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Tumors of the Thyroid Gland (C-Cells)

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Book cover Surgical Pathology of Endocrine and Neuroendocrine Tumors

Part of the book series: Current Clinical Pathology ((CCPATH))

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Abstract

Medullary thyroid carcinoma (MTC) comprises 5–10% of all thyroid malignancies and occurs sporadically in 75% of cases, while the remainder develop in association with multiple endocrine neoplasia (MEN) 2A, MEN2B, or familial MTC (FMTC). The MEN2 syndromes, which have autosomal dominant patterns of inheritance, occur as the result of germline mutations in the RET proto-oncogene (10q 11.2). Guidelines for age-stratified prophylactic thyroidectomies in MEN2A, MEN2B, and FMTC are now based on the results of mutational analyses. The tumors exhibit a remarkably wide spectrum of histological appearances and may mimic every type of thyroid malignancy. A small proportion of the cases represent mixed follicular and C cell neoplasms. The heritable tumors are preceded in their development by phases of C cell hyperplasia although there is a growing body of data to support the view that the proliferating C cells represent preinvasive malignancies (carcinomas in situ). C cell hyperplasia (CCH) also occurs in patients with hypercalcemia, hypergastrinemia, Hashimoto's disease, and in the immediate vicinity of follicular cell neoplasms (peritumoral CCH). The latter types of hyperplasia have been classified as physiological or secondary CCH as compared to the C cell proliferative changes in the MEN2 syndromes. In contrast to physiological hyperplasia in which C cells most often appear normal cytologically, the C cells in MEN2 syndromes are frequently dysplastic. However, these distinctions may not always be apparent in individual cases, including some normal glands, which may demonstrate considerable variations in C cell distribution and morphology.

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  1. Pathologist-in-Chief, Lifespan AMC Pathology Laboratories, Professor and Associate Chair of Pathology and Laboratory Medicine, The Warren Alpert Medical School of Brown University, Department of Pathology, Rhode Island Hospital, Providence, RI 02903, USA

    Ronald A. DeLellis

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    Ashraf Khan

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DeLellis, R.A. (2009). Tumors of the Thyroid Gland (C-Cells). In: Khan, A. (eds) Surgical Pathology of Endocrine and Neuroendocrine Tumors. Current Clinical Pathology. Humana Press. https://doi.org/10.1007/978-1-60327-396-1_6

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