Abstract Background Parathyroid carcinoma (PC) is a rare thyroid tumor. PC with sarcomatoid differentiation(PCSD) is even rarer and its exact etiology remains unclear. We here report a case of PCSD, and present the clinicopathological features and pathological diagnosis and review the literature. Case presentation A 71-year-old man presented with a mass of 4.5?cm?×?3.5?cm in the right neck. The tumor was composed of nest-like transparent cells, and the septum had heterotypic rhabdoid cells with sarcomatoid differentiation. Immunophenotype was as follows: myogenic differentiation 1(MyoD1), myogenin and desmin were positive; clear cells were positive for chromogranin A(CGA), synaptophysin(Syn) and GATA-3; and Ki-67 proliferation index was 40%. Hematoxylin and eosin staining and immunohistochemistry were performed. The patient was diagnosed with PCSD, and died 6?months after surgery. Conclusions PCSD is a rare type of primary parathyroid tumor with high malignancy and poor prognosis. Definitive diagnosis should be based on histopathological morphology and immunophenotype, and surgical treatment should be performed as soon as possible.
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