AACE CLINICAL CASE REPORTS Vol 4 No. 5 September/October 2018 e383 Copyright © 2018 AACE Case Report ECTOPIC ATYPICAL PARATHYROID NEOPLASM IN A PATIENT WITH MULTIPLE ENDOCRINE NEOPLASIA TYPE I Rimesh Pal, MD 1 ; Sanjay Kumar Bhadada, MD 1 ; Pinaki Dutta, MD 1 ; Arunanshu Behera, MS 2 ; Shibojit Talukder, MS 2 ; Uma Nahar Saikia, MD 3 ; Suvradeep Mitra, MD 3 ; Gurjeet Kaur, PhD 1 ; Anil Bhansali, MD 1 Submitted for publication February 17, 2018 Accepted for publication March 17, 2018 From the 1 Department of Endocrinology, 2 Department of General Surgery, and 3 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Address correspondence to Dr. Anil Bhansali, Department of Endocrinology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012, India. E-mail: [email protected]. DOI:10.4158/ACCR-2018-0002 To purchase reprints of this article, please visit: www.aace.com/reprints. Copyright © 2018 AACE. ABSTRACT Objective: Present a case of an atypical parathyroid adenoma in the mediastinum as a part of multiple endo- crine neoplasia type 1 (MEN1). Methods: Clinical, laboratory, imaging, and histolo- pathogic analyses were performed. Results: A 50-year-old man who was diagnosed with chronic kidney disease secondary to bilateral nephroli- thiasis was referred for evaluation of incidentally detected hypercalcemia. He had an acromegaloid facies and lipo- mas over the forehead. Laboratory evaluation revealed hypercalcemia (3.33 mmol/L), hyperphosphatemia, and elevated intact parathyroid hormone level (215 pmol/L). Serum insulin-like growth factor 1 was elevated and growth hormone was non-suppressible on oral glucose load. Contrast-enhanced magnetic resonance imaging of the sella showed a pituitary microadenoma. A scan using 99mTc-Sestamibi and combined positron emission and computed tomography using fluorocholine F-18 revealed a mediastinal ectopic parathyroid adenoma, thereby attrib- uting hypercalcemia to primary hyperparathyroidism. A clinical diagnosis of MEN1 was made. Genetic analysis revealed an A>T substitution at cDNA 253 in exon 2 of the MEN1 gene. He underwent median sternotomy and a 3 × 3-cm mass was excised, weighing 14 g. Histopathology showed parathyroid tissue with moderate pleomorphism, pushing margins, fibrous trabeculae, atypical mitoses, and low Ki-67 index, suggestive of atypical parathyroid neoplasm. After surgery the patient became normocal- cemic. On follow-up, he is asymptomatic without any evidence of recurrence of primary hyperparathyroidism. Conclusion: Parathyroid involvement in MEN1 is almost exclusively in the form of benign hyperplasia. Atypical parathyroid adenomas and parathyroid carcino- mas in MEN1 are very rare, limited to only a few anecdotal case reports to which ours contributes a new case. (AACE Clinical Case Rep. 2018;4:e383-e387) Abbreviations: CKD = chronic kidney disease; iPTH = intact parathy- roid hormone; MEN1 = multiple endocrine neoplasia type 1 INTRODUCTION Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome characterized by the presence of 2 of the following 3 tumors: parathyroid, duodeno-pancreatic neuroendocrine, and anterior pitu- itary. Parathyroid involvement, in the form of multiple benign hyperplastic eutopic parathyroid glands manifest- ing as primary hyperparathyroidism, is the most common presenting feature of MEN1 (1). Rarely, ectopic parathy- roid adenomas have also been reported in MEN1, often located in the thymus, thyroid gland, anterior mediastinum, pericardium, or surrounding the trachea, esophagus, and the carotid arteries (2). Occasionally, MEN1 is associated with parathyroid carcinomas (3-10). More scarce is the association of atypical parathyroid neoplasm with MEN 1 with only one report to date in the literature (11). This This is an Open Access article under the CC-BY-NC license. -ND
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