A 45-Year-Old Female with Hypokalemic Rhabdomyolysis due to VIP-Producing Composite Pheochromocytoma

Abstract

The watery diarrhea, hypokalemia and achlorhydria (WHDA) syndrome due to vasoactive intestinal polypeptide (VIP)-producing extra-pancreatic tumors is rare. We report on a 45-year-old woman who suffered from persistent secretory diarrhea for six years and who was admitted to hospital with complaints of muscular weakness and myalgia. Biochemical testing revealed pronounced rhabdomyolysis due to severe hypokalemia. Gastrointestinal evaluation of long-standing diarrhea including endoscopy of the upper and lower gastrointestinal tract and the small intestine did not show any pathologies. An abdominal computed tomography scan revealed a mass of 4 × 5 cm in the left adrenal gland demonstrating a strong uptake in the ¹²³I-labelled metaiodobenzylguanidine scintigraphy. Plasma levels of chromogranin A, calcitonin, parathormone, basal renin and most prominently VIP were increased in line with a increased 24 hour urinary secretion of noradrenaline, dopamine, normetanephrine and vanillymandelic acid. A WDHA (watery diarrhea, hypokalaemia, achlorhydria) syndrome with hypokalemic rhabdomyolysis due to a VIP-producing adrenal tumor was diagnosed that was removed surgically. The histological evaluation demonstrated a composite pheochromocytoma. Diarrhea stopped immediately after surgery together with a normalization of laboratory parameters. In conclusion, this case report focuses on the rare clinical presentation of secretory diarrhea and electrolyte disturbances in combination with hypokalemic rhabdomyolysis which was caused by a VIP-producing composite pheochromocytoma.

Zusammenfassung

Wir berichten über eine 45-jährige Patientin mit seit 6 Jahren persistierender Diarrhö, die wegen Muskelschwäche und -schmerzen stationär aufgenommen wurde. Die Laboruntersuchungen ergaben eine ausgeprägte Rhabdomyolyse bei schwerer Hypokaliämie. Die gastroenterologische Abklärung der Diarrhö einschließlich endoskopischer Untersuchungen des unteren, mittleren und oberen Gastrointestinaltrakts ergaben keine Auffälligkeiten. Eine Computertomografie des Abdomens zeigte eine 4 × 5 cm große Raumforderung im Bereich der linken Nebenniere, die in der Jod-123-MIBG-Szintigrafie eine umschriebene Mehranreicherung aufwies. Eine eingeleitete Hormondiagnostik ergab erhöhte Serumkonzentrationen für Chromogranin A, Kalzitonin, Parathormon, Renin und besonders ausgeprägt für das vasoaktive intestinale Polypeptid (VIP). Im 24-Stunden-Sammel-Urin fanden sich erhöhte Werte für Noradrenalin, Dopamin, Normetanephrin und Vanillinmandelsäure. Aufgrund der Laborkonstellation und der bildgebenden Diagnostik wurde die Verdachtsdiagnose eines WDHA-Syndroms (wässrige Diarrhö, Hypokaliämie, Achlorhydrie) mit hypokaliämischer Rhabdomyolyse aufgrund eines VIP-produzierenden Nebennierentumors gestellt. Der Nebennierentumor wurde laparoskopisch entfernt. Die histologische Aufarbeitung ergab einen Kombinationstumor aus einem Phäochromozytom mit einem Ganglioneurom der linken Nebenniere. Unmittelbar nach der operativen Entfernung des Tumors kam es zu einem Sistieren der Durchfälle sowie zu einer Normalisierung der auffälligen Laborparameter.

Key words

gastro-entero-pancreatic tumors - diarrhea - vasoactive intestinal polypeptide

Schlüsselwörter

gastro-entero-pankreatische Tumoren - Diarrhö - vasoaktives Intestinal-Polypeptid

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