Multiple Endocrine Neoplasia Type I and Cushings Syndrome Due to an Aggressive ACTH Producing Thymic Carcinoid

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概要

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• Thymic carcinoid in multiple endocrine neoplasia type 1 (MEN 1) is previously reported as a nonACTH producing tumor. The present case is a 39yearold man with mortal outcome from thymic carcinoid and Cushings syndrome with high plasma ACTH. The symptom was first observed at age 29 and was relieved after extended thymectomy, with reduction of ACTH level. The tumor was positive for ACTH, Grimelius silver staining and Chromogranin A. The finding of primary hyperparathyroidism, pituitary adenoma, and a novel germline nonsense mutation (W423X) established the diagnosis of MEN 1. Cushings syndrome due to ACTH producing thymic carcinoid should be also considered as one phenotype of the MEN 1 spectrum.

著者

• Hattori Yoshinobu

  Department Of Ophthalmology Kitami Red Cross Hospital

• NOGIMORI Tsuyoshi

  Konanshowa Hospital

• Takagi Junko

  Department Of Chemistry And Research Center For Materials Science Nagoya University

• YOSHIKAWA Kazuhiro

  Department of Applied Chemistry, School of Engineering, Nagoya University

• IKEDA HIROSHI

  Deparment of Biology, Faculty of Science, Tokyo Metropolitan University

• HIROOKA Yoshifumi

  Department of Laboratory Medicine

• Kato Harumichi

  Division of Respiratory Medicine, Aichi Medical University School of Medicine

• Otake Kazuo

  Division of Endocrinology, Metabolism, and Diabetes, Aichi Medical University School of Medicine

• Morishita Munehiko

  Division of Respiratory Medicine, Aichi Medical University School of Medicine

• Nakao Naoki

  Division of Neurology, Aichi Medical University School of Medicine

• Larsson Catharina

  Department of Molecular Medicine, Karolinska Hospital

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