原發性腎臟類癌之案例報告與文獻回顧
瞿琤1、黃逸修1,2、黃志賢1,2
臺北榮民總醫院 泌尿部1；國立陽明大學醫學院泌尿學科及書田泌尿科學研究中心2
Primary renal carcinoid: a case report and literature review
Cheng Chu1, Eric Y.H. Huang1,2, William J. Huang1,2
Department of Urology, Taipei Veterans General Hospital
Department of Urology, School of Medicine and Shu-Tien Urological Science Research Center,
National Yang-Ming University, Taipei, Taiwan2
Introduction:
Carcinoid tumors are found in the gastrointestinal tract or the respiratory tract in more than 90% of cases. They are rarely found in the genitourinary system and primary renal carcinoid is extremely rare. The clinical behavior of renal carcinoid is rarely associated with neuroendocrine symptoms. Hence these tumors pose a diagnostic and therapeutic challenge. We reported a primary renal carcinoid without associated symptoms.
Case presentation:
A 63-year-old male was noted to have an incidental right renal massby abdominal sonography. He did not present with abdominal pain, fever, weight loss, or hematuria. Contrast enhanced computerized tomography (CECT) showed a 11.1 cm x12 cm, well-defined heterogeneous and calcified mass at upper pole of right kidney with para-caval enlarged lymph nodes. Radiological diagnosis with of renal cell carcinoma (RCC) was impressed.Robotic assisted laparoscopic right radial nephrectomy and lymph node dissection was smoothly performed. Gross examination of the tumor revealed a tan friable 12 cmx 9.2 cm x 9 cm tumor, replacing the renal parenchyma with necrosis and calcification on cut section. On microscopic exmamiation, tumor cells are positive for INSM1, synaptophysin and CD56, while being negative for chromogranin A, PAX8, GATA3 and CK7 by immunohistochemical stains. The Ki67 proliferation index is approximately 1%. Paracaval lymph nodes were posivie for metastasis. A well-differentiated NET (Carcinoid) was diagnosed. He was referred to oncologist for further treatment.
Conclusion:
Primary renal carcinoid tumors are very rare, malignant tumors composed of neuroendocrine cells. Normally, neuroendocrine cells are not found in adult renal parenchyma. Therefore, the pathogenesis of renal carcinoid is unclear. Primary renal carcinoid has an indolent clinical course but occasionally has lymph node metastasis, subsequently progressing to metastatic disease. The primary treatment for primary renal carcinoidis complete surgical resection and can be curative for localized disease. Residual local disease can be treated with radiotherapy and chemotherapy or palliation can be used for metastatic renal NETs. Due to its rarity, there is no standard treatment for locally advanced or metastatic renal carcinoid. The prognosis and clinical behavior of renal carcinoid remain unclear due to its rarity.