701 results match your criteria: "VIPomas"
Vasoactive Intestinal Polypeptide Secreting MS Neuroblastoma.
J Indian Assoc Pediatr Surg
November 2024
Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
We present a rare case of a 17-month-old child diagnosed with MS-stage neuroblastoma and associated chronic diarrhea due to elevated vasoactive intestinal peptide (VIP) levels. The unusual occurrence of a VIP-secreting tumor in a patient with MS neuroblastoma is a rare combination, not previously reported in the literature. The patient underwent exploratory laparotomy and excision of the tumor, leading to a significant decline in VIP levels and resolution of symptoms.
View Article and Find Full Text PDFCarcinoid heart findings in vasoactive intestinal peptide-secreting tumour.
BMJ Case Rep
November 2024
Division of Cardiology, Department of Medicine, Harbor-UCLA Medical Center, Torrance, California, USA.
Article Synopsis
- - Diagnosis of tricuspid valve disease in patients with carcinoid heart syndrome is often done with echocardiography, especially in those already diagnosed with carcinoid syndrome.
- - VIPoma is a rare type of tumor occurring in 0.05%-2% of cases, causing symptoms like flushing, diarrhea, and electrolyte issues without typically affecting heart valves.
- - In a notable case, detection of tricuspid regurgitation and stenosis during echocardiography led to a quicker investigation for cancer, ultimately revealing the presence of VIPoma.
VIPoma: An Unusual Cause of Chronic Diarrhea.
Acta Medica (Hradec Kralove)
October 2024
Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
Chronic diarrhea is a significant challenge in clinical practice because of its high prevalence and various causes. Comprehensive clinical assessment and stepwise laboratory approach are crucial for an accurate diagnosis. This report presents a case of an adult woman who experienced chronic watery diarrhea, complicated by renal impairment and multiple electrolyte imbalances, including hypokalemia, hypophosphatemia, and metabolic acidosis.
View Article and Find Full Text PDFEfficacy of Racecadotril in a Patient Affected by a Therapy-Refractory VIPoma and Carcinoid Syndrome.
JCEM Case Rep
October 2024
Division of Endocrinology, Diabetes and Clinical Nutrition, Department of Internal Medicine I, University Hospital Schleswig-Holstein, Campus Kiel, 24105 Kiel, Germany.
Article Synopsis
- - Neuroendocrine neoplasms (NENs) are diverse tumors from neuroendocrine cells, with about 30% causing hormone-related syndromes that can significantly impact patient health beyond the tumor size itself.
- - A 49-year-old male patient with an ileal NEN exhibited concurrent VIP and serotonin release, leading to a severe diarrhea syndrome after receiving peptide-radio-receptor therapy, despite ongoing medical treatments.
- - As traditional treatment options were no longer effective, an off-label use of racecadotril helped manage the patient's condition, allowing him to leave the ICU after five days of treatment.
Vasoactive Intestinal Peptide-Producing Neuroblastic Tumors: A Rare Cause of Refractory Diarrhea.
Cureus
August 2024
Pediatric Oncology, Grupo de Apoio ao Adolescente e a Criança com Câncer (GRAACC) / Universidade Federal de São Paulo (UNIFESP), Sao Paulo, BRA.
Neuroblastic tumors are the most common malignant extracranial solid tumors of childhood. A small subgroup presents chronic incoercible diarrhea due to the tumor's production of vasoactive intestinal peptide (VIP). The hypothesis of an occult tumor is not always considered, which delays and impairs treatment.
View Article and Find Full Text PDFEndoscopic Ultrasound-Guided Radiofrequency Ablation of Metastatic Pancreatic VIPoma: A Novel Treatment.
Cureus
August 2024
Department of Gastroenterology and Hepatology, Asian Institute of Gastroenterology, Hyderabad, IND.
VIPomas are a rare type of functional pancreatic neuroendocrine tumors (PNETs), causing symptoms due to their hypersecretion in the gastrointestinal tract. The rare association of PNETs with tumors of endocrine organs such as pituitary and parathyroid glands is called multiple endocrine neoplasia (MEN1) syndrome. Due to their indolent course, VIPomas often present late in the illness and may already have metastatic disease.
View Article and Find Full Text PDFManagement of functional neuroendocrine tumors.
Curr Probl Cancer
October 2024
ENETS Center of Excellence, Division of Endocrinology and Metabolism, University of Iowa, 200 Hawkins Drive, Room E400 GH, Iowa City, Iowa, 52242, USA. Electronic address:
Article Synopsis
- * Managing the symptoms associated with functional NENs may require approaches like tumor removal, chemotherapy, or specialized therapies aimed at reducing hormone production and its effects on the body.
- * This review emphasizes the importance of addressing the hormonal symptoms of NENs alongside traditional tumor reduction strategies to enhance the overall wellbeing of patients.
Characteristics, therapy, and outcome of rare functioning pancreatic neuroendocrine neoplasms.
Sci Rep
August 2024
Department of Visceral, Thoracic and Vascular Surgery, University Hospital Marburg, Philipps University Marburg, Marburg, Germany.
Article Synopsis
- * Out of 216 patients with pNENs treated from 2002 to 2022, 12 were identified as rf-pNENs, including different subtypes like vasoactive intestinal polypeptide, glucagon, and calcitonin-producing tumours.
- * Following surgery, most patients showed positive outcomes, with a median follow-up of 75 months indicating that those who had complete resections (R0) and no liver metastases had a better prognosis, with six patients remaining alive and disease-free.
A cross-species transcriptomic analysis reveals a novel 2-dimensional classification system explaining the invasiveness heterogeneity of pancreatic neuroendocrine tumor.
Cancer Lett
July 2024
Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China; State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China. Electronic address:
Article Synopsis
- Pancreatic neuroendocrine tumors (PanNETs) show a wide range of invasiveness, and existing genomic knowledge sheds some light on this variability, especially between different types like insulinomas and other PanNET variants.
- Researchers used a 2D classification system based on RNA sequencing of PanNETs from both mouse models and humans to categorize these tumors primarily into benign insulinomas and various invasive subtypes.
- By integrating the 2D classification, specific mutation status (DAXX/ATRX), and tumor size, they identified a subset of PanNETs with low recurrence risk, helping to clarify the complexity of these tumors and enhance their prognostic evaluation.
Interference With VIP to Distinguish Between Real and False VIPoma: National Study From the French Endocrine Tumors Group.
J Endocr Soc
May 2024
Service de Biochimie, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron 69500, France.
Article Synopsis
- VIPomas are rare tumors that cause life-threatening hormonal issues by secreting vasoactive intestinal peptide (VIP), and plasma VIP can be measured to confirm diagnosis, although interference in tests can occur.
- Researchers developed three methods to detect potential interference in VIP radioimmunoassay (RIA) tests and studied both patients suspected of false positive VIP results and those with confirmed VIPomas.
- Their results showed that all false positive patients had interference in the tests, whereas no such issues were found in confirmed VIPoma patients, indicating the importance of using their developed techniques when test results don't match clinical symptoms.
Successful liver transplantation as rescue therapy in a patient with metastases from a vasoactive intestinal peptide producing neuroendocrine tumor.
J Surg Case Rep
May 2024
ENETS Center of Excellence, Copenhagen University Hospital, Rigshospitalet, 2100 Copenhagen, Denmark.
Article Synopsis
- A 40-year-old patient with a high-grade neuroendocrine tumor from the pancreas underwent successful liver transplantation after experiencing rapid disease progression and severe symptoms requiring extensive intravenous support.
- This case challenges existing guidelines which suggest liver transplantation should only be for tumors with a Ki-67 score below 20% and under control for over 6 months.
- Eighteen months post-transplant, the patient shows no sign of tumor recurrence and is in good health, indicating that transplantation may be a viable option for select NET patients despite higher tumor grades.
A Rare Presentation of Lymph Node Metastasis of VIPoma After Three Years of Resection: A Case Report.
Cureus
April 2024
Department of Hepato-Biliary-Pancreatic Surgery, Ehime University Graduate School of Medicine, Ehime, JPN.
Article Synopsis
- * In this case, a 60-year-old male, previously treated for VIPoma, was found to have lymphadenomegaly suggesting rare lymph node metastasis during a routine follow-up CT scan.
- * The patient underwent a successful lymphadenectomy, marking the first documented case of metachronous lymph node metastasis in someone who had previously undergone surgery for VIPoma, highlighting the importance of ongoing monitoring for recurrence.
Advancements in medical treatment for pancreatic neuroendocrine tumors: A beacon of hope.
World J Gastroenterol
March 2024
Department of Endocrinology and Metabolism, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, 605006, India.
Article Synopsis
- The editorial discusses significant advancements in treatment strategies for pancreatic neuroendocrine tumors (pan-NETs), focusing on personalized approaches for different tumor subtypes.
- Key treatments like cytoreductive surgery and somatostatin analogs (SSAs) are highlighted, while more aggressive options are reserved for patients unresponsive to SSAs.
- The article emphasizes the need for distinct therapies for different types of neuroendocrine tumors and suggests that understanding their genetics and exploring immunotherapies could lead to better future treatment options.
All you need to know about VIPoma: Review on the latest studies.
Presse Med
June 2024
Faculty of Medicine, Riga Stradins University, 16 Dzirciema Street, Riga LV-1007, Latvia. Electronic address:
Article Synopsis
- Vasoactive intestinal peptide secreting tumor (VIPoma) is a rare, often malignant neuroendocrine tumor that leads to symptoms like watery diarrhea, low potassium levels, and lack of stomach acid.
- Diagnosis typically involves identifying symptoms, blood and stool tests, imaging studies, and specific lab findings.
- Treatment options focus on stabilizing the patient's condition through fluid and electrolyte replacement, medication with somatostatin analogs, and may include surgery or chemotherapy.
Synchronous Insulinoma and Glucagonoma: A Review of the Literature.
In Vivo
November 2023
NS Christeas Laboratory of Experimental Surgery and Surgical Research, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Article Synopsis
- Pancreatic neuroendocrine tumors (PNETs) are classified into functioning and non-functioning types, with non-functioning tumors being the most common and associated with higher morbidity due to potential tissue invasion and metastasis.
- Insulinoma is the most prevalent functioning PNET, leading to symptoms from excess insulin, while glucagonoma is another type that causes hyperglycemia from high glucagon levels, and the co-occurrence of both is extremely rare.
- A literature review identified 8 cases of patients with both insulinoma and glucagonoma from 1992 to 2021, highlighting the need for a multidisciplinary approach for effective diagnosis and treatment.
Chronic Diarrhea Caused by Vasoactive Intestinal Peptide-Secreting Tumor.
Life (Basel)
September 2023
First Pediatric Clinic, Disturbances of Growth and Development on Children Research Center, "Victor Babeș" University of Medicine and Pharmacy, 300041 Timișoara, Romania.
Article Synopsis
- * In children, VIPomas are less common and often linked to neurogenic tumors located in the retroperitoneum or mediastinum, rather than the pancreas.
- * A case study of an infant with chronic diarrhea revealed a VIPoma in the retroperitoneum, diagnosed through imaging, leading to successful tumor removal and normalization of both diarrhea and electrolyte levels, confirming the tumor as ganglioneuroblastoma through further tests.
Pancreatic Vasoactive Intestinal Peptide-Producing Tumor as a Rare Cause of Acute Diarrhea and Severe Hypokalemia.
J Med Cases
October 2023
Department of Internal Medicine, General Hospital of Kavala, Greece.
Article Synopsis
- Pancreatic vasoactive intestinal peptide-producing tumor (VIPoma) is a rare type of tumor that causes chronic watery diarrhea and various electrolyte imbalances, primarily hypokalemia and hypercalcemia, often leading to delayed diagnosis.
- The case discussed involves a 45-year-old woman with sudden, severe diarrhea and significant electrolyte disturbances that required urgent medical attention and further investigation for a functional neuroendocrine tumor.
- After confirming VIPoma through MRI and biochemical tests, the patient underwent surgery to remove the tumor, but experienced complications post-surgery, including severe anemia and a retroperitoneal cystic lesion.
Calcium-sensing receptor activator cinacalcet for treatment of cyclic nucleotide-mediated secretory diarrheas.
Transl Res
January 2024
Department of Pediatrics, University of California, San Francisco, California. Electronic address:
Article Synopsis
- * The FDA-approved drug cinacalcet, which activates the extracellular calcium-sensing receptor (CaSR), shows promise by significantly inhibiting components of secretory currents in various cell and animal models of diarrhea.
- * Cinacalcet demonstrated a reduction in intestinal fluid accumulation by about 55% in a mouse model of cholera and has potential for repurposing as an effective treatment for cyclic nucleotide-mediated secretory diarrheas due to its strong safety profile.
European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes.
J Neuroendocrinol
August 2023
Department of Visceral, Thoracic and Vascular Surgery, Phillips-University Marburg and University Hospital Gießen Marburg GmbH, Marburg, Germany.
Article Synopsis
- * A functioning syndrome is characterized by a clinical presentation and high levels of specific hormones due to the presence of NETs, with several types identified, including insulinoma and gastrinoma.
- * The recommendations include approaches for biochemical, genetic, and imaging assessments, as well as management strategies for different hormonal syndromes in pancreatic NETs, particularly for well-differentiated tumors.
Analysis of Clinical Characteristics and Survival in Patients With Functional Neuroendocrine Tumors of Gastrointestinal Origin.
Pancreas
October 2022
From the Department of Hematology-Oncology, Albert Einstein College of Medicine-Jacobi Medical Center.
Article Synopsis
- The study analyzed survival trends in patients with functional neuroendocrine tumors (FNETs), which produce excess hormones and are relatively rare.
- A total of 529 FNET patients were evaluated, with gastrinoma and insulinoma being the most common types, primarily located in the pancreas.
- Findings revealed that older age, lack of surgical treatment, presence of metastasis, and poor tumor differentiation negatively affected patient survival, with median overall survival at 9.8 years.
Recurrent primary hepatic VIPoma treated with a combination of surgical resection and loco-regional therapy.
Future Sci OA
January 2023
Gastroeneterology Department, Sahloul University Hospital, 4052, Tunisia.
Article Synopsis
- * The management of VIPomas is complex and lacking clear guidelines, posing challenges for healthcare providers.
- * A unique case study illustrates the recurrence of a primary hepatic VIPoma in a woman 22 years post-surgery, successfully treated with transarterial chemoembolization, emphasizing the need for long-term patient monitoring for potential recurrence.