LAUSR

A 38-year-old man with a history of tobacco abuse presented with recurrent symptomatic nephrolithiasis. Evaluation revealed elevated calcium of 11.8 mg/dl and elevated parathyroid hormone. He was diagnosed with primary hyperparathyroidism and was referred for endocrine surgery for parathyroidectomy. Preoperative localization 99mTc-sestaMIBI scan suggested possible right-sided adenoma; however, intraoperative exploration revealed four-gland hyperplasia necessitating subtotal parathyroidectomy. His intraoperative PTH was reduced from 132 to 45 pg/mL postparathyroidectomy, and pathology revealed nodular hyperplasia of all four glands. Due to noted tracer activity in the right mediastinum and medial lung on preoperative sestaMIBI scan (Fig. 1), a chest CT was recommended, which revealed a right upper lobe obstructing bronchial mass, enlarged right hilar lymph nodes, and thyroid nodules in the isthmus and inferior left lobe. Biopsy of the lung nodule revealed carcinoid tumor, and octreotide scan revealed intense radiotracer uptake in the right hilar region corresponding to the carcinoid tumor seen on chest CT. A 24-h urinary 5-HIAA was normal at 4.9 mg/ 24 h, prolactin 30.2 ng/ml, and fasting gastrin 208 pg/ml. He underwent right upper lung lobectomy, revealing an 8 mm carcinoid tumor without lymph node involvement and negative surgical margins. The pathology test result by fine-needle aspiration of both thyroid nodules was benign. Geneticscreening test for multiple endocrine neoplasia type 1 revealed a novel K224T missense mutation in the MEN1 gene (Fig. 2) resulting in a substitution of A– > C at position 671 (coordinates 64575136). His 5-year old daughter was tested and found to have the same mutation. His living parent and siblings have deferred testing at this point but have no clinical signs or symptoms of disease. Further tumor surveillance included an MRI of the abdomen, which revealed a 1.7-cm lesion in the left hepatic lobe with normal-appearing pancreas. Subsequent labs revealed a fasting gastrin level of 478 pg/mL (reference range < 100 pg/ mL), normal pancreatic polypeptide (305 pg/nL), normal glucagon level (121 pg/ml), normal insulin level (17.5 IU/ml), chromogranin A 44 ng/mL (normal < 15 ng/mL), and vasoactive intestinal polypeptide 50 pg/mL (reference range 20– 42 pg/mL). He was referred to GI and underwent EGD, which revealed gastritis and duodenitis. An EUS did not reveal any evidence of pancreatic or duodenal masses. A follow-up prolactin level was elevated at 58.6 ng/mL, and pituitary MRI demonstrated a 4-mm microadenoma. His testosterone at the time was low at 222 ng/dL, and, after treatment with cabergoline, has normalized to 502 ng/dL. Due to rising gastrin levels with a new mesenteric lymph node on MRI abdomen with negative EGD, a Gallium 68-DOTATATE CT was obtained, which revealed a small duodenal lesion and uptake in the mesenteric lymph nodes. The patient is currently undergoing work-up for these new findings.