Ampulla Of Vater Small Cell Neuroendocrine Carcinoma C1332250
A rare, aggressive neuroendocrine cancer of the Ampulla of Vater arising from hormone-producing cells. It tends to grow and spread quickly. Treatment may involve surgery, chemotherapy, and other targeted approaches.
Key facts at a glance
- ICD-10
- C80.1
- ICD-11
- 2A00.0
- Body system
- oncologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Obstructive jaundice
Generalized symptoms
(oncologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Weight loss
(constitutional)Abdominal pain
(gastrointestinal)Carcinoid syndrome (flushing and diarrhea)
(endocrine)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Platinum-Based Chemotherapy
First-line systemic chemotherapy using platinum agents combined with etoposide for small cell neuroendocrine carcinoma of the ampulla of Vater, targeting the aggressive neuroendocrine component.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Radical Surgical Resection
Aggressive surgical resection including pancreaticoduodenectomy with lymphadenectomy when the tumor is localized, as these tumors have high metastatic potential even when seemingly localized.
Additional treatment options
Topotecan Therapy
Second-line chemotherapy option using topotecan for patients with progressive disease following platinum-based regimens, particularly for refractory or relapsed small cell carcinoma.
medicationEndoscopic Biliary Decompression
Endoscopic placement of biliary stents or nasobiliary drains to relieve malignant biliary obstruction caused by the tumor, improving quality of life and liver function.
procedureNutritional Support
Specialized nutritional intervention including pancreatic enzyme supplementation and dietary modifications to maintain nutritional status during intensive chemotherapy treatment.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Ampulla Of Vater Small Cell Neuroendocrine Carcinoma C1332250?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Benson AB, Ajani JA, Engelstein BJ, et al. NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine and Adrenal Tumors. J Natl Compr Canc Netw. 2023.
Zhang L, Wang LW, Chen Z, et al. Small Cell Neuroendocrine Carcinoma of the Ampulla of Vater: A Systematic Review. Pancreas. 2021.
Kim JH, Lee SH, Choi EK, et al. Neuroendocrine Tumors of the Ampulla of Vater: Clinicopathological Features and Prognostic Factors. Ann Surg Oncol. 2020.
Washington MK, Goldberg RM, Chang GJ, et al. Protocol for the Examination of Specimens from Patients with Carcinoma of the Ampulla of Vater. Arch Pathol Lab Med. 2019.
Medication Comparisons
Compare medications used to treat Ampulla Of Vater Small Cell Neuroendocrine Carcinoma C1332250. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy