Primary Paraganglioma of Thyroid Gland: A Clinicopathologic and Immunohistochemical Analysis of Three Cases with a Review of the Literature

Thyroid paraganglioma is an extremely rare tumor and frequently mistaken for other thyroid neoplasms. Increased awareness of its potential presentation in thyroid and its characteristic features is essential for avoiding diagnostic and therapeutic pitfalls. We describe here three additional cases of primary thyroid paraganglioma and analyze their clinical findings and pathological characteristics. Patients included two women and one man presenting with asymptomatic thyroid nodules. Radiological examinations were nonspecific and none had been diagnosed correctly before surgery. On intraoperative frozen section consultation they were all misdiagnosed as carcinomas, either primary or metastatic. However, the permanent sections showed features consistent with paraganglioma. Of note, two cases displayed extension into adjacent thyroid tissues, one of which exhibited increased mitotic activity, confluent tumor necrosis and vascular invasion. Immunohistochemically, the neoplastic chief cells expressed chromogranin, synaptophysin, neuron-specific enolase and CD56, whereas the sustentacular cells were highlighted by S100 protein. All three patients were well with normal hormone secretion, without local recurrence or distant metastasis at last follow-up (range 10–47 months). We further reviewed the literature to summarize the characteristics of this distinctive entity. Albeit being very rare, paraganglioma should be included in the differential diagnosis of hypervascular thyroidal neoplasms. Accurate diagnosis relies on the histopathogical findings and adjunctive immunohistochemcial studies. To date, all the reported cases have pursued a benign course. Although atypical features seem to have no association with clinical behavior, long time postoperative surveillance with biochemical screening of hormone secretion, cervical ultrasonography and whole-body CT scan is recommended.