A case of watery diarrhoea syndrome due to an adrenal phaeochromocytoma secreting vasoactive intestinal polypeptide with coincidental autoimmune thyroid disease

Fisher, B. M.; Macphee, G. J. A.; Davies, David; McPherson, Stuart; Brown, Ivan; Goldberg, A.

doi:10.1530/acta.0.1140340

Cited by 13 publications

(8 citation statements)

References 12 publications

(22 reference statements)

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“…Muscle weakness was commonly reported in these published cases, possibly related to associated hypokalemia [16,22,23,27,29,30,33]. In our case, the patient suffered from muscular weakness and myalgia associated with severe rhabdomyolysis.…”

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confidence: 48%

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

et al. 2012

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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.

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confidence: 48%

“…Adrenal pheochromocytoma could be one of the extra-pancreatic VIPomas. In the past, more than 20 cases of VIP-producing adrenal pheochromocytoma have been reported so far [9, [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32]. Most of these cases had clinical symptoms of watery diarrhea.…”

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confidence: 99%

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

et al. 2012

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“…Eeckhout et colleagues described a pheochromocytoma in which VIP was only observed in scattered cells among pheochromocytes. Other researchers, on the other hand, have discovered VIP positive in the pheochromocytes themselves [4,[14][15][16][17][18][19][20][21][22][23].…”

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confidence: 99%

Etiology, Assessment and Management of WDHA (Watery Diarrhea, Hypokalemia, and Achlorhydria) and VIPoma

Hussein

Shaqaqiq

Aldhuaina

et al. 2021

JPRI

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The syndrome of watery diarrhoea, hypokalemia, and achlorhydria (WDHA syndrome) is an uncommon disorder marked by severe, watery diarrhoea caused by non–beta pancreatic islet cell oversecretion of vasoactive intestinal peptide (VIP). The onset of the disease is gradual, and diagnosis is often months or years later. Long-term dehydration, electrolyte and acid-base abnormalities, and chronic renal failure are all linked to morbidity. Pancreatic endocrine tumours are extremely rare, with less than 10 incidences per million people. VIPomas are uncommon tumours that affect between 0.05 and 2.0 percent of people. The most prevalent symptom is diarrhoea, which affects at least 89 percent of patients. VIPoma is treated with a combination of medicine and surgery The goal of first medical treatment is to reduce symptoms and restore fluids and electrolytes as quickly as possible

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“…Previously, sixteen cases of VIP-producing adrenal pheochromocytoma have been reported (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). Thirteen of those cases had clinical symptoms of watery diarrhea.…”

Section: Wdha Syndrome Is Caused By Vip-producing Tumors (Vipomas) Al...mentioning

confidence: 99%

Hypokalemic Rhabdomyolysis due to WDHA Syndrome Caused by VIP-producing Composite Pheochromocytoma: A Case in Neurofibromatosis Type 1

Onozawa¹,

Fukuhara²,

Minoguchi³

et al. 2005

Japanese Journal of Clinical Oncology

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A 47-year-old woman with neurofibromatosis type 1 suffered from general muscle weakness and watery diarrhea. Laboratory findings showed elevated muscular enzymes, severe hypokalemia and excessive production of catecholamines and vasoactive intestinal polypeptide (VIP). A computed tomography scan showed a 10 cm left adrenal mass, in which [(131)I]-metaiodobenzylguanidine scintigraphy showed high uptake. After she underwent surgical removal of the tumor, all the symptoms and signs subsided. A histological study revealed that the mass consisted of pheochromocytoma and ganglioneuroma, respectively producing catecholamines and VIP. In immunohistochemical staining of neurofibromin, pheochromocytoma and ganglion cells showed positive staining, whereas the staining was negative for nerve bundles and Schwann cells. We concluded that the patient had hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome, which was induced by a VIP-producing composite pheochromocytoma. Composite pheochromocytoma is a neuroendocrine tumor that is composed of pheochromocytoma and ganglioneuroma, both derived from the neural crest. Deficiency of neurofibromin in Schwann cells might have played an important role in the development and the growth of the composite pheochromocytoma in this patient.

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A case of watery diarrhoea syndrome due to an adrenal phaeochromocytoma secreting vasoactive intestinal polypeptide with coincidental autoimmune thyroid disease

Cited by 13 publications

References 12 publications

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

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

Etiology, Assessment and Management of WDHA (Watery Diarrhea, Hypokalemia, and Achlorhydria) and VIPoma

Hypokalemic Rhabdomyolysis due to WDHA Syndrome Caused by VIP-producing Composite Pheochromocytoma: A Case in Neurofibromatosis Type 1

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