Anticancer effect of sirolimus in renal allograft recipients with de novo malignancies

The inhibition of mTOR is a target for anticancer drugs in posttransplant malignancies. The influence of conversion to sirolimus after malignancy diagnosis was investigated on patient and renal allograft survivals. The 20 renal allograft recipients (4 women, 16 men) of ages 26 to 73 years (mean, 59 years) developed malignancies within 6 to 172 months (mean, 53 months) after transplantation. Three patients developed posttransplant lymphoproliferative disease (PTLD); four, Kaposi sarcoma, three, lung cancer; two, malignant melanoma; two, breast cancer; two, renal cell carcinoma; one, Merkel cell carcinoma; one, cutaneous T-cell lymphoma; one, larynx cancer; and one, gingival cancer. After tumor diagnosis, calcineurin inhibitors, azathioprine, or mycophenolate mofetil (MMF) were discontinued abruptly and sirolimus introduced (2 mg/d; target trough level, 4.0 to 8.0 ng/mL). Prednisone was maintained. The observation time of sirolimus therapy was 4 to 48 months (mean, 14 months). Two patients with PTLD (large B-cell lymphoma) and four with Kaposi sarcoma had full regressions. Eleven patients (larynx cancer, melanoma, breast cancer, T-cell lymphoma, renal cell carcinoma, Merkel cell carcinoma, and skin lymphoma) in addition to sirolimus therapy, underwent oncologic treatment, namely, surgery and/or chemotherapy. Six patients died from disseminated malignancy 4 to 9 months after conversion. One patient with T-cell lymphoma lost his graft; in the remaining patients, serum creatinine level was stable. In conclusion, Conversion to sirolimus resulted in regression of large B-cell lymphoma and Kaposi sarcoma. In patients with advanced or disseminated malignancy, the tumors progressed. Graft function was preserved after conversion to sirolimus.     

Carcinomas of the urinary bladder simulating malignant lymphoma. A report of five cases

We report five carcinomas of the urinary bladder, four of them transitional cell carcinomas and one undifferentiated carcinoma, with unusual features that have received little or no comment in the literature and may be the cause of diagnostic difficulty because of their possible confusion with malignant lymphoma. Four patients were male and one female. They ranged from 61 to 76 years of age. Three tumors from these patients had a prominent (2 cases) or massive (1 case) lymphoid infiltrate that partially obscured the invasive carcinoma in two cases and largely obscured it in the third case, which closely resembled a lymphoepithelioma. The diagnosis of malignant lymphoma was only excluded with confidence in the last case after thorough immunohistochemical study. The lymphoid infiltrate was composed of numerous T-cells (UCHL-1 and Leu 22 positive) and polytypic plasma cells with admixed eosinophils; occasional germinal centers were present in one case. The tumors were deeply invasive in two patients, one of whom is alive with no evidence of disease 4 years after treatment with chemotherapy and radiation therapy; the other two cases are too recent for meaningful follow-up. Two other transitional cell carcinomas had diffuse patterns that simulated lymphoma or plasmacytoma. Recognition of these patterns of vesical carcinoma is important in order to avoid the misdiagnosis of the very rare malignant lymphoma of the urinary bladder.     

Adrenal tumors associated with renal cell carcinoma

We experienced two cases with renal cell carcinoma who showed enlargement of the contralateral adrenal gland. In case 1, the enlarged adrenal gland was a non-functioning adrenal adenoma, and in case 2, it was a metastatic adrenal tumor. Non-functioning adrenal adenomas are benign tumors of the adrenal cortex often found incidentally at autopsy or on computed tomography (CT) studies of the upper abdomen. Adrenal adenomas have been reported to occur in 9.5-14% of patients with renal cell carcinoma, while the incidence being 1.5-8.7% in the general population. Since metastases of renal cell carcinoma to the adrenal glands are not uncommon, it is important to distinguish between non-functioning adrenal adenomas and metastatic tumors. Therefore, we studied the incidence of adrenal tumors (including metastatic tumors and benign tumors) in patients with renal cell carcinoma who underwent the abdominal CT study in our hospital. From 1982 to 1989, CT had been performed in 67 patients with renal cell carcinoma. Six adrenal masses were identified with CT in these patients. Three out of the 6 patients probably had metastatic tumors and one of the 3 adrenal masses was pathologically diagnosed as metastatic tumor. The other three masses were benign on pathology and only one of them was pathologically diagnosed as non-functioning adrenal adenoma. The incidence of adrenal adenomas on the CT study was lower than that of previous studies based on autopsy. The reasons of this difference in results between their studies and ours are not clear.(ABSTRACT TRUNCATED AT 250 WORDS)     

Worth a second look: outcomes of patients with initial finding of regular renal tissue in CT-guided renal tumor biopsies

Introduction and objective

Renal tumor biopsy is recommended for histological diagnosis of radiologically indeterminate renal masses, to select patients with small-renal masses for surveillance approaches, before ablative treatments and to confirm metastatic spread of renal cell cancer (RCC), according to the EAU guidelines. We aimed to determine outcomes of patients with suspicious renal masses with initial finding of regular renal tissue in renal tumor biopsies.

Methods

Retrospective database analysis of 101 patients undergoing CT-guided-, percutaneous renal tumor biopsies in local anesthesia.

Results

In 23/101 patients, histopathologic evaluation of the biopsies showed regular renal tissue. Of these, two patients underwent simultaneous radiofrequency ablation (RFA), 2/23 underwent radical nephrectomy, despite negative biopsy because of radiological suspicious aspect. Overall, 12 patients underwent a second set of biopsies due to persistent clinical suspicion. Of these, five were diagnosed with RCC: three clear cell renal cell carcinoma (ccRCC) and two papillary renal cell carcinoma (pRCC). Benign tumours were found in two patients. A lymphoma was found in two patients. In 3/12 patients, also the second set of biopsies showed regular renal tissue.

Conclusion

An unsuspicious histology in CT-guided renal tumor biopsy does not preclude patients with suspicious renal masses from being diagnosed with malignancies.

     

Most nasal/nasopharyngeal lymphomas are peripheral T-cell neoplasms

Eleven consecutive cases of nasal/nasopharyngeal lymphoma were studied histologically and with a panel of monoclonal antibodies. The disease showed a male predominance and occurred over a wide age range, with a median of 52 years. Five patients had midfacial destructive disease, and six had gross tumor masses involving the nose/nasopharynx. Three cases were classified as small cleaved cell, two cases as mixed cell, five cases as large cell, and one case as immunoblastic lymphoma. Those belonging to the first two categories also satisfied the diagnostic criteria of so-called polymorphic reticulosis. Epithelial invasion, angioinvasion, and coagulative necrosis were demonstrated in seven, eight, and nine cases respectively. Immunohistochemical studies confirmed that the atypical cells of all 11 cases exhibited T-cell markers. All but one case had lost one or more T-cell markers associated with peripheral T cells, particularly T1 and T3. Loss of T-cell markers in the five cases histologically consistent with polymorphic reticulosis provided further support to indicate that the lesion was neoplastic. A significant proportion of cases also expressed the activated T-cell markers 12 (HLA-DR) and interleukin-2 receptor (IL-2R1).     

肾盂鳞状细胞癌临床特点分析

目的 探讨肾盂鳞状细胞癌的诊治特点.方法 回顾性分析1991年10月至2009年5月收治8例肾盂鳞状细胞癌患者资料.临床表现血尿8例,腰痛7例,腹部包块1例.B超检查8例,IVU检查8例,CT检查4例.术前诊断为肿瘤3例,诊断为肾结石5例,结石术中发现肿瘤并经冰冻病理确诊2例.8例患者均经手术治疗,行根治性肾输尿管切除4例、单纯性肾切除3例、姑息性切除术1例.结果 8例病理诊断均为鳞状细胞癌.中分化6例,高分化和低分化各1例;pT1 1例,pT2 1例,pT3 3例,pT4 3例;淋巴结转移2例.获随访7例,失访1例.术后生存时间2～42个月,中位时间6个月,患者均死于肿瘤复发及转移.结论 肾盂鳞状细胞癌恶性程度高,常合并结石,术前诊断困难,确诊时多为中晚期,术后短期内易复发转移,预后极差.

Abstract：

Objective To review the diagnosis and treatment of squamous cell carcinoma of renal pelvis. Methods The clinical data from October 1991 to May 2009 of eight cases of squamous cell carcinoma of renal pelvis were reviewed and analyzed retrospectively. The symptoms of the patients were hematuria (eight cases), pain (seven cases) and abdominal mass (one case). All patients underwent B-ultrasound and IVU examination and four cases underwent CT scan. Three cases were diagnosed as having a tumor before surgery. Five cases were diagnosed as renal calculus, two of the five cases were diagnosed by intraoperative frozen section. Radical nephroureterectomy were performed in four cases, nephrectomy in three cases and palliative resection in one case. Results Histological classification revealed that six cases were moderately differentiated, one case was well differentiated and one case was poorly differentiated. Two cases had stage pT1/pT2 and six cases had stage pT3/pT4. 2 cases had regional lymph nodes metastasis. Seven cases were followed-up. All patients died of tumor recurrence or metastasis. The median tumor specific survive time was six months (range from two months to 42 months). Conclusions Squamous cell carcinoma of renal pelvis is often occurs concurrently with urolithiasis which could lead to difficulty in diagnose before operation. As the most of the patients were diagnosed with advanced stage disease, squamous cell carcinoma of renal pelvis tended to early recurrence and metastasis and the prognosis was very poor.     

COINCIDENT RENAL CELL CARCINOMA AND NONHODGKIN'S LYMPHOMA: THE M.D. ANDERSON EXPERIENCE AND REVIEW OF THE LITERATURE

Purpose

We recently observed nonHodgkin's lymphoma and renal cell carcinoma occurring simultaneously in several patients. We determine whether the incidence of co-occurrence of the 2 malignancies is greater than expected based on the incidence of each disease in the general population.

Materials and Methods

Patients diagnosed with simultaneous renal cell carcinoma and nonHodgkin's lymphoma were identified through the medical informatics data base at our cancer center. Charts of all patients were reviewed to collect detailed demographic information and confirm both diagnoses. United States population based cancer statistics (Surveillance, Epidemiology and End Results Program data) were used to arrive at the expected age adjusted incidence of co-occurrence of these diagnoses, and statistical analysis was performed to ascertain any differences between expected and observed incidences.

Results

We identified 41 cases with both diagnoses between 1954 and 1995, including 21 diagnosed after 1987. The latter group was used for statistical analysis. The observed rates of renal cell carcinoma developing in the nonHodgkin's lymphoma population (1.86) and nonHodgkin's lymphoma developing in the renal cell carcinoma population (2.67) were greater than expected and both reached statistical significance.

Conclusions

There is a higher than expected incidence of co-occurrence of renal cell carcinoma and nonHodgkin's lymphoma. The cause remains speculative.     

Skeletal Muscle Metastasis from a Conventional Renal Cell Carcinoma,Two Years after Nephrectomy: a Case Report

In this paper, we describe a case of skeletal muscle metastasis from a conventional (clear cell) renal cell carcinoma, two years after nephrectomy. Our first clinical tentative diagnosis was soft tissue tumour, showing the mimicking capacity of renal cell carcinoma. A review of literature shows that skeletal muscle metastases from renal cell carcinoma are extremely rare: only 16 cases have been described. In 5/16, the muscle mass was the initial manifestation of the renal tumour. In the other 11/16, the muscle masses were metachronous (10 months-16 years). We conclude that, when confronted with a patient with a muscle mass and a history of renal cell carcinoma, one should always keep in mind the possibility of a (late) renal cell carcinoma metastasis.     

Skeletal muscle metastasis from a conventional renal cell carcinoma,two years after nephrectomy: a case report

In this paper, we describe a case of skeletal muscle metastasis from a conventional (clear cell) renal cell carcinoma, two years after nephrectomy. Our first clinical tentative diagnosis was soft tissue tumour, showing the mimicking capacity of renal cell carcinoma. A review of literature shows that skeletal muscle metastases from renal cell carcinoma are extremely rare: only 16 cases have been described. In 5/16, the muscle mass was the initial manifestation of the renal tumour. In the other 11/16, the muscle masses were metachronous (10 months-16 years). We conclude that, when confronted with a patient with a muscle mass and a history of renal cell carcinoma, one should always keep in mind the possibility of a (late) renal cell carcinoma metastasis.     

老年患者双肾肿瘤的半保守治疗

目的:评价老年双肾肿瘤半保守治疗安全性。方法:对6例老年双肾肿瘤患者的临床资料进行回顾性分析。6例共12枚肿瘤,每例患者肿瘤均一大一小,影像学均高度怀疑肾癌。大肿瘤直径4.5~10.0cm,平均7.0cm均行根治性切除;小肿瘤直径1.8~2.7cm,平均2.3cm,予保守治疗。结果:手术均未发生严重的并发症,6例大肿瘤均为肾细胞癌:5枚混合细胞癌(以透明细胞为主型),分级Ⅱ级4枚、Ⅰ级1枚;1枚嫌色细胞癌,分级Ⅰ级。随访12~45个月,平均34个月,小肿瘤体积平均每年增大2.96(1.15~8.66)cm3,无局部浸润征象。手术侧未见复发,亦未见远处转移。结论:老年双肾肿瘤(一侧大肿瘤一侧小肿瘤)患者半保守治疗安全、有效。     

Surgical enucleation for renal cell carcinoma. A case report

We present a case of bilateral synchronous renal cell carcinoma treated with surgical enucleation. Urological consultation was asked to evaluate masses of the kidneys, which were detected during a diagnostic imaging on a 58-year-old man with hepatic disorder. Excretory urograms showed definite bilateral upper pole renal masses. Bilateral selective renal angiograms disclosed neovascularity in the small masses. Surgical enucleation of the left renal tumor by a flank approach was performed. The surgical specimen being 3.5 by 2.5 by 2 cm (29 g) was pseudoencapsulated and was identified renal cell carcinoma of the clear cell type, grade 1. INF alpha, pathologically. Seven weeks after, a pseudoencapsulated tumor of 3 by 2.5 by 2 cm (16 g) in the right upper pole was removed by simple enucleation. The pathological diagnosis was renal cell carcinoma of clear cell type, grade 2, INF alpha. The patient is well without evidence of recurrent or any residual disease at 21 months after the second operation. Renal function remains with in normal limits (CCr 110 ml/min). Of our collected cases of bilateral synchronous renal carcinomas treated by bilateral conservative surgery, clinical data are available for 11. Including our case, a total of 12 cases are reviewed.     

An evaluation of Bosniak's radiological classification of cystic renal masses

OBJECTIVE: To determine the clinical usefulness of Bosniak's classification of cystic renal masses, the differentiation of which remains difficult despite significant advances in diagnostic imaging. PATIENTS AND METHODS: The computed tomography (CT) findings of all histopathologically examined cystic renal masses diagnosed at our institution were analysed retrospectively; 35 patients with cystic renal masses were treated between 1986 and 1998. Tissues surgically removed were examined pathologically and the final diagnosis compared with the preoperative CT category of Bosniak's classification. RESULTS: The histopathological examined showed cystic renal cell carcinoma in 21 patients, a benign renal cyst in 12, haemangiosarcoma in one and transitional cell carcinoma in one. Most of the 35 masses (26, 74%) were found incidentally during evaluation for an unrelated disease or a routine health check. All 11 masses of Bosniak category I were benign and one category II mass was malignant. All 10 masses of category III and 12 of category IV were malignant. CONCLUSIONS: Bosniak's classification is useful for differentiating category I, III and IV cystic renal masses. There were too few samples to allow meaningful conclusions to be drawn for category II renal masses. It is critical to differentiate between complicated cysts of category II and III because of the major implications for prognosis and clinical management.     

Multiple renal masses in patients with renal cell carcinoma: diagnostic pitfalls and surgical implications.

Additional renal masses were found in the same or contralateral kidney in 41 of 131 patients with renal cell carcinoma. In 35 of the patients the additional renal masses were simple renal cysts situated in the kidney contralateral to the renal cell carcinoma in 16 patients, in the same kidney in 11 and bilaterally in 8 patients. Four patients had bilateral renal cell carcinomas. One patient had polycystic kidney disease as well as renal cell carcinoma and another patient had a benign tumour in the contralateral kidney. In 3 of the 30 patients with additional renal masses in the kidney contralateral to the renal cell carcinoma the radiologic examinations were insufficient to exclude malignancy. Only explorative surgery could establish a true diagnosis of these masses.     

Inflammatory myofibroblastic tumors of the kidney: a clinicopathologic and immunohistochemical study of 12 cases

Inflammatory myofibroblastic tumor is a rare entity composed of spindle cells admixed with variable amounts of extracellular collagen, lymphocytes, and plasma cells. In the genitourinary tract, inflammatory myofibroblastic tumor most commonly occurs in the bladder. Isolated case studies of inflammatory myofibroblastic tumor of the kidney, renal pelvis, and ureter have been previously reported. Our series includes 12 cases of inflammatory myofibroblastic tumor occurring in the renal pelvis (six cases), renal parenchyma (four cases), and immediate perirenal soft tissue (two cases). Clinical presentation included flank pain (two patients), painless gross hematuria (one patient), and ureteropelvic junction stenosis with hydronephrosis (one patient). The remaining eight patients were asymptomatic. All patients underwent nephrectomy. The tumors were characterized by firm white tissue or had a myxoid "gelatinous" appearance. Three histologic patterns were identified in the tumors, including a myxoid vascular pattern, a compact spindle cell pattern, and a hypocellular fibrous pattern. Immunohistochemical and electron microscopic studies supported a myofibroblastic proliferation. All cases were negative for anaplastic lymphoma kinase. Follow-up was available in eight cases and ranged from 1 to 17 years with no evidence of recurrence. Based on this series, renal inflammatory myofibroblastic tumor is a proliferative lesion of myofibroblasts of uncertain pathogenesis with no identified potential for recurrence or metastases.     

Analysis of spinal cord hemangioblastoma in von Hippel-Lindau disease]

Von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary disorder showing various clinical features. We analyzed 6 patients with spinal cord hemangioblastoma associated with VHL disease in four families. All patients had cerebellar hemangioblastomas. Four cases carried retinal hemangioblastoma and 5 cases showed visceral lesions; renal cell carcinoma (2 cases), renal cyst (2 cases), pancreas cyst (2 cases) and paraovarian tumor. In four cases, spinal cord hemangioblastomas were multiple. Ten symptomatic or rapidly growing lesions in 5 patients were surgically resected. Two of these lesions were extramedullary spinal root hemangioblastoma. Operative results were good except for a case of ventrally placed thoracic spinal intramedullary hemangioblastoma who showed paraparesis postoperatively. One patient who suffered from complete paraplegia preoperatively did not recover after surgery. In two patients, renal cell carcinoma was detected and nephrectomy was undergone. It was noteworthy that metastasis of renal cell carcinoma to the hemangioblastoma was histologically proved with anti-cytokeratin immunostaining in two patients with VHL-associated renal cell carcinoma. Molecular genetic analysis showed a missence mutation in one family and possible intragenic deletion in another family. However, two families showed no VHL gene mutation with single strand conformational polymorphism or Southern blot analyses. Spinal cord hemangioblastomas in VHL disease are often multiple and located at various sites and seem to be underestimated. Surveillance should start in childhood and requires annual follow-up with imaging of the central nervous system and abdominal viscera. Presymptomatic diagnosis by gene analysis can be very useful for early detection of this disease.     

囊性肾癌诊治体会

目的 提高囊性肾癌的诊治水平. 方法 回顾分析10例囊性肾癌患者术前影像学特点、病理特征和治疗方法.男7例,女3例.年龄38～74岁,平均56岁.患侧腰酸3例,体检偶然发现7例,有肾囊肿病史者2例.囊腔直径3.5～8.2 cm.术前B超检查诊断为肾癌6例,CT诊断为肾癌7例.8例术中行冰冻病理:肾细胞癌6例,未发现恶性倾向2例.10例均行根治性肾切除术. 结果 术后病理诊断:肾透明细胞癌9例,颗粒细胞癌1例.病理学分型:肾癌囊性坏死6例,多房囊性肾癌2例,肾囊肿恶变型2例.8例随访6个月～5年,6例无瘤存活,2例分别于术后13、20个月死于肿瘤转移. 结论 重视囊性肾癌独特的影像学特点、病理学特征,术中行冰冻病理检查,是提高囊性肾癌诊治水平的关键.     

Renal involvement in lymphoma: prevalence and various patterns of involvement on abdominal CT

Objective The kidney is a frequent site of involvement in lymphoproliferative disorders. The aim of this study was to demonstrate the prevalence and spectrum of morphologic appearances of renal involvement in patients with lymphoma on helical computed tomographic (CT) scan. Methods Three phases of post-contrast helical CT of the abdomen in 74 patients with lymphoma were reviewed for possible renal involvement: the cortico-medullary, nephrographic and delayed excretory phases. Tumor characteristics, patterns of distribution and enhancement features were evaluated. Results Of the 74 patients with lymphoma, 11 had CT evidence of renal involvement—ten with non-Hodgkin’s lymphoma and one with Hodgkin’s lymphoma—representing 15% of all patients scanned for routine staging of histologically diagnosed lymphoma. Five types of renal involvement were observed: enlarged lobular non-enhancing kidneys (four patients); bilateral multiple renal masses (two patients); focal single non-enhancing mass (two patients); perirenal infiltrations from retroperitoneal extension (two patients); bilateral diffuse areas of non-enhancing hypo-densities (one patient). Conclusion Five distinct patterns of renal involvement with lymphoma were detected with helical CT. The most common appearance was enlarged lobular kidneys. CT with intravenous contrast enhancement is currently the approach of choice for both the evaluation of renal involvement as well as for accurate staging of lymphoma. Awareness of different patterns of renal involvement in lymphoma allows proper differentiation from other similar diseases.     

Treatment of oropharyngeal cancer in renal transplant recipients without cessation of immunosuppressive therapy

INTRODUCTION: Renal transplantation and immunosuppression are associated with an increased incidence of malignancy. Reduction or cessation of immunosuppressive therapy has been advocated in these cases to prevent tumor progression and recurrence. We evaluated the outcome of treatment of oropharyngeal cancer (OC) after renal transplantation without cessation of immunosuppressive therapy. METHODS: The database of patients with OC after renal transplantation was analyzed with respect to age, sex, type of immunosuppression, interval between transplantation and diagnosis of cancer, as well as method of treatment and survival. RESULTS: Thirty one (2.06%) renal transplant recipients developed malignancy including 6 (20%) with OC. Lingual cancer was seen in three, and one each showed an isolated tonsillar lymphoma, a parotid carcinoma, or a carcinoma of the larynx with only the last having had two other malignancies in the past. Three subjects were on immunosuppression with azathioprine and prednisolone, and the others were prescribed cyclosporine and prednisolone. Average time from transplantation to diagnosis of OC was 106 months. The interval was the shortest (2 years) for tonsillar lymphoma in an 18-year-old patient who received cyclosporine and showed features of left follicular tonsillitis. The patient with advanced carcinoma of the larynx did not receive any treatment and succumbed within 3 months. The dose of cyclosporine was reduced in the lymphoma case but immunosuppression was not altered in the other patients. All subjects were treated with a standard protocol. During a mean follow-up of 33 months, one had local recurrence of parotid carcinoma and the others showed well functioning renal grafts. CONCLUSION: Comprehensive treatment of OC after renal transplantation without withdrawing the immunosuppression prolonged the life of these patients with functioning grafts.     

Tubulocystic renal carcinoma: a clinical perspective

Introduction

Tubulocystic renal carcinoma (TCRC) is a recently described neoplastic entity. To date, clinicopathological features on less than hundred cases of these rare tumours have been characterized exclusively in the pathological literature. Herein, we present five additional cases emphasizing clinical aspects on these rare renal neoplasms.

Material and method

Cases diagnosed as TCRC were retrieved and reviewed from the routine and consultation files of the Pilsen tumour registry comprising over 20,000 cases of renal tumours.

Results

All patients were men, mean age 56 years (range 29–70). Features on computed tomography (CT) were in two cases Bosniak III, one IV and two were solid tumours. In four patients, nephrectomy was performed, and one patient underwent resection. At the time of surgery, two patients had metastases. In one case, both primary tumour and metastases were active on FDG positron emission tomography (PET)/CT. Both patients with metastatic disease were treated with sunitinib with partial response. One patient died 26 months postoperatively and the other patient is alive 5 months after surgery. Three patients with localized tumours are without evidence of disease 31, 28 and 7 months after surgery. In one case, the resected tumour was histologically combined with a papillary renal cell carcinoma (PRCC).

Conclusion

TCRC occurs predominantly in men with a wide age range. TCRC frequently displays a cystic component which may render a radiological classification of Bosniak III or IV. FDG PET/CT is helpful in the detection of metastases. TCRC has definitive malignant potential. Our findings support a possible relationship to PRCC. The tyrosine kinase inhibitor sunitinib may be used a therapeutical agent with partial response and temporary effect.     

Multifocal renal oncocytoma in a patient with Von Hippel-Lindau mutation

Von Hippel-Lindau disease (VHL) is a rare genetic disease with a lifetime risk of clear cell renal cell carcinoma in approximately 70% of cases. We present a case of a 63-year-old man with bilateral, multifocal renal masses. Genetic testing results were consistent with a VHL deletion. The patient had no other disease manifestations consistent with VHL. The patient underwent staged bilateral nephron-sparing procedures. Pathology of all renal masses revealed oncocytoma. To our knowledge, we describe the first reported case of multiple renal oncocytomas in a male patient with a germline VHL mutation.