Primary Renal Fibrosarcoma with Massive Tumor Size and Inferior Vena Cava Extension Mimicking Renal Cell Carcinoma: A Rare Case Report

Primary renal fibrosarcoma is an exceedingly rare malignant mesenchymal tumor that accounts for only 1-3% of adult renal malignancies, often mistaken for sarcomatoid renal cell carcinoma (RCC) or leiomyosarcoma due to overlapping morphology. Thus, accurate disease diagnosis is crucial for its management. We report a case of a 49-year-old female, a chronic smoker, who presented with right flank pain and progressive abdominal swelling. Clinical examination revealed a firm mass in the right abdomen, and contrast-enhanced computed tomography demonstrated a large exophytic right renal mass with areas of necrosis, infiltration of the psoas muscle, and extension of tumor thrombus into the inferior vena cava (IVC). The patient underwent right radical nephrectomy with IVC thrombectomy, and a 20 × 15 cm irregular renal mass with 2 cm IVC thrombus was excised intraoperatively. Histopathology revealed a high-grade spindle cell neoplasm with interlacing fascicles and herringbone patterns, brisk mitoses (10-12 mitotic figures per 10HPF), and 20% necrosis. Immunohistochemistry showed diffuse vimentin positivity with negative staining for epithelial, myogenic, neural, and renal lineage markers, confirming the diagnosis of high-grade primary renal fibrosarcoma. This case is notable for being the largest renal fibrosarcoma reported to date, with rare IVC extension, features typically associated with advanced RCC rather than fibrosarcoma. Despite aggressive pathology, no metastases were identified, and the patient remained recurrence-free at 6 months postoperatively, with chemotherapy reserved for recurrence or metastasis. This report emphasizes the diagnostic challenges, surgical complexity, and clinical significance of primary renal fibrosarcoma and highlights the importance of including it in the differential diagnosis of large renal masses with vascular involvement.

Oncologic Outcomes Following Surgical Resection of Renal Cell Carcinoma with Inferior Vena Caval Thrombus Extending Above the Hepatic Veins: A Contemporary Multicenter Cohort

A Decade of Robotic-Assisted Radical Nephrectomy with Inferior Vena Cava Thrombectomy: A Systematic Review and Meta-Analysis of Perioperative Outcomes.

Haemodynamic changes during radical nephrectomy with inferior vena cava thrombectomy: A pilot study.

Positron Emission Tomography Enhancing Tumor Thrombus in Patient With Renal Cell Carcinoma

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V11-04 ROBOTIC-ASSISTED LAPAROSCOPIC RIGHT RADICAL NEPHRECTOMY WITH RIGHT IVC THROMBECTOMY AND REPAIR WITH BOVINE GRAFT

Risk factors for acute kidney injury after radical nephrectomy and inferior vena cava thrombectomy for renal cell carcinoma

V10-09 CHALLENGING RENAL CELL CARCINOMA CASES WITH INFERIOR VENA CAVA INVOLVEMENT: FROM EXTIRPATION TO CAVAL REPLACEMENT

Renal cell carcinoma (RCC) presents with inferior vena cava (IVC) thrombus in 10%-30% cases and surgical management forms the mainstay of the treatment. The objective of this study is to assess the outcomes of the patients undergoing radical nephrectomy with IVC thrombectomy. A retrospective analysis of patients undergoing open radical nephrectomy with IVC thrombectomy between 2006 till 2018 was done. A total of 56 patients were included. The mean (±standard deviation) age was 57.1 (±12.2) years. The number of patients with levels I, II, III, and IV thrombus were 4, 29,10, and 13, respectively. The mean blood loss was 1851.8 mL, and the mean operative time was 303.3 minutes. Overall, the complication rate was 51.7%, while the perioperative mortality rate was 8.9%. The mean duration of hospital stay was 10.6 ± 6.4 days. The majority of the patients had clear cell carcinoma (87.5%). There was a significant association between grade and stage of thrombus (P = 0.011). Using Kaplan-Meier survival analysis, the median overall survival (OS) was 75 (95% confidence interval [CI] = 43.5-106.5) months, and the median recurrence-free survival (RFS) was 48 (95% CI = 33.1-62.3) months. Age (P = 0.03), presence of systemic symptoms (P = 0.01), radiological size (P = 0.04), histopathological grade (P = 0.01), level of thrombus (P = 0.04), and invasion of thrombus into IVC wall (P = 0.01) were found to be significant predictors of OS. The management of RCC with IVC thrombus poses a major surgical challenge. Experience of a center along with high-volume and multidisciplinary facility particularly cardiothoracic facility provides better perioperative outcome. Though surgically challenging, it offers good overall-survival and recurrence-free survival.

V05-07 ROBOTIC-ASSISTED LEFT RADICAL NEPHRECTOMY AND IVC THROMBECTOMY IN CROSS-FUSED RENAL ECTOPIA

To evaluate the outcome of patients of renal cell carcinoma (RCC) with inferior vena caval (IVC) thrombus treated by radical nephrectomy and IVC thrombectomy in terms of clinical and pathological factors and prognosis. Sixty-three consecutive patients of RCC with IVC thrombus who underwent radical nephrectomy with IVC thrombectomy between June 1993 and May 2003 were included in this retrospective analysis. Data was analyzed in terms of clinical factors, such as level of thrombus and pathological factors, such as grade, local invasion and N status. Tumor thrombus level was infrahepatic in 35 patients, retrohepatic in 20 and suprahepatic in 8, including 5 with right atrial thrombus. The immediate post-operative mortality was 3% and the incidence of major post-operative complications was 34%, but most of them improved after conservative management except one who needed surgery for burst abdomen. The disease free survival (DFS) was 48.5%, 50.6%, 66.6% and 40% for infrahepatic, retrohepatic, suprahepatic and intra-atrial tumors, respectively. Of the histological types, patients with clear cell tumors had the best prognosis; those with granular cell had the worst prognosis (DFS of 53.5% vs 33.3%, though statistically not significant). Grade-2 tumors had better prognosis than grade-4 tumors (DFS 66.6% vs 0%, P < 0.001). Sixty-eight percent of patients without perinephric fat invasion were free of disease as compared to 31% of those with perinephric fat invasion (P < 0.01). Further, N status showed DFS of 60.9% in patients with negative nodes and 30% in patients with positive nodes (P < 0.05). Though surgery for RCC with IVC thrombus has high morbidity, it can give good results in terms of prolonged DFS in expert hands. Regarding long-term survival, pathological factors, such as local stage and grade, are more important than clinical factors, such as level of thrombus.

Long-term outcomes of nephrectomy and inferior vena cava thrombectomy in patients with advanced renal cell carcinoma: A single-center experience

Background/Purpose: This study evaluated the long-term outcomes of nephrectomy and inferior vena cava (IVC) thrombectomy in patients with advanced renal cell carcinoma (RCC). Methods: From January 1996 to December 2013, we retrospectively collected the clinical data of 31 patients with pathologically confirmed RCC and IVC tumor thrombus. All patients received nephrectomy and IVC thrombectomy. We examined the clinical outcomes, including cancer-specific survival, progression-free survival, and postoperative complications, during a 90-day period. The associated prognostic factors were also analyzed. This study is registered with the National Taiwan University Hospital Research Ethics Committee (number 201305059RINC). Results: The median follow-up period was 24 months. Of 20 patients with nonmetastatic disease, 15 developed metastases during the study period. The overall 5-year cancer-specific survival rate was 30.8% in the nonmetastatic group versus 12.5% in the metastatic group. The level of IVC thrombus (Levels I–II vs. Levels III–IV) was not significantly associated with the 5-year cancer-specific survival (P = 0.43). The Fuhrman grade and sarcomatoid type were statistically significant predictors of cancer-specific survival (Fuhrman Grades III–IV vs. Grades I–II: Hazard ratio [HR] = 0.11, P = 0.04; sarcomatoid type: HR = 0.136, P = 0.001). Fuhrman grade, capsular invasion, and positive surgical margins were associated with 1-year progression-free survival (Fuhrman Grades III–IV vs. Grades I–II: HR = 0.08, P = 0.04; capsular invasion: HR = 0.15, P = 0.04; positive surgical margins: HR = 0.16, P = 0.05). The most common perioperative complication was massive blood loss. The most common recurrence sites were bones, liver, and lungs. Conclusion: The pathologic Fuhrman grade, sarcomatoid type, positive surgical margins, and capsular invasion were significant predictors of oncological outcomes in patients with advanced RCC and IVC thrombus.

ABSTRACTObjective:Vascular involvement in the form of renal vein (RV) or inferior vena cava (IVC) thrombus can be seen in 4-10% of patients presented with RCC. In patients without presence of metastasis, surgical treatment in the form of radical nephrectomy remains the treatment of choice with 5-year survival rates of 45-70%. Open surgery is still the first treatment option of choice at the moment for RCC patients with IVC thrombus.Materials and Methods:In our study, we are reporting a case of patient with RCC and level I IVC thrombus treated with laparoscopy. Our patient is a 72 years old man with underlying co-morbidity of hypertension and chronic kidney disease (CKD) presented with right-sided RCC. The CT scan done showed a large right renal upper pole tumor measuring 8.4x5.2cm with level I IVC thrombus (Figure-1). There were no regional lymphadenopathy and the staging scans were negative.Results:The operative time was 124 minutes and blood loss was minimal. The patient was progressed to diet on POD 1 with bowel movement on POD 2. There was no significant change in the pre and post-operative glomerular filtration rate (GFR). The surgical drain was removed on POD2. The patient was discharged well on POD 5. There were no perioperative complications. The pathology was pT3bN0M0 Fuhrman grade II clear cell RCC.Conclusions:As a conclusion, laparoscopic radical nephrectomy and IVC thrombectomy is a complex and technically demanding surgery. With advancement of surgical skills as well as technology, more cases of minimally invasive laparoscopic radical nephrectomy and IVC thrombectomy can performed to improve the perioperative outcomes of carefully selected patients in a high volume center.