Abstract

Background Primary squamous cell carcinoma of the ampulla of Vater is a rare tumor, and the treatment and prognosis of which is not well known because of limited number of reported cases in the literature. Here, we report the case of primary squamous cell carcinoma of the ampulla of Vater, treated with multimodality therapy (surgery and adjuvant chemotherapy), and have a longer survival than the reported cases in the literature. Case presentation A Sixty-five-year-old woman presented with painless progressive jaundice and generalized itching. The clinical presentation, radiographic and endoscopic investigations strongly suggest periampullary carcinoma. We performed Pylorus-preserving pancreaticoduodenectomy with curative intent. Histopathological examination and immunohistochemistry analysis revealed primary squamous cell carcinoma of ampulla of vater. The postoperative course was uneventful. The patient received adjuvant chemotherapy, and patient is on follow up with no tumor recurrence, and having a good quality of life for last 1 year after multimodality therapy. Conclusion Primary squamous cell carcinoma of the ampulla of Vater is a rare histological type. The literature review revealed the poor prognosis of all the reported cases of primary squamous cell carcinoma of the ampulla of Vater. The optimal treatment and prognosis is unclear. To the best of our knowledge, this is the first report of primary squamous cell carcinoma of the ampulla of Vater that was treated with multimodality therapy. With our experience, we suggest that multimodality treatment should be the mainstay therapy for primary squamous cell carcinoma of the ampulla of Vater. However, more number of studies on clinico-pathological findings and treatment modality are required to determine appropriate treatment strategy and prognosis of this tumor.