ACTH-secreting thymic carcinoid associated with multiple endocrine neoplasia type 1.
Thymic carcinoids are classified into three categories: (1) nonsecretory tumors, (2) hormonal secretory tumors, and (3) tumors associated with multiple endocrine neoplasia type 1. We report a rare case with adrenocorticotropic hormone secreting thymic carcinoid with multiple endocrine neoplasia type 1. Radiologic examination showed an anterior mediastinal mass and a parathyroid tumor. Blood analysis revealed high levels of parathyroid hormone and adrenocorticotropic hormone. Urine cortisol and 17-hydroxycorticoids levels were also elevated. Extended thymectomy was performed. Subsequently adjuvant radiation therapy and parathyroid tumor resection were performed. A germline mutation of exon 7 in the multiple endocrine neoplasia type 1 gene was detected and a somatic mutation of exon 9 was demonstrated in the thymic tumor.
Yano
Motoki
M
Department of Surgery II, Nagoya City University Medical School, Nagoya, Aichi, Japan. motoki@med.nagoya-cu.ac.jp
Fukai
Ichiro
I
Kobayashi
Yoshihiro
Y
Mizuno
Kotaro
K
Konishi
Akimitsu
A
Haneda
Hiroshi
H
Suzuki
Eriko
E
Endo
Katsuhiko
K
Fujii
Yoshitaka
Y
D000182:ACTH Syndrome, Ectopic / Q000097:blood / Q000209:etiology*; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D002276:Carcinoid Tumor / Q000097:blood / Q000235:genetics / Q000378:metabolism* / Q000628:therapy; D003131:Combined Modality Therapy; D004252:DNA Mutational Analysis; D004273:DNA, Neoplasm / Q000235:genetics; D005091:Exons / Q000235:genetics; D005483:Flushing / Q000209:etiology; D016147:Genes, Tumor Suppressor; D006801:Humans; D006934:Hypercalcemia / Q000209:etiology; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000097:blood / Q000150:complications*; D015282:Octreotide / Q000627:therapeutic use; D010281:Parathyroid Hormone / Q000097:blood / Q000378:metabolism; D010282:Parathyroid Neoplasms / Q000235:genetics / Q000378:metabolism / Q000628:therapy; D016105:Parathyroidectomy; D010997:Pleural Neoplasms / Q000556:secondary / Q000601:surgery; D018714:Radiotherapy, Adjuvant; D013934:Thymectomy; D013953:Thymus Neoplasms / Q000097:blood / Q000235:genetics / Q000378:metabolism* / Q000628:therapy
D018931:Antineoplastic Agents, Hormonal; D004273:DNA, Neoplasm; D010281:Parathyroid Hormone; D015282:Octreotide