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

Onozawa, Masahiro; Fukuhara, Takashi; Minoguchi, Madoka; Takahata, Mutsumi; Yamämoto, Yasushi; Miyake, Takayoshi; Kanagawa, Koichi; Kanda, Makoto; Maekawa, I

doi:10.1093/jjco/hyi139

Cited by 30 publications

(19 citation statements)

References 23 publications

(34 reference statements)

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“…Based on these investigations, along with the patient’s clinical manifestations, we could exclude the possibility of MEN2. So far, the only genetic manifestation of pheochromocytoma associated with WDHA syndrome was found being a NF1 gene mutation, with two cases reported in the literature [8, 18]. A pheochromocytoma secreting calcitonin has been previously reported in a MEN2A patient bearing a triple RET gene mutation [19].…”

Section: Discussionmentioning

confidence: 99%

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

Jiang

Zhang

et al. 2014

BMC Cancer

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BackgroundA 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 presentationA 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.ConclusionWe report an extremely rare case of pheochromocytoma causing WDHA syndrome and uremia, which the patient completely recovered from after tumor resection.

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Section: Discussionmentioning

confidence: 99%

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

Jiang

Zhang

et al. 2014

BMC Cancer

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“…However, a history of persistent diarrhea was present and presumably caused by secretion of VIP within the pheochromocytoma. VIPomas mostly arise in the pancreas, but adrenal pheochromocytomas could very well be one of the extrapancreatic sites, and previous cases of such VIPomas have been documented [5, 9, 10]. VIP is a potent stimulant of adenylate cyclase, which causes hypersecretion of water and electrolytes by the intestinal mucosa leading to profuse watery diarrhea, hypokalemia and achlorhydria or WDHA [5, 11, 12].…”

Section: Discussionmentioning

confidence: 99%

Adrenal Pheochromocytoma Producing Vasoactive Intestinal Peptide and Masking Hypertension

Øzbay¹,

Obukhau

Buhl

et al. 2008

Horm Res Paediatr

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Aim: To report a case of a pheochromocytoma secreting vasoactive intestinal peptide (VIP). Methods: We studied a 77-year-old woman who had suffered from persistent diarrhea and episodes of sweating and palpitations. Results: She had neither previous or current anamnesis of hypertension nor any known familial dispositions to endocrine diseases. Initially gastrointestinal investigations were carried out based on longstanding diarrhea with hypopotassemia, but radiological imaging revealed a unilateral adrenal mass. Biochemical testing showed increased levels of catecholamine and VIP, and ¹²³I-metaiodobenzylguanidine scintigraphy confirmed the adrenal origin as well as the diagnosis of a VIP-producing pheochromocytoma. The patient underwent surgical removal of the tumor which led to relief of symptoms and normalized laboratory values. Conclusion: This case report focuses on the protean mode of presentation seen in pheochromocytomas as well as their capacity to produce several neuropeptides, ultimately intensifying the need for early examination for this condition despite unrelated symptoms.

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“…Also, hypertension, either sustained or paroxysmal, is the principal feature of PHEO (3,12). Composite PHEO-GNs usually secrete excessive catecholamines and their metabolites, and they rarely secrete vasoactive intestinal polypeptide (VIP) (13,16,17,(26)(27)(28). Rarely, watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome due to excessive VIP secretion can be observed in patients with composite PHEO-GN.…”

Section: Ementioning

confidence: 99%

“…Rarely, watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome due to excessive VIP secretion can be observed in patients with composite PHEO-GN. These cases are frequently normotensive because of vasodilatory action of VIP and lack typical symptoms such as headaches (14,16,28,29). Composite adrenal PHEO-GN may be incidentally discovered, manifested as catecholamine-induced acute relapsing pancreatitis without hypertension (19).…”

Section: Ementioning

confidence: 99%

Composite Adrenal Pheochromocytoma-Ganglioneuroma in an Adult Patient

Erem¹

2014

Acta Endo (Buc)

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Background. Composite adrenal pheochromocytoma-ganglioneuromas (PHEO-GNs) are well-defined neoplasms of the adrenal medulla, consisting of both endocrine and neural components. They are extremely rare. To date, only 46 cases have been reported in the English literature.Case report. We describe an adult case of endocrinologically active adrenal composite PHEO-GN diagnosed in a 62-year-old male patient with history of dizziness, headache, nausea, vomiting, and uncontrolled hypertension including intermittant hypertension attacks. On physical examination, he had a blood pressure (BP) of 170/110 mmHg. 18-fluorodeoxyglucose positron emission tomography-computed tomography showed a right adrenal tumor with increased metabolic activity. Urinary levels of catecholamines and their metabolites were prominently elevated. Right adrenalectomy was performed for treatment purposes. The histological diagnosis of the resected tumor was composite adrenal PHEO-GN.Conclusions. Composite adrenal PHEO-GN is a rare entity and preoperative diagnosis is difficult. Its hormonal activity and imaging characteristics are frequently very similar to those of other adrenal tumors, especially pure PHEO and adrenal carcinoma. Therefore, careful evaluation by endocrine tests and multiple imaging procedures are needed for providing a differential diagnosis. However, definitive diagnosis composite adrenal PHEO-GN is established by histological and immunochemical studies. To our knowledge, the present case is the first report that describes composite adrenal PHEO-GN in a patient from Turkey. We discuss this case and review the literature on this unusual entity.

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

Cited by 30 publications

References 23 publications

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

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

Adrenal Pheochromocytoma Producing Vasoactive Intestinal Peptide and Masking Hypertension

Composite Adrenal Pheochromocytoma-Ganglioneuroma in an Adult Patient

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