Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
BMC Cancer
Published in: BMC Cancer 1/2014

Open Access 01-12-2014 | Case report

A rare case of watery diarrhea, hypokalemia and achlorhydria syndrome caused by pheochromocytoma

Authors: Jingjing Jiang, Li Zhang, Zhaodi Wu, Zhilong Ai, Yingyong Hou, Zhiqiang Lu, Xin Gao

Published in: BMC Cancer | Issue 1/2014

Login to get access

Abstract

Background

A rare syndrome of watery diarrhea, hypokalemia and achlorhydria (WDHA) is usually caused by pancreatic endocrine tumors that secrete excessive vasoactive intestinal polypeptide (VIP). Here we report a rare case of WDHA caused by a pheochromocytoma.

Case presentation

A 45-year old male presented with persistent and progressive watery diarrhea for half a year, and was treated with dialysis due to azotemia, hypokalemia, hypercalcemia and metabolic acidosis. A right adrenal mass was found by ultrasonography, and Positron Emission Tomography-Computed Tomography (PET-CT) showed the tumor was hyper-metabolic. Levels of plasma normetanephrine (NMN) and serum chromogranin A (CgA) were significantly elevated. Immunohistochemistry analysis of the adrenal tumor was strongly positive for CgA, synaptophysin and VIP. The patient fully recovered from WDHA syndrome soon after surgery, as reflected in that diarrhea stopped, levels of plasma NMN, serum CgA, and electrolytes returned to normal thus no dialysis was needed. The patient remained disease free in a 12-months follow-up period.

Conclusion

We report an extremely rare case of pheochromocytoma causing WDHA syndrome and uremia, which the patient completely recovered from after tumor resection.
Appendix
Available only for authorised users
Literature
1.
Verner JV, Morrison AB: Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. Am J Med. 1958, 25 (3): 374-380.CrossRefPubMed
2.
3.
Cooperman AM, 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): 325-328.CrossRefPubMedPubMedCentral
4.
Fisher BM, MacPhee GJ, Davies DL, McPherson SG, Brown IL, 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): 340-344.
5.
Matta MK, Prorok JJ, Trimpi HD, Sheets JA, Stasik JJ, Khubchandani IT: WDHA syndrome caused by pheochromocytoma: report of a case. Dis Colon Rectum. 1978, 21 (4): 297-301.CrossRefPubMed
6.
Ozbay A, Obukhau A, Buhl L, Brondt Hartlev L, Logstrup Poulsen P: Adrenal pheochromocytoma producing vasoactive intestinal peptide and masking hypertension. Horm Res. 2008, 70 (3): 188-192.CrossRefPubMed
7.
Pais SO: Angiographic demonstration of a vasoactive intestinal polypeptide-secreting pheochromocytoma in a patient with WDHA syndrome. AJR Am J Roentgenol. 1978, 130 (1): 172-174.CrossRefPubMed
8.
Quarles Van Ufford-Mannesse P, Castro Cabezas M, Vroom TM, Van Gils A, Lips CJ, 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): 231-234.CrossRefPubMed
9.
Sackel SG, Manson JE, Harawi SJ, Burakoff R: Watery diarrhea syndrome due to an adrenal pheochromocytoma secreting vasoactive intestinal polypeptide. Dig Dis Sci. 1985, 30 (12): 1201-1207.CrossRefPubMed
10.
Smith SL, Slappy AL, Fox TP, Scolapio JS: Pheochromocytoma producing vasoactive intestinal peptide. Mayo Clin Proc. 2002, 77 (1): 97-100.CrossRefPubMed
11.
Van Eeckhout P, Shungu H, Descamps FX, Lanthier P, Castelain T, Saey JP, Rettman R, Drese C, Colin IM: Acute watery diarrhea as the initial presenting feature of a pheochromocytoma in an 84-year-old female patient. Horm Res. 1999, 52 (2): 101-106.CrossRefPubMed
12.
Viale G, Dell’Orto 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): 1099-1106.CrossRefPubMed
13.
14.
Lundberg P, Lie A, Bjurholm A, Lehenkari PP, Horton MA, Lerner UH, Ransjo M: Vasoactive intestinal peptide regulates osteoclast activity via specific binding sites on both osteoclasts and osteoblasts. Bone. 2000, 27 (6): 803-810.CrossRefPubMed
15.
Lundberg P, Lerner UH: Expression and regulatory role of receptors for vasoactive intestinal peptide in bone cells. Microsc Res Tech. 2002, 58 (2): 98-103.CrossRefPubMed
16.
Eisenhofer G, Lenders JW, Linehan WM, Walther MM, Goldstein DS, Keiser HR: Plasma normetanephrine and metanephrine for detecting pheochromocytoma in von Hippel-Lindau disease and multiple endocrine neoplasia type 2. N Engl J Med. 1999, 340 (24): 1872-1879.CrossRefPubMed
17.
Eisenhofer G, Walther MM, Huynh TT, Li ST, Bornstein SR, Vortmeyer A, Mannelli M, Goldstein DS, Linehan WM, Lenders JW, Pacak K: Pheochromocytomas in von Hippel-Lindau syndrome and multiple endocrine neoplasia type 2 display distinct biochemical and clinical phenotypes. J Clin Endocrinol Metab. 2001, 86 (5): 1999-2008.CrossRefPubMed
18.
Onozawa M, Fukuhara T, Minoguchi M, Takahata M, Yamamoto Y, Miyake T, Kanagawa K, Kanda M, Maekawa I: 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): 559-563.CrossRefPubMed
19.
Conzo G, Circelli L, Pasquali D, Sinisi A, Sabatino L, Accardo G, Renzullo A, Santini L, Salvatore F, Colantuoni V: Lessons to be learned from the clinical management of a MEN 2A patient bearing a novel 634/640/700 mutation of the RET proto-oncogene. Clin Endocrinol. 2012, 77 (6): 934-936.CrossRef
Metadata
Title
A rare case of watery diarrhea, hypokalemia and achlorhydria syndrome caused by pheochromocytoma
Authors
Jingjing Jiang
Li Zhang
Zhaodi Wu
Zhilong Ai
Yingyong Hou
Zhiqiang Lu
Xin Gao
Publication date
01-12-2014
Publisher
BioMed Central
Published in
BMC Cancer / Issue 1/2014
Electronic ISSN: 1471-2407
DOI
https://doi.org/10.1186/1471-2407-14-553

Other articles of this Issue 1/2014

BMC Cancer 1/2014 Go to the issue

Research article

Randomized phase II study of pemetrexed/cisplatin with or without axitinib for non-squamous non-small-cell lung cancer

Research article

PARP inhibition and the radiosensitizing effects of the PARP inhibitor ABT-888 in in vitrohepatocellular carcinoma models

Research article

Use of NQO1 status as a selective biomarker for oesophageal squamous cell carcinomas with greater sensitivity to 17-AAG

Research article

ALDH1A1 expression correlates with clinicopathologic features and poor prognosis of breast cancer patients: a systematic review and meta-analysis

Research article

The development and validation of oral cancer staging using administrative health data

Case report

Leptomeningeal metastasis from hepatocellular carcinoma with other unusual metastases: a case report
Webinar | 19-02-2024 | 17:30 (CET)

Keynote webinar | Spotlight on antibody–drug conjugates in cancer

Watch now

Antibody–drug conjugates (ADCs) are novel agents that have shown promise across multiple tumor types. Explore the current landscape of ADCs in breast and lung cancer with our experts, and gain insights into the mechanism of action, key clinical trials data, existing challenges, and future directions.

Dr. Véronique Diéras
Prof. Fabrice Barlesi
Developed by: Springer Medicine
Image Credits