Ectopic Growth Hormone-Releasing Hormone Secretion by a Neuroendocrine Tumor Causing Acromegaly: Long-Term Follow-Up Results
- 21 March 2009
- journal article
- case report
- Published by Springer Science and Business Media LLC in Endocrine Pathology
- Vol. 20 (2), 127-132
- https://doi.org/10.1007/s12022-009-9067-1
Abstract
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 × 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 indium111 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.Keywords
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