Parathyroid/Bone Disorders
Anuja Choure, MD
Endocrinology Fellow
University of Arizona college of Medicine
Chandler, Arizona, United States
Pt is a 58 yr old male who was referred for hypercalcemia in the setting of MEN1 syndrome. He complained of anorexia, weight loss and muscle weakness and Ca was elevated to 11.2.
Pt was diagnosed at age 8 with primary hyperparathyroidism and underwent a partial parathyroidectomy at age 12 with removal of 3 glands. Around age 24, he had recurrence of hypercalcemia and underwent removal of the 4th parathyroid gland. Half of the gland was transplanted into his left arm into 7 little pockets. He was then diagnosed with pancreatic NET (gastrinoma) requiring partial pancreatectomy. Pt also reported a history of multiple lipomas and angiolipomas, some of which required resection. He also had an obstructing colonic lipoma s/p resection and path confirming lipoma. He again had recurrence of hyperparathyroidism and underwent resection of all pockets except for one with path showing hyperplastic parathyroid gland. Family history of MEN-1 in multiple relatives on maternal side. MENI 1 genetic testing was positive.
Labs: Calcium 11.5, Glucagon 110 ph/ml, Chromogranin A 96ng/ml (Ref < 93), gastrin 33 pg/mL, VIP < 50, Prolactin 21.5 ng/mL (3.46-19.4), PTH 109 with Ca 2.5.
Imaging: 1. MRI Brain with 4.5 mm left pituitary microadenoma. 2. Dotatate scan: six well-defined radiotracer avid enhancing pancreatic masses ranging in size from 1.1-2.5 cm within the pancreatic body and tail consistent with multifocal neuroendocrine tumors in the setting of MEN1. 3. Sestamibi scan left forearm: Tracer uptake over lateral aspect of flexor aspect of left forearm, suggestive of site of transplanted parathyroid tissue. 4. Dexa Scan: Low bone mass.
Plan: Pt initiated on Cinacalcet. Repeat Calcium level 10, with PTH of 43.