A Rare Case of Synchronous Fumarate Hydratase-Deficient Renal Cell Carcinoma and Clear Cell Renal Cell Carcinoma With Fumarate Hydratase and von Hippel-Lindau Gene Mutations: A Clinicopathologic and Molecular Study.

Fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) is a rare and aggressive tumor characterized by pathogenic alterations in the fumarate hydratase (FH) gene. Clear cell renal cell carcinoma (clear cell RCC) is a common renal cell carcinoma (RCC) associated with von Hippel-Lindau (VHL) gene variations. Here, we reported a case of bilateral RCCs. A 60-year-old man was admitted to hospital with a 3.6 cm × 3.3 cm mass in the right kidney and a 2.8 cm × 2.3 cm nodule in the left kidney. Pathologically, the right tumor showed a nested growth pattern of cells with clear cytoplasm and was FH positive and 2-succinylcysteine (2SC) negative. The left tumor demonstrated a high-grade papillary pattern and was FH negative and 2SC positive. Whole-exome sequencing and Sanger sequencing identified a germline FH c.563A > T mutation in both the tumors and an additional somatic VHL c.479_480insA mutation in the right tumor, confirming the diagnosis of clear cell RCC and FH-deficient RCC in the right and left kidneys, respectively. We reported a rare case of synchronous bilateral clear cell RCC (right) and FH-deficient RCC (left) likely driven by somatic VHL mutation and germline FH mutation, respectively.

Retroperitoneal lipoma and bilateral renal cell carcinoma in a rare co-existence.

INTRODUCTION: Giant retroperitoneal lipomas are rarely observed clinically, and a retroperitoneal lipoma accompanied by renal cell carcinoma is even more unusual. We present a case of a large retroperitoneal lipoma with bilateral renal cell carcinoma that was definitively diagnosed after resection. CASE PRESENTATION: A huge retroperitoneal mass was incidentally discovered in a 58-year-old male with end stage renal disease being evaluated for a kidney transplant. Imaging studies revealed a mixed solid and fat-containing mass displacing the left kidney. Repeat imaging discovered concurrent unilateral renal cell carcinoma and interval enlargement of the mass. Histopathology showed benign adipose tissue, bland spindle cells, and mixed inflammatory infiltrate that was negative for MDM2 amplification. Resection of the mass and bilateral nephrectomy was performed. Final histopathological examination was consistent with bilateral renal cell carcinoma and a large benign retroperitoneal lipoma. DISCUSSION: The presented case provides a prime example of the diagnostic challenges encountered with retroperitoneal tumors, The final diagnosis of lipoma in this case was only made after review of the resected specimen in its entirety. Retroperitoneal lipomas can present differently based on tumor size and involvement of adjacent organs. The concomitant occurrence of a renal cell carcinoma and retroperitoneal lipoma is extremely rare, and this is the first report published in the literature. CONCLUSION: We document a rare case of retroperitoneal lipoma with concurrent bilateral renal cell carcinoma and illustrate frequently encountered challenges during the evaluation of retroperitoneal masses.

[Bilateral papillary renal cell carcinoma following kidney transplantation: A case report].

With the continuous development of kidney transplantation technique, the survival time after kidney transplantation is gradually prolonged. Thus, the malignant tumor has been the important influencing factor on the long-term survival for kidney transplantation patients. Renal cell carcinoma is a relatively common tumor after kidney transplantation. Besides, clear cell renal cell carcinoma and papillary renal cell carcinoma are the relatively common pathological types for renal cell carcinoma following kidney transplantation. However, bilateral renal cell carcinoma following kidney transplantation is comparatively rare. In this article, we presented a case of bilateral papillary renal cell carcinoma, which occurred after kidney transplantation. And the diagnosis and treatment were introduced in detail. The patient was 37 years old, and he underwent kidney transplantation 13 years ago in our hospital, because of kidney failure. After kidney transplantation, he had regular medical check-up every year. In this year, his urological ultrasound results indicated bilateral renal tumors. And then, he received abdominal and pelvic computed tomography, and the result also showed bilateral renal tumors, which were likely to be malignant tumors. After adequate consultation, the patient chose surgical treatment. The patient received long-term immunosuppressive therapy, because of kidney transplantation. Considering this, the surgeon decided to choose a staging surgical treatment, in order to reduce the bad influence of one-stage surgery. Then, the patient first underwent retroperitoneal laparoscopic radical nephrectomy for right renal tumor in our hospital, and he had no complications after operation. The pathological results showed papillary renal cell carcinoma. He was discharged successfully. He underwent retroperitoneal laparoscopic radical nephrectomy for left renal tumor in our hospital one month later, and he had no complications after operation. The pathological results also showed papillary renal cell carcinoma. He was discharged successfully two days after surgery. In the 3-month follow-up, the patient was recovering well. To sum up, the incidence of bilateral renal cell carcinoma following kidney transplantation is relatively rare, and bilateral radical nephrectomy is effective and safe treatment. Above all, it is the patient's condition that determines the choice of staging surgery or simultaneous surgery.

Bilateral papillary renal cell carcinoma following kidney transplantation: A case report / 北京大学学报(医学版)

With the continuous development of kidney transplantation technique, the survival time after kidney transplantation is gradually prolonged. Thus, the malignant tumor has been the important influencing factor on the long-term survival for kidney transplantation patients. Renal cell carcinoma is a relatively common tumor after kidney transplantation. Besides, clear cell renal cell carcinoma and papillary renal cell carcinoma are the relatively common pathological types for renal cell carcinoma following kidney transplantation. However, bilateral renal cell carcinoma following kidney transplantation is comparatively rare. In this article, we presented a case of bilateral papillary renal cell carcinoma, which occurred after kidney transplantation. And the diagnosis and treatment were introduced in detail. The patient was 37 years old, and he underwent kidney transplantation 13 years ago in our hospital, because of kidney failure. After kidney transplantation, he had regular medical check-up every year. In this year, his urological ultrasound results indicated bilateral renal tumors. And then, he received abdominal and pelvic computed tomography, and the result also showed bilateral renal tumors, which were likely to be malignant tumors. After adequate consultation, the patient chose surgical treatment. The patient received long-term immunosuppressive therapy, because of kidney transplantation. Considering this, the surgeon decided to choose a staging surgical treatment, in order to reduce the bad influence of one-stage surgery. Then, the patient first underwent retroperitoneal laparoscopic radical nephrectomy for right renal tumor in our hospital, and he had no complications after operation. The pathological results showed papillary renal cell carcinoma. He was discharged successfully. He underwent retroperitoneal laparoscopic radical nephrectomy for left renal tumor in our hospital one month later, and he had no complications after operation. The pathological results also showed papillary renal cell carcinoma. He was discharged successfully two days after surgery. In the 3-month follow-up, the patient was recovering well. To sum up, the incidence of bilateral renal cell carcinoma following kidney transplantation is relatively rare, and bilateral radical nephrectomy is effective and safe treatment. Above all, it is the patient's condition that determines the choice of staging surgery or simultaneous surgery.

Non-Familial Synchronous Bilateral Renal Cell Carcinoma with Bilateral Synchronous Renal Vein Extension and Inferior Vena Cava Thrombus.

Bilateral renal cell carcinoma with tumor thrombus extension into the renal vein and/or inferior vena cava - clinical stage T3a+ - is rare. The majority of these cases arise due to a genetic predisposition. We present a case report of a 47-year-old male with bilateral, synchronous renal cell carcinoma with bilateral renal vein and inferior vena cava tumor thrombi with no identifiable familial predisposition.

A case of bilateral renal cell carcinoma associated with long-term dialysis showing false-positive immunoreactivity for TFE3 as Xp11 translocation renal cell carcinoma.

Renal carcinomas associated with Xp11.2 translocations/transcription factor 3 (TFE3) gene fusion (Xp11 translocation RCC) are a rare subtype of renal cell carcinoma. A middle-aged Japanese man, who had a medical history of dialysis for more than 12 years, had bilateral renal cancers with a background of acquired cystic disease of the kidney and remarkable deposition of calcium oxalate in the tumorous area. The right renal tumor showed papillary architecture of clear cells with diffuse and strong immunoreactivity for TFE3 and focal and weak positivity for cathepsin K, suggesting a possibility of Xp11 translocation RCC. However, RT-PCR failed to detect any type of the reported fusion genes involving TFE3. Thus, the sample was sent for a TFE3 break-apart FISH assay in a renal tumor consultation service, which reported no evidence of TFE3 gene rearrangement. The right renal tumor was finally diagnosed as papillary renal cell carcinoma with cystic change. We report here a case of bilateral renal cell carcinoma in a patient undergoing long-term dialysis, which showed false-positive immunoreactivity for TFE3 immunostaining. Titration of TFE3 immunohistochemical staining (IHC) should be performed and cross-referenced with the FISH or RT-PCR results to avoid the misinterpretation of TFE3 IHC results.

A Case of Unrecognized Bilateral Renal Cell Carcinoma / 대한비뇨기과학회지

Renal cell carcinoma is a relatively rare tumor, accounting for approximately 3 percent of adult malignancies. Bilateral renal cell carcinoma, either synchronous or asynchronous, very rarely occurs and makes another urological dilemma. We experienced a case of unrecognized bilateral renal cell carcinoma ; which had been treated by radical nephrectomy after diagnosed as a left renal cell carcinoma, Robson stage IIIa, about 18 months later another mass was found in remaining kidney. Therefore, we performed wedge resection of the mass, and it was also diagnosed as a renal cell carcinoma. Reviewing of the initial abdomen-pelvis CF scan, mass in Rt. kidney had already been existed when left. renal cell carcinoma was diagnosed. This case was finally diagnosed with unrecognized bilateral synchronous renal cell carcinoma.

The Clinical Experience With Bilateral Renal Cell Carcinoma / 대한비뇨기과학회지

Bilateral renal cell carcinoma is very rare. In the past, bilateral renal cell carcinoma indicated a poor prognosis. But recently, aggressive operative intervention with nephron salvaging procedure can improve the survival of bilateral renal cell carcinoma. The conservative treatment of bilatera1 renal cell carcinoma depends on the size, the location and the stage of the tumor. Of several conservative surgical procedures, either enucleation or extracorporeal partial nephrectomy with autotransplantation were performed followed by radical nephrectomy in our cases. The advantages of an extracorporeal approach in such case include an optimum exposure, a bloodless surgical field, an ability to perform a more precise operation with maximum conservation of renal parenchyma, and a greater protection of the kidney from prolonged ischemia. Although this technique involves increased operative time with greater potential morbidity, we performed this procedure without much difficulties with the surgical skills culminated from renal transplantation.