Related Experiment Video
Updated: Oct 3, 2026

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
Published on: May 17, 2024
Composite pheochromocytoma-ganglioneuroma secreting vasoactive intestinal peptide
Benjamin Brochu1, Matthieu St-Jean1, Yves Collin2
1Division of Endocrinology, Department of Medicine, Centre Hospitalier Universitaire de Sherbrooke , Sherbrooke, Canada.
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.
Related Concept Videos
Sympathetic Pathways: Collateral Ganglia and Adrenal Medulla
Collateral Ganglia
Sympathetic preganglionic axons reach the collateral ganglia along the route of splanchnic nerves. These nerves bypass the sympathetic trunk and communicate with sympathetic postganglionic neurons housed in the prevertebral ganglia. These ganglia supply the organs of the abdominopelvic cavity.
The greater splanchnic nerve, formed by the...
Chemotherapy-Induced Nausea and Vomiting: Neurokinin-1 Receptor Antagonists
Esophageal Achalasia
Drugs Acting on Autonomic Ganglia: Stimulants
Ganglionic stimulants activate NM nicotinic receptors in autonomic ganglia, falling into two categories: nicotine mimetics [e.g., lobeline, dimethylpiperazine, tetramethylammonium] and muscarinic receptor agonists [e.g., muscarine, methacholine]. The first category's action is rapid and blocked by nicotinic receptor antagonists, while the second category's action is delayed and blocked by atropine-like agents. Nicotine, an alkaloid, affects the heart rate by stimulating sympathetic or...
Sympathetic Pathways: Sympathetic Chain Ganglia
Peripheral Nervous System: Ganglia and Nerves
Nerves
The nerve is a bundle of axons that serves as the communication highway in the PNS. Each nerve is ensheathed in a protective layer of connective tissue called the epineurium. This outermost layer safeguards the nerve and supports the...