Watery Diarrhea, Hypokalemia and Achlorhydria Syndrome Due to an Adrenal Pheochromocytoma

Overview

Journal World J Gastroenterol

Publisher Baishideng Publishing Group

Specialty Gastroenterology

Date 2007 Aug 31

PMID 17729424

Citations 7

Authors

Shin-ichi Ikuta

Chiaki Yasui

Masahiro Kawanaka

Tsukasa Aihara

Hidenori Yoshie

Hidenori Yanagi

Masao Mitsunobu

Ayako Sugihara

Naoki Yamanaka

Affiliations

Soon will be listed here.

Abstract

Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma, which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.

Citing Articles

Review of the Pathophysiologic and Clinical Aspects of Hypokalemia in Children and Young Adults: an Update.

Bamgbola O Curr Treat Options Pediatr. 2023; 8(3):96-114.

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Vasoactive Intestinal Peptide-Secreting Pheochromocytoma: A Case Report and Review of Literature.

Yee S, Meyer J, Wong L AACE Clin Case Rep. 2022; 8(4):158-162.

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Vasoactive intestinal peptide producing pheochromocytoma and intracardiac thrombosis.

Hermel M, Jones D, Olson C, Sherman M, Srivastava A Rare Tumors. 2021; 13:20363613211007792.

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Case Report: Irreversible Watery Diarrhea, Severe Metabolic Acidosis, Hypokalemia and Achloridria Syndrome Related to Vasoactive Intestinal Peptide Secreting Malignant Pheochromocytoma.

Negro A, Verzicco I, Tedeschi S, Campanini N, Zanelli M, Negri E Front Endocrinol (Lausanne). 2021; 12:652045.

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Octreotide reverses shock due to vasoactive intestinal peptide-secreting adrenal pheochromocytoma: A case report and review of literature.

Hu X, Cao W, Zhao M World J Clin Cases. 2018; 6(14):862-868.

PMID: 30510956 PMC: 6264997. DOI: 10.12998/wjcc.v6.i14.862.

References

WEICHERT 3rd R . The neural ectodermal origin of the peptide-secreting endocrine glands. A unifying concept for the etiology of multiple endocrine adenomatosis and the inappropriate secretion of peptide hormones by nonendocrine tumors. Am J Med. 1970; 49(2):232-41. DOI: 10.1016/s0002-9343(70)80079-1. View

Matsuoka I, Satake R, Kurihara K . Cholinergic differentiation of clonal rat pheochromocytoma cells (PC12) induced by factors contained in glioma-conditioned medium: enhancement of high-affinity choline uptake system and reduction of norepinephrine uptake system. Brain Res. 1986; 389(1-2):145-52. DOI: 10.1016/0165-3806(86)90182-3. View

Fisher B, Macphee G, Davies D, McPherson S, Brown I, GOLDBERG A . A case of watery diarrhoea syndrome due to an adrenal phaeochromocytoma secreting vasoactive intestinal polypeptide with coincidental autoimmune thyroid disease. Acta Endocrinol (Copenh). 1987; 114(3):340-4. DOI: 10.1530/acta.0.1140340. View

Viale G, DellOrto P, Moro E, Cozzaglio L, Coggi G . Vasoactive intestinal polypeptide-, somatostatin-, and calcitonin-producing adrenal pheochromocytoma associated with the watery diarrhea (WDHH) syndrome. First case report with immunohistochemical findings. Cancer. 1985; 55(5):1099-106. DOI: 10.1002/1097-0142(19850301)55:5<1099::aid-cncr2820550527>3.0.co;2-3. View

Cooperman A, DeSantis D, WINKELMAN E, Farmer R, Eversman J, Said S . Watery diarrhea syndrome. Two unusual cases and further evidence that VIP is a humoral mediator. Ann Surg. 1978; 187(3):325-8. PMC: 1396422. DOI: 10.1097/00000658-197803000-00020. View

Mendelsohn G, Eggleston J, Olson J, Said S, Baylin S . Vasoactive intestinal peptide and its relationship to ganglion cell differentiation in neuroblastic tumors. Lab Invest. 1979; 41(2):144-9. View

van Ufford-Mannesse P, Cabezas M, Vroom T, van Gils A, Lips C, Niermeijer P . A patient with neurofibromatosis type 1 and watery diarrhoea syndrome due to a VIP-producing adrenal phaeochromocytoma. J Intern Med. 1999; 246(2):231-4. DOI: 10.1046/j.1365-2796.1999.00533.x. View

Loehry C, Kingham J, Whorwell P . Watery diarrhoea and hypokalaemia associated with a phaeochromocytoma. Postgrad Med J. 1975; 51(596):416-9. PMC: 2496073. DOI: 10.1136/pgmj.51.596.416. View

Nigawara K, Suzuki T, Tazawa H, Funyu T, Yagihashi S, Yamaya K . A case of recurrent malignant pheochromocytoma complicated by watery diarrhea, hypokalemia, achlorhydria syndrome. J Clin Endocrinol Metab. 1987; 65(5):1053-6. DOI: 10.1210/jcem-65-5-1053. View

10.

Onozawa M, Fukuhara T, Minoguchi M, Takahata M, Yamamoto Y, Miyake T . Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma: a case in neurofibromatosis type 1. Jpn J Clin Oncol. 2005; 35(9):559-63. DOI: 10.1093/jjco/hyi139. View

11.

Trump D, Livingston J, Baylin S . Watery diarrhea syndrome in an adult with ganglioneuroma-pheochromocytoma: identification of vasoactive intestinal peptide, calcitonin, and catecholamines and assessment of their biologic activity. Cancer. 1977; 40(4):1526-32. DOI: 10.1002/1097-0142(197710)40:4<1526::aid-cncr2820400425>3.0.co;2-7. View

12.

Bloom S, Polak J, Pearse A . Vasoactive intestinal peptide and watery-diarrhoea syndrome. Lancet. 1973; 2(7819):14-6. DOI: 10.1016/s0140-6736(73)91947-8. View

13.

Said S . Evidence for secretion of vasoactive intestinal peptide by tumours of pancreas, adrenal medulla, thyroid and lung: support for the unifying APUD concept. Clin Endocrinol (Oxf). 1976; 5 Suppl:201S-204S. DOI: 10.1111/j.1365-2265.1976.tb03828.x. View

14.

Matta M, Prorok J, TRIMPI H, SHEETS J, Stasik Jr J, Khubchandani I . WDHA syndrome caused by pheochromocytoma: report of a case. Dis Colon Rectum. 1978; 21(4):297-301. DOI: 10.1007/BF02586708. View

15.

Pais S . Angiographic demonstration of a vasoactive intestinal polypeptide-secreting pheochromocytoma in a patient with WDHA syndrome. AJR Am J Roentgenol. 1978; 130(1):172-4. DOI: 10.2214/ajr.130.1.172. View

16.

Van Eeckhout P, Shungu H, Descamps F, Lanthier P, Castelain T, Saey J . Acute watery diarrhea as the initial presenting feature of a pheochromocytoma in an 84-year-old female patient. Horm Res. 2000; 52(2):101-6. DOI: 10.1159/000023443. View

17.

Schwartz C, KIMBERG D, Sheerin H, Field M, Said S . Vasoactive intestinal peptide stimulation of adenylate cyclase and active electrolyte secretion in intestinal mucosa. J Clin Invest. 1974; 54(3):536-44. PMC: 301586. DOI: 10.1172/JCI107790. View

18.

Sano T, Saito H, Inaba H, Hizawa K, Saito S, Yamanoi A . Immunoreactive somatostatin and vasoactive intestinal polypeptide in adrenal pheochromocytoma. An immunochemical and ultrastructural study. Cancer. 1983; 52(2):282-9. DOI: 10.1002/1097-0142(19830715)52:2<282::aid-cncr2820520215>3.0.co;2-j. View

19.

Schmid K, Dockhorn-Dworniczak B, Fahrenkamp A, Kirchmair R, Totsch M, Fischer-Colbrie R . Chromogranin A, secretogranin II and vasoactive intestinal peptide in phaeochromocytomas and ganglioneuromas. Histopathology. 1993; 22(6):527-33. DOI: 10.1111/j.1365-2559.1993.tb00172.x. View

20.

VERNER J, Morrison A . Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med. 1958; 25(3):374-80. DOI: 10.1016/0002-9343(58)90075-5. View