A Rare Presentation of Low-grade Neuroendocrine Tumour
R Koul, WK Dolata, A Dubey
Hong Kong J Radiol 2014;17:e9-14
DOI: 10.12809/hkjr1413191
Neuroendocrine tumours are believed to arise from various cells of the neuroendocrine system. Neuroendocrine cells are present not only in endocrine glands throughout the body that produce various hormones, but also diffusely in body tissues. The majority of neuroendocrine tumours are well differentiated as they usually retain the organ architecture typical of the neuroendocrine organ from where they originate, and they have low proliferative index. Clinical course is highly variable. Small tumours without poor prognostic features are cured by surgical resection. Even if the tumour is advanced and has metastasised where curative surgery is not feasible, surgery often has a role in neuroendocrine cancers for controlling symptoms and, possibly, improves survival. Patients who have metastasis at presentation may live long, thus improving quality of life is important in management. Unfortunately, response to conventional chemotherapy is very low. With the availability of newer agents, disease control rates may increase. Here we report a case with low-grade neuroendocrine tumour with widespread metastases at presentation. The patient was put on everolimus, an oral inhibitor of mammalian target of rapamycin, and has shown good clinical and radiological response.
中文摘要
低度惡性神經內分泌腫瘤的罕見報導
R Koul、WK Dolata、A Dubey
神經內分泌腫瘤可由神經內分泌系統的多種細胞起源。神經內分泌細胞不僅存在於全身的內分泌腺體，產生多種激素，也瀰漫分佈於全身組織中。大多數神經內分泌腫瘤由於保持了起源神經內分泌器官的典型組織結構，多屬分化良好，並具低增殖指數。該病的臨床表現差異很大。小腫瘤無不良預後因素，手術切除即可治癒。即使腫瘤已屬局部晚期或出現遠處轉移，難以根治性切除，手術仍能有效緩解症狀，並有可能提高生存率。神經內分泌腫瘤患者，即使出現遠處轉移，仍有可能長期生存，因此，改善生存質量是治療的重要考慮因素。可惜，患者對常規化療的反應偏低。隨着新藥物的出現，疾病控制率有可能提升。本文報告了一已出現廣泛轉移的低度惡性神經內分泌腫瘤病例。Everolimus是一種哺乳動物雷帕黴素靶蛋白的口服抑製劑，病人接受everolimus治療後，臨床症狀改善，影像學反應良好。