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Tuberous sclerosis patient with neuroendocrine carcinoma of the esophagogastric junction: A case report
- Source :
- World Journal of Gastroenterology
- Publication Year :
- 2020
- Publisher :
- Baishideng Publishing Group Inc, 2020.
-
Abstract
- Background Tuberous sclerosis complex (TSC) is a rare inherited disease with non-cancerous tumor growths in the skin, brain, kidneys, heart, and lungs. The co-occurrence of neuroendocrine neoplasm (NEN) with TSC is even rarer. There have been few reports on the relationship between TSC and neuroendocrine tumors (NETs), and fewer on the relationship between TSC and neuroendocrine carcinoma (NEC), a subtype of NEN. This is the first reported case of NEC occurring at the esophagogastric junction in a patient with TSC. Case summary A 46-year-old woman visiting our hospital for the treatment of TSC was admitted to the emergency department with tarry stools and dizziness. Computed tomography scans revealed thickness of the gastric cardia, multiple metastatic lesions of the liver, and enlarged lymph nodes near the lesser curvature of the stomach. Esophagogastroduodenoscopy revealed a type 3 tumor located from the esophagogastric junction to the fundus, and the pathological diagnosis by biopsy was NEC. The patient was treated with seven courses of cisplatin + irinotecan, followed by eight courses of ramucirumab + nab-paclitaxel, one course of nivolumab, and two courses of S-1 + oxaliplatin. Twenty-three months after the first treatment, the patient died because of disease progression and deterioration of the general condition. Conclusion This case of NEC occurring in a patient with TSC indicates a difference in the occurrence of NETs and NECs.
- Subjects :
- medicine.medical_specialty
congenital, hereditary, and neonatal diseases and abnormalities
mTOR inhibitor
Neuroendocrine tumors
Gastroenterology
Ramucirumab
03 medical and health sciences
Tuberous sclerosis
0302 clinical medicine
Neuroendocrine tumor
Internal medicine
Biopsy
Case report
medicine
Chemotherapy
neoplasms
medicine.diagnostic_test
Esophagogastroduodenoscopy
business.industry
Stomach
General Medicine
medicine.disease
Curvatures of the stomach
digestive system diseases
Esophagogastric junction
medicine.anatomical_structure
Tuberous sclerosis complex
030220 oncology & carcinogenesis
Neuroendocrine carcinoma
030211 gastroenterology & hepatology
Nivolumab
business
Subjects
Details
- Language :
- English
- ISSN :
- 22192840 and 10079327
- Volume :
- 26
- Issue :
- 45
- Database :
- OpenAIRE
- Journal :
- World Journal of Gastroenterology
- Accession number :
- edsair.doi.dedup.....37dc13dfefbcfc3395ba496401a31b2c