Composite pheochromocytoma-ganglioneuroma secreting vasoactive intestinal peptide.
This case highlights a rare occurrence of VIP secretion in a composite pheochromocytoma-ganglioneuroma, with normalization of VIP levels following surgical intervention.
Where it sits
this study against the rest of the vip (vasoactive intestinal polypeptide) corpusSummary and findings
An 80-year-old female with a 10-year history of diarrhea and chronic hyponatremia was found to have a 6.8-centimeter right adrenal mass. Elevated levels of vasoactive intestinal peptide (VIP) were noted, and surgical resection confirmed a composite pheochromocytoma-ganglioneuroma. Postoperatively, plasma levels of VIP and normetanephrines normalized, along with resolution of diarrhea and other symptoms.
Abstract
Summary An 80-year-old female patient presented with a 10-year history of progressively worsening diarrhea and chronic hyponatremia. An abdominal computed tomography (CT) revealed a 6.8-centimeter right adrenal mass. A Ga-68 DOTATATE positron emission tomography (PET)/CT scan subsequently demonstrated a significant expression of somatostatin receptors on the tumoral shell (maximal SUV of 14.4), associated with a hypometabolic central portion. Endocrine investigation indicated elevated levels of both urinary and plasmatic normetanephrines. Given the watery diarrhea, VIP levels were measured, which were markedly elevated. Surgical resection confirmed a composite pheochromocytoma-ganglioneuroma with VIP immunoreactivity in both tumor components. In the postoperative period, the plasma levels of VIP and normetanephrines returned to normal with resolution of diarrhea, hyponatremia, and hypertension. This case illustrates a rare instance of composite pheochromocytoma-ganglioneuroma with co-secretion of catecholamines and VIP, with evidence of VIP production in both tumor components. The finding of VIP secretion in the pheochromocytoma and the ganglioneuroma suggests that both tumors might originate from the same cell of neural crest origin. Learning points
Background
Not reported in abstract.
Methods
Not reported in abstract.
Results
Not reported in abstract.
Interpretation
Not reported in abstract.
Limitations
Not reported in abstract.