PMID- 27212590 OWN - NLM STAT- MEDLINE DCOM- 20170316 LR - 20181202 IS - 1743-9159 (Electronic) IS - 1743-9159 (Linking) VI - 31 DP - 2016 Jul TI - Parathyroid carcinoma and atypical parathyroid neoplasms in MEN1 patients; A clinico-pathologic challenge. The MD Anderson case series and review of the literature. PG - 10-6 LID - S1743-9191(16)30118-2 [pii] LID - 10.1016/j.ijsu.2016.05.035 [doi] AB - BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a genetic disorder characterized by usually benign tumors of the parathyroid glands, pancreatic islet cells, and anterior pituitary. Hyperparathyroidism (HPT) occurs in 90% of MEN1 patients. In rare cases, it is associated with parathyroid carcinoma (PC) or atypical parathyroid neoplasm (APN). We present a cohort of 3 such patients. METHODS: We performed a retrospective review of our institution's MEN1 database to identify patients who underwent operations for HPT and had a histopathologic diagnosis of PC or APN. Clinical features, genetics, and outcomes were summarized. RESULTS: Of 291 MEN1 patients, 242 had HPT (83.2%). Two of the 242 patients (0.8%) had a histopathologic diagnosis of PC, and 1 (0.4%) had a diagnosis of APN. The patients with PC were male, ages 62 and 56 years at the time of surgery; the patient with APN was female, age 32 years. All patients also had a pancreatic endocrine tumor. The observed genetic mutations in the PC patients were c.703G > A (p.E235K) in exon 4 and c.1378C > T (p.R460X) in exon 10. All 3 patients had recurrence of hypercalcemia, and 2 patients underwent reoperation; pathologic analysis revealed the presence of a hyperplastic gland, not tumor recurrence. No cases had distant metastasis. CONCLUSIONS: This is the first report of APN in an MEN1 patient. Although rare, the presence of PC or APN in MEN1 is noteworthy because it affects the management if hypercalcemia recurs, possibly requiring an open approach rather than the minimally invasive techniques used in the reoperative setting for benign disease. CI - Copyright (c) 2016 IJS Publishing Group Ltd. Published by Elsevier Ltd. All rights reserved. FAU - Christakis, Ioannis AU - Christakis I AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Busaidy, Naifa L AU - Busaidy NL AD - Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Cote, Gilbert J AU - Cote GJ AD - Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Williams, Michelle D AU - Williams MD AD - Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Hyde, Samuel M AU - Hyde SM AD - Department of Clinical Cancer Genetics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Silva Figueroa, Angelica M AU - Silva Figueroa AM AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Kwatampora, Lily Joy AU - Kwatampora LJ AD - Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Clarke, Callisia N AU - Clarke CN AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Qiu, Wei AU - Qiu W AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; The First Hospital of Jilin University, Hepatobiliary Pancreatic Surgical Department, Changchun, Jilin, China. FAU - Lee, Jeffrey E AU - Lee JE AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. FAU - Perrier, Nancy D AU - Perrier ND AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA. Electronic address: nperrier@mdanderson.org. LA - eng PT - Case Reports PT - Journal Article PT - Review DEP - 20160519 PL - United States TA - Int J Surg JT - International journal of surgery (London, England) JID - 101228232 SB - IM MH - Adult MH - Female MH - Humans MH - Hyperparathyroidism/genetics/*pathology/surgery MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/genetics/*pathology MH - Mutation MH - Parathyroid Glands/pathology/surgery MH - Parathyroid Neoplasms/genetics/*pathology/surgery MH - Retrospective Studies OTO - NOTNLM OT - Hypercalcemia OT - Multiple endocrine neoplasia type 1 (MEN 1) OT - Neoplasm OT - Parathyroid carcinoma OT - Parathyroid gland OT - Parathyroidectomy OT - Primary hyperparathyroidism EDAT- 2016/05/24 06:00 MHDA- 2017/03/17 06:00 CRDT- 2016/05/24 06:00 PHST- 2016/02/03 00:00 [received] PHST- 2016/03/22 00:00 [revised] PHST- 2016/05/15 00:00 [accepted] PHST- 2016/05/24 06:00 [entrez] PHST- 2016/05/24 06:00 [pubmed] PHST- 2017/03/17 06:00 [medline] AID - S1743-9191(16)30118-2 [pii] AID - 10.1016/j.ijsu.2016.05.035 [doi] PST - ppublish SO - Int J Surg. 2016 Jul;31:10-6. doi: 10.1016/j.ijsu.2016.05.035. Epub 2016 May 19.