Less than 1% cases of acromegaly is caused by ectopic secretion of growth hormone releasing hormone (GHRH).
A 25-year-old woman was admitted to the hospital, with iron deficiency anemia, acromegaly and a 6×6 cm mass in infrahepatic location near to the pancreatic head. Sellar magnetic resonance (MR) imaging indicated pituitary enlargement without obvious evidence of a pituitary adenoma. The patients underwent abdominal exploration. Histopathological diagnosis was a well-differentiated neuroendocrine carcinoma of duodenum with the invasion of tunica muscularis and 9 metastatic lymph nodes (the greatest size 5×5×2.5 cm in diameter). Neoplastic cells showed cytoplasmic immunoreactivity to GHRH and GHRH-receptor. Because increased IGF-1 concentrations persisted after the operation, octreotide LAR 20 mg/month was begun. Growth hormone and IGF-1 levels normalized. At the end of six years of follow-up, a left paraaortic mass, showing uptake of indium111 octreotide was detected. Operation showed a metastatic lymph node of 2.5×1.5×1.4 cm in diameter. After the operation IGF-1 concentration was mildly elevated. OctreotideLAR 10 mg/day was begun and continued until the present time.
We suggest that octreotide treatment may lead to a delay in tumor growth and the clinical and radiological diagnosis of recurrence and have a beneficial effect on disease course during a total follow-up of 7 years for our case. Expression of both GHRH and GHRH-R may indicate the autocrine/paracrine role of GHRH for proliferation of tumor tissue itself.