Vipoma asociado a dermatomiosistis, tiroiditis de Hashimoto y pancreatitis autoinmune (síndrome poliglandular tipo 3C)

Mujer de 72 años con diarrea crónica secretora, disfagia orofaringea, debilidad muscular proximal, compromiso dérmico y bocio multinodular; con imágenes y estudios auxiliares compatible con un VIPoma benigno asociado a una dermatomiositis y un síndrome poliglandular tipo IIIC (tiroiditis de Hashimoto y pancreatitis autoinmune). Fue tratado con corticoides, inmunosupresores y análogos de somatostatina con buena evolución clínica. Sería el primer caso reportado en nuestro país.

VIPoma: a rare cause of diarrhea. A case report / VIPoma: una causa poco frecuente de diarrea. Case report

Abstract Introduction: Vasoactive intestinal peptide-secreting tumor (VIPoma) is a rare functional pancreatic neuroendocrine tumor (F-PNET) characterized by secretory diarrhea, hypokalemia, and hypochlorhydria. Its low incidence and high risk of malignancy pose a clinical challenge that requires a high degree of clinical suspicion. Case presentation: A 61-year-old woman visited the emergency department of a tertiary care hospital in Medellín, Colombia, due to chronic diarrhea (7 months) that led to dehydration, renal failure, metabolic acidosis, and hypokalemia. As a result, a treatment based on loperamide, intravenous fluids and broad-spectrum antibiotics was started. In addition, chromogranin A levels of 477 ug/L (<100) were reported, while an abdominal MRI showed a 33x30mm mass in the head and uncinate process of the pancreas, so outpatient surgical management was decided. However, three days after discharge, and due to the persistence of clinical signs, the patient was admitted to another hospital (also a tertiary care hospital), where, given the high suspicion of VIPoma, and once the diarrhea was solved, the mass was removed (Whipple procedure) without any complication. Finally, the diagnosis was confirmed based on serum vasoactive intestinal peptide levels (930 pg/mL (RV<75)) and the pathology report (PNET tumor grade 2). Two years after the surgery, the patient was asymptomatic, and no residual lesions or metastases were evident in a control MRI. Conclusion: Late diagnosis of VIPoma is associated with worsened quality of life, severe complications, and high prevalence of metastasis. Therefore, it should be suspected in patients with chronic secretory diarrhea that is not caused by an infection, since early diagnosis and timely treatment can contribute to achieving better survival rates in these patients.

Resumen Introducción. El tumor secretor de péptido intestinal vasoactivo o VIPoma es un tumor funcional neuroendocrino pancreático (F-PNET) raro caracterizado por diarrea secretora, hipokalemia e hipoclorhidria. Su baja incidencia y alto riesgo de malignidad representan un reto clínico que requiere un alto grado de sospecha clínica. Presentación del caso. Mujer de 61 años quien consultó al servicio de urgencias de un hospital de tercer nivel en Medellín, Colombia, por diarrea crónica (7 meses) que llevó a des-hidratación, falla renal, acidosis metabólica e hipokalemia, por lo que se inició manejo con loperamida, líquidos endovenosos y antibióticos de amplio espectro. Además, se reportaron niveles de cromogranina A de 477 ug/L (<100) y, mediante resonancia magnética (RM) abdominal, se identificó masa de 33x30mm en cabeza y proceso uncinado de páncreas, por lo que se decidió manejo quirúrgico ambulatorio. Sin embargo, tres días después del alta, la paciente ingresó, por persistencia de los signos, a un segundo hospital (también de tercer nivel), donde ante la alta sospecha de VIPoma, y una vez superada la diarrea, se extirpó la masa (procedimiento de Whipple). Finalmente, con base en los niveles séricos de péptido intestinal vasoactivo (930 pg/ml (VR<75)) y el informe de patología (tumor PNET grado 2), se confirmó el diagnóstico. Dos años después del procedimiento, la paciente se encontraba asintomática y sin evidencia de lesiones residuales ni metástasis en RM de control. Conclusión. El diagnóstico tardío de VIPoma se asocia con detrimento de la calidad de vida, complicaciones graves y alta prevalencia de metástasis, por lo que debe sospecharse en pacientes con diarrea crónica secretora no causada por infecciones, pues de diagnosticarse a tiempo e iniciarse el tratamiento oportuno se pueden lograr mejores tasas de supervivencia en estos pacientes.

Diarrea crónica como manifestación de un tumor neuroendocrino / Chronic diarrhea as a manifestation of a neuroendocrine tumor

Resumen Las neoplasias neuroendocrinas (Neuroendocrine Neoplasms, NEN) constituyen un grupo heterogéneo de neoplasias poco frecuentes, que se originan en las células endocrinas, con la capacidad de secretar aminas y polipéptidos hormonales. Las NEN de localización pancreática (pNEN) pueden ser funcionales o no funcionales. Las pNEN funcionales secretan hormonas como la gastrina, la insulina y el glucagón y otras menos frecuentes como el péptido intestinal vasoactivo (PIV), por lo que sus características sindromáticas dependen del péptido secretado. Los vipomas se manifiestan con diarrea crónica de características secretoras, que usualmente conducen a trastornos hidroelectrolíticos e incluso a complicaciones serias asociadas como la falla renal. A continuación, se describe el caso de un hombre de 37 años con diarrea crónica de 6 meses de evolución y frecuentes hospitalizaciones por trastornos hidroelectrolíticos, generados por hipocalemia severa y lesión renal aguda por deshidratación. Después de múltiples estudios, se considera el diagnóstico de una diarrea secretora por NEN funcional, secretora de PIV. Por tanto, se inicia una terapia empírica con octreotida y se logra controlar la diarrea, así como corregir el trastorno hidroelectrolítico. Además, se amplían los estudios, para documentar las pNEN tratadas mediante intervención quirúrgica, con respuesta clínica favorable y remisión completa de la sintomatología.

Abstract Neuroendocrine tumors (NETs) are a heterogeneous group of rare neoplasms that originate in endocrine cells with the ability to secrete amines and hormonal polypeptides. Pancreatic neuroendocrine tumors (PNETs) can be functional or non-functional. Functional PNETs secrete common hormones such as gastrin, insulin and glucagon and much less frequent hormones such as vasoactive intestinal peptide (VIP). Their characteristics depend on the peptide secreted. Vipomas are characterized by chronic diarrhea of ​​secretory characteristics that usually lead to hydroelectrolytic disorders and can lead to serious complications associated with renal failure. This article describes the case of a 37-year-old man who had suffered chronic diarrhea with frequent hospitalization for hydroelectrolytic disorders for six months due to severe hypokalemia and acute renal damage due to dehydration. After multiple studies, a diagnosis of secretory diarrhea due to a VIP secretory functional NET was considered. Empirical therapy with Octreotide was begun to control diarrhea and correct the hydroelectrolytic disorder. More studies of PNETS are being published. They have been treated surgically intervention with favorable clinical results and complete remission of symptoms.

VIPoma pancreático tratado com pancreatectomia distal e preservação esplênica com ligadura da artéria esplênica / Pancreatic VIPoma treated with distal pancreatectomy and splenic preservation with ligature of splenic artery

O VIPoma é um tumor neuroendócrino de baixa malignidade derivado das células das ilhotas não-beta do pâncreas, apresenta incidência de 1:10.000.000 pessoas na população e sua localização mais comum é no corpo e cauda do pâncreas. Seu tratamento curativo é cirúrgico, sendo geralmente realizada a pancreatectomia distal com esplenectomia. O caso relatado foi o de uma paciente do sexo feminino, de 31 anos, diagnosticada com VIPoma pancreático. O tratamento realizado foi a pancreatectomia distal com preservação esplênica e ligadura da artéria esplênica. A preservação esplênica diminui a morbidade perioperatória, sem prejudicar o resultado oncológico nos tumores de baixo grau de malignidade.

VIPoma is a low grade malignancy neuroendocrine tumor derived from non-beta pancreatic islet cells, it has an incidence of 1:10.000.000 individuals in the general population and its commoner location is in the body and tail of the pancreas. The curative treatment is surgery, and distal pancreatectomy with splenectomy is usually the employed technique. The case reported was of a female patient, 31 years-old, diagnosed with pancreatic VIPoma. The chosen treatment was distal pancreatectomy with splenic preservation and ligature of the splenic artery. Splenic preservation reduces the perioperatory morbidity, with no prejudice to the oncologic effect in the low grade malignancy tumors.

VIPoma en retroperitoneo de adulto mayor, causa de diarrea crónica en localización infrecuente / VIPoma in retroperitoneum of elderly, cause of chronic diarrhea in uncommon location

La diarrea crónica secretora es una entidad frecuente. Las causas son múltiples, por lo que llegar al diagnóstico definitivo puede resultar demorado con las complicaciones que esto ocasiona en el estado general del paciente. Se presenta el caso de un adulto mayor que cursa con diarrea crónica asociada a hipokalemia leve y acidosis metabolica hiperclorémica secundaria a un VIPoma a nivel de retroperitoneo, la cual es una localización inusual de este tipo de tumores, que en este caso fue diagnosticado mediante una biopsia guiada por tomografía...

Chronic secretory diarrhea is a frequent entity. Causes are multiple, that is why reaching final diagnosis can result in delay with complications that this causes in the general condition of the patient. We present the case of one older adult with chronic diarrhea, mild hypokalemia and metabolic hyperchloremic acidosis secondary to a VIPoma in retroperitoneum which is an unusual location of this type of tumor that was diagnosed by biopsy guided by tomography...

Significant Symptom Relief with Hepatic Artery Embolization in a VIPoma with Liver Metastases / 대한내과학회지

Vasoactive intestinal polypeptide-secreting tumors (VIPomas) cause VIPoma syndrome, which is characterized by watery diarrhea, hypokalemia, and achlorhydria. The treatment options for metastatic VIPomas include somatostatin analogs, cytoreductive surgery, and chemotherapy. We report the case of a 54-year-old male who presented with a peripancreatic mass with multiple hepatic metastases on computed tomography. After resection, the peripancreatic mass was demonstrated pathologically to be a neuroendocrine tumor. Although the patient received systemic chemotherapy and somatostatin analogs for the hepatic metastatic masses, the tumor increased in size. The patient then experienced severe diarrhea, despite treatment with the somatostatin analogs. Elevated serum VIP levels (3,260 pg/mL) and typical symptoms confirmed the diagnosis of VIPoma. We performed hepatic artery embolization (HAE) to reduce the tumor volume and control his symptoms, which led to a very rapid symptomatic response. The patient has remained symptom-free for 18 months with repeated HAE.

VIPoma pancreático: Caso clínico / Pancreatic VIPoma: Report of one case

Neuroendocrine tumors are uncommon, including VIPoma that produces vasoactive intestinal polypeptide. We report a 45-year-old female presenting with a history of diarrhea lasting three months. An abdominal CAT scan showed a solid tumor in the body of the pancreas. A fine needle aspiration biopsy of the tumor was compatible with a neuroendocrine tumor. The patient was subjected to a partial pancreatectomy, excising a 4 cm diameter tumor. The pathological study was compatible with a neuroendocrine carcinoma. There was no regional lymph node involvement. During the postoperative period the results of serum vasoactive intestinal polypeptide were received. These were 815.9 pg/ml before surgery and normalized after the operation.

A Case of Multiple Endocrine Neoplasia Associated with VIPoma / 대한내분비학회지

Multiple endocrine neoplasia I(MEN I) is a genetic disorder that consists of neoplasia of neuroendocrine type in the parathyroid glands, in the islets of Langerhans in the pancreas, and in the anterior pituitary gland. Primary hyperparathyroidism is the most common feature and occurs in approximately 95% of MEN I patients. Pancreatic islet cell tumors occur in 40% of MEN I patients. Most of these tumors produce excessive amounts of hormones, such as gastrin, insulin, glucagon and vasoactive intestinal polypeptide(VIP). VIP-producing pancreatic tumors(VIPoma) associated with MEN I are rare and so far only one has been reported in Korea. Recently, we came across a case of MEN I, associated VIPoma presented persistent hypercalcemia after a parathyroidectomy. A 70 year old man had suffered from large amount of watery diarrhea, severe general weakness and paralysis of lower limbs for 3 months which suggests symptoms of hypercalcemia. Before the patient visited our hospital, he underwent subtotal parathyroidectomy due to hyperparathyroidism. Even though he was operation, there was no subsidization of the symptoms and abnormal findings of blood chemistry such as hypercalcemia, hypocalemia were remained unchanged. However, the parathyroid hormone level was still within normal limits. Abdominal computerized tomography scan demonstrated a mass of 2.5cm diameter in tail of the pancreas. As serum level of VIP hormone was also elevated, distal pancreatectomy was carried out to performed. There was improvement in the symptoms towards the normal condition and the level of biochemical parameters such as serum potassium, calcium and VIP, were also within the normal limits. In a direct sequence analysis, GAC-->CAT(Asp-->His) point mutation, at codon 383 of exon 9 of the MEN I gene was identified in both the patient and his son. The authors report a rare case of VIPoma associated with MEN I with review of literature on MEN I.

A Case of Multiple Endocrine Neoplasia Type I Presenting with a Watery Diarrhea / 대한내분비학회지

MEN1 is characterized by the combined occurrence of tumors of the parathyroids, pancreatic islet cells and the anterior pituitary. Pancreatic islet cell tumors occur in 40% of MEN1 patients. Pancreatic polypeptidomas occur most commonly but they are asymptomatic. Gastrinomas are the second most common type. VIPomas are rare and there has been no report of a case in Korea so far. We encountered a case of pancreatic VIPoma in MEN Type I. A 49 year old man was referred from his local hospital presenting with a sudden onset of an explosive watery diarrhea of 3 months duration. Abnormal findings in his blood chemistry were hypercalcemia and hypokalemia. The 99mTc-sestamibi sintigraphy showed an increased uptake in right lower parathyroid gland. Abdominal CT demonstrated a mass of 6x4 cm in tail of the pancreas and multiple lesions in both hepatic lobes. Serum levels of VIP hormones were elevated. Subtotal parathyroidectomy and subtotal pancreatectomy were done. Postoperatively his symptoms were improved transiently, however the patient showed repetitive attacks of watery diarrhea. So in order to palliate his symptoms, an RF ablation of the metastatic liver masses was performed. After that therapy his clinical symptoms were reduced dramatically. Unfortunately, the patients condition worsened again. Despite of continuous octreotide therapy, interferon and two courses of combination chemotherapy, the hepatic metastases failed to regress and the patient died 10 months after the diagnosis of a metastatic VIPoma. This is the first report of pancreatic VIPoma in MEN type I in Korea.

A case of WDHA(watery diarrhea hypokalemia achlorhydria) syndrome

No abstract available.