Endocrine Abstracts

Introduction: Pancreatic neuroendocrine tumors (PNET) are a heterogeneous group with various clinical presentations and lineage. They have an incidence of one per 100 000 individuals per year and represent about 1–2% of all pancreatic tumors. Non functional PNET (NF-PNET) are incidentally discovered in most cases. Pulmonary neuroendocrine tumors constitue a distinct category of tumors with morphologic and biologic neuroendocrine features, they present 20–30% of all NETs and 11–14% of all lung cancers. The association of the two tumors has never been reported.

Case report: We report a case of 46-year old men without particular pathological history admitted to our unit in October 2017 for impairment of general condition and abdominal pain. Clinical examination found a weakened (BMI: 17 kg/m²) anorexic patient emaciated and jaundice. No carcinoid syndrome or clinical signs of tumor hypersecretion. The thoraco-abdomino-pelvic CT scan objectified a left mediastino-pulmonary mass measuring 162*91 mm with a necrotic pancreatic cephalic mass measuring 34*32 mm. Transthoracic pulmonary biopsy was realized; the pathological study concluded to a large cell lung primitive neuroendocrine carcinoma with positive immuno-histo-chemical study to chromogranin; synaptophysin; TTF1; and a Ki67 very high at 80%. We completed our investigation with a echoendoscopic biopsy of the pancreatic mass. The pathological and immunohistochimical study concluded to a primitive low differentiated pancreatic neuroendocrine carcinoma grade 3 (chromogranine − synaptophysine +; CD56+; Ki67: 60%). Brain scan has found multiple cerebral secondary locations. Chemotherapy has been proposed to our patient, unfortunately he died before starting this treatment.

Conclusion: The combination of two primitive neuroendocrine tumors at the same time is very rare, the case of our patient is probably the first case reported in the literature.