Case Report: Irreversible Watery Diarrhea, Severe Metabolic Acidosis, Hypokalemia and Achloridria Syndrome Related to Vasoactive Intestinal Peptide Secreting Malignant Pheochromocytoma.
Acidosis
/ complications
Adrenal Gland Neoplasms
/ complications
Adrenalectomy
Aged
Chromaffin Cells
/ cytology
Diarrhea
/ complications
Humans
Hypokalemia
/ complications
Male
Peptides, Cyclic
/ therapeutic use
Peripheral Nervous System Neoplasms
Pheochromocytoma
/ physiopathology
Radionuclide Imaging
Receptors, Somatostatin
/ therapeutic use
Somatostatin
/ analogs & derivatives
Syndrome
Tomography, X-Ray Computed
Vasoactive Intestinal Peptide
/ chemistry
Weight Loss
arterial hypotension
metabolic acidosis
pheochromocytoma
vasointestinal peptide (VIP)
watery diarrhea hypokalemia achlorhydria syndrome
Journal
Frontiers in endocrinology
ISSN: 1664-2392
Titre abrégé: Front Endocrinol (Lausanne)
Pays: Switzerland
ID NLM: 101555782
Informations de publication
Date de publication:
2021
2021
Historique:
received:
11
01
2021
accepted:
01
03
2021
entrez:
5
4
2021
pubmed:
6
4
2021
medline:
8
1
2022
Statut:
epublish
Résumé
Pheochromocytoma (PHEO) clinical manifestations generally mirror excessive catecholamines secretion; rarely the clinical picture may reflect secretion of other hormones. Watery diarrhea, hypokalemia and achlorhydria (WDHA) is a rare syndrome related to excessive secretion of vasoactive intestinal peptide (VIP). A 73-year-old hypotensive man affected by adrenal PHEO presented with weight loss and watery diarrhea associated with hypokalemia, hyperchloremic metabolic acidosis (anion gap 15 mmol/l) and a negative urinary anion gap. Abdominal computed tomography scan showed a right adrenal PHEO, 8.1 cm in maximum diameter, with tracer uptake on A rare case of WDHA syndrome caused by malignant VIP-secreting PHEO was diagnosed. High levels of circulating VIP were responsible of the rapidly evolving clinical picture with massive dehydration and weight loss along with severe hyperchloremic metabolic acidosis and hypokalemia due to the profuse untreatable diarrhea. The rescue treatment with lanreotide was unsuccessful because of the paucity of somatostatin-receptor-2A on VIP-secreting PHEO chromaffin cells.
Sections du résumé
Background
Pheochromocytoma (PHEO) clinical manifestations generally mirror excessive catecholamines secretion; rarely the clinical picture may reflect secretion of other hormones. Watery diarrhea, hypokalemia and achlorhydria (WDHA) is a rare syndrome related to excessive secretion of vasoactive intestinal peptide (VIP).
Clinical Case
A 73-year-old hypotensive man affected by adrenal PHEO presented with weight loss and watery diarrhea associated with hypokalemia, hyperchloremic metabolic acidosis (anion gap 15 mmol/l) and a negative urinary anion gap. Abdominal computed tomography scan showed a right adrenal PHEO, 8.1 cm in maximum diameter, with tracer uptake on
Conclusions
A rare case of WDHA syndrome caused by malignant VIP-secreting PHEO was diagnosed. High levels of circulating VIP were responsible of the rapidly evolving clinical picture with massive dehydration and weight loss along with severe hyperchloremic metabolic acidosis and hypokalemia due to the profuse untreatable diarrhea. The rescue treatment with lanreotide was unsuccessful because of the paucity of somatostatin-receptor-2A on VIP-secreting PHEO chromaffin cells.
Identifiants
pubmed: 33815297
doi: 10.3389/fendo.2021.652045
pmc: PMC8010837
doi:
Substances chimiques
Peptides, Cyclic
0
Receptors, Somatostatin
0
lanreotide
0G3DE8943Y
Vasoactive Intestinal Peptide
37221-79-7
Somatostatin
51110-01-1
somatostatin receptor 2
D73QL0OMU2
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
652045Informations de copyright
Copyright © 2021 Negro, Verzicco, Tedeschi, Campanini, Zanelli, Negri, Farnetti, Nicoli, Palladini, Santi, Cunzi, Calvi, Coghi, Gerra, Volpi, Graiani and Cabassi.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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