Endocrine Abstracts

Introduction: The GEP NEN (gastroentero-pancreatic neuroendocrine neoplasms) are 70% of all neuroendocrine tumors, but are rare in general population – constituting only 2% of the gastrointestinal tract neoplasms. Carcinoid stands for 50% of GEP NEN, most often located in the appendix and/or ileum. Although the detection of NEN is rising, their prevalence is still likely underestimated. The clinical presentation is not characteristic at the early stages of the disease, it varies among patients, what may make the diagnosis difficult to establish. We present a case of a man with certain clinical characteristics of carcinoid. Specific points suggesting a GEP NEN and critical to the clinical practice are discussed.

Case report: A 65-year-old man with the previous history of hypertension, atrial fibrillation with tricuspid valve insufficiency, DM2 and a few small liver haemangiomas discovered in 2013, was referred to internal diseases department in Wroclaw in 2016. He actually complained of poor hypertension controll, sporadic diarhoea, not significant weight loss. Performed abdominal CT scans revealed multiple large liver lesions both with enlarged regional lymph nodes of suspected metastastatic origin. Other CT scans and endoscopic investigations found no primary tumor, except few gastric erosions, diverticulosis coli and benign rectal polyp. On account of the patient’s slight recurrent flushes reported in a deepened interview and echocardiography revealing endocardial fibrosis with advanced degeneration of the tricuspid valve (regurgitation), the GEP NEN was suspected. Performed Tectrotide somatostatin receptor scintigraphy SPECT CT Tc99m was negative, CgA concentration was 425ng/ml and finally liver biopsy material with immunohistochemistry confirmed metastatic NEN G1. Next PET CT 18F-FDG scan revealed metabolically active lesion in the ileum, which was confirmed in Galium 68 PET(neoplasm with high expression of somatostatin receptors of the smal intestine from dissemination to mesenteric lymph nodes and liver). Due to advanced heart failure the primary tumor of the ileum was not treated surgically. The patient receives chronically Sandostain LAR, he underwent also 177Lu Dotatate therapy in Oncology Center, Gliwice. The size and number of the liver lesions are under controll.

Conclusion: Carcinoid diagnosis needs various imaging techniques and histopathological evaluation, but clinically accuracy is of greatest importance. Incidental occurrence of liver lesions suggests the need for the GEP NEN screening, at least evaluation of CgA and/or CT control, especially in the case of accompanying the right heart valve abnormalities. Early treatment is crucial to avoid dissemination of the disease and life-threatening complications.