Ectopic growth hormone-releasing hormone secretion by a neuroendocrine tumor causing acromegaly: long-term follow-up results.
Ectopic secretion of growth hormone-releasing-hormone (GHRH) is a rare cause of acromegaly-representing less than 1% of patients. A 25-year-old woman was admitted to the hospital with acromegaly and a 6 x 6 cm infrahepatic mass. Sellar magnetic resonance imaging indicated diffuse pituitary enlargement consistent with hyperplasia. The infrahepatic mass was resected, and the histopathological diagnosis was a well-differentiated invasive neuroendocrine carcinoma of the duodenum with metastases to local lymph nodes. The tumor cells contained cytoplasmic immunoreactivity for GHRH. Because increased IGF-1 concentrations persisted after the operation, the patient was treated with octreotide long-acting repeatable (LAR) injections of 20 mg/month. Growth hormone and IGF-1 levels normalized. After 6 years of surveillance, a left paraaortic mass was detected by uptake of indium 111 octreotide. Surgical exploration revealed metastatic neuroendocrine carcinoma in a 2.5-cm lymph node. Postoperatively, the IGF-1 concentration was mildly elevated. Octreotide LAR therapy is being continued at 10 mg/month. This case suggests that octreotide treatment may have a beneficial effect on disease course and can be maintained for as long as 7 years in a patient with acromegaly due to a GHRH-secreting neuroendocrine carcinoma.
Colak Ozbey
Neşe
N
Istanbul Medical Faculty, Department of Medicine, Division of Endocrinology, Istanbul University, 34390, Capa, Istanbul, Turkey. nozbey@hotmail.com
Kapran
Yersu
Y
Bozbora
Alp
A
Erbil
Yeşim
Y
Tascioglu
Cemil
C
Asa
Sylvia L
SL
D000172:Acromegaly / Q000209:etiology*; D000328:Adult; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D018278:Carcinoma, Neuroendocrine / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism*; D004379:Duodenal Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism*; D005260:Female; D005500:Follow-Up Studies; D005951:Glucose Tolerance Test; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006729:Hormones, Ectopic / Q000378:metabolism*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D015282:Octreotide / Q000627:therapeutic use; D012008:Recurrence
D018931:Antineoplastic Agents, Hormonal; D006729:Hormones, Ectopic; D007334:Insulin-Like Growth Factor I; D013007:Growth Hormone-Releasing Hormone; D015282:Octreotide