Multiple endocrine neoplasia type I and Cushing's syndrome due to an aggressive ACTH producing thymic carcinoid

Junko Takagi; Kazuo Otake; Munehiko Morishita; Harumichi Kato; Naoki Nakao; Kazuhiro Yoshikawa; Hiroshi Ikeda; Yoshifumi Hirooka; Yoshinobu Hattori; Catharina Larsson; Tsuyoshi Nogimori

doi:10.2169/internalmedicine.45.1427

Multiple endocrine neoplasia type I and Cushing's syndrome due to an aggressive ACTH producing thymic carcinoid

Intern Med. 2006;45(2):81-6. doi: 10.2169/internalmedicine.45.1427. Epub 2006 Feb 15.

Authors

Junko Takagi¹, Kazuo Otake, Munehiko Morishita, Harumichi Kato, Naoki Nakao, Kazuhiro Yoshikawa, Hiroshi Ikeda, Yoshifumi Hirooka, Yoshinobu Hattori, Catharina Larsson, Tsuyoshi Nogimori

Affiliation

¹ Department of Laboratory Medicine, Aichi Medical University School of Medicine, Nagakute, Aichi.

PMID: 16484744
DOI: 10.2169/internalmedicine.45.1427

Abstract

Thymic carcinoid in multiple endocrine neoplasia type 1 (MEN 1) is previously reported as a non-ACTH producing tumor. The present case is a 39-year-old man with mortal outcome from thymic carcinoid and Cushing's 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. Cushing's syndrome due to ACTH producing thymic carcinoid should be also considered as one phenotype of the MEN 1 spectrum.

Publication types

Case Reports

MeSH terms

ACTH Syndrome, Ectopic / blood
ACTH Syndrome, Ectopic / etiology*
Adrenocorticotropic Hormone / blood
Adult
Antineoplastic Agents, Hormonal / therapeutic use
Antineoplastic Combined Chemotherapy Protocols / administration & dosage
Carcinoid Tumor / blood
Carcinoid Tumor / complications*
Carcinoid Tumor / pathology
Carcinoid Tumor / therapy
Combined Modality Therapy
Cushing Syndrome / blood
Cushing Syndrome / etiology*
Fatal Outcome
Germ-Line Mutation
Humans
Male
Multiple Endocrine Neoplasia Type 1 / blood
Multiple Endocrine Neoplasia Type 1 / diagnosis*
Multiple Endocrine Neoplasia Type 1 / genetics
Multiple Endocrine Neoplasia Type 1 / therapy
Octreotide / therapeutic use
Radiotherapy, Adjuvant
Thymectomy
Thymus Neoplasms / blood
Thymus Neoplasms / complications*
Thymus Neoplasms / pathology
Thymus Neoplasms / therapy

Substances

Antineoplastic Agents, Hormonal
Adrenocorticotropic Hormone
Octreotide