Endocrine Abstracts (2016) 40 P12 | DOI: 10.1530/endoabs.40.P12

Ectopic cushing syndrome by an aggressive gastroenteropancreatic neuroendocrine carcinoma

C Robles Lázaro, L P Llamocca Portella, A I Sánchez Marcos, A Herrero Ruiz, H S Villanueva Alvarado, J S Cabezas Andrade, R Cáceres Martín, J M Miralles García & J J Corrales Hernández


Hospital Clínico de Salamanca, Salamanca, Spain


Introduction: Gastroenteropancreatic neuroendocrine tumors have a highly variable presentation, which can range from benign neoplastic growths to rapidly aggressive malignancies. Neuroendocrine carcinomas of the digestive system are relatively rare. The incidence of GEP NEC in Spain is estimated to be 2.5 – five cases per 100 000 inhabitants.

Case: We describe the case of a 67-year-old man, previously diagnosed with hypertension and dyslipidemia. He was admitted due to hyperglycemia. The clinical examination was not remarkable and the first blood sample showed elevated transaminases (with progressive worsening) and hypokalemic metabolic alkalosis. The CT scan showed hypodense liver lesions with peripheral enhancement, suggestive of metastases, and lymph nodes located in pancreatoduodenal space, that were associated with a thickening of the third duodenal portion wall. The result of the biopsy sample was a neuroendocrine carcinoma with Ki- 67 of 80% and a rate of mitosis of 10/10 HPF representing WHO Grade 3. Because there was contraindication of other alternative treatment due to the hepatic involvement, and the positive uptake in the octreoscan, symptomatic treatment with somatostatin analogues was considered. Also, the patient received treatment with cisplatin and etoposide. Despite the treatment, patient’s condition progressively deteriorated and he died 1 month after the diagnosis of NEC.

Conclusion: Neuroendocrine carcinomas are high-grade malignancy tumors that can be metastatic at diagnosis and have a high index of cell proliferation, with poor prognosis for all stages of disease, with a median survival of 5 months for metastatic disease from the time of diagnosis.