S2131 Rare Case of Synchronous Association of Adenocarcinoma of the Ampulla of Vater With Sporadic Squamous Cell Carcinoma of the Oropharynx

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Introduction: Concomitant adenocarcinoma of the Ampulla of Vater (AOV) with other sporadic primary cancers is rare with a majority of cases presenting with gastrointestinal (GI) primary cancers. This case represents a unique occurrence of synchronous sporadic adenocarcinoma of AOV and squamous cell carcinoma (SCC) of the oropharynx. Case Description/Methods: 65-year-old male with hyperbilirubinemia and transaminitis was initially suspected to have non-alcoholic fatty-liver disease. Abdominal CT showed dilated intra and extrahepatic bile ducts without obstruction and mildly prominent mesenteric retroperitoneal lymph nodes. A year later, he visited the ER for fever and dark urine. Total bilirubin was 3.2, and he was found to have Klebsiella bacteremia. There was concern for cholangitis prompting MRCP, which showed common bile duct (CBD) dilation and a filling defect at the AOV. Indeterminate lymphadenopathy was noted in the thorax and abdomen. ERCP demonstrated a 2 cm ulcerated mass at the AOV; a plastic stent was placed for drainage. Pathology showed poorly differentiated adenocarcinoma with signet ring features. Subsequently, distal paraoesophageal lymph node biopsy showed poorly differentiated adenocarcinoma. PET scan showed anterior oropharyngeal mass concerning for another potential primary malignancy with adenopathy in the neck, mediastinum, and retroperitoneum. Tongue biopsy demonstrated poorly differentiated invasive SCC. He underwent 6 months of mFOLFIRINOX and started FOLFIRI maintenance therapy for AOV carcinoma. He received palliative radiation for SCC of the tongue. Repeat imaging showed good response to therapy. Discussion: AOV adenocarcinoma, a subset of GI cancers, accounts for 0.2% of cases, and signet ring morphology is exceptionally rare. Patients with primary neoplasms are prone to developing second malignancies compared to the general population. While previous reports highlighted associations between AOV carcinoma and synchronous GI malignancies, there have been limited cases of synchronous cancers including lung, kidney, prostate, female breast, and corpus uteri. This case is the only documented instance of synchronous SCC of the tongue occurring with AOV adenocarcinoma. Clinicians should be vigilant of other synchronous cancers in similar patients.

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