Squamous cell carcinoma of the ampulla of vater – A case report

Introduction: Ampullary carcinoma comprises up to 2% of all gastrointestinal malignancies. Primary squamous cell carcinoma of the ampulla is a very rare neoplasm, with only three reported cases in literature. Due to its rarity, the biological behavior is largely unknown. We highlight such a case, and its diagnostic workup. Case Report: A 61 year old woman presented with painless obstructive jaundice of 2 months duration. MRI scan showed a stricture in the distal common bile duct suggestive of a neoplasm. Biopsy at ERCP showed a poorly differentiated ampullary carcinoma. The specimen received at Whipple’s surgery showed a white tumour surrounding the ampulla, 25 mm in maximum dimension. Histology and immunohistochemistry (CK 7+/20 -, CA 19-9+, CK5/6 focally+) pointed towards a poorly differentiated squamous cell carcinoma. A primary squamous carcinoma elsewhere was excluded. The patient is alive nine months after surgery. Discussion: Ampullary squamous cell carcinoma is most often metastatic from sites such as the larynx, esophagus, lung and uterus. A squamous carcinoma therefore requires ruing out of a primary site elsewhere or a glandular component (adenosquamous carcinoma). These were excluded in this patient. Immunohistochemistry differed from that of primary adenocarcinoma of the ampulla.