Adenosquamous carcinoma of the ampulla of Vater - a rare disease at unusual location

Adenosquamous carcinoma is defined as a tumor in which both glandular and squamous elements are histologically malignant. Although some published studies have analyzed and discussed adenosquamous carcinomas, hybrid malignancy of the ampulla of Vater has rarely been discussed thus far in the literature. In this study, we report the case of a 64-year-old man who presented with jaundice and intermittent abdominal dull pain that persisted for several weeks. The patient was diagnosed with adenosquamous carcinoma of the ampulla of Vater and underwent pancreaticoduodenectomy. The final diagnosis was adenosquamous carcinoma of the ampulla of Vater, T3N1M0, stage IIB. Although R0 resection was performed, he had multiple liver metastases 2 months after the operation; he died 4 months later. Upon reviewing the medical records of our institute, we identified 4 patients who were diagnosed with adenosquamous carcinoma of the ampulla of Vater in the past 2 decades. We also identified only five reported cases of this lesion in the English literature. Adenosquamous carcinoma of the ampulla of Vater is a rare disease with a dismal prognosis. Surgical intervention does not appear to prolong patient survival. Early recurrence and distal metastasis may be encountered after surgery.