Abstract
Introduction: Ectopic secretion of GHRH is a rare cause of acromegaly. However, its recognition is clinically important because different therapeutic approaches are required. Case Presentation: We present a challenging case of acromegaly secondary to ectopic GHRH secretion from pancreatic neuroendocrine tumor in a 52-year-old female. The patient is treated with different modalities which include pegvisomant in an attempt to control the stimulated GH-axis considering the limited data about its use in treatment of ectopic acromegaly. Conclusion: GHRH-secreting tumor is a rare cause of acromegaly. Surgical resection of the tumor is the therapy of choice whenever possible. However, further studies are warranted for unresectable tumor or resistant cases.
Highlights
Ectopic secretion of Growth Hormone-Releasing Hormone (GHRH) is a rare cause of acromegaly
GHRH-secreting neuroendocrine tumors (NETs) are the commonest cause of ectopic GHRH secretion causing acromegaly with bronchial carcinoids accounting for 54% of cases followed by pancreatic NETs in 30% of cases [2]
GHRH producing pancreatic neuroendocrine tumors are associated with Multiple Endocrine Neoplasia (MEN) 1 in 16% of cases, and more than 60% are malignant [5]
Summary
A 52-year-old woman presented initially in May 2007 with a 2.5 cm firm non-tender nodule over her right triceps area and significant weight loss over 1 year. Coarse facial features noticed this admission prompted a work-up for acromegaly This diagnosis was confirmed on the basis of elevated serum insulin-like growth factor 1 (IGF-I) 77 nmol/L (reference range, 10 - 29.4 nmol/L) and growth. The remaining pituitary function normal included a serum prolactin of 5.6 mcg/L (reference range, 3.3 - 26.7 mcg/L) She started on octreotide LAR monthly injections. Post-surgical CT imaging showed no residual pancreatic tumor, progression of liver involvement was reported. Her clinical complaints of hot flushes, diarrhea and spinal cord compression symptoms became significant at this point. The radiological imaging showed massive metastatic lesions in the liver, bone, lungs and new lesion in right breast which were confirmed pathologically to have the same nature of the other tumors foci. At this point we lost further contact with the patient
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