Renal cell carcinoma (RCC) with rhabdoid features is a rare histology and exhibits clinically aggressive behavior. We report a case of a married couple in whom RCC with rhabdoid features concurrently occurred. The rarity of this event suggests that environmental factors may contribute to the etiology of RCC with rhabdoid features.
A 76-year-old Japanese woman was diagnosed with a hypervascular mass in the right kidney and tumor thrombus extending into the right atrium by enhanced computed tomography (CT). She underwent radical nephrectomy and tumor thrombectomy following systemic therapy with the tyrosine kinase inhibitor sunitinib. The histological evaluation denoted clear cell RCC with rhabdoid features. The patient died of cancer 12 months postoperatively. A 76-year-old man, her husband, presented with gross hematuria 2 weeks after his wife had undergone surgery. He had a long history of asbestos exposure. An abdominal CT scan revealed a hypervascular mass in the right kidney and tumor thrombus extending into the inferior vena cava. He also underwent radical nephrectomy and tumor thrombectomy. The histological evaluation also showed clear cell RCC with rhabdoid features. Bone metastasis occurred 12 months postoperatively, but he died of an unrelated cause 18 months after surgery.
Concurrent occurrence of RCC with rhabdoid features may not to be coincidental. Although further studies are warranted, asbestos exposure may contribute to the etiology of clear cell RCC with rhabdoid features.
Renal cell carcinoma; Rhabdoid features; Married couple; Asbestos
The clinical impact of a tumor thrombus in renal cell carcinoma (RCC) patients awaiting radical nephrectomy and thrombectomy is unknown.
To determine the incidence of venous thromboembolism (VTE) in RCC patients with tumor thrombus prior to nephrectomy.
Patients and methods
We conducted a retrospective cohort study including all late-stage (stage 3–4 excluding T1–2 N0M0) RCC patients who underwent radical nephrectomy at our institution between 1 January 2005 and 1 July 2012. Tumor thrombus was defined as the presence of an intraluminal filling defect in the renal vein, hepatic vein, portal vein, or inferior vena cava, directly extending from a renal mass detected on computed tomography.
A total of 176 patients were included in the study. Fifty-three (30.1%) patients had tumor thrombus diagnosed on imaging Three patients with tumor thrombus (5.7%; 95% confidence interval [CI] 1.4–16.8) developed a VTE while awaiting radical nephrectomy, whereas none (0%; 95% CI 0–2.9) of the patients without a tumor thrombus had an event (P = 0.026). All three events were deep vein thrombosis. Times from tumor thrombus diagnosis to VTE were 5, 15 and 21 days.
Tumor thrombus on imaging is a frequent finding among RCC patients awaiting nephrectomy. The presence of tumor thrombus in these patients increases the incidence of preoperative VTE.
neoplasm; nephrectomy; renal cell carcinoma; thrombosis; venous thromboembolism
Management of renal cell carcinoma (RCC) with tumor thrombus extending to the renal vein and inferior vena cava (IVC) is challenging. The aim of this study was to evaluate the benefit of surgical management in such patients.
From February 1995 to February 2013, 520 patients were treated for RCC at Hirosaki University Hospital, Hirosaki, Japan. The RCC patients with tumor thrombus extending to the renal vein (n = 42) and IVC (n = 43) were included in this study. The records of these 85 patients were retrospectively reviewed to assess the relevant clinical and pathological variables and survival. Prognostic factors were identified by multivariate analysis. The benefit of surgical management was evaluated using propensity score matching to compare overall survival between patients who received surgical management and those who did not.
RCC was confirmed by pathological examination of surgical or biopsy specimens in 74 of the 85 patients (87%). Sixty-five patients (76%) received surgical management (radical nephrectomy with thrombectomy). Distant metastasis was identified in 45 patients (53%). The proportion of patients with tumor thrombus level 0 (renal vein only), I, II, III, and IV was 49%, 13%, 18%, 14%, and 5%, respectively. The estimated 5-year overall survival rate was 70% in patients with thrombus extending to the renal vein and 23% in patients with thrombus extending to the IVC. Multivariate analysis identified thrombus extending to the IVC, presence of distant metastasis, surgical management, serum albumin concentration, serum choline esterase concentration, neutrophil-lymphocyte ratio, and Carlson comorbidity index as independent prognostic factors. In propensity score-matched patients, overall survival was significantly longer in those who received surgical management than those who did not.
Surgical management may improve the prognosis of RCC patients with thrombus extending to the renal vein and IVC.
Renal cell carcinoma; Radical nephrectomy with thrombectomy; Tumor thrombus; Prognostic factors
Metastasis of renal cell carcinoma to the contralateral ureter is extremely rare. To date, only 50 cases of metastatic RCC to the ureter have been reported, among whom 6 cases occur at the contralateral site. We herein report a rare case of metastatic RCC in the contralateral ureter 4 years after radical nephrectomy.
Presentation of case
A 74-year-old man presented with gross, painless hematuria for one month. Computed tomography scan confirmed that a 1.5 cm × 0.5 cm tumor occurred in the contralateral distal ureter. A 3.5 cm segment of ureter was resected and a uretero-vesical anastomosis with psoas hitch was accomplished.
The reappearance of hematuria after radical nephrectomy is the most common manifestation of the metastasis to the bladder or ureter. The mechanism of metastasis is not clear. In pathology, vimentin and cytokeratins might help to differentiate between metastatic clear cell renal cell carcinoma and clear cell transitional cell carcinoma.
Metastasis of renal cell carcinoma to the contralateral ureter is rare. Early recognition is extremely important in protecting the remaining renal function and prolonging life-expectancy for post-nephrectomy patients. Complete metastectomy suitable anastomosis have been shown to improve survival.
Renal cell carcinoma; Ureteral metastasis
Clear cell renal cell carcinoma (ccRCC) characterized by a tumor thrombus (TT) extending into the inferior vena cava (IVC) generally indicates poor prognosis. Nevertheless, the risk for tumor recurrence after nephrectomy and thrombectomy varies. An applicable and accurate prediction system to select ccRCC patients with TT of the IVC (ccRCC/TT) at high risk after nephrectomy is urgently needed, but has not been established up to now. To our knowledge, a possible role of microRNAs (miRs) for the development of ccRCC/TT or their impact as prognostic markers in ccRCC/TT has not been explored yet. Therefore, we analyzed the expression of the previously described onco-miRs miR-200c, miR-210, miR-126, miR-221, let-7b, miR-21, miR-143 and miR-141 in a study collective of 74 ccRCC patients. Using the expression profiles of these eight miRs we developed classification systems that accurately differentiate ccRCC from non-cancerous renal tissue and ccRCC/TT from tumors without TT. In the subgroup of 37 ccRCC/TT cases we found that miR-21, miR-126, and miR-221 predicted cancer related death (CRD) accurately and independently from other clinico-pathological features. Furthermore, a combined risk score based on the expression of miR-21, miR-126 and miR-221 was developed and showed high sensitivity and specificity to predict cancer specific survival (CSS) in ccRCC/TT. Using the combined risk score we were able to classify ccRCC/TT patients correctly into high and low risk cases. The risk stratification by the combined risk score (CRS) will benefit from further cohort validation and might have potential for clinical application as a molecular prediction system to identify high- risk ccRCC/TT patients.
To evaluate oncological and clinical outcome in patients with renal cell carcinoma (RCC) and tumor thrombus involving inferior vena cava (IVC) treated with nephrectomy and thrombectomy.
We identified 50 patients with a median age of 65 years, who underwent radical surgical treatment for RCC and tumor thrombus of the IVC between 1997 and 2010. The charts were reviewed for pathological and surgical parameters, as well as complications and oncological outcome.
The median follow-up was 26 months. In 21 patients (42%) distant metastases were already present at the time of surgery. All patients underwent radical nephrectomy, thrombectomy and lymph node dissection through a flank (15 patients/30%), thoracoabdominal (14 patients/28%) or midline abdominal approach (21 patients/42%), depending upon surgeon preference and upon the characteristics of tumor and associated thrombus. Extracorporal circulation with cardiopulmonary bypass (CPB) was performed in 10 patients (20%) with supradiaphragmal thrombus of IVC. Cancer-specific survival for the whole cohort at 5 years was 33.1%. Survival for the patients without distant metastasis at 5 years was 50.7%, whereas survival rate in the metastatic group at 5 years was 7.4%. Median survival of patients with metastatic disease was 16.4 months.
On multivariate analysis lymph node invasion, distant metastasis and grading were independent prognostic factors. There was no statistically significant influence of level of the tumor thrombus on survival rate. Indeed, patients with supradiaphragmal tumor thrombus (n = 10) even had a better outcome (overall survival at 5 years of 58.33%) than the entire cohort.
An aggressive surgical approach is the most effective therapeutic option in patients with RCC and any level of tumor thrombus and offers a reasonable longterm survival. Due to good clinical and oncological outcome we prefer the use of CPB with extracorporal circulation in patients with supradiaphragmal tumor thrombus. Cytoreductive surgery appears to be beneficial for patients with metastatic disease, especially when consecutive therapy is performed. Although sample size of our study cohort is limited consistent with some other studies lymph node invasion, distant metastasis and grading seem to have prognostic value.
Renal cell carcinoma; Inferior vena cava; Thrombectomy; Tumor thrombus
Cutaneous metastasis of renal cell carcinoma (RCC) is very rare. The author herein report two cases of RCC with cutaneous metastasis. Case 1: is a 75-year-old man with right lumbago. Imaging modalities including CT and MRI revealed a right renal tumor. Nephrectomy was performed. Pathological diagnosis of the renal tumor was RCC of clear cell type (Fuhrman's grade II). He denied follow-up. Nine years later, he (at the age of 84 years), a neck skin tumor emerged. Clinical diagnosis was hemangioma. Imaging modalities including CT and MRI showed several tumors in both lungs. The resection of the neck tumor was performed. The tumor was composed of clear cell type arranged in a trabecular pattern. Immunohistochemically, the tumor cells were positive for pancytokeratins, cytokeratin 18, CD10, Ki-67 (labeling=13%), but negative for CD34, factor-VIII-related antigen, CEA, EMA, melanosome (HMB45), S100 protein, p53, and HepPar-1. Metastatic RCC was diagnosed. Despite interferon therapy, he died of 6 months after the second admission. Case 2 is a 66-year-old man with gross hematuria. Imaging modalities revealed left renal tumor. A nephrectomy was performed. The pathological diagnosis was RCC of clear cell type (grade II). The tumor was invasive into the renal pelvis. He was treated by chemoradiation, but metastases of lungs, skin (thigh), and lib emerged, and died of cachexia 9 months after the admission. Necropsy of the skin tumor was performed. The skin tumor was composed of clear cells arranged in a trabecular pattern. Immunohistochemically, the tumor cells were positive for pancytokeratins (AE1/3, CAM5.2), CD10, p53, and Ki-67 (labeling=20%), but negative for CD34, factor-VIII-related antigen, CEA, melanosome (HMB45), S100 protein, and HepPar-1. A diagnosis of RCC (grade II) was diagnosed.
Skin; metastasis; renal cell carcinoma; immunohistochemistry
Renal cell carcinoma with tumor thrombus extension into the inferior vena cava occurs in approximately 5% of cases. Despite such situations, an aggressive surgical approach is recommended. However, intraoperative prevention of pulmonary embolism by a fragmended tumor thrombus is necessary. To prevent pulmonary embolism, placement of a temporary suprarenal filter has been attempted, however, the precise placement of a temporary filter between the level of the hepatic vein and right atrium is not always easy because of its migration, tilting, and strut fracture. Here we report a method for early occlusion control of the intrapericardial inferior vena cava to prevent pulmonary embolism during nephrectomy in level II or III renal cell carcinoma tumor thrombus.
Our first case was a 37-year-old Japanese man with left renal cell carcinoma extending into the inferior vena cava below the main hepatic vein (level II) and our second was a 75-year-old Japanese man with right renal cell carcinoma extending into the retrohepatic inferior vena cava at the main hepatic vein (level III). En block resection of the kidney and the tumor thrombus was performed with the aid of partial extracorporeal circulation; the postoperative course of both patients was uneventful.
Control of intrapericardial inferior vena cava is a feasible method to prevent pulmonary embolism.
Intrapericardial inferior vena cava; Renal cell carcinoma; Tumor thrombus; Pulmonary embolism
Budd-Chiari syndrome (BCS) is a poorly understood entity in urology. It results from obstruction of the hepatic veins and the subsequent complications. It has been infrequently reported to be secondary to hepatic venous obstruction from invasion by an inferior vena cava (IVC) tumor thrombus in renal cell carcinoma (RCC). We report the largest known series of patients with RCC and BCS.
Patients and Methods:
Ten patients presented to a tertiary hospital with locally advanced RCC with IVC tumor thrombus. All were evaluated and had clinical or radiographic evidence of BCS. All underwent nephrectomy, IVC thrombectomy or ligation, and tumor removal from the hepatic veins. The perioperative and pathological factors were measured. These included estimated blood loss (EBL) and transfusions. Inpatient factors including duration of intubation, length of intensive care unit (ICU) stay, and overall length of stay (LOS) were recorded. The tumor-free status was evaluated.
The average age was 59 years. No intraoperative deaths occurred. Two intraoperative complications were noted. The mean EBL was 4244 cc; mean surgery length was 8 hours 12 minutes; and the mean ICU stay was nine days. The overall LOS averaged 13.25 days. One patient died postoperatively of sepsis and multisystem organ failure. One patient required reoperation for an abdominal wall hematoma caused by subcutaneous enoxaparin administration. Average follow-up was 28 months. Five patients are alive with no evidence of disease.
Budd-Chiari syndrome is a rare entity in urology, with a potential for significant morbidity and mortality. Surgical excision of the primary tumor along with thrombectomy results in alleviation of BCS and improvement in the patient.
Budd-Chiari syndrome; hepatic vein thrombus; inferior vena cava thrombus; renal cell carcinoma
We report an extremely rare case of squamous cell carcinoma (SCC) of the renal pelvis associated with an incompletely duplicated renal pelvis and ureter. A 71-year-old woman presented with left lower back pain and gross hematuria. Urinary cytology showed atypical squamous cells. Computed tomography, magnetic resonance imaging and retrograde pyelography revealed left incompletely duplicated renal pelvis and ureter and a mass in the left upper renal pelvis. A clinical diagnosis of left renal pelvic cancer was made and the patient underwent total nephroureterectomy. Histological examination of the resected specimen revealed SCC with marked keratinization in the upper renal pelvis. The tumor had invaded the renal parenchyma and perinephric fat. There was no urothelial carcinoma component. The pathological stage was pT4 N0. There was no evidence of recurrence 6 months postoperatively. Because the prognosis of SCC of the upper urinary tract is poor, urologists and pathologists should be aware that SCC may develop in duplicated urinary systems.
Cancer; congenital anomaly; duplex kidney; immunohistochemistry; pathological diagnosis; prognosis
We describe an unusual case of renal cell carcinoma (RCC) involving the entire upper urinary tract. A 51-year-old female was referred to us because of macroscopic hematuria. Computed tomography revealed a renal tumor filling renal pelvis and ureter, which turned to be a clear cell RCC after nephroureterectomy.
Although germline mutations of met proto-oncogene on human chromosome 7q31-34 have been known as useful molecular markers of hereditary papillary renal cell carcinoma (RCC), the expression of MET, a product of met proto-oncogene, has not been fully studied in sporadic RCC, along with its clinical significance. We investigated the expression of MET by immunohistochemistry in 182 cases of renal neoplasm encompassing 145 RCC, 25 urothelial carcinomas of renal pelvis, and 12 oncocytomas. MET was diffusely and strongly expressed in 90% of papillary RCC, all collecting duct carcinomas, and 92% of urothelial carcinomas of renal pelvis. On the contrary, clear cell RCC, chromophobe RCC, and oncocytomas were negative or focally positive for MET expression. In clear cell RCC, MET expression was positively correlated with high nuclear grade, presence of infiltrative growth, tumoral necrosis, papillary architecture, sarcomatoid component, tumoral involvement of the renal pelvis or ureter, involvement of the calyx, and lymphatic invasion. In conclusion, diffuse and strong expression of MET in papillary RCC and collecting duct carcinoma might be helpful in discriminating from the other subtypes of RCC with tubular or papillary growth. In case of MET expression observed in clear cell RCC, it might correlate with those clinicopathological parameters implying aggressive behavior.
Proto-Oncogene Proteins c-met; Carcinoma, Renal Cell; Kidney Neoplasms; Kidney; Immunohistochemistry
The surgical anatomy of a horseshoe kidney (HK) is unique in many ways, ranging from its anomalous circulation, shared renal parenchyma between the right and left renal moieties, and its anterior renal pelvis, to the fact that it obscures access to the vena cava and aorta. While renal cell carcinomas (RCCs) are known to occur in HKs, the surgical approach to an RCC with tumour thrombus extending to the right atrium has not been reported in the literature. We report an unusual presentation of RCC and the technical aspects of our successful experience with managing RCC of a HK extending to the inferior vena cava and right atrium.
Nephrectomy with inferior vena cava (IVC) thrombectomy for advanced renal cell carcinoma (RCC) is a challenging and morbid surgical case. We describe the use of a simple endoluminal technique to occlude the suprahepatic IVC during thrombectomy. A 60-year-old male presented with a large right-sided RCC and IVC tumour thrombus. The tip of the thrombus, which was non-adherent to the caval wall, extended to the level of the hepatic veins. After complete dissection of the kidney, we obtained suprahepatic control of the IVC by a large compliant balloon, introduced through the right internal jugular vein and inflated just below the level of the diaphragm. The IVC thrombectomy was performed in a bloodless field. Mean blood pressure remained stable during IVC balloon inflation with a total occlusion time of 10 minutes. Intraprocedural completion cavogram and postoperative Doppler ultrasonography showed no residual IVC clot. Blood loss during the thrombectomy portion of the case was scant. The patient’s postoperative course was uncomplicated and, at the last follow-up, he had stable metastatic disease on sunitinib therapy. For the surgical treatment of RCC with retrohepatic IVC tumour extension, transjugular balloon occlusion of the suprahepatic IVC offers an alternative to extensive hepatic mobilization to obtain suprahepatic thrombus control. Advantages over traditional surgical methods may include decreased surgical time, lower risk of liver injury and tumour embolism. We suggest this method for further evaluation.
To evaluate the impact of tumor histology on clinicopathologic outcomes for patients with renal cell carcinoma (RCC) and venous tumor thrombus (VTT).
We identified 807 patients with RCC and VTT who underwent nephrectomy at our institution between 1970–2008. All pathologic specimens were re-reviewed by a single urologic pathologist. Patients with non-clear cell RCC (non-ccRCC) (n=56) were matched 1:2 to patients with clear cell RCC (ccRCC) VTT based onsymptoms at presentation, regional lymph node involvement, distant metastases, tumor thrombus level, nuclear grade and sarcomatoid differentiation. Survival was estimated using the Kaplan-Meier method and compared with the log-rank test.
The 56 patients with non-ccRCC VTT included 26 papillary, 11 chromophobe, 5 collecting duct tumors, and 14 RCC not otherwise specified. Compared to unmatched patients with ccRCC VTT (n=751), patients with non-ccRCC VTT presented with larger tumor size (p=0.02), higher nuclear grade (p=0.04), and more frequent sarcomatoid differentiation (p<0.001) and lymph node invasion (p<0.001). However, when patients with non-ccRCC were matched to patients with cc-RCC, no significant differences were noted with regard to 5-year metastases-free survival (41% versus 34%; p=0.24) or cancer-specific survival (25% versus 27%; p=0.97).
Non-ccRCC VTT is associated with a high rate of adverse pathologic features. Nevertheless, when matched to patients with ccRCC, patients with non-ccRCC VTT did not have increased rates of recurrence or adverse survival. Aggressive surgical resection represents the mainstay of treatment in these cases, while continued efforts to optimize a multimodal management approach to such patients remain necessary.
renal cell carcinoma; tumor thrombus; kidney cancer; histology
We aimed to evaluate the feasibility and clinical significance of using a modified liver-mobilization technique to treat renal cell carcinoma (RCC) combined with intrahepatic inferior vena cava (IVC) thrombosis.
A total of 11 level III thrombus patients underwent radical nephrectomy with resection of the tumor thrombus from intrahepatic IVC. A father clamp was used in combination with hepatic portal blocking to control the IVC.
The intraoperative mortality and postoperative complications were reduced in 11 cases of RCC with intrahepatic IVC thrombosis. The mean blood loss was 800 mL, and mean patient hospital stay was 13 days. Follow-up was conducted for one to four months, with only two cases of recurrence recorded.
The proposed modified liver-mobilization technique could safely and effectively treat RCC and reduce intrahepatic IVC thrombosis.
Renal cell carcinoma; Modified liver-mobilization technique; Thrombosis; Intrahepatic inferior vena cava; Father clamp
Tumor resection and caval tumor thrombectomy, with or without cavotomy and inferior vena cava (IVC) replacement are sometimes performed in patients with renal cell carcinoma (RCC) extending into the IVC or liver tumors invading the IVC. Two such cases were treated. Case 1: a 68-year-old female was transferred with a diagnosis of right RCC with tumor thrombus extending into the IVC. A plication was performed to prevent extension into the right atrium before the nephrectomy and cavotomy with removal of the tumor thrombus was accomplished, because the IVC was almost completely obstructed and the hemodynamics were stable during cross-clamping of the IVC. Case 2: a 37-year-old female was transferred with a diagnosis of a giant metastatic liver tumor. A trisegmentectomy with resection of the invaded IVC and IVC replacement was performed while the abdominal aorta was cross-clamped to maintain the hemodynamics. Therefore, abdominal aortic cross-clamping was convenient to maintain the hemodynamics when the IVC replacement was performed during IVC cross-clamping.
Inferior vena cava tumor thrombus; Inferior vena cava invasion; Renal cell carcinoma
Adrenocortical carcinomas are rare aggressive tumors. Their annual incidence is approximately one to two per million among the population of the United States of America. Patients with active endocrine tumors often present with Cushing's syndrome accompanied by virilizing features. Conversely, patients with non-functioning tumors may present with symptoms related to a mass-occupying lesion, such as abdominal pain and flank pain. Although varicoceles and acute kidney injuries are common problems in medicine, they are uncommon presentations of these rare tumors and easy to miss. We report a case of a large adrenocortical carcinoma that presented as testicular pain, varicocele, and acute kidney injury secondary to renal vein thrombosis.
A 54-year-old Caucasian man with a left-sided varicocele presented to our emergency department with lower abdominal pain and a decrease in urination. Four months previously, he had noticed pain and swelling in his left groin and had been diagnosed with left-sided varicocele. For one week, he began developing left-sided abdominal pain and decreased urination frequency, so he came to our emergency department for evaluation. His physical examination revealed a hard mass occupying the entire left side of his abdomen, crossing the midline, and extending to the pelvic brim. His blood tests showed acute kidney injury and mild anemia. Computed tomography of his abdomen showed a large retroperitoneal mass on the left side, displacing the left kidney inferiorly and the spleen superiorly with thoracic epidural compression. Thrombus was also identified in his left renal vein and inferior vena cava. Computed tomography of his chest showed bilateral pulmonary nodules. A computed tomography-guided abdominal mass biopsy was performed, and the diagnosis of adrenocortical carcinoma was made on the basis of pathology and immunohistochemistry. His hormonal evaluations were normal. His kidney function improved with intravenous hydration and anti-coagulation treatment. Unfortunately, the adrenal mass was unresectable because of the extent of the tumor. Treatment with mitotane, an adrenocorticolytic drug, was started with concomitant with irradiation of a lesion at T5, followed by combination chemotherapy thereafter.
Unilateral right-sided varicoceles are rare and should alert the clinician to possible underlying pathology causing inferior vena caval obstruction. Left-sided varicoceles, in contrast, are common secondary to the venous anatomy of the left testis; however, the enlargement of the left testicle can be associated with blockage of the left testicular vein by tumor invasion of the left renal vein. Varicoceles could be an early presentation of a non-functioning adrenocortical carcinoma. Acute kidney injury can occur as a result of mass effect or thrombosis of renal vessels. Large tumors can cause abdominal pain as a late manifestation. Physicians should perform a complete abdominal examination in every patient with varicocele or testicular pain.
To evaluate the clinical and oncological outcomes and to identify prognostic factors for survival in Chinese patients with renal cell carcinoma (RCC) and venous tumor thrombus (VTT).
A total of 86 patients who underwent nephrectomy and tumor thrombectomy for RCC and venous tumor thrombus extension from 2003 to 2013 were included in this retrospective study. The records of these patients were reviewed. Kaplan-Meier analysis was used to determine cancer-specific survival (CSS). Prognostic factors for CSS were identified by univariate and multivariate analyses using the Cox proportional hazards regression mode.
All patients in this cohort received radical nephrectomy and tumor thrombectomy. Median follow-up period was 27.0 months (range 3–111). No patients died intraoperatively, and the complication rate was 36.0%. The 1-, 3-, and 5-year CSS rates for all patients were 93.0%, 70.9%, and 58.1%, respectively, and those for patients without distant metastasis at presentation were 95.3%, 82.6%, and 68.6%, respectively. Multivariate Cox regression analysis showed that lymph node invasion, distant metastasis at presentation, and invasion of the inferior vena cava (IVC) wall were the independent prognostic factors for CSS in all patients. For patients without distant metastasis, tumor grade, lymph node invasion, and perinephric fat invasion were significantly associated with CSS on multivariate analysis.
Survival rates for patients with RCC and VTT were still poor. Our results indicated that lymph node invasion, distant metastasis at presentation, and invasion of the IVC wall were independent negative prognostic factors.
Renal cell carcinoma; Venous tumor thrombus; Cancer-specific survival; Prognostic factors
This is the first case ever reported showing a combination of renal cell carcinoma (RCC) with tumour thrombus into inferior vena cava (IVC), horseshoe kidney and doubled right kidney that was successfully treated. Even in advanced tumour lesions of the kidney, curative treatment is a feasible and safe option by using interdisciplinary cooperation and expertise. However, this requires an adequate diagnostic work-up to clarify resectability and optimal perioperative and postoperative care, and also advanced surgical skills exhausting all potential options for complete tumour resection in a centre of excellence. Achieving R0 resection with a reasonable risk-benefit ratio for the patient, which should be the primary aim, can distinctly improve survival chances as published cases in literature have indicated. RCC-derived IVC tumour thrombus as an extra-renal tumour manifestation by continuous intravascular tumour growth (also classified as secondary IVC tumour lesion) can be considered no serious contraindication to aim for curative surgery.
Renal cell carcinoma (RCC) with sarcomatoid differentiation is invasive, refractory to treatment, and has a higher mortality. Therefore, systemic therapy is still challenging, and the curative resection of localized or locally advanced RCC with sarcomatoid differentiation is very important. Axitinib is a potent and selective second-generation vascular endothelial growth factor receptor tyrosine kinase inhibitor with improved safety and tolerability. Axitinib is generally recommended as second-line therapy for advanced RCC because the phase III axitinib versus sorafenib in advanced RCC (AXIS) trial demonstrated that it achieved longer progression-free survival than sorafenib in patients with metastatic RCC after failure of an approved first-line regimen.
We present a 73-year-old man who had a large (13 cm in diameter) right RCC with sarcomatoid differentiation that directly invaded the duodenum and inferior vena cava. The patient presented with gastrointestinal bleeding, was unable to eat solid food, and had become emaciated. Thus, his classification was poor risk with anemia, hypercalcemia, and poor performance status, according to the Memorial Sloan-Kettering Cancer Center criteria. He seemed unlikely to survive if radical nephrectomy, cavotomy with thrombectomy, and pancreatoduodenectomy were performed. To reduce the tumor burden and potential operative complications, we administered axitinib as first-line neoadjuvant therapy.
Six weeks of treatment reduced the tumor burden without causing severe toxicities. Subsequently, radical right nephrectomy, cavotomy with thrombectomy, and pancreatoduodenectomy were performed successfully. The pathological treatment effect of axitinib was grade 2 (two-thirds necrosis). The resected tumor showed a heterogeneous reaction for phosphorylated Akt (Ser-473) by Western blotting and immunohistochemistry, indicating that parts of the tumor were sensitive to axitinib and other parts were not.
Axitinib might be promising as preoperative or neoadjuvant therapy for locally advanced RCC (>cT3b or >cTanyN1).
renal cell carcinoma; sarcomatoid differentiation; axitinib; tyrosine kinase inhibitors; phosphorylated Akt
Herein, we present the case of a patient with recurrent hepatocellular carcinoma (HCC) who had paracaval lymph node (LN) metastases with an inferior vena cava (IVC) tumor thrombus after a hepatectomy. A 65-year-old man with chronic hepatitis B virus infection received an extended anterior segmentectomy because of two hepatic tumors, located in segments 7 and 8. Histological examination of both resected specimens showed mostly moderately differentiated HCC with some poorly differentiated areas, and liver cirrhosis (A2/F4). Because the patient had an elevated α-fetoprotein serum level, abdominal computed tomography (CT) was performed. Abdominal CT revealed a 9-mm-diameter recurrent tumor in hepatic segment 3 and paracaval LN metastases with an IVC tumor thrombus at 8 months after the first operation. The patient received transcatheter arterial chemoembolization as treatment for the intrahepatic recurrence, following resection of the paracaval LN metastases and removal of the IVC tumor thrombus. In this case, the paracaval LN metastases had directly infiltrated the IVC via the lumbar veins, resulting in an IVC tumor thrombus, which usually develops from an intrahepatic tumor via the hepatic vein. The development of an IVC tumor thrombus with HCC recurrence, as in this case, is very rare, and based on a PubMed search, we believe this report may be the first to describe this condition.
Hepatocellular carcinoma; Lymph node metastases; Inferior vena cava tumor thrombus
Background: The multilocular cystic nephroma (MLCN) is a unilateral cystic neoplasm of the kidney exhibiting benign biological behavior. The etiology and histopathogenesis of the disease is controversial (dysplastic/hamartomous/neoplastic). MLCNs show bimodal age distribution, with peak incidence occurring at 2-4 years of age and between the fourth and sixth decades. The male to female ratio in patients aged below 4 years is 3:1, which reverses to 1:8 between the fourth and sixth decades. Patients and methods: A 59-year-old female patient presented with left flank pain and abdominal pain. Ultrasound (US) revealed 220×109×82 mm multiple septated hyperechoic kidney cysts with a semi-solid appearance. MRI showed a 245×119×98 mm multilocular cystic renal mass in the left kidney with hypointense appearance in T1-weighted images and hyperintense in T2-weighted images, and multicystic appearance in ureter projection, the largest portion measuring 17 mm in diameter. Radical nephrectomy was planned with the pre-diagnosis of multilocular cystic nephroma or multicystic renal cell carcinoma. Results: The patient underwent transperitoneal radical nephroureterectomy. The immunohistopathological examination revealed MLCN with ureteral invagination. Conclusion: The etiology, pathogenesis, and genetic basis of multilocular cystic nephroma are currently unknown. This tumor is confused with cystic partially differentiated nephroblastoma and cystic Wilms tumor in childhood, and multilocular cystic renal cell carcinoma, clear cell papillary renal cell carcinoma, and tubulocystic carcinoma in adults. The association of this tumor with pleuropulmonary blastoma in children exhibits genetic inheritance. US control is particularly recommended in siblings of these children. Albeit rare, the disease can occur as a bilateral synchronous or metachronous lesion. There are four reports of cases with recurrence in the literature. The laparoscopic partial nephrectomy is the recommended treatment method in patients with sufficient renal reserve that are found to be free of malignancy in the frozen section examination. The symptoms of hematuria and flank pain can be associated with invagination of the cysts into the pelvis and intrarenal rupture of the cysts. The invagination of cysts into the pelvis has been previously described. The authors consider that this was the first case of MLCN in the literature exhibiting invagination into the ureter.
Multilocular cystic nephroma; renal cystic mass; multilocular cystic renal tumors; bosniak classification of renal cysts
We report a case of primary small cell carcinoma of the ureter with squamous cell and transitional cell carcinomatous components associated with ureteral stone, which is unique in that the patient has remained free of tumor recurrence for 36 months after the surgery without adjuvant chemotherapy or radiotherapy. A 60-yr-old man presented himself with a right flank pain. Computed tomography revealed an ill-defined mass and a stone in the lower one third of the right ureter, and hydronephroureterosis above the stone-impacted site. The patient underwent right nephroureterectomy and stone removal. Upon gross examination, a 3.8 x 1.8 x 1.2 cm white and partly yellow mass was noted in the anterior part of the ureter, resulting in indentation of the ureteral lumen on the posterior side. Light microscopic examination revealed that the mass was mainly composed of small cell carcinoma, and partly squamous cell and transitional cell carcinomatous components. The overlying ureteral mucosa and renal pelvis also contained multifocal dysplastic transitional epithelium and transitional cell carcinoma in situ. There was no vascular invasion, and the surgical margins were free of tumor. The small cell carcinomatous component was positive for chromogranin, neuron specific enolase, synaptophysin, and pancytokeratin but negative for high molecular-weight cytokeratin (K-903) by immunohistochemistry.
Extraskeletal osteoclast-like giant cell (OGC) tumors are uncommon and have mainly been found in the breast and pancreas. OGC neoplasms of the urinary tract are extremely rare. Most cases found in the renal pelvis and bladder are associated with either an in situ urothelial malignancy or a conventional high-grade urothelial carcinoma. These malignancies tend to be associated with a poor prognosis and disease course. To our knowledge, no cases of OGC tumors of the distal ureter only have been published. Here, we present the case of a 76-year-old man who underwent hand-assisted laparoscopic nephroureterectomy because of painless gross hematuria with right flank pain. Pathologic examination showed OGC carcinoma of the right distal ureter. No local tumor recurrence or distant metastasis was found at the 5-month follow-up.
Giant cell carcinoma; Osteoclasts; Ureter