Ectopic Growth Hormone-Releasing Hormone Secretion by a Neuroendocrine Tumor Causing Acromegaly: Long-Term Follow-Up Results |
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Authors: | Ne?e Colak Ozbey Yersu Kapran Alp Bozbora Ye?im Erbil Cemil Tascioglu Sylvia L. Asa |
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Affiliation: | (1) Istanbul Medical Faculty, Department of Medicine, Division of Endocrinology, Istanbul University, 34390, Capa, Istanbul, Turkey;(2) Istanbul Medical Faculty, Department of Pathology, Istanbul University, Istanbul, Turkey;(3) Istanbul Medical Faculty, Department of Surgery, Istanbul University, Istanbul, Turkey;(4) Istanbul Medical Faculty, Department of Medicine, Istanbul University, Istanbul, Turkey;(5) Department of Pathology, University Health Network, Toronto, ON, Canada |
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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. |
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Keywords: | GHRH ectopic acromegaly octreotide neuroendocrine carcinoma recurrence |
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