Abstract. While there is no doubt that total thyroidectomy is necessary for medullary thyroid carcinoma (MTC) in multiple endocrine neoplasia type 2A (Men2A) patients, there is still controversy regarding the management of the parathyroid glands. Although most, but not all, endocrine surgeons leave normal-appearing parathyroid glands in situ during thyroid surgery for Men2A, we have employed total parathyroidectomy with autotransplantation. Between 1994 and 2006, 12 Men2A patients underwent therapeutic total or completion thyroidectomy and lymph nodes dissection at least in the central compartment for MTC. Total or completion parathyroidectomy with autotransplantation was performed concurrently with above-mentioned surgery. All patients were over 25 years old, and the median age was 48.5 years. There were 5 males and 7 females from 8 families. The average number of transplanted parathyroid glands was 3. Serum calcium and intact PTh levels have been maintained during the median follow up of 107 months in all patients except for one who of died of advanced MTC one year after surgery. Total parathyroidectomy with autotransplantation at the time of primary surgery for MTC, i.e. total thyroidectomy with bilateral central neck dissection, is a feasible approach for managing the risk of hyperparathyroidism.

Neuroendocrine differentiation was identified in a subset (26%) of Japanese breast cancer patients, but this appeared to have no relationship with established prognostic factors or patient outcome.

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We report a rare case with pheochromocytoma as the first manifestation of multiple endocrine neoplasia type 2A with RET mutation S891A. Bilateral pheochromocytomas were identified in a 54-year-old woman. Screening for RET revealed a rare S891A mutation located in the intracellular tyrosine kinase domain. This mutation was previously recognized as one of the mutations only in cases manifesting solely medullary thyroid carcinomas (MTCs). Since calcitonin stimulation test indicated positive result, total thyroidectomy was performed 1 year after the bilateral adrenalectomy, and C-cell hyperplasia was diagnosed by histopathological examination. Our report suggests that cases with S891A mutation, akin to those with other RET mutations, require screening for pheochromocytoma. In addition, it is indicated that calcitonin stimulation test should be performed even in the unaffected elder cases with S891A mutation although the mutation is classified as lowest risk group on MTC in guidelines.

We herein report the case of a patient with critical hyperkalemia after unilateral adrenalectomy (ADX) for aldosterone-producing adenomas, which were coexisting with primary hyperparathyroidism. A right adrenal tumor oversecreting mineral corticoid was identified in a 62-year-old female whose kidney function had been impaired due to primary hyperaldosteronism and hyperparathyroidism. The ADX improved her hypertension with normalization of the plasma aldosterone concentration, but without adequately increasing her plasma renin activity. Her eGFR further decreased postoperatively, hyperkalemia appeared and the serum potassium level rose to 6.3 mEq/L at 3 months after ADX. Then, treatment with calcium polystyrene sulfonate jelly was started. Eight months after ADX, a left lower parathyroidectomy was performed, and the serum calcium and intact parathyroid hormone levels decreased to the normal range. The hyperkalemia was difficult to control within 20 months postoperatively without treatment with calcium polystyrene sulfonate jelly or hydrocortisone. This suggests that unmasking the renal impairment and relative hypoaldosteronism after ADX might induce critical hyperkalemia.