fusion re-arrangement associated with synovial sarcoma. This study emphasizes the importance of molecular approaches in modern soft tissue pathology. Detecting the expression of neuroendocrine antigens in synovial sarcoma is a pre-requisite to avoid misdiagnosis of metastatic neuroendocrine tumor, malignant peripheral nerve sheath tumor with glandular differentiation, and carcinosarcoma. © 2022. The Author(s).

Citation

Ying Chen, Ning Zhou, Deyu Guo, Xiaodong Wang, Xin He, Yujuan Xu. Predominantly epithelial-type synovial sarcoma with overwhelming neuroendocrine differentiation: a potential diagnostic pitfall. Diagnostic pathology. 2022 Jul 11;17(1):59