多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房性囊性肾癌一例报告及文献复习

Objective To discuss the diagnosis and outcome of multilocular cystic renal cell car-cinoma. Methods The clinic data of 1 case of multilocular cystic renal cell carcinoma were reviewed with its clinical manifestation, imaging, pathology and therapy in our hospital. The male patient aged 49 was incidentally found to have a right renal tumor. The CT scan showed a multiloeular cystic tumor in the low pole of the right kidney with a clear outline. The thin septa were found in the tumor, which were enhanced in the enhancement CT scan. The enhancement MRI showed the cystic wall was en-hanced. Results The patient received a partial nephrectomy as his treatment. The pathological char-acteristics were as following: the multilocular cystic tumor was about 3 cm×2 cm×2 cm with clear serosity in it. The cystic wall was smooth with the width of 0.1-0.2 cm. Most of the cystic cavities were covered by monostratified or stratified cubic clear cells, and some were covered by squamous epi-thelium or no epithelium at all. The septum was composed of collagen fiber, and clear cells were found in it. The clear cells form small collections but do not form expansile nodules. The final pathological diagnosis was multilocular cystic renal cell carcinoma. The follow-up was 20 months, without local re-currence or distant metastasis was found. Conclusions Multilocular cystic renal cell carcinoma (MCRCC) is a rare variant of renal cell carcinoma with a good prognosis. MCRCC is an uncommon tumor of the kidney composed of multiple cysts with clear cells in the septa indistinguishable from grade I renal cell carcinoma. Most patients are asymptomatie and the tumors are discovered inciden-tally. The preoperative diagnosis of MCRCC immediately depends on imaging studies. Pathology is the key to diagnosis.     

多房囊性肾细胞癌的临床分析及其与vonHippel-Lindau基因突变的相关性研究

目的 提高对多房囊性肾细胞癌(MCRCC)的认识及诊治水平,分析MCRCC中von Hippel-Lindau(VHL)基因的作用.方法 回顾性分析2000-2010年17例MCRCC患者资料,占同期收治512例肾癌的3.32%.男11例,女6例.年龄37～61岁,平均46岁.术前常规行B超、CT等检查,误诊为肾盂旁囊肿1例.应用PCR、PCR产物直接测序等方法分析11例MCRCC组织和相应远离病灶的正常组织中VHL基因突变的情况.结果 17例患者行根治性肾切除术14例,其中1例先行囊肿去顶术,术中囊壁组织冰冻病理检查提示透明细胞癌,改行根治性肾切除术;行肾部分切除术3例.肿瘤直径2.2～6.0 cm,平均(3.6±1.2)cm.术后病理均为MCRCC,镜下主要表现为纤维囊壁组织内衬单层或数层肿瘤细胞,核小致密,胞质透亮.TNM分期均为T1N0M0,病理分级G1 14例,G2 3例.术后随访9～36个月,平均12个月,均未见复发及转移.11例MCRCC组织中7例(64%)存在VHL基因突变,而正常组织标本均未发现VHL基因突变.结论 MCRCC作为肾细胞癌的一种少见的独立亚型,病理及临床上易漏诊或误诊.CT等影像学检查有助于术前诊断,因其预后良好,手术方式推荐保留肾单位手术.VHL基因突变与MCRCC的发生存在一定关系.

Abstract：

Objective To discuss the diagnosis and surgical management of multilocular cystic renal cell carcinoma (MCRCC) and to evaluate the gene function of the mutation of von Hippel-Lindau (VHL) gene in MCRCC. Methods Seventeen MCRCC cases (11 men and 6 women) out of 512 cases of renal cell carcinoma from 2000 to 2010 were retrospectively analyzed. The mean age of the 17 patients was 46 years (37-61 years). Ultrasonography and CT were available in all 17 cases, and 1 case was misdiagnosed as parapelvic renal cyst. The mutation of VHL gene was detected by PCR in the specimens of can-cerous tissue and adjacent normal tissue from 11 cases of MCRCC. Results Three of 17 cases underwent nephron sparing surgery, the others underwent radical nephrectomy. One case underwent unroofing of parapelvic renal cyst, but the rapid frozen pathology of the cyst wall showed renal cell carcinoma of clear type. As a result, radical nephrectomy was eventually performed. All 17 cases were confirmed as MCRCC by eva-luating pathological characteristics, such as the cyst wall lined by single or several layers of clear tumor cells and the nuclei which were small and anachromasis. Clinical stages of all cases were T1N0M0, in which there were 14 cases with pathological T1G1 and 3 cases with pathological T1G2. All patients underwent a follow-up of 9 to 36 months (mean, 12 months) without recurrence or metastasis. Mutation of VHL gene was detected in 7 of 11 cases (64%), but all adjacent normal tissues were negative. Conclusions As a rare subtype of renal cell carcinoma, MCRCC is difficult to diagnose. CT is an essential measure in diagnosis of MCRCC preoperatively. Because of the good prognosis of reported cases, nephron sparing surgery for the treatment of MCRCC is recommended. VHL gene mutations may play an important role in the carcinogenesis of MCRCC.