PMID- 28070481 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20201001 IS - 2213-0071 (Print) IS - 2213-0071 (Electronic) IS - 2213-0071 (Linking) VI - 20 DP - 2017 TI - Lung adenocarcinoma and adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1. PG - 77-81 LID - 10.1016/j.rmcr.2016.12.002 [doi] AB - Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder caused by heterozygous germline mutations in the tumor suppressor gene MEN1, which encodes a nuclear protein, menin. MEN1 is characterized by the combined occurrence of tumors involving the pituitary gland, pancreatic islets, and parathyroid glands. Additionally, patients with MEN1 often exhibit adrenal tumors. Although most MEN1-associated tumors are benign, malignant lesions arising in these endocrine organs have been reported. Additionally, malignant diseases of non-endocrine organs concomitant with MEN1 have also been reported. Here, we report a rare case of a MEN1 patient who exhibited adrenocortical carcinoma (ACC) and lung adenocarcinoma (LAC). A 53-year-old Japanese woman was diagnosed with genetically proven MEN1 that initially manifested as parathyroid, pancreatic, and adrenal tumors. During the course of the disease, she developed LAC harboring the epidermal growth factor receptor gene mutations and cortisol-secreting ACC. Both tumors were surgically resected. The tumor cells were immunohistochemically negative for menin. Studies have suggested a causative link between MEN1 gene mutations and ACC, and menin expression may decrease in MEN1-related ACCs. In contrast, there are few reports suggesting a specific role of MEN1 gene mutations in LAC. Menin is often inactivated in the LACs of patients without MEN1. Thus, our patient's ACC probably occurred as part of MEN1, whereas the latter had no evident etiological association with her LAC. This case demonstrates the need for physicians to consider the potential development of malignant diseases originating from both endocrine and non-endocrine organs in MEN1 patients. FAU - Ohara, Nobumasa AU - Ohara N AD - Department of Endocrinology and Metabolism, Nagaoka Red Cross Hospital, Niigata, Japan; Department of Endocrinology and Metabolism, Uonuma Institute of Community Medicine, Niigata University Medical and Dental Hospital, Niigata, Japan. FAU - Kaneko, Masanori AU - Kaneko M AD - Department of Endocrinology and Metabolism, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Ikeda, Masahiro AU - Ikeda M AD - Department of Urology, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Ishizaki, Fumio AU - Ishizaki F AD - Department of Urology, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Suzuki, Kazuya AU - Suzuki K AD - Department of Urology, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Maruyama, Ryo AU - Maruyama R AD - Department of Urology, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Komeyama, Takeshi AU - Komeyama T AD - Department of Urology, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Sato, Kazuhiro AU - Sato K AD - Department of Respiratory Medicine, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Togashi, Kenichi AU - Togashi K AD - Department of Thoracic Surgery, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Usuda, Hiroyuki AU - Usuda H AD - Department of Pathology, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Yamazaki, Yuto AU - Yamazaki Y AD - Department of Pathology, Tohoku University Graduate School of Medicine, Miyagi, Japan. FAU - Sasano, Hironobu AU - Sasano H AD - Department of Pathology, Tohoku University Graduate School of Medicine, Miyagi, Japan. FAU - Kaneko, Kenzo AU - Kaneko K AD - Department of Endocrinology and Metabolism, Nagaoka Red Cross Hospital, Niigata, Japan. FAU - Kamoi, Kyuzi AU - Kamoi K AD - Department of Internal Medicine, Ojiya General Hospital, Niigata, Japan; Center of Diabetes, Endocrinology and Metabolism, Joetsu General Hospital, Niigata, Japan. LA - eng PT - Case Reports DEP - 20161218 PL - England TA - Respir Med Case Rep JT - Respiratory medicine case reports JID - 101604463 PMC - PMC5219631 OTO - NOTNLM OT - ACC, adrenocortical carcinoma OT - ALK, anaplastic lymphoma kinase OT - Adrenocortical carcinoma OT - CT, computed tomography OT - EGFR, epidermal growth factor receptor OT - Epidermal growth factor receptor gene mutation OT - Immunohistochemistry OT - LAC, lung adenocarcinoma OT - Lung adenocarcinoma OT - MEN1, multiple endocrine neoplasia type 1 OT - MRI, magnetic resonance imaging OT - Menin OT - Multiple endocrine neoplasia type 1 OT - SF-1, steroidogenic factor OT - TTF-1, thyroid transcription factor-1 EDAT- 2017/01/11 06:00 MHDA- 2017/01/11 06:01 PMCR- 2016/12/18 CRDT- 2017/01/11 06:00 PHST- 2016/07/01 00:00 [received] PHST- 2016/12/14 00:00 [revised] PHST- 2016/12/16 00:00 [accepted] PHST- 2017/01/11 06:00 [entrez] PHST- 2017/01/11 06:00 [pubmed] PHST- 2017/01/11 06:01 [medline] PHST- 2016/12/18 00:00 [pmc-release] AID - S2213-0071(16)30216-7 [pii] AID - 10.1016/j.rmcr.2016.12.002 [doi] PST - epublish SO - Respir Med Case Rep. 2016 Dec 18;20:77-81. doi: 10.1016/j.rmcr.2016.12.002. eCollection 2017.