Vasoactive intestinal polypeptide-, somatostatin-, and calcitonin-producing adrenal pheochromocytoma associated with the watery diarrhea (WDHH) syndrome. First case report with immunohistochemical findings.
A 30-year-old man presenting with watery diarrhea, hypokalemia, and hypochlorhydria (Verner-Morrison syndrome, WDHH syndrome) had raised plasma levels of vasoactive intestinal polypeptide (VIP), somatostatin (SRIF), calcitonin, and gastrin, as well as high urinary excretion of vanillylmandelic acid. A right adrenal pheochromocytoma was found and excised. The neoplastic cell population was immunohistochemically shown to contain VIP, SRIF, and calcitonin. Gross, histologic, and immunohistochemical evaluation of the pancreas revealed no abnormalities, whereas a marked hyperplasia of the gastrin-producing cells of the gastric antral mucosa was demonstrated. Postoperatively, the patient recovered from his symptoms and the plasma hormone levels returned to normal values. The clinical and histogenetic implications of this most unusual tumor of neural crest derivatives are discussed.
Viale
G
G
Dell'Orto
P
P
Moro
E
E
Cozzaglio
L
L
Coggi
G
G
D007516:Adenoma, Islet Cell / Q000378:metabolism*; D000310:Adrenal Gland Neoplasms / Q000378:metabolism* / Q000473:pathology; D000328:Adult; D002116:Calcitonin / Q000096:biosynthesis*; D005755:Gastrins / Q000096:biosynthesis; D006651:Histocytochemistry; D006729:Hormones, Ectopic / Q000096:biosynthesis*; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D010673:Pheochromocytoma / Q000378:metabolism* / Q000473:pathology; D013004:Somatostatin / Q000096:biosynthesis*; D014660:Vasoactive Intestinal Peptide / Q000096:biosynthesis*; D003969:Vipoma / Q000378:metabolism*
D005755:Gastrins; D006729:Hormones, Ectopic; D014660:Vasoactive Intestinal Peptide; D013004:Somatostatin; D002116:Calcitonin