Abstract. Primary hyperparathyroidism is the most common hormonal manifestation associated with multiple endocrine neoplasia 1 (MEN1). It is generally caused by parathyroid hyperplasia, and parathyroid carcinoma is rare. Here, we report a case of MEN1 with parathyroid carcinoma in two parathyroid glands causing primary hyperparathyroidism. A 40-year-old man with primary hyperparathyroidism due to MEN1 underwent a total parathyroidectomy. His corrected calcium and intact PTH (i-PTH) serum levels were 10.8 mg/dL and 203 pg/mL, respectively. Although three glands were successfully removed, the left upper parathyroid gland could not be detected. Since the right lower parathyroid lesion had invaded into the thyroid, right lobectomy was performed. A portion of the left lower parathyroid tissue was transplanted into his forearm. The histological findings of the left lower and the right upper parathyroid glands were consistent with hyperplasia while that of the right lower parathyroid gland was parathyroid carcinoma. Since the post-surgical i-PTH levels remained high, the intrathyroidal lesion of the left lobe, which was initally diagnosed as an adenomatous nodule, was suspected to contain parathyroid tumor. A fine needle aspiration of the tumor revealed a high concentration of i-PTH. One week after the first surgery, a left thyroid lobectomy was performed. The pathological diagnosis of the tumor was parathyroid carcinoma. After the surgery, calcium and i-PTH levels were normal. Although it is rare, parathyroid carcinoma should be considered as a cause of hyperparathyroidism in MEN1 patients. Since it is difficult to diagnose parathyroid carcinoma before surgery, intraoperative findings are important for the appropriate treatment. nancy that is found in less than 5% of PHPT patients [2]. PC occurs only in 2% of MEN1 patients that have received surgery for PHPT [3] in contrast to the 15% of PC found in hyperparathyroidism-jaw tumor syndrome patients [4]. Here, we report a case of MEN1 with PC causing primary hyperparathyroidism.
Case presentationA 40-year-old man was referred for follow up of MEN1. His mother was diagnosed as PHPT due to MEN1, 3 years before by his previous doctor but she had not yet been treated. She also had non-functioning pancreatic tumor, lung cartinoid and was suspected as having prolactinoma as her serum PRL level elavated and pituitary gland swelled. At that time, the previous doctor examined his serum calcium (Ca) and intact-PTH (i-PTH) level and detected hypercalcemia and elevation of