Endocrine Abstracts

Introduction: Neuroendocrine neoplasms are rare heterogeneous malignancies originating from neuroendocrine cells throughout the body. The most common primary sites are the gastrointestinal and respiratory tracts, but can originate from almost any organ, some in unusual locations. The diagnosis is based on the confirmation of the endocrine nature of the tumor and secondly the confirmation of its primary nature. We describe two cases with a very rare primary localization of neuroendocrine tumor: one in the breast and one hepatic.

Discussion: Case 1 A 43-year-old woman presents after self-diagnosing a left breast lump. Breast ultrasound showed a hypoechoic formation of 1.1/0.6 cm, left breast sectorectomy is performed. Histopathological result: G2 mammary neuroendocrine tumor, positive for hormone receptors (estrogen Alfred 7, progesterone Alfred 6), ki67% 30%, synaptophysin, and HER2 negative. SSTR-2 and SSTR-5 receptors, were positive but no other lesions with somatostatin receptor expression on Tektrotyd scintigraphy. Imagistics was negative, preoperative and at 4 months postoperatively. No carcinoid syndrome with normal serotonin. Chromogranin A was 7.7 times than normal but normalized 6 months post-operatively. Polychemotherapy with Epirubicin and Cyclophosphamide was initiated. It is desired to initiate adjuvant therapy with somatostatin analogue, as evidence supports benefits to survival when endocrine therapies are used in the adjuvant setting. Case 2 A 55-year-old woman known to have extensive right hepatectomy with resection of the main bile duct, lympho-dissection of hepatic and retrocephalopancreatic pedicle. Histopathological result shows: G1/G2 hepatic neuroendocrine tumor, with multiple secondary lymph node determinations, positive for synaptophysin, chromogranin A, CD-56, ki67 4%. Postoperatively, CT TAP, abdominal MRI, cholangio-MRI were performed periodically without evidence of other determinations. Biochemically, within normal limits: neuronal specific enolase, chromogranin A, 5-hydroxyindoleacetic acid/urinary 24 h, with a single slightly elevated serum serotonin 271.10 ng/ml (20-206). Treatment with somatostatin analogue was initiated. Octreoscan scintigraphy is awaited.

Conclusion: Neuroendocrine tumors are a very rare entity, breast and hepatic primary location are unusual. The diagnosis can be challenging and is based on pathological examination and immunohistochemistry, as well as the result of the Octreoscan. The gold-standard treatment is represented by surgical resection and long-term prognosis remains favorable.