Case Review
Transitional Cell Carcinoma of the Renal Pelvis With Venous Tumor Thrombus Onkar Singh, MCh (Urol), Arun Jacob Phillip George, MCh (Urol), J. Chandra Singh, MCh (Urol), DipNB (Urol), Antony Devasia, MCh (Urol), FRCSEd, FRCS (Urol) Department of Urology, Christian Medical College and Hospital, Vellore, Tamilnadu, India
Renal cell carcinoma (RCC) is the most common malignancy that results in venous tumor thrombosis. Transitional cell carcinoma of the renal pelvis with renal or vena cava thrombus is extremely rare. Fewer than 40 cases have been reported. We report a similar case of a patient who underwent radical nephrectomy with a preoperative diagnosis of RCC. [Rev Urol. 2017;19(2):145–148 doi: 10.3909/riu0743]
®
© 2017 MedReviews , LLC
Key words Transitional cell carcinoma • Urothelial carcinoma • Kidney • Inferior vena cava • Thrombus
T
ransitional cell carcinoma (TCC) of the renal pelvis is an uncommon lesion, accounting for approximately 5% of all urinary tract tumors.1 Renal vein (RV) and/or inferior vena cava (IVC) tumor thrombus usually develops in renal cell carcinoma (RCC), and is a rare feature of renal TCC. The management and outcome of RCC and renal TCC are completely different. Occasionally, it can be difficult to distinguish between these two conditions preoperatively. We present a case of renal TCC with tumor thrombus in the RV extending to the IVC, for which the preoperative diagnosis on radiologic evidence was RCC with RV thrombus extending to the IVC.
Case Report
A 55-year-old hypertensive woman had right flank pain for 2 years. She had a 30 pack-year history of smoking. She had no hematuria or weight loss. Results of her physical examination were unremarkable. Results of routine blood tests and urinalysis were within normal limits. Ultrasonography revealed a large heteroechoic mass involving the right kidney. On contrast-enhanced computed tomography (CT) scan, an 8.7 3 7.3 3 7.6-cm heterogeneously enhancing lesion with a few areas of necrosis, perinephric fat stranding, and involvement of the Gerota fascia was seen in the lower and interpolar region of the right kidney (Figure 1). Vol. 19 No. 2 • 2017 • Reviews in Urology • 145
TCC of the Renal Pelvis With Venous Tumor Thrombus continued Figure 1. Contrast-enhanced computed tomography coronal image showing a heterogeneously enhancing lesion with a few areas of necrosis and perinephric fat stranding involving the right kidney.
ipsilateral adrenalectomy were also performed. Her postoperative period was uneventful. Histopathologic investigation revealed a high-grade urothelial carcinoma with involvement of
4% to 10% of cases of RCC, respectively.2 However, renal TCC with RV/IVC thrombus is an uncommon, late finding.3-5 The incidence of IVC involvement in RCC has been estimated to be 48-fold higher
Histopathologic investigation revealed a high-grade urothelial carcinoma with involvement of the renal parenchyma, sinus, proximal ureter, and adrenal gland.
Multiple right renal hilar lymph nodes were present; the largest was 2 cm in size. A hypodense, nonenhancing filling defect was seen in the right RV extending to the junction of the right RV and IVC (Figure 2). A radiologic diagnosis of RCC with RV and IVC thrombus (cT4N1M0) was made, and the patient underwent radical nephrectomy via a chevron incision. The RV contained a thrombus extending to its ostium. After ligation of the right renal artery, the thrombus retracted into the RV. A Satinsky clamp was placed distal to the RV ostium. The RV was ligated just beyond the thrombus. Hilar lymphadenectomy and
the renal parenchyma, sinus, proximal ureter, and adrenal gland. The RV thrombus and lymph nodes were involved by the tumor. Perinephric fat was free of tumor and the ureteric margin had carcinoma in situ.
than in renal TCC.4 Huber and colleagues4 reviewed their series of 102 cases of renal TCC managed over 20 years and found an incidence of 5%. However, fewer than 40 cases have been reported to date.5 Approximately 90% of cases
The incidence of IVC involvement in RCC has been estimated to be 48-fold higher than in renal TCC.
On reviewing the imaging, CT revealed the mass to be more central than exophytic, without distortion of the reniform shape of the kidney. It also had a few dilated calyces, which may have suggested a tumor of urothelial origin rather than RCC. Adjuvant chemotherapy with cisplatin and gemcitabine was recommended.
Discussion
Thrombosis of the RV and IVC has been reported in 21% to 35% and
have been reported involving the right kidney,6 and most cases are high-grade tumors.7 Moreover, renal TCC more commonly presents with a higher stage than TCC of the urinary bladder because of the thin muscle layer of the renal pelvis.8 Large, infiltrating TCC may be difficult to differentiate from other common and uncommon masses such as RCC with RV thrombus, xanthogranulomatous pyelonephritis, lymphoma, or metastases.3 However, the renal mass in our patient appeared to have more of an
Figure 2. Computed tomography axial and coronal images showing a hypodense, nonenhancing filling defect in the right renal vein extending up to the junction of right renal vein and inferior vena cava (red arrows). Hilar lymph nodes are seen posterior to the right renal vein in Panel A.
A
146 • Vol. 19 No. 2 • 2017 • Reviews in Urology
B
TCC of the Renal Pelvis With Venous Tumor Thrombus infiltrative radiologic pattern, with a preserved reniform shape of the kidney, which is not typical of RCC. The first differential diagnosis given by the radiologist was RCC in view of the RV and IVC thrombus.
tumor is usually locally advanced in such cases. More than 50% of patients die within the first year of diagnosis.5,13 Most reported cases were treated surgically. When there is a strong
An infiltrative pattern of growth that preserves the reniform renal shape is more suggestive of urothelial carcinoma or lymphoma.
An infiltrative pattern of growth that preserves the reniform renal shape is more suggestive of urothelial carcinoma or lymphoma.9 Percutaneous needle biopsy may be indicated in such cases.10 With RV/IVC thrombus, preoperative differentiation between upper tract TCC and RCC is important for both management and prognostication; renal TCC with RV/IVC tumor thrombus has a poorer prognosis than RCC with venous thrombus.7,11 In renal TCC, those with venous involvement have poor prognosis, with an estimated median survival of 8.9 months,4 as compared with 12.9 months for patients with locally advanced renal TCC without venous involvement.12 The
suspicion of renal TCC on preoperative imaging, examination of an intraoperative frozen section helps in the choice of surgical procedure.14 If the frozen section reveals TCC, nephroureterectomy with thrombectomy and bladder cuff excision must be performed. The role of liver transplantation techniques in the surgical management of renal TCC with IVC thrombus has been described.15 Recently, IVC resection has been reported in one case of renal TCC with IVC invasion.16 Although radical surgery with IVC thrombectomy prolongs survival in patients with RCC,17 the exact role of radical extirpative surgery in the management of renal TCC with venous thrombus is not defined due to the limited available
literature, as well as its aggressive nature and advanced presentation. In our patient, the absence of hematuria and presence of the venous thrombus were the two key misleading points that clouded our vision toward correct preoperative diagnosis. Thus, renal pelvic TCC should be included in the differential diagnosis of an infiltrative renal mass with venous involvement and maintained reniform shape. Percutaneous needle biopsy in such doubtful cases may be considered. References 1.
2.
3.
4.
5.
6.
In our patient, the absence of hematuria and presence of the venous thrombus were the two key misleading points that clouded our vision toward correct preoperative diagnosis.
7.
Hall MC, Womack S, Sagalowsky AI, et al. Prognostic factors, recurrence and survival in transitional cell carcinoma of the upper urinary tract; a 30-year experience in 252 patients. Urology. 1998;52:594-601. Fernández López-Peláez MS, García Gómez JM, Ortíz Vico F, Roldán Ramos J. Tumor thrombosis of the left renal vein and inferior vena cava secondary to renal cell carcinoma. Findings with ultrasonography, EchoDoppler and Computerized tomography [in Spanish]. Actas Urol Esp. 2000;24:664-668. Perez-Montiel D, Wakely PE, Hes O, Suster S. Highgrade urothelial carcinoma of the renal pelvis: clinicopathologic study of 108 cases with emphasis on unusual morphologic variants. Mod Pathol. 2006;19:494-503. Huber J, Teber D, Hatiboglu G, et al. Does a venous tumor thrombus exclude transitional cell carcinoma? Implications for neo-adjuvant treatment strategies. Anticancer Res. 2014;34:1031-1035. Miyazato M, Yonou H, Sugaya K, et al. Transitional cell carcinoma of the renal pelvis forming tumor thrombus in the vena cava. Int J Urol. 2001;8:575-577. Prando A, Prando P, Prando D. Urothelial cancer of the renal pelvicaliceal system: unusual imaging manifestations. Radiographics. 2010;30:1553-1566. Hwang CD, Hsieh TS, Wang TH, et al. Renal transitional cell carcinoma with tumor thrombus into the vena cava: a case report. J Urol R.O.C. 2000;11:126-130.
Main Points • Transitional cell carcinoma (TCC) of the renal pelvis is an uncommon lesion, accounting for approximately 5% of all urinary tract tumors. Renal vein (RV) and/or inferior vena cava (IVC) tumor thrombus usually develops in renal cell carcinoma (RCC), and is a rare feature of renal TCC. • It can be difficult to distinguish between these two conditions preoperatively; however, the management and outcome of RCC and renal TCC are completely different. • With RV/IVC thrombus, preoperative differentiation between upper tract TCC and RCC is important for both management and prognostication; renal TCC with RV/IVC tumor thrombus has a poorer prognosis than RCC with venous thrombus. • Renal pelvic TCC should be included in the differential diagnosis of an infiltrative renal mass with venous involvement and maintained reniform shape; percutaneous needle biopsy may be indicated.
Vol. 19 No. 2 • 2017 • Reviews in Urology • 147
TCC of the Renal Pelvis With Venous Tumor Thrombus continued 8.
9.
10.
11.
Raman JD, Messer J, Sielatycki JA, Hollenbeak CS. Incidence and survival of patients with carcinoma of the ureter and renal pelvis in the USA, 1973-2005. BJU Int. 2011;107:1059-1064. Tseng YS, Chen KH, Chiu B, et al. Renal urothelial carcinoma with extended venous thrombus. South Med J. 2010;103:813-814. Huben RP, Mounzer AM, Murphy GP. Tumor grade and stage as prognostic variables in upper tract urothelial tumors. Cancer. 1988;62:2016-2020. Fujimoto M, Tsujimoto Y, Nonomura N, et al. Renal pelvic cancer with tumor thrombus in the vena cava
12.
13.
14.
inferior; a case report and review of the literature. Urol Int. 1997; 59:263-265. Libertino JA, Zinman L, Watkins E Jr. Long-term results of resection of renal cell cancer with extension into inferior vena cava. J Urol. 1987;137:21-24. Cerwinka WH, Manoharan M, Soloway MS, Ciancio G. The role of liver transplantation techniques in the surgical management of advanced renal urothelial carcinoma with or without inferior vena cava thrombus. Int Braz J Urol. 2009;35:19-23. Nam JK, Moon KM, Park SW, Chung MK. Surgical treatment of inferior vena cava invasion in patients with
148 • Vol. 19 No. 2 • 2017 • Reviews in Urology
15.
16.
17.
renal pelvis transitional cell carcinoma by use of human cadaveric aorta. Korean J Urol. 2012;53:285-287. Al Otaibi M, AbouYoussif T, Alkhaldi A, et al. Renal cell carcinoma with inferior vena caval extention: impact of tumour extent on surgical outcome. BJU Int. 2009;104:1467-1470. Dyer R, DiSantis D J, McClennan BL. Simplified imaging approach for evaluation of the solid renal mass in adults. Radiology. 2008;247:331-343. Caoili EM, Davenport MS. Role of percutaneous needle biopsy for renal masses. Semin Intervent Radiol. 2014;31:20-26.
Transitional Cell Carcinoma of the Renal Pelvis With Venous Tumor Thrombus Onkar Singh, MCh (Urol), Arun Jacob Phillip George, MCh (Urol), J. Chandra Singh, MCh (Urol), DipNB (Urol), Antony Devasia, MCh (Urol), FRCSEd, FRCS (Urol) Department of Urology, Christian Medical College and Hospital, Vellore, Tamilnadu, India
Renal cell carcinoma (RCC) is the most common malignancy that results in venous tumor thrombosis. Transitional cell carcinoma of the renal pelvis with renal or vena cava thrombus is extremely rare. Fewer than 40 cases have been reported. We report a similar case of a patient who underwent radical nephrectomy with a preoperative diagnosis of RCC. [Rev Urol. 2017;19(2):145–148 doi: 10.3909/riu0743]
®
© 2017 MedReviews , LLC
Key words Transitional cell carcinoma • Urothelial carcinoma • Kidney • Inferior vena cava • Thrombus
T
ransitional cell carcinoma (TCC) of the renal pelvis is an uncommon lesion, accounting for approximately 5% of all urinary tract tumors.1 Renal vein (RV) and/or inferior vena cava (IVC) tumor thrombus usually develops in renal cell carcinoma (RCC), and is a rare feature of renal TCC. The management and outcome of RCC and renal TCC are completely different. Occasionally, it can be difficult to distinguish between these two conditions preoperatively. We present a case of renal TCC with tumor thrombus in the RV extending to the IVC, for which the preoperative diagnosis on radiologic evidence was RCC with RV thrombus extending to the IVC.
Case Report
A 55-year-old hypertensive woman had right flank pain for 2 years. She had a 30 pack-year history of smoking. She had no hematuria or weight loss. Results of her physical examination were unremarkable. Results of routine blood tests and urinalysis were within normal limits. Ultrasonography revealed a large heteroechoic mass involving the right kidney. On contrast-enhanced computed tomography (CT) scan, an 8.7 3 7.3 3 7.6-cm heterogeneously enhancing lesion with a few areas of necrosis, perinephric fat stranding, and involvement of the Gerota fascia was seen in the lower and interpolar region of the right kidney (Figure 1). Vol. 19 No. 2 • 2017 • Reviews in Urology • 145
TCC of the Renal Pelvis With Venous Tumor Thrombus continued Figure 1. Contrast-enhanced computed tomography coronal image showing a heterogeneously enhancing lesion with a few areas of necrosis and perinephric fat stranding involving the right kidney.
ipsilateral adrenalectomy were also performed. Her postoperative period was uneventful. Histopathologic investigation revealed a high-grade urothelial carcinoma with involvement of
4% to 10% of cases of RCC, respectively.2 However, renal TCC with RV/IVC thrombus is an uncommon, late finding.3-5 The incidence of IVC involvement in RCC has been estimated to be 48-fold higher
Histopathologic investigation revealed a high-grade urothelial carcinoma with involvement of the renal parenchyma, sinus, proximal ureter, and adrenal gland.
Multiple right renal hilar lymph nodes were present; the largest was 2 cm in size. A hypodense, nonenhancing filling defect was seen in the right RV extending to the junction of the right RV and IVC (Figure 2). A radiologic diagnosis of RCC with RV and IVC thrombus (cT4N1M0) was made, and the patient underwent radical nephrectomy via a chevron incision. The RV contained a thrombus extending to its ostium. After ligation of the right renal artery, the thrombus retracted into the RV. A Satinsky clamp was placed distal to the RV ostium. The RV was ligated just beyond the thrombus. Hilar lymphadenectomy and
the renal parenchyma, sinus, proximal ureter, and adrenal gland. The RV thrombus and lymph nodes were involved by the tumor. Perinephric fat was free of tumor and the ureteric margin had carcinoma in situ.
than in renal TCC.4 Huber and colleagues4 reviewed their series of 102 cases of renal TCC managed over 20 years and found an incidence of 5%. However, fewer than 40 cases have been reported to date.5 Approximately 90% of cases
The incidence of IVC involvement in RCC has been estimated to be 48-fold higher than in renal TCC.
On reviewing the imaging, CT revealed the mass to be more central than exophytic, without distortion of the reniform shape of the kidney. It also had a few dilated calyces, which may have suggested a tumor of urothelial origin rather than RCC. Adjuvant chemotherapy with cisplatin and gemcitabine was recommended.
Discussion
Thrombosis of the RV and IVC has been reported in 21% to 35% and
have been reported involving the right kidney,6 and most cases are high-grade tumors.7 Moreover, renal TCC more commonly presents with a higher stage than TCC of the urinary bladder because of the thin muscle layer of the renal pelvis.8 Large, infiltrating TCC may be difficult to differentiate from other common and uncommon masses such as RCC with RV thrombus, xanthogranulomatous pyelonephritis, lymphoma, or metastases.3 However, the renal mass in our patient appeared to have more of an
Figure 2. Computed tomography axial and coronal images showing a hypodense, nonenhancing filling defect in the right renal vein extending up to the junction of right renal vein and inferior vena cava (red arrows). Hilar lymph nodes are seen posterior to the right renal vein in Panel A.
A
146 • Vol. 19 No. 2 • 2017 • Reviews in Urology
B
TCC of the Renal Pelvis With Venous Tumor Thrombus infiltrative radiologic pattern, with a preserved reniform shape of the kidney, which is not typical of RCC. The first differential diagnosis given by the radiologist was RCC in view of the RV and IVC thrombus.
tumor is usually locally advanced in such cases. More than 50% of patients die within the first year of diagnosis.5,13 Most reported cases were treated surgically. When there is a strong
An infiltrative pattern of growth that preserves the reniform renal shape is more suggestive of urothelial carcinoma or lymphoma.
An infiltrative pattern of growth that preserves the reniform renal shape is more suggestive of urothelial carcinoma or lymphoma.9 Percutaneous needle biopsy may be indicated in such cases.10 With RV/IVC thrombus, preoperative differentiation between upper tract TCC and RCC is important for both management and prognostication; renal TCC with RV/IVC tumor thrombus has a poorer prognosis than RCC with venous thrombus.7,11 In renal TCC, those with venous involvement have poor prognosis, with an estimated median survival of 8.9 months,4 as compared with 12.9 months for patients with locally advanced renal TCC without venous involvement.12 The
suspicion of renal TCC on preoperative imaging, examination of an intraoperative frozen section helps in the choice of surgical procedure.14 If the frozen section reveals TCC, nephroureterectomy with thrombectomy and bladder cuff excision must be performed. The role of liver transplantation techniques in the surgical management of renal TCC with IVC thrombus has been described.15 Recently, IVC resection has been reported in one case of renal TCC with IVC invasion.16 Although radical surgery with IVC thrombectomy prolongs survival in patients with RCC,17 the exact role of radical extirpative surgery in the management of renal TCC with venous thrombus is not defined due to the limited available
literature, as well as its aggressive nature and advanced presentation. In our patient, the absence of hematuria and presence of the venous thrombus were the two key misleading points that clouded our vision toward correct preoperative diagnosis. Thus, renal pelvic TCC should be included in the differential diagnosis of an infiltrative renal mass with venous involvement and maintained reniform shape. Percutaneous needle biopsy in such doubtful cases may be considered. References 1.
2.
3.
4.
5.
6.
In our patient, the absence of hematuria and presence of the venous thrombus were the two key misleading points that clouded our vision toward correct preoperative diagnosis.
7.
Hall MC, Womack S, Sagalowsky AI, et al. Prognostic factors, recurrence and survival in transitional cell carcinoma of the upper urinary tract; a 30-year experience in 252 patients. Urology. 1998;52:594-601. Fernández López-Peláez MS, García Gómez JM, Ortíz Vico F, Roldán Ramos J. Tumor thrombosis of the left renal vein and inferior vena cava secondary to renal cell carcinoma. Findings with ultrasonography, EchoDoppler and Computerized tomography [in Spanish]. Actas Urol Esp. 2000;24:664-668. Perez-Montiel D, Wakely PE, Hes O, Suster S. Highgrade urothelial carcinoma of the renal pelvis: clinicopathologic study of 108 cases with emphasis on unusual morphologic variants. Mod Pathol. 2006;19:494-503. Huber J, Teber D, Hatiboglu G, et al. Does a venous tumor thrombus exclude transitional cell carcinoma? Implications for neo-adjuvant treatment strategies. Anticancer Res. 2014;34:1031-1035. Miyazato M, Yonou H, Sugaya K, et al. Transitional cell carcinoma of the renal pelvis forming tumor thrombus in the vena cava. Int J Urol. 2001;8:575-577. Prando A, Prando P, Prando D. Urothelial cancer of the renal pelvicaliceal system: unusual imaging manifestations. Radiographics. 2010;30:1553-1566. Hwang CD, Hsieh TS, Wang TH, et al. Renal transitional cell carcinoma with tumor thrombus into the vena cava: a case report. J Urol R.O.C. 2000;11:126-130.
Main Points • Transitional cell carcinoma (TCC) of the renal pelvis is an uncommon lesion, accounting for approximately 5% of all urinary tract tumors. Renal vein (RV) and/or inferior vena cava (IVC) tumor thrombus usually develops in renal cell carcinoma (RCC), and is a rare feature of renal TCC. • It can be difficult to distinguish between these two conditions preoperatively; however, the management and outcome of RCC and renal TCC are completely different. • With RV/IVC thrombus, preoperative differentiation between upper tract TCC and RCC is important for both management and prognostication; renal TCC with RV/IVC tumor thrombus has a poorer prognosis than RCC with venous thrombus. • Renal pelvic TCC should be included in the differential diagnosis of an infiltrative renal mass with venous involvement and maintained reniform shape; percutaneous needle biopsy may be indicated.
Vol. 19 No. 2 • 2017 • Reviews in Urology • 147
TCC of the Renal Pelvis With Venous Tumor Thrombus continued 8.
9.
10.
11.
Raman JD, Messer J, Sielatycki JA, Hollenbeak CS. Incidence and survival of patients with carcinoma of the ureter and renal pelvis in the USA, 1973-2005. BJU Int. 2011;107:1059-1064. Tseng YS, Chen KH, Chiu B, et al. Renal urothelial carcinoma with extended venous thrombus. South Med J. 2010;103:813-814. Huben RP, Mounzer AM, Murphy GP. Tumor grade and stage as prognostic variables in upper tract urothelial tumors. Cancer. 1988;62:2016-2020. Fujimoto M, Tsujimoto Y, Nonomura N, et al. Renal pelvic cancer with tumor thrombus in the vena cava
12.
13.
14.
inferior; a case report and review of the literature. Urol Int. 1997; 59:263-265. Libertino JA, Zinman L, Watkins E Jr. Long-term results of resection of renal cell cancer with extension into inferior vena cava. J Urol. 1987;137:21-24. Cerwinka WH, Manoharan M, Soloway MS, Ciancio G. The role of liver transplantation techniques in the surgical management of advanced renal urothelial carcinoma with or without inferior vena cava thrombus. Int Braz J Urol. 2009;35:19-23. Nam JK, Moon KM, Park SW, Chung MK. Surgical treatment of inferior vena cava invasion in patients with
148 • Vol. 19 No. 2 • 2017 • Reviews in Urology
15.
16.
17.
renal pelvis transitional cell carcinoma by use of human cadaveric aorta. Korean J Urol. 2012;53:285-287. Al Otaibi M, AbouYoussif T, Alkhaldi A, et al. Renal cell carcinoma with inferior vena caval extention: impact of tumour extent on surgical outcome. BJU Int. 2009;104:1467-1470. Dyer R, DiSantis D J, McClennan BL. Simplified imaging approach for evaluation of the solid renal mass in adults. Radiology. 2008;247:331-343. Caoili EM, Davenport MS. Role of percutaneous needle biopsy for renal masses. Semin Intervent Radiol. 2014;31:20-26.
