A rare, life-threatening debut of pancreatic adenocarcinoma: Pulmonary tumor thrombotic microangiopathy

Abstract

Pulmonary tumor thrombotic microangiopathy (PTTM) is a paraneoplastic syndrome associated with mucin secreting adenocarcinomas, usually gastric. Mucin secretion releases the coagulation cascade and activates endothelial growth factors, causing thrombosis and endothelial proliferation with secondary luminal stenosis. PTTM, which is usually fatal in a very short time, presents a significant ante-mortem diagnostic challenge. We report the case of a 59-year-old woman with metastatic pancreatic adenocarcinoma who developed PTTM, leading to cor pulmonale and lethal cardiogenic shock. This is the fourth case report of PTTM secondary to pancreatic adenocarcinoma. The clinical suspicion was confirmed in the autopsy when a second, previously undetected tumor was found. This report highlights the need for postmortem studies in current oncology practice.

Keywords

• Pulmonary tumor thrombotic microangiopathy
• Paraneoplastic syndrome
• Cor pulmonale
• Pathological autopsy
• Familial pancreatic cancer