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Large Renal Cavernous Hemangioma With Renal Vein Thrombosis: Case Report and Review of Literature
Case Report Large Renal Cavernous Hemangioma With Renal Vein Thrombosis: Case Report and Review of Literature Xiaokun Zhao, Jinqing Zhang, Zhaohui Zhong, Chester J. Koh, Hui Wen Xie, and Brian E. Hardy Kidney cavernous hemangiomas are rare. Renal cavernous hemangioma complicated by secondary chronic thrombosis of the renal vein has not been previously reported. A 17-year-old boy was initially diagnosed with a renal mass with tumor thrombosis. Radical nephrectomy was performed, and the pathologic examination revealed a renal cavernous hemangioma with chronic renal vein thrombosis. We also reviewed the previous diagnostic and treatment methods described in previous reports. UROLOGY 73: 443.e1– 443.e3, 2009. © 2009 Elsevier Inc.
R
enal hemangioma is a rare, benign, vascular malformation also known as renal angiomatous dysplasia. We report the first case of renal cavernous hemangioma complicated by thrombosis in the renal vein.
CASE REPORT A 17-year-old boy was admitted to our hospital with hematuria of 5 days’ duration. On physical examination, the patient was thin with a large palpable mass below the left costal margin that was nontender. Serum studies were normal, and urinalysis revealed red blood cell count of 0 to 5 per high power field and numerous white blood cells. Renal ultrasonography revealed a solid renal mass with clear demarcations in the middle and lower poles of the left kidney, with some hypoechoic/cystic components. Computed tomography (CT) revealed an 8 ⫻ 7 ⫻ 8-cm, low-density, left kidney tumor that was slightly enhanced at 72.09 Hounsfield units after contrast injection (Fig. 1). Soft tissue consistent with left renal vein thrombosis was also seen. During radical nephrectomy, engorged veins were seen on the renal capsule surface. The renal vein thrombosis extended to just within the inferior vena cava. Frozen section analysis revealed a renal hemangioma with a renal vein thrombosis. Gross pathologic examination revealed a 10 ⫻ 7 ⫻ 8-cm mass within the left kidney that had replaced most of the middle and lower
This study was supported by grants from the Natural Science Fund of Hunan Province (grant 05JJ30056) and the Chang Sha Scientific Bureau 2006 fund. From the Department of Urology, Second Xiangya Hospital, Central South University School of Medicine, Xiangya, People’s Republic of China; and Division of Pediatric Urology, Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California Address for correspondence: Chester J. Koh, M.D., Division of Pediatric Urology, Children’s Hospital Los Angeles, 4650 Sunset Boulevard, Mailstop 114, Los Angeles, CA 90027. E-mail: [email protected] Submitted: January 8, 2008, accepted (with revisions): February 21, 2008
© 2009 Elsevier Inc. All Rights Reserved
poles, with involvement of the renal hilum. The left renal vein contained a gray, tenacious thrombosis. Histopathologic examination revealed a renal cavernous hemangioma with extensive intratumoral hemorrhage and chronic renal vein thrombosis (Fig. 2). Immunohistochemical stains showed reactivity of the tumor cells to factor Vim and CD34 and no reactivity to HHF35, epithelial membrane antigen, LCK, or S-100, confirming the benign nature of the tumor. After 1 year, the patient was in good health, and follow-up studies showed no evidence of recurrence.
COMMENT Renal hemangioma is a rare, benign, vascular malformation also known as renal angiomatous dysplasia. Virchow reported the first case in 1867.1 A distinction is usually made between the capillary and cavernous forms. The kidney is the most commonly affected organ of the urinary tract, because it can be found in the lamina propria of the pelvis (48.7%), the pyramid of the kidney (42.1%), and the renal cortex (9.2%).2 Most renal hemangiomas are unilateral and have no sex predilection; affected patients range in age from 4 days to 70 years old,1,3 with a peak incidence between 30 and 40 years of age.4 Most pediatric cases have been reported in the right kidney. Renal hemangiomas tend to be small and usually vary between 1 and 2 cm in diameter. Although patients with renal hemangiomas can be asymptomatic, spontaneous hematuria is the most common presenting symptom. The hematuria ranges from microscopic to gross and can even be life-threatening, with blood transfusions required.5 Hemorrhage is thought to occur as a result of thrombosis, infarction, or renewed vessel growth after erosion of the urothelium. Flank pain is another common symptom that can vary from slight to colicky pain. On physical examination, a flank mass can 0090-4295/09/$34.00 443.e1 doi:10.1016/j.urology.2008.02.049
Figure 1. (A) CT scan without intravenous contrast showing large, soft-tissue mass in left renal parenchyma. (B) CT scan with intravenous contrast showing sharp border to kidney tissue. Renal pedicle appears to be involved and tumor thrombus in left renal vein is visible.
be palpable and may even be occasionally associated with flank tenderness. Most renal hemangiomas are small and might not be seen on imaging studies. The most common ultrasound appearance is a hyperechoic lesion6,7; however, 1 case was of a hypoechoic lesion.8 The ultrasound examination in our case revealed a large quantity of vessel walls and stromatic tissue contained in the pseudotumor. CT and magnetic resonance imaging might help to differentiate hemangioma from other renal or retroperitoneal neoplasms. On CT with contrast injection, a renal cavernous hemangioma typically appears as a mass with low density.7–9 For capillary hemangioma, one might instead see a small, enhancing, heterogeneous solid mass.10 Magnetic resonance imaging results were reported for a small number of cavernous hemangioma cases. In 1 case, the mass was described as having lowsignal intensity on T1-weighted images and high-signal intensity on T2-weighted images. In addition, several low-intensity ring or tube-like areas resembling flow voids were seen in the mass.8 In the other cases, the mass had 443.e2
Figure 2. (A) On hematoxylin-eosin staining (100⫻), hemangioma seen that is partly racemosum hemangioma, partly cavernous hemangioma, and partly capillary hemangioma. (B) Thrombosis with organization seen in left renal vein. Original magnification ⫻40.
a similar isointensity to that of the surrounding soft tissue, but fluid attenuation was also observed with enhancement.9 Selective renal angiography was recommended by many investigators as the best diagnostic method to detect renal hemangiomas.4,10 –12 The angiographic findings are generally characterized by hypervascularity, but hypovascularity with slow blood flow in the tumor can also be seen.4,5,13 In differentiating between hemangioma and renal carcinoma using angiography, only carcinomas tend to have a mass effect, staining or pooling during the nephrogram phase, and an irregular vascular encasement.14 Ureterorenoscopy could help with the diagnosis, as well as with concomitant treatment of the hemangioma, such that the patient might avoid partial or complete nephrectomy. One can see a clear visualization of mulberry-like lesions over the papilla, characteristic of hemangioma1; however, sometimes only blood clots can be found. Moody et al.7 described the use of percutaneous needle biopsy before surgery, but this technique was not sufficient to lead to a correct diagnosis. UROLOGY 73 (2), 2009
Currently most cases are still diagnosed after surgical exploration and pathologic review, because it has been difficult to differentiate benign renal hemangioma from renal cancer with current imaging techniques.7,10,14 On pathologic examination, the gross specimen is typically described as dark brown and friable with clotted blood in the vasculature. On histopathologic examination, hemangiomas are usually described as epithelium-lined spaces of varying sizes (small, capillary; large, cavernous) that are filled with red blood cells and thrombosis.15 Calcifications are rarely seen.7 Our report describes the first case of renal cavernous hemangioma with renal vein thrombosis. The differential diagnosis of renal hemangioma must include papillary necrosis, ectopic papilla, hemorrhagic papillitis, and urothelial carcinoma, among others. Asymptomatic hemangiomas tend to resolve spontaneously. If the lesion is detected on arteriography, selective embolization might be useful as the primary treatment, but additional treatment could be required in some patients.4,10,13 If the lesion is identified solely in the renal pelvis, ureterorenoscopy, with coagulation of the renal hemangioma by electrocautery or yttrium-aluminum-garnet or holmium laser, is effective, with similar success rates.1,6 For severe hematuria, partial nephrectomy or total nephrectomy is still considered the ideal treatment modality. It is difficult to diagnosis a large renal cavernous hemangioma before the surgery, because most patients with this type of flank mass will undergo nephrectomy to either control profuse bleeding or for suspected malignancy. In our case, an extremely large renal cavernous hemangioma with a renal vein thrombus that extended to the inferior vena cava was identified.
CONCLUSIONS Renal cavernous hemangioma is a rare disease. This is the first reported case of a large renal cavernous hemangioma with renal vena thrombosis. As a clinical algorithm, we suggest the use of CT, magnetic resonance imaging, and angiography for the workup of a renal mass. We recommend that all suspicious renal masses should be treated as suspected malignancies and should be excised with either
UROLOGY 73 (2), 2009
partial or total nephrectomy, with the extent of surgery being determined by the size and location of the kidney mass. Other potential treatment options include ureterorenoscopic coagulation and selective renal embolization in treating selective patients with this renal anomaly if the pathologic diagnosis has been confirmed.
References 1. Daneshmand S, and Huffman JL: Endoscopic management of renal hemangioma. J Urol 167: 488-489, 2002. 2. Jahn H, and Nissen HM: Haemangioma of the urinary tract: review of the literature. Br J Urol 68: 113-117, 1991. 3. Akel SR, Rassi A, Tawil A, et al: Isolated renal haemangioma in children: presentation and management. BJU Int 90: 758-760, 2002. 4. Ekelund L, and Gothlin J: Renal hemangiomas: an analysis of 13 cases diagnosed by angiography. AJR Am J Roentgenol 125: 788794, 1975. 5. Costa Neto TF, Renteria JM, and Di Biase Filho G: Renal hemangioma. Int Braz J Urol 30: 216-218, 2004. 6. Fujii Y, Ajima J, Oka K, et al: Benign renal tumors detected among healthy adults by abdominal ultrasonography. Eur Urol 27: 124127, 1995. 7. Moody JA, Litwin MS, Cochran ST, et al: Renal cavernous hemangioma in a patient with the acquired immunodeficiency syndrome. J Urol 156: 1759-1760, 1996. 8. Geenen RW, Den Bakker MA, Bangma CH, et al: Sonography, CT, and MRI of giant cavernous hemangioma of the kidney: correlation with pathologic findings. AJR Am J Roentgenol 182: 411-414, 2004. 9. Haas CA, Resnick MI, and Abdul-Karim FW: Cavernous hemangioma presenting as a renal hilar mass. J Urol 160(6 Pt 1): 21392140, 1998. 10. Hull GW III, Genega EM, and Sogani PC: Intravascular capillary hemangioma presenting as a solid renal mass. J Urol 162(3 Pt 1): 784-785, 1999. 11. Abercrombie JF, Holmes SA, and Ball AJ: Diagnosis and management of renal angioma. J R Soc Med 85: 625-627, 1992. 12. Michel F, Gattegno B, Valade S, et al: Renal haemangioma: emergency partial nephrectomy. Br J Urol 55: 336-337, 1983. 13. Gordon R, Rosenmann E, Barzilay B, et al: Correlation of selective angiography and pathology in cavernous hemangioma of the kidney. J Urol 115: 608-609, 1976. 14. Crissey MM, Kearnery GP, Sos T, et al: Renal hemangioma: diagnostic aspects and management techniques. Cardovasc Intervent Radiol 3: 170-174, 1980. 15. Gupta NP, Kumar P, Goel R, et al: Renal sinus hemangioma simulating renal mass: a diagnostic challenge. Int Urol Nephrol 36: 485-487, 2004.
443.e3
R
enal hemangioma is a rare, benign, vascular malformation also known as renal angiomatous dysplasia. We report the first case of renal cavernous hemangioma complicated by thrombosis in the renal vein.
CASE REPORT A 17-year-old boy was admitted to our hospital with hematuria of 5 days’ duration. On physical examination, the patient was thin with a large palpable mass below the left costal margin that was nontender. Serum studies were normal, and urinalysis revealed red blood cell count of 0 to 5 per high power field and numerous white blood cells. Renal ultrasonography revealed a solid renal mass with clear demarcations in the middle and lower poles of the left kidney, with some hypoechoic/cystic components. Computed tomography (CT) revealed an 8 ⫻ 7 ⫻ 8-cm, low-density, left kidney tumor that was slightly enhanced at 72.09 Hounsfield units after contrast injection (Fig. 1). Soft tissue consistent with left renal vein thrombosis was also seen. During radical nephrectomy, engorged veins were seen on the renal capsule surface. The renal vein thrombosis extended to just within the inferior vena cava. Frozen section analysis revealed a renal hemangioma with a renal vein thrombosis. Gross pathologic examination revealed a 10 ⫻ 7 ⫻ 8-cm mass within the left kidney that had replaced most of the middle and lower
This study was supported by grants from the Natural Science Fund of Hunan Province (grant 05JJ30056) and the Chang Sha Scientific Bureau 2006 fund. From the Department of Urology, Second Xiangya Hospital, Central South University School of Medicine, Xiangya, People’s Republic of China; and Division of Pediatric Urology, Children’s Hospital Los Angeles, University of Southern California Keck School of Medicine, Los Angeles, California Address for correspondence: Chester J. Koh, M.D., Division of Pediatric Urology, Children’s Hospital Los Angeles, 4650 Sunset Boulevard, Mailstop 114, Los Angeles, CA 90027. E-mail: [email protected] Submitted: January 8, 2008, accepted (with revisions): February 21, 2008
© 2009 Elsevier Inc. All Rights Reserved
poles, with involvement of the renal hilum. The left renal vein contained a gray, tenacious thrombosis. Histopathologic examination revealed a renal cavernous hemangioma with extensive intratumoral hemorrhage and chronic renal vein thrombosis (Fig. 2). Immunohistochemical stains showed reactivity of the tumor cells to factor Vim and CD34 and no reactivity to HHF35, epithelial membrane antigen, LCK, or S-100, confirming the benign nature of the tumor. After 1 year, the patient was in good health, and follow-up studies showed no evidence of recurrence.
COMMENT Renal hemangioma is a rare, benign, vascular malformation also known as renal angiomatous dysplasia. Virchow reported the first case in 1867.1 A distinction is usually made between the capillary and cavernous forms. The kidney is the most commonly affected organ of the urinary tract, because it can be found in the lamina propria of the pelvis (48.7%), the pyramid of the kidney (42.1%), and the renal cortex (9.2%).2 Most renal hemangiomas are unilateral and have no sex predilection; affected patients range in age from 4 days to 70 years old,1,3 with a peak incidence between 30 and 40 years of age.4 Most pediatric cases have been reported in the right kidney. Renal hemangiomas tend to be small and usually vary between 1 and 2 cm in diameter. Although patients with renal hemangiomas can be asymptomatic, spontaneous hematuria is the most common presenting symptom. The hematuria ranges from microscopic to gross and can even be life-threatening, with blood transfusions required.5 Hemorrhage is thought to occur as a result of thrombosis, infarction, or renewed vessel growth after erosion of the urothelium. Flank pain is another common symptom that can vary from slight to colicky pain. On physical examination, a flank mass can 0090-4295/09/$34.00 443.e1 doi:10.1016/j.urology.2008.02.049
Figure 1. (A) CT scan without intravenous contrast showing large, soft-tissue mass in left renal parenchyma. (B) CT scan with intravenous contrast showing sharp border to kidney tissue. Renal pedicle appears to be involved and tumor thrombus in left renal vein is visible.
be palpable and may even be occasionally associated with flank tenderness. Most renal hemangiomas are small and might not be seen on imaging studies. The most common ultrasound appearance is a hyperechoic lesion6,7; however, 1 case was of a hypoechoic lesion.8 The ultrasound examination in our case revealed a large quantity of vessel walls and stromatic tissue contained in the pseudotumor. CT and magnetic resonance imaging might help to differentiate hemangioma from other renal or retroperitoneal neoplasms. On CT with contrast injection, a renal cavernous hemangioma typically appears as a mass with low density.7–9 For capillary hemangioma, one might instead see a small, enhancing, heterogeneous solid mass.10 Magnetic resonance imaging results were reported for a small number of cavernous hemangioma cases. In 1 case, the mass was described as having lowsignal intensity on T1-weighted images and high-signal intensity on T2-weighted images. In addition, several low-intensity ring or tube-like areas resembling flow voids were seen in the mass.8 In the other cases, the mass had 443.e2
Figure 2. (A) On hematoxylin-eosin staining (100⫻), hemangioma seen that is partly racemosum hemangioma, partly cavernous hemangioma, and partly capillary hemangioma. (B) Thrombosis with organization seen in left renal vein. Original magnification ⫻40.
a similar isointensity to that of the surrounding soft tissue, but fluid attenuation was also observed with enhancement.9 Selective renal angiography was recommended by many investigators as the best diagnostic method to detect renal hemangiomas.4,10 –12 The angiographic findings are generally characterized by hypervascularity, but hypovascularity with slow blood flow in the tumor can also be seen.4,5,13 In differentiating between hemangioma and renal carcinoma using angiography, only carcinomas tend to have a mass effect, staining or pooling during the nephrogram phase, and an irregular vascular encasement.14 Ureterorenoscopy could help with the diagnosis, as well as with concomitant treatment of the hemangioma, such that the patient might avoid partial or complete nephrectomy. One can see a clear visualization of mulberry-like lesions over the papilla, characteristic of hemangioma1; however, sometimes only blood clots can be found. Moody et al.7 described the use of percutaneous needle biopsy before surgery, but this technique was not sufficient to lead to a correct diagnosis. UROLOGY 73 (2), 2009
Currently most cases are still diagnosed after surgical exploration and pathologic review, because it has been difficult to differentiate benign renal hemangioma from renal cancer with current imaging techniques.7,10,14 On pathologic examination, the gross specimen is typically described as dark brown and friable with clotted blood in the vasculature. On histopathologic examination, hemangiomas are usually described as epithelium-lined spaces of varying sizes (small, capillary; large, cavernous) that are filled with red blood cells and thrombosis.15 Calcifications are rarely seen.7 Our report describes the first case of renal cavernous hemangioma with renal vein thrombosis. The differential diagnosis of renal hemangioma must include papillary necrosis, ectopic papilla, hemorrhagic papillitis, and urothelial carcinoma, among others. Asymptomatic hemangiomas tend to resolve spontaneously. If the lesion is detected on arteriography, selective embolization might be useful as the primary treatment, but additional treatment could be required in some patients.4,10,13 If the lesion is identified solely in the renal pelvis, ureterorenoscopy, with coagulation of the renal hemangioma by electrocautery or yttrium-aluminum-garnet or holmium laser, is effective, with similar success rates.1,6 For severe hematuria, partial nephrectomy or total nephrectomy is still considered the ideal treatment modality. It is difficult to diagnosis a large renal cavernous hemangioma before the surgery, because most patients with this type of flank mass will undergo nephrectomy to either control profuse bleeding or for suspected malignancy. In our case, an extremely large renal cavernous hemangioma with a renal vein thrombus that extended to the inferior vena cava was identified.
CONCLUSIONS Renal cavernous hemangioma is a rare disease. This is the first reported case of a large renal cavernous hemangioma with renal vena thrombosis. As a clinical algorithm, we suggest the use of CT, magnetic resonance imaging, and angiography for the workup of a renal mass. We recommend that all suspicious renal masses should be treated as suspected malignancies and should be excised with either
UROLOGY 73 (2), 2009
partial or total nephrectomy, with the extent of surgery being determined by the size and location of the kidney mass. Other potential treatment options include ureterorenoscopic coagulation and selective renal embolization in treating selective patients with this renal anomaly if the pathologic diagnosis has been confirmed.
References 1. Daneshmand S, and Huffman JL: Endoscopic management of renal hemangioma. J Urol 167: 488-489, 2002. 2. Jahn H, and Nissen HM: Haemangioma of the urinary tract: review of the literature. Br J Urol 68: 113-117, 1991. 3. Akel SR, Rassi A, Tawil A, et al: Isolated renal haemangioma in children: presentation and management. BJU Int 90: 758-760, 2002. 4. Ekelund L, and Gothlin J: Renal hemangiomas: an analysis of 13 cases diagnosed by angiography. AJR Am J Roentgenol 125: 788794, 1975. 5. Costa Neto TF, Renteria JM, and Di Biase Filho G: Renal hemangioma. Int Braz J Urol 30: 216-218, 2004. 6. Fujii Y, Ajima J, Oka K, et al: Benign renal tumors detected among healthy adults by abdominal ultrasonography. Eur Urol 27: 124127, 1995. 7. Moody JA, Litwin MS, Cochran ST, et al: Renal cavernous hemangioma in a patient with the acquired immunodeficiency syndrome. J Urol 156: 1759-1760, 1996. 8. Geenen RW, Den Bakker MA, Bangma CH, et al: Sonography, CT, and MRI of giant cavernous hemangioma of the kidney: correlation with pathologic findings. AJR Am J Roentgenol 182: 411-414, 2004. 9. Haas CA, Resnick MI, and Abdul-Karim FW: Cavernous hemangioma presenting as a renal hilar mass. J Urol 160(6 Pt 1): 21392140, 1998. 10. Hull GW III, Genega EM, and Sogani PC: Intravascular capillary hemangioma presenting as a solid renal mass. J Urol 162(3 Pt 1): 784-785, 1999. 11. Abercrombie JF, Holmes SA, and Ball AJ: Diagnosis and management of renal angioma. J R Soc Med 85: 625-627, 1992. 12. Michel F, Gattegno B, Valade S, et al: Renal haemangioma: emergency partial nephrectomy. Br J Urol 55: 336-337, 1983. 13. Gordon R, Rosenmann E, Barzilay B, et al: Correlation of selective angiography and pathology in cavernous hemangioma of the kidney. J Urol 115: 608-609, 1976. 14. Crissey MM, Kearnery GP, Sos T, et al: Renal hemangioma: diagnostic aspects and management techniques. Cardovasc Intervent Radiol 3: 170-174, 1980. 15. Gupta NP, Kumar P, Goel R, et al: Renal sinus hemangioma simulating renal mass: a diagnostic challenge. Int Urol Nephrol 36: 485-487, 2004.
443.e3