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Surgical Management of Parathyroid and Adrenal Glands in Inherited Medullary Thyroid Carcinoma

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Medullary Thyroid Cancer

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Abstract

Hereditary medullary thyroid cancer is a component of the type 2 multiple endocrine neoplasia syndromes (MEN). This chapter focuses on the management of the adrenal glands and parathyroid glands in classical MEN2A and MEN2B. Classical MEN2A is associated with pheochromocytoma and primary hyperparathyroidism, which both have varied frequency/penetrance depending on the specific RET mutation. In MEN2B pheochromocytoma will develop in approximately 50% of patients and tends to be diagnosed at a younger age. Pheochromocytomas in MEN2 are usually benign, multicentric, bilateral, and confined to the adrenal gland. Surgical approach to pheochromocytoma in MEN2 should be aimed at removing the secreting lesion while trying to preserve adrenal function to avoid corticosteroid dependence with associated risks of acute adrenal insufficiency, which may be fatal. Cortical-sparing adrenalectomy is a safe surgical option discussed in depth in this chapter. Primary hyperparathyroidism is reported to develop in 20–30 % of patients with MEN2 and is generally mild. It may be diagnosed synchronously with medullary thyroid cancer or more likely, after thyroidectomy, but it is rarely the presenting diagnosis for a patient with MEN2A. Careful management of the parathyroid glands and the central neck contents as described in detail in this chapter help avoid hypoparathyroidism.

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  1. Section of Endocrine Surgery, Division of Surgical Oncology, Department of Surgery, Medical College of Wisconsin, 9200 West Wisconsin Ave, Milwaukee, WI, 53226, USA

    Azadeh A. Carr & Douglas B. Evans

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  2. Douglas B. Evans
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Correspondence to Azadeh A. Carr .

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  1. Associate Professor of Surgery; Chief, Section of Endocrine Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

    Tracy S. Wang

  2. Donald C. Ausman Family Foundation Professor in Surgery and Chair, Medical College of Wisconsin, Milwaukee, Wisconsin, USA

    Douglas B. Evans

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Carr, A.A., Evans, D.B. (2016). Surgical Management of Parathyroid and Adrenal Glands in Inherited Medullary Thyroid Carcinoma. In: Wang, T., Evans, D. (eds) Medullary Thyroid Cancer. Springer, Cham. https://doi.org/10.1007/978-3-319-39412-1_10

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