Department of Medical Oncology, Dr. Lutfi Kirdar Kartal Education and Research Hospital, Istanbul, Turkey.
Crit Rev Oncol Hematol. 2013 Sep;87(3):256-64. doi: 10.1016/j.critrevonc.2013.02.003. Epub 2013 Mar 9.
Primary carcinoid tumors of the kidney are very rare, malignant tumors consisting of neuroendocrine cells. The pathogenesis of renal carcinoid is unclear because neuroendocrine cells are not normally found in adult renal parenchyma. Electron microscopy, immunohistochemistry, octreotide scan, positron emission tomography along with conventional radiographic imaging techniques are used in diagnosis and follow-up. Presenting symptoms usually include flank pain and haematuria. Early stage disease is treated with surgery only. However, randomized trials are lacking because of the very low number of reported cases. Thus, the role of debulking surgery, chemotherapy, radiotherapy, octreotide and targeted therapy in the management of advanced disease remains an open question. In this article the clinicopathologic features and prognosis of this very rare disease along with treatment outcomes of the reported cases are reviewed. In addition, we report a new case of a metastatic primary renal atypical carcinoid tumor treated with octreotide therapy.
肾脏原发性类癌非常罕见，是由神经内分泌细胞组成的恶性肿瘤。由于神经内分泌细胞在成人肾实质中通常不存在，因此肾脏类癌的发病机制尚不清楚。电子显微镜、免疫组织化学、奥曲肽扫描、正电子发射断层扫描以及常规放射影像学技术均用于诊断和随访。常见的临床表现为腰痛和血尿。早期疾病仅采用手术治疗。然而，由于报告的病例数量非常少，因此缺乏随机试验。因此，在晚期疾病的治疗中，去势手术、化疗、放疗、奥曲肽和靶向治疗的作用仍然是一个悬而未决的问题。本文回顾了这种非常罕见疾病的临床病理特征和预后，以及报告病例的治疗结果。此外，我们还报告了一例采用奥曲肽治疗的转移性原发性肾脏非典型类癌肿瘤的新病例。
