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Renal Liposarcoma: Case Report
Vol. 115, June Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
RENAL LIPOSARCOMA: CASE REPORT JESUS Y. CANO
AND
RONALD A. D'ALTORIO
From the Department of Radiology, The Western Pennsylvania Hospital, Pittsburgh, Pennsylvania
ABSTRACT
A case of renal liposarcoma is described. The clinical presentation, angiographic findings and treatment are discussed. Liposarcoma is one of the most common soft tissue sarcomas but it is rarely encountered as an isolated lesion of the kidney. Although the diagnosis is usually established postoperatively, the characteristic clinical presentation and the frequent association with tuberous sclerosis may help determine the diagnosis preoperatively. There are also angiographic changes, which may be helpful in establishing a diagnosis. Since there have been few reports in the English literature of liposarcoma of the kidney 1-• we herein present the following case with emphasis on the arteriographic findings and some comment about treatment of these tumors.
the world literature in 1973 Lien found 35 cases of renal liposarcoma and added 2 of his own. 1 Williams and Savage noted that 10 of 29 cases of renal liposarcoma were associated with tuberous sclerosis. 2 This implied a generalized defect of
CASE REPORT
J. S., a 60-year-old white woman, was in good health until April 4, 197 4 when severe pain developed in the back and right flank, associated with nausea and vomiting. She was admitted to the hospital and examination of the abdomen revealed a large mass and tenderness in the right flank. The remainder of the physical examination was unremarkable. An excretory urogram (IVP) revealed a large mass in the upper portion of the right kidney compressing the caliceal system (fig. 1). Multiple gallstones were noted. On April 10 she underwent an exploratory operation. A large tumor of the right kidney measured 25 by 5 by 8 cm. The tumor bled easily. No metastases were noted in the abdomen. A biopsy was performed and pathological examination revealed a well differentiated liposarcoma of the kidney (fig. 2). No attempt was made to remove the tumor and the patient was treated postoperatively with 2,440 rads of cobalt 60 radiation therapy during the following 3 weeks. On November 1 she was readmitted to the hospital for further evaluation. An arteriogram showed a large mass in the upper pole of the right kidney. There was stretching and displacement of the intrarenal arterial branches. Neovascularity and pooling of the contrast material were noted in portions of the tumor (fig. 3). An inferior venacavogram was performed. The tumor displaced the vena cava but did not invade it. The patient was started on chemotherapy consisting of 600 mg. cyclophosphamide, 1 mg. cosmegen and 2 mg. vincristine. She received 2 courses of this regimen and on January 9 and March FIG. 1. IVP shows large mass of upper pole of right kidney and 4, 1975 received a repeat course with the addition of compression of callceal system. Multiple faceted gallstones are presadriamycin. On June 4 a repeat arteriogram showed considera- ent. (Gallstones also are seen in figures 3 and 4.) ble shrinkage of the tumor (fig. 4). The patient was re-explored on June 25 and residual tumor could be seen on the upper lateral aspect of the right kidney, although the biopsies of this tissue combination with the production of hamartomas and area revealed no evidence of tumor. Convalescence has re- renal liposarcomas occurring as a malignant form of hamarmained uneventful. toma. Conversely, renal liposarcoma can occur without tuberous sclerosis. Renal liposarcoma is usually associated with pain, renal DISCUSSION mass, anorexia and weight loss. Symptoms may be present for Although liposarcoma is the most common neoplasm of the as long as several years. These tumors may become large but retroperitoneum, it is a rare tumor of the kidney. In a review of metastases are uncommon. One of the most interesting aspects of these tumors is the absence of hematuria even when the Accepted for publication November 21, 1975. tumor attains a large size. Apparently this is because the 747
748
CANO AND D'ALTORIO
tumors are usually located peripherally in the kidney. Although the tumor was more centrally located in our patient there was still an absence of hematuria. Arteriography was done in a few of the reported cases and in each instance the description was one of neovascularity, pooling of contrast material and arteriovenous communication similar to the angiographic findings of hypernephroma. However, unlike hypernephromas, there were stretching of the vessels about the tumor and relatively little tumor neovascularity extending into the mass. Most of the vascularity is about the periphery of the tumor, which produces a cystic-like picture probably owing to hemorrhage into the tumor because of the highly vascular nature and large amount of fat in the tumor. An operation is the treatment of choice when the tumor is confined to the kidney. The survival rate of patients with resectable tumors is reasonably good with several long-term survivals reported! Unfortunately, the tumor frequently extends into the retroperitoneum, rendering operation impossible. These patients have a poor prognosis and there is a need for improved therapy in such cases. The effect of ionizing radiation on liposarcoma is extremely variable, with only an occasional favorable response having been reported. Unfortunately, the tumor is usually radioresistant. • There are few reports in the literature evaluating chemotherapy in the treatment of renal liposarcoma. Most reports
FIG. 2. Well differentiated liposarcoma of kidney composed of adult fat cells and immature lipoblasts. Nuclei are hyperchromatic and contain small amount of finely vacuolated cytoplasm. H & E, reduced from x240.
of soft tissue sarcomas rece1vmg chemotherapy have been disappointing.• James and associates reported a remarkable result of treatment of a massive recurrent intra-abdominal liposarcoma with a combination of vincristine and cyclophosphamide. 5 The tumor completely disappeared and the patient was well 1 ½ years later. In our case, marked reduction in the tumor size as demonstrated by arteriography was obtained after multiple drug chemotherapy. Although this tumor was still not resectable others receiving such therapy may become resectable. There are too few cases available for evaluating the proper therapy but a combination of chemotherapy and subsequent operation may indeed be the treatment of choice for large renal liposarcomas.
FIG. 4. Post-treatment angiogram reveals marked shrinkage of tumor. There are still tumor vessels and pooling of contrast m periphery of tumor (arrow).
FIG. 3. A, selective arteriogram shows stretching and displacement of vessels about tumor. There are tumor vessels extending into tumor (straight arrow) and pooling of contrast material (crossed arrow and arrowhead). B, note relative avascularity in tumor substance producing cystlike appearance.
Lien, \rV. I\11. Liposarcoma of ths kid~ey. :?ostgrad. ]:Vied. J., 49: 660, 1973 2. Williams, J P. and Savage, P. T .. Liposarcoma of kidney. Brit. J. Surg., 46: 225, 1958-1959. 3. MacDermott, E. N. and Kennedy, J. D.: Liposarcoma of the kidney.
32: 282, 1960. 4, Syittle) IvL F J>Jev.,ton, K. A. and 1n,ac,,t,1z1e. D. I-L. Liposa:rco1na. A review of 60 cases. Brit. J. Canser 24: 1970. 5. James, D. H., Jr., Johnson, W. W. and Wrenn, E. L., Jr.: Effective chemotherapy of an abdominal liposarcoma. J. Pediat., 68: 311, 1966. 1
THE JOURNAL OF UROLOGY
Copyright© 1976 by The Williams & Wilkins Co.
RENAL LIPOSARCOMA: CASE REPORT JESUS Y. CANO
AND
RONALD A. D'ALTORIO
From the Department of Radiology, The Western Pennsylvania Hospital, Pittsburgh, Pennsylvania
ABSTRACT
A case of renal liposarcoma is described. The clinical presentation, angiographic findings and treatment are discussed. Liposarcoma is one of the most common soft tissue sarcomas but it is rarely encountered as an isolated lesion of the kidney. Although the diagnosis is usually established postoperatively, the characteristic clinical presentation and the frequent association with tuberous sclerosis may help determine the diagnosis preoperatively. There are also angiographic changes, which may be helpful in establishing a diagnosis. Since there have been few reports in the English literature of liposarcoma of the kidney 1-• we herein present the following case with emphasis on the arteriographic findings and some comment about treatment of these tumors.
the world literature in 1973 Lien found 35 cases of renal liposarcoma and added 2 of his own. 1 Williams and Savage noted that 10 of 29 cases of renal liposarcoma were associated with tuberous sclerosis. 2 This implied a generalized defect of
CASE REPORT
J. S., a 60-year-old white woman, was in good health until April 4, 197 4 when severe pain developed in the back and right flank, associated with nausea and vomiting. She was admitted to the hospital and examination of the abdomen revealed a large mass and tenderness in the right flank. The remainder of the physical examination was unremarkable. An excretory urogram (IVP) revealed a large mass in the upper portion of the right kidney compressing the caliceal system (fig. 1). Multiple gallstones were noted. On April 10 she underwent an exploratory operation. A large tumor of the right kidney measured 25 by 5 by 8 cm. The tumor bled easily. No metastases were noted in the abdomen. A biopsy was performed and pathological examination revealed a well differentiated liposarcoma of the kidney (fig. 2). No attempt was made to remove the tumor and the patient was treated postoperatively with 2,440 rads of cobalt 60 radiation therapy during the following 3 weeks. On November 1 she was readmitted to the hospital for further evaluation. An arteriogram showed a large mass in the upper pole of the right kidney. There was stretching and displacement of the intrarenal arterial branches. Neovascularity and pooling of the contrast material were noted in portions of the tumor (fig. 3). An inferior venacavogram was performed. The tumor displaced the vena cava but did not invade it. The patient was started on chemotherapy consisting of 600 mg. cyclophosphamide, 1 mg. cosmegen and 2 mg. vincristine. She received 2 courses of this regimen and on January 9 and March FIG. 1. IVP shows large mass of upper pole of right kidney and 4, 1975 received a repeat course with the addition of compression of callceal system. Multiple faceted gallstones are presadriamycin. On June 4 a repeat arteriogram showed considera- ent. (Gallstones also are seen in figures 3 and 4.) ble shrinkage of the tumor (fig. 4). The patient was re-explored on June 25 and residual tumor could be seen on the upper lateral aspect of the right kidney, although the biopsies of this tissue combination with the production of hamartomas and area revealed no evidence of tumor. Convalescence has re- renal liposarcomas occurring as a malignant form of hamarmained uneventful. toma. Conversely, renal liposarcoma can occur without tuberous sclerosis. Renal liposarcoma is usually associated with pain, renal DISCUSSION mass, anorexia and weight loss. Symptoms may be present for Although liposarcoma is the most common neoplasm of the as long as several years. These tumors may become large but retroperitoneum, it is a rare tumor of the kidney. In a review of metastases are uncommon. One of the most interesting aspects of these tumors is the absence of hematuria even when the Accepted for publication November 21, 1975. tumor attains a large size. Apparently this is because the 747
748
CANO AND D'ALTORIO
tumors are usually located peripherally in the kidney. Although the tumor was more centrally located in our patient there was still an absence of hematuria. Arteriography was done in a few of the reported cases and in each instance the description was one of neovascularity, pooling of contrast material and arteriovenous communication similar to the angiographic findings of hypernephroma. However, unlike hypernephromas, there were stretching of the vessels about the tumor and relatively little tumor neovascularity extending into the mass. Most of the vascularity is about the periphery of the tumor, which produces a cystic-like picture probably owing to hemorrhage into the tumor because of the highly vascular nature and large amount of fat in the tumor. An operation is the treatment of choice when the tumor is confined to the kidney. The survival rate of patients with resectable tumors is reasonably good with several long-term survivals reported! Unfortunately, the tumor frequently extends into the retroperitoneum, rendering operation impossible. These patients have a poor prognosis and there is a need for improved therapy in such cases. The effect of ionizing radiation on liposarcoma is extremely variable, with only an occasional favorable response having been reported. Unfortunately, the tumor is usually radioresistant. • There are few reports in the literature evaluating chemotherapy in the treatment of renal liposarcoma. Most reports
FIG. 2. Well differentiated liposarcoma of kidney composed of adult fat cells and immature lipoblasts. Nuclei are hyperchromatic and contain small amount of finely vacuolated cytoplasm. H & E, reduced from x240.
of soft tissue sarcomas rece1vmg chemotherapy have been disappointing.• James and associates reported a remarkable result of treatment of a massive recurrent intra-abdominal liposarcoma with a combination of vincristine and cyclophosphamide. 5 The tumor completely disappeared and the patient was well 1 ½ years later. In our case, marked reduction in the tumor size as demonstrated by arteriography was obtained after multiple drug chemotherapy. Although this tumor was still not resectable others receiving such therapy may become resectable. There are too few cases available for evaluating the proper therapy but a combination of chemotherapy and subsequent operation may indeed be the treatment of choice for large renal liposarcomas.
FIG. 4. Post-treatment angiogram reveals marked shrinkage of tumor. There are still tumor vessels and pooling of contrast m periphery of tumor (arrow).
FIG. 3. A, selective arteriogram shows stretching and displacement of vessels about tumor. There are tumor vessels extending into tumor (straight arrow) and pooling of contrast material (crossed arrow and arrowhead). B, note relative avascularity in tumor substance producing cystlike appearance.
Lien, \rV. I\11. Liposarcoma of ths kid~ey. :?ostgrad. ]:Vied. J., 49: 660, 1973 2. Williams, J P. and Savage, P. T .. Liposarcoma of kidney. Brit. J. Surg., 46: 225, 1958-1959. 3. MacDermott, E. N. and Kennedy, J. D.: Liposarcoma of the kidney.
32: 282, 1960. 4, Syittle) IvL F J>Jev.,ton, K. A. and 1n,ac,,t,1z1e. D. I-L. Liposa:rco1na. A review of 60 cases. Brit. J. Canser 24: 1970. 5. James, D. H., Jr., Johnson, W. W. and Wrenn, E. L., Jr.: Effective chemotherapy of an abdominal liposarcoma. J. Pediat., 68: 311, 1966. 1