Vol. 116, December
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1976 by The Williams & Wilkins.Co.
BENIGN RENAL ANGIOMYOLIPOMA WITH REGIONAL LYMPH NODE INVOLVEMENT F. MICHAEL BUSCH, C. JACK BARK
AND
H. ROBERT CLYDE
From the Departments of Urology and Pathology, Alvarado Community Hospital and the Department of Urology, Naval Regional Medical Center, San Diego, California
ABSTRACT
The 2 cases reported herein involve benign renal angiomyolipomas, showing the same angiomyolipoma changes in the regional lymph nodes. It is concluded that these lymph node changes are caused by a multicentric origin of the angiomyolipoma rather than true metastasis. A plea is made not to over treat these tumors since all evidence points to the fact that they are indeed benign. We have seen 2 patients with benign renal angiomyolipomas and involvement of regional lymph nodes. An angiomyolipoma is a benign neoplastic tumor or a hamartial lesion containing tissues normally present in the organ. As the name implies the tumors are composed of variable mixtures of smooth muscle, blood vessels and fat. It is impossible to determine if they arise as an anomaly of development or as a true neoplasm.'
much more common and usually presents as a large, unilateral, symptomatic renal mass, frequently associated with intrarenal or retroperitoneal hemorrhage because of its vascular nature. The hemorrhage with resultant pain and/or mass prompts these patients to seek medical attention. This type is completely independent of the tuberous sclerosis complex but is histologically indistinguishable from the first type. 2
FIG. 1. A, 3 components of tumor are seen, as well as scattered tubules and glomerulus. B, regional lymph nodes, lymphoid tissue and tumor components similar to kidney.
There have been about 200 cases of this rare tumor reported to date. The reader is referred to the complete review of the literature by Bissada and associates• and the excellent management protocol of McCullough and associates. 4 Price and Mostofi have described thoroughly the gross and microscopic features of the lesion. 5 Involvement of regional lymph nodes Accepted for publication May 21, 1976. Read at annual meeting of Western Section, American Urological with similar malformation is not well recognized and often Association, Coronado, California, February 22-26, 1976. raises the fear of metastatic disease.•
There are 2 distinct types of renal angiomyolipomas. The first type is seen in about 50 per cent of the patients with the tuberous sclerosis complex and are small, asymptomatic, usually bilateral and found only at autopsy. The second type is
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BUSCH, BARK AND CLYDE
Fm. 2. A, renal tubules and 3 tumor components. B, regional lymph node with similar involvement CASE REPORTS
DISCUSSION
Case 1. A 21-year-old male college student was hospitalized on May 15, 1973 with a painful, large, right upper quadrant mass 12 hours in duration. All laboratory studies, including urinalysis, were normal. Nephrotomograms and ultrasound studies demonstrated a solid, avascular mass in the central portion of the right kidney. The left kidney was normal. Preoperative diagnosis was renal carcinoma and right radical nephrectomy, including regional lymphadenectomy, was done. Examination of the specimen revealed an 11 by 11 by 6.5 cm. mass in the upper pole of the right kidney. On cross section the entire central portion of the mass was hemorrhagic. Microscopic examination revealed an angiomyolipoma containing various amounts of bland smooth muscle, blood vessels and fatty components (fig. 1, A). Also noted were microscopic areas of angiomyolipoma nodules away from the main mass. Of even greater surprise was the involvement of regional lymph nodes with the angiomyolipoma occupying as much as half of the architecture of several nodes present. Again, little cellular atypia was noted and all 3 tumor components were present (fig. 1, B). No evidence of malignancy or distant metastasis nor any stigmas of the tuberous sclerosis was present in this patient. Case 2. A 49-year-old woman presented with an asymptomatic right upper abdominal mass, which she had been aware of for about a year. She had none of the stigmas of the tuberous sclerosis complex. An excretory urogram was negative but angiography revealed a large vascular mass arising from the lower pole of the right kidney. Preoperative diagnosis was adenocarcinoma and right radical nephrectomy was done on December 7, 1973. Gross and microscopic findings were typical of an angiomyolipoma with the same changes noted in 1 of the 16 regional lymph nodes (fig. 2). There has been no evidence of malignancy.
The finding of regional lymph node involvement in a case of renal angiomyolipoma leads to the question of metastasis versus multicentric origin. The 2 cases reported were reviewed by persons at the Genitourinary Branch of the Armed Forces Institute of Pathology and they concurred with the diagnosis of a benign angiomyolipoma. They commented on having seen several cases with typical lesions of renal angiomyolipomas with regional node involvement in the last several years. They noted the benign appearance of the renal and lymph node lesions, rather than a sarcomatous picture of any of the 3 elements. 7 They also concurred in the authors' impression that the evidence points to the fact that the nodal involvement by the tumor represents multicentricity of the angiomyolipoma. Therefore, the main thrust of our report is to reassure urologists who have removed a kidney containing a benign angiomyolipoma, associated with the same changes in the regional nodes. Although long-term followups are not available we believe that the patient can be assured that the prognosis is not grave and that it is not necessary to give further treatment, such as radiation therapy or chemotherapy. This theory is confirmed by noting that not a single case of distant metastasis or death from a renal angiomyolipoma has been reported. 8 ' 9 REFERENCES
Rusche, C.: Renal hamartoma (angiomyolipoma): report of three cases. J. Urol., 67: 823, 1952. 2, Hajdu, S. I. and Foote, F. W., Jr.: Angiomyolipoma of the kidney: report of 27 cases and review of the literature. J. Urol., 102: 396, 1969. 3. Bissada, N. K., White, H.J., Sun, C. N., Smith, P. L., Barbour, G. L. and Redman, J. F.: Tuberous sclerosis complex and renal angiomyolipoma. Urology, 6: 105, 1975. 1.
BENIGN RENAL ANGIOMYOLIPOMA WITH LYMPH NODE INVOLVEMENT
4. McCullough, D. L., Scott, R., Jr. and Seybold, H. M.: Renal angiomyolipoma (hamartoma): review of the literature and report of 7 cases. J. Urol., 105: 32, 1971. 5. Price, E. B., Jr. and Mostofi, F. K.: Symptomatic angiomyolipoma of the kidney. Cancer, 18: 761, 1965. 6. Scott, M. B., Halpern, M. and Cosgrove, M. D.: Renal angiomyolipoma, two varieties. Urology, 6: 768, 1975.
717
7. Genitourinary Branch, Armed Forces Institute of Pathology, personal communication, 1973-1974. 8. Allen, T. D. and Risk, W.: Renal angiomyolipoma. J. Urol., 94: 203, 1.965. 9. Waldbaum, R. S., Marshall, V. F., Muecke, E. C. and Watson, R.: Tuberous sclerosis with bilateral angiomyolipomas of the kidney: case report with 8-year followup. J. Urol., 106: 180, 1971.
THE JOURNAL OF UROLOGY
Printed in U.S.A.
Copyright© 1976 by The Williams & Wilkins.Co.
BENIGN RENAL ANGIOMYOLIPOMA WITH REGIONAL LYMPH NODE INVOLVEMENT F. MICHAEL BUSCH, C. JACK BARK
AND
H. ROBERT CLYDE
From the Departments of Urology and Pathology, Alvarado Community Hospital and the Department of Urology, Naval Regional Medical Center, San Diego, California
ABSTRACT
The 2 cases reported herein involve benign renal angiomyolipomas, showing the same angiomyolipoma changes in the regional lymph nodes. It is concluded that these lymph node changes are caused by a multicentric origin of the angiomyolipoma rather than true metastasis. A plea is made not to over treat these tumors since all evidence points to the fact that they are indeed benign. We have seen 2 patients with benign renal angiomyolipomas and involvement of regional lymph nodes. An angiomyolipoma is a benign neoplastic tumor or a hamartial lesion containing tissues normally present in the organ. As the name implies the tumors are composed of variable mixtures of smooth muscle, blood vessels and fat. It is impossible to determine if they arise as an anomaly of development or as a true neoplasm.'
much more common and usually presents as a large, unilateral, symptomatic renal mass, frequently associated with intrarenal or retroperitoneal hemorrhage because of its vascular nature. The hemorrhage with resultant pain and/or mass prompts these patients to seek medical attention. This type is completely independent of the tuberous sclerosis complex but is histologically indistinguishable from the first type. 2
FIG. 1. A, 3 components of tumor are seen, as well as scattered tubules and glomerulus. B, regional lymph nodes, lymphoid tissue and tumor components similar to kidney.
There have been about 200 cases of this rare tumor reported to date. The reader is referred to the complete review of the literature by Bissada and associates• and the excellent management protocol of McCullough and associates. 4 Price and Mostofi have described thoroughly the gross and microscopic features of the lesion. 5 Involvement of regional lymph nodes Accepted for publication May 21, 1976. Read at annual meeting of Western Section, American Urological with similar malformation is not well recognized and often Association, Coronado, California, February 22-26, 1976. raises the fear of metastatic disease.•
There are 2 distinct types of renal angiomyolipomas. The first type is seen in about 50 per cent of the patients with the tuberous sclerosis complex and are small, asymptomatic, usually bilateral and found only at autopsy. The second type is
715
716
BUSCH, BARK AND CLYDE
Fm. 2. A, renal tubules and 3 tumor components. B, regional lymph node with similar involvement CASE REPORTS
DISCUSSION
Case 1. A 21-year-old male college student was hospitalized on May 15, 1973 with a painful, large, right upper quadrant mass 12 hours in duration. All laboratory studies, including urinalysis, were normal. Nephrotomograms and ultrasound studies demonstrated a solid, avascular mass in the central portion of the right kidney. The left kidney was normal. Preoperative diagnosis was renal carcinoma and right radical nephrectomy, including regional lymphadenectomy, was done. Examination of the specimen revealed an 11 by 11 by 6.5 cm. mass in the upper pole of the right kidney. On cross section the entire central portion of the mass was hemorrhagic. Microscopic examination revealed an angiomyolipoma containing various amounts of bland smooth muscle, blood vessels and fatty components (fig. 1, A). Also noted were microscopic areas of angiomyolipoma nodules away from the main mass. Of even greater surprise was the involvement of regional lymph nodes with the angiomyolipoma occupying as much as half of the architecture of several nodes present. Again, little cellular atypia was noted and all 3 tumor components were present (fig. 1, B). No evidence of malignancy or distant metastasis nor any stigmas of the tuberous sclerosis was present in this patient. Case 2. A 49-year-old woman presented with an asymptomatic right upper abdominal mass, which she had been aware of for about a year. She had none of the stigmas of the tuberous sclerosis complex. An excretory urogram was negative but angiography revealed a large vascular mass arising from the lower pole of the right kidney. Preoperative diagnosis was adenocarcinoma and right radical nephrectomy was done on December 7, 1973. Gross and microscopic findings were typical of an angiomyolipoma with the same changes noted in 1 of the 16 regional lymph nodes (fig. 2). There has been no evidence of malignancy.
The finding of regional lymph node involvement in a case of renal angiomyolipoma leads to the question of metastasis versus multicentric origin. The 2 cases reported were reviewed by persons at the Genitourinary Branch of the Armed Forces Institute of Pathology and they concurred with the diagnosis of a benign angiomyolipoma. They commented on having seen several cases with typical lesions of renal angiomyolipomas with regional node involvement in the last several years. They noted the benign appearance of the renal and lymph node lesions, rather than a sarcomatous picture of any of the 3 elements. 7 They also concurred in the authors' impression that the evidence points to the fact that the nodal involvement by the tumor represents multicentricity of the angiomyolipoma. Therefore, the main thrust of our report is to reassure urologists who have removed a kidney containing a benign angiomyolipoma, associated with the same changes in the regional nodes. Although long-term followups are not available we believe that the patient can be assured that the prognosis is not grave and that it is not necessary to give further treatment, such as radiation therapy or chemotherapy. This theory is confirmed by noting that not a single case of distant metastasis or death from a renal angiomyolipoma has been reported. 8 ' 9 REFERENCES
Rusche, C.: Renal hamartoma (angiomyolipoma): report of three cases. J. Urol., 67: 823, 1952. 2, Hajdu, S. I. and Foote, F. W., Jr.: Angiomyolipoma of the kidney: report of 27 cases and review of the literature. J. Urol., 102: 396, 1969. 3. Bissada, N. K., White, H.J., Sun, C. N., Smith, P. L., Barbour, G. L. and Redman, J. F.: Tuberous sclerosis complex and renal angiomyolipoma. Urology, 6: 105, 1975. 1.
BENIGN RENAL ANGIOMYOLIPOMA WITH LYMPH NODE INVOLVEMENT
4. McCullough, D. L., Scott, R., Jr. and Seybold, H. M.: Renal angiomyolipoma (hamartoma): review of the literature and report of 7 cases. J. Urol., 105: 32, 1971. 5. Price, E. B., Jr. and Mostofi, F. K.: Symptomatic angiomyolipoma of the kidney. Cancer, 18: 761, 1965. 6. Scott, M. B., Halpern, M. and Cosgrove, M. D.: Renal angiomyolipoma, two varieties. Urology, 6: 768, 1975.
717
7. Genitourinary Branch, Armed Forces Institute of Pathology, personal communication, 1973-1974. 8. Allen, T. D. and Risk, W.: Renal angiomyolipoma. J. Urol., 94: 203, 1.965. 9. Waldbaum, R. S., Marshall, V. F., Muecke, E. C. and Watson, R.: Tuberous sclerosis with bilateral angiomyolipomas of the kidney: case report with 8-year followup. J. Urol., 106: 180, 1971.
