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.     

Bilateral renal cell carcinoma in a patient receiving long-term dialysis

Bilateral renal cell carcinoma developing in a 55-year-old male receiving long-term dialysis is reported. The patient, who had undergone maintenance hemodialysis for 12 years, was admitted in July, 1986 for the purpose of extensive examination of a right renal mass. CT scan and ultrasonography demonstrated an enlarged bilateral kidney associated with multiple cysts, containing a solid mass in the right upper pole. The patients underwent right nephrectomy under the diagnosis of right renal tumor. The solid tumor 4 x 3 cm in size revealed a grade 1 to 2 renal cell carcinoma pathologically. Two small tumors were recognized in other portions of the kidney. Multiple cysts of varying sizes, the largest 2 cm in diameter, replaced the renal parenchyma. The cyst walls frequently contained hyperplastic changes. The patient has been subsequently followed up for 2 years and 9 months and underwent left nephrectomy for suspected left renal tumor. The surface of the left kidney was covered by numerous cysts. The result of pathological examination was renal cell carcinoma, which was recognized in a total of 7 regions of the left kidney. The patient remains well on hemodialysis, with no evidence of recurrence or metastasis.     

Juvenile renal cell carcinoma as first manifestation of von Hippel-Lindau disease

Von Hippel-Lindau (VHL) disease is an autosomal dominant syndrome characterized by germline mutations in the VHL tumor suppressor gene located at chromosome 3p25-26 and pleomorphic clinical picture. The major clinical manifestations include retinal angiomas, central nervous system hemangioblastomas, pheopleochromocytoma, pancreatic cysts, epididymal cystoadenomas and renal lesions. Recently, we observed a 58-year-old male patient with macrohematuria and a history of nephrectomy due to renal cell carcinoma (RCC). The patient showed retinal angiomatosis, cerebellar hemangioblastomas, multiple pancreatic cysts, right kidney with polycystic features plus two RCC. The patient's offspring, two females and one male, showed VHL lesions, such as retinal angiomatosis, cerebellar hemangioblastomas and polycystic kidney disease (PKD). The affected family members were screened for mutations in the VHL gene. Data suggested the presence of a deletion encompassing exon 1 of the VHL gene. Early diagnosis of VHL disease in patients and their relatives is important for clinical and geneticreasons. VHL disease patients have an increased incidence of malignant carcinomas and the syndrome can mimic the presentation of other cystic kidney diseases. Early diagnosis and molecular genetic testing of family members is essential to improve the clinical management of patients and to allow an accurate risk assessment in asymptomatic individuals. In conclusion, nephrologists and urologists must carefully evaluate patients with PKD and RCC to confirm or exclude VHL disease, and physicians must play a crucial role in the clinical process of therapeutical decisions and choices for VHL patients.     

多房性囊性肾癌一例报告及文献复习

目的 提高多房性囊性肾癌的诊治水平.方法多房性囊性肾癌患者1例,男,49岁.体检B超偶然发现右肾下极多囊性肿物1个月.囊内无回声区,其间混杂低回声区;CT检查右肾下极见多囊性低密度病变,边界清楚、光整,内见薄壁分隔,增强扫描分隔可见强化;MRI检查示右肾下极多囊性病变,增强扫描囊壁可见强化.行右肾部分切除术,完整切除肿瘤.结果 病理报告:肾被膜下见多房状肿物,大小约3.0 cm×2.0 cm×2.0 cm,囊壁光滑,腔内含清亮的浆液性及血性液体,囊壁厚约0.1～0.2 cm,与肾周脂肪粘连.镜下多数囊腔内衬单层或复层立方状透明细胞,细胞核小而圆,位于细胞中央,无明显核仁;有的囊腔内衬扁平上皮细胞或无内衬上皮,偶见由透明细胞覆盖的小乳头;囊腔间隔由胶原纤维组成,部分间隔内可见灶状透明细胞,但未形成肉眼可见的结节.病理诊断:多房性囊性肾癌.术后随访20个月未见复发和转移.结论 多房性囊性肾癌是肾癌的一种罕见亚型,发病率低,是一种完全由囊腔构成的肿瘤;影像学检查可提供直接依据,确诊需依靠病理学检查;外科手术治疗预后良好.     

von Hippel-Lindau病肾癌的自然病程研究

目的 探讨von Hippel-Lindau(VHL)病肾癌的自然病程. 方法 初诊后未立即手术的VHL病肾癌患者11例,男5例,女6例,平均年龄45岁.共32个肿瘤,肿瘤直径平均2.5(0.5～6.2)cm.采用积极监测策略对其进行临床管理.对患者临床资料、随访结果及肿瘤生长速度进行分析. 结果 中位随访时间70(32～258)个月.双肾实性占位6例;行手术治疗8例,包括保留肾单位手术7次,根治性肾切除6次;肿瘤复发4例.死于肿瘤转移2例,死于非肿瘤因素1例.32个肿瘤中位随访时间51(19～106)个月.肿瘤平均生长速度0.55 cm/年;直径＞3 cm、≤3 cm肿瘤生长速度分别为0.55、0.44 cm/年,差异无统计学意义(P=0.40).肿瘤生长快3个(1.13～1.75cm/年),肿瘤生长慢29个(0.17～0.88 cm/年).肿瘤进展转移2例.随访结束时,肿瘤＞3 cm者27个(84%),直径≤4 cm肿瘤未发现转移.结论 多数VHL病肾癌生长缓慢,不发生转移;肿瘤直径≤4 cm者很少转移;对≤4 cm VHL病肾癌采用积极监测策略可行.     

A case of pheochromocytoma associated with incidental multiple renal cell carcinoma originating from acquired cystic disease of the kidney in hemodialysis

The patient was a 52-year-old woman maintained on peritoneal dialysis for 9 years and on hemodialysis for 7 years. This patient complained of palpitations and hypertensive attack during the hemodialysis. Computed tomography and magnetic resonance imaging demonstrated the presence of right adrenal tumor(1.5 x 1.5 cm) and bilateral multiple renal cysts. Right adrenal pheochromocytoma was confirmed by elevated serum level of catecholamines and diagnosed by 131I-MIBG scintigram. This patient underwent right adrenalectomy and nephrectomy in consideration of future generation of cancer in the acquired cystic kidney. Histological examination confirmed malignant pheochromocytoma and small multiple renal cell carcinomas. The patient was alive without any recurrence 6 months postoperatively. To our knowledge, this is the fifth case of adrenal pheochromocytoma and renal cell carcinoma associated ACDK in a hemodialysis patient in the Japanese literature.     

A case of inflammatory myofibroblastic tumor recognized in the retroperitoneum and the kidney

A 61-year-old man consulted his local physician because of abdominal pain and was referred to our hospital after bilateral renal tumors were suspected on CT. CT and MRI showed a tumor, approximately 3 cm in diameter in the right renal pelvis and a cyst in the left kidney. There were no malignant cells detected on cytological studies of two urine specimens that were voided commonly and obtained from the right renal pelvis, respectively. Under a diagnosis of right renal pelvic tumor, the patient underwent laparoscopic right nephroureterectomy. Pathological examination demonstrated two inflammatory myofibroblastic tumors; one was located in the upper portion of the right kidney and the other was in the fatty tissue of the right renal hilus. There has not been any evidence of recurrence detected on follow-up for three months postoperatively.     

原发性肾窦肿瘤三例报告

目的 总结原发性肾窦肿瘤的临床特点及治疗方法.方法 原发性肾窦肿瘤患者3例.女2例,男1例.平均年龄40(33～55)岁.临床表现为腰痛伴血尿1例,单纯腰部疼痛2例.肿瘤位于右侧2例,左侧1例.直径平均5.5(3.5～8.5)cm.3例均经行手术治疗. 结果行右肾窦肿瘤探查术1例,术中病理提示良性肿瘤,单纯切除肿瘤,病理报告为血管平滑肌瘤,随访3年未见复发.1例术中因肿瘤出血及肾盂破坏严重,行右肾切除,病理报告为血管平滑肌脂肪瘤,随访10个月未见复发.1例因肿瘤包绕肾动静脉,局部严重黏连,行左肾切除术,病理报告为脂肪瘤,随访4年未见肿瘤复发.结论 原发性肾窦肿瘤临床罕见,多为良性,易误诊为肾盂肿瘤,CT、MRI、IVU检查有助于鉴别;良性肿瘤直径<4 cm且无症状者可密切随访,有症状或直径>4 cm者可行肿瘤切除;肿瘤累及肾蒂并严重破坏肾盂者可行单纯肾切除;恶性肿瘤应行根治性肾切除.     

Single case of renal cell carcinoma and endocrine pancreatic head cancer occurring with von Hippel-Lindau disease

Von Hippel-Lindau (VHL) disease is an autosomal dominant genetic disease in which various neoplastic lesions occur in multiple organs. Reported here is a case of VHL disease with concurrent renal cell carcinoma and endocrine pancreatic cancer. The patient was a 43-year old woman. On this occasion, the patient had sought treatment from her local physician, complaining chiefly of yellowing of the skin and bulbar conjunctiva. Abdominal ultrasound and computed tomography scans revealed a mass in the right kidney and a mass in the pancreatic head. Peripheral blood genetic analysis revealed an Arg/stop heteroconjugative mutation in codon 113 in exon 1 of the VHL gene on the short arm of chromosome 3 (p25–26). After various tests were performed, the patient was diagnosed with right renal cell carcinoma, malignant tumor of the pancreatic head, and multiple pancreatic cysts accompanying von Hippel-Lindau disease. Right nephrectomy and pancreatoduodenectomy were performed. Based on the histopathological results, the patient was diagnosed with right renal cell carcinoma and highly differentiated endocrine pancreatic cancer. Immunohistologically, a large number of atypical cells were found to be positive for both anti-chromogranin and anti-synaptophysin antibodies in the endocrine tumor. Immunostaining for each type of gut hormone was also performed, but all results were negative. Based on the above findings, nonfunctioning, highly differentiating endocrine cancer was diagnosed. This is the first confirmed case of renal cell carcinoma and endocrine pancreatic cancer occurring concurrently with VHL. This is an important case, so it is presented here along with a short discussion of the literature.     

Clear cell renal carcinoma presenting as a bleeding cyst in pregnancy: inaugural manifestation of a von Hippel-Lindau disease

Von Hippel-Lindau (VHL) disease is a dominant autosomal disorder inducing the development of many tumors, such as hemangioblastomas in the central nervous system and retina, cysts or tumors (benign or malignant) in the kidneys and/or the pancreas. We report the case of a pregnant woman who presented with a voluminous hemorrhagic cyst of the right kidney with an exophytic lesion detected in the lower median part of the cyst wall. As an anamnestic inquiry resulted in a familial history of VHL disease, a screening imaging was performed and detected three medullary hemangioblastomas. Considering the active bleeding of the renal cyst and its potential malignancy, a unilateral nephrectomy was carried out after pregnancy interruption. Histological analysis confirmed a multilocular clear cell renal carcinoma. This case underlines the importance of screening procedures such as abdominal ultrasonography and medullary magnetic resonance imaging in all pregnant women with a familial history of VHL disease.     

Renal cell carcinomas arising from the allograft and bilateral native kidneys

Abstract:   We present the case of a young lady who developed renal cell carcinomas (RCC) in the allograft and bilateral native kidneys metachronously within one year. She received a living donor kidney transplantation from her father. A solid tumor of 4 cm in diameter was uncovered first in the allograft kidney 103 months after transplantation, and was treated with graftectomy. Six months after graftectomy, a right renal tumor measuring 3.5 cm and left renal tumors emerged in the native kidneys. She underwent laparoscopic right and left radical nephrectomy in separate sessions. The pathological diagnosis in the allograft and right renal tumors was clear cell RCC with eosinophilic cytoplasm and that in the left kidney was clear cell carcinoma. Fluorescence in situ hybridization and human leukocyte antigen typing showed that each tumor was most probably primary disease. She was free of disease 18 months postoperatively. This is the first report on RCC arising both in the allograft and bilateral native kidneys.