ABSTRACT
Patients with neurofibromatosis type 1 are at increased risk of developing carcinoid tumours of the duodenum, particularly in the ampulla of Vater. Aggressive surgery with pancreatoduodenectomy is recommended for all ampullary carcinoid tumours because of their propensity to metastasise. We report a case of a 43-year-old woman with neurofibromatosis type 1, who presented with recurrent epigastric pain for 15 months. Evaluation using magnetic resonance cholangiopancreatography and side-viewing duodenoscopy revealed a submucosal tumour at the ampulla of Vater causing pancreatobiliary ductal obstruction and dilation. The ampullary tumour was overlooked initially by forward-viewing endoscopy. The patient subsequently underwent pancreatoduodenectomy. Histological examination of the surgically resected specimen confirmed the presence of a carcinoid tumour, with metastasis to peri-pancreatic lymph nodes. She remained asymptomatic 10 months after surgery.
Subject(s)
Abdominal Pain/etiology , Ampulla of Vater , Carcinoid Tumor/etiology , Common Bile Duct Neoplasms/etiology , Neurofibromatosis 1/complications , Adult , Carcinoid Tumor/diagnosis , Carcinoid Tumor/surgery , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Female , HumansABSTRACT
Lymphoepithelioma-like carcinoma is a rare tumour in the oral cavity and is characterized histologically by non-keratinizing, undifferentiated squamous cell carcinoma with lymphocytic infiltration. Three consecutive cases of intraoral lymphoepithelioma-like carcinoma are reported. A review of the literature reveals a similar biological behaviour to that of nasopharyngeal lymphoepithelioma: a high incidence of cervical nodal spread and remarkable radiosensitivity. Chemotherapy should be considered when nodal or distant metastases are present. The association of the Epstein-Barr virus with this tumour remains unclear but our experience suggests a positive correlation in Chinese individuals.
