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Presented by Dr. Lisa Rooper and prepared by Dr. Tatianna Larman.
A 40 year old woman presents for thyroid lobectomy after fine needle aspiration showed atypia of unknown significance.
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Question 1 of 1
1. Question
A 40 year old woman presents for thyroid lobectomy after fine needle aspiration showed atypia of unknown significance. Choose the correct diagnosis.
Correct
Answer: PARAGANGLIOMA
Histology: The tumor is actually centered outside the thyroid gland. It consists of a nested proliferation of cells with abundant amphophilic cytoplasm embedded in a variably vascular and fibrotic stroma. Nuclei show moderate variation in size with speckled chromatin and occasional prominent nucleoli. Tumor cells are positive for synaptophysin, INSM1, and GATA3, and negative for AE1/AE3 with retained SDHB. Sustentacular cells are positive for S100.
Discussion: In the head and neck, paragangliomas most commonly arise in the carotid body but can affect a wide range of sites, including within or adjacent to the thyroid gland. Because these tumors frequently arise from parasympathetic paraganglia, they are most often non-functional and generally present with mass effect. Historically, tumor behavior has been regarded as unpredictable, without good correlation to histologic features. However, most malignant paragangliomas have recently been associated with SDH complex gene mutations. The most reliable means of differentiating a perithyroidal paragangliomas from other neuroendocrine tumors of this region, including medullary thyroid carcinoma and parathyroid neoplasms, is performing cytokeratin; paragangliomas are a cytokeratin-negative neuroendocrine tumor. They also are helpfully positive for GATA3. Loss of staining for SDHB protein correlates with SDH complex gene mutations and is frequently used as a prognostic marker.
Incorrect
Answer: PARAGANGLIOMA
Histology: The tumor is actually centered outside the thyroid gland. It consists of a nested proliferation of cells with abundant amphophilic cytoplasm embedded in a variably vascular and fibrotic stroma. Nuclei show moderate variation in size with speckled chromatin and occasional prominent nucleoli. Tumor cells are positive for synaptophysin, INSM1, and GATA3, and negative for AE1/AE3 with retained SDHB. Sustentacular cells are positive for S100.
Discussion: In the head and neck, paragangliomas most commonly arise in the carotid body but can affect a wide range of sites, including within or adjacent to the thyroid gland. Because these tumors frequently arise from parasympathetic paraganglia, they are most often non-functional and generally present with mass effect. Historically, tumor behavior has been regarded as unpredictable, without good correlation to histologic features. However, most malignant paragangliomas have recently been associated with SDH complex gene mutations. The most reliable means of differentiating a perithyroidal paragangliomas from other neuroendocrine tumors of this region, including medullary thyroid carcinoma and parathyroid neoplasms, is performing cytokeratin; paragangliomas are a cytokeratin-negative neuroendocrine tumor. They also are helpfully positive for GATA3. Loss of staining for SDHB protein correlates with SDH complex gene mutations and is frequently used as a prognostic marker.
