- Email: [email protected]
Renal Hemangioma
THE JOUR)l"AL OF UROLOGY
Vol. 74, No. 2, August 1955 Printed in U.S.A.
RENAL HEMANGIOMA J. I. WALLER, M.A. THROCKMORTON
AND
E. BARBOSA
From the Department of Urology, Hertzler Clinic and Hertzler Research Foundation, Halstead, Kansas
While relatively few cases of renal hemangioma have been reported in the literature, the condition is probably much more common than reports would indicate. Many cases of unexplained or so-called 'essential hematuria' are most likely due to renal hemangioma. These lesions may be small and cause only a few red blood cells in the urine from time to time, and in such cases may be clinically ignored. However, in larger lesions causing profuse hemorrhage, one is faced with the problem of controlling bleeding to save life . .Judd and Simon stated that angioma is the rarest of benign tumors of the kidney. They reported a case from the Mayo Clinic and the second case to be reported from that clinic was described by Rives and Pool in 1944. Riley and Swan stated that among 13,219 necropsies at the Boston City Hospital up to .January 1, 19:39, no cases of renal angioma were found. The first renal hemangioma was found at autopsy in 1867 by Virchow. Fenwick reported the first case of a capillary hemangioma successfully removed by nephrectomy. Marks and Lazarus made an exhaustive review of the world literature from 1867 to 1947 and reported 70 cases of renal hemangioma. Since 1947 eight more cases have been found. In 1948 Heckel and Penick reported a mixed tumor described as a lipomyohemangioma. One of us (.J. I. W.) was a resident in urology at Presbyterian Hospital at that time and was privileged to see that patient. Dukes reported a case in 1949 while in 1950 Waterfall reported 1 case treated by nephrectomy. During 1951 Butt and Perry, Paletz and Sewell, Weyrauch and Berger, and l\!IcCrea each reported a case of renal hemangioma. Anderson et al. reported a case in J 95;3 while Malashock and Kopp found another at autopsy and reported it in 1954. This brings the total of reported cases of pure hemangioma to seventy-eight. We wish to add another case to the literature. An analysis of previously reported cases reveals that renal hemangioma occurs about equally in both sexes. If anything, a few n10re cases were found in men than in women. About half the cases occurred in the third or fourth decades of life, although the youngest was 4 days old and the oldest reported was 67 years of age. Virchow considered angiomas as originating from imperfectly formed vessels. Two types are encountered, capillary and cavernous. The capillary type is the most common. They may be single or multiple and may be located in the cortex, the medulla, or in the calyx. Lazarus and Marks came to the conclusion that the lesion is usually very small, but that its size may vary from a pinhead to a mass one-third the size of the kidney. Dukes felt that hematuria resulted from rupture of the hemangioma into the pelvis of the kidney. All of the lesions have been Accepted for publication February 23, 1955. 186
RENAL HEMANGIOMA
187
benign and almost all were unilateral. The most predominant symptom is painless, gross hematuria. However, in many cases renal colic or lumbar pain is associated with hematuria due to formation of blood clots in the pelvis of the kidney or in the ureter. Where infection is present, symptoms of nocturia, frequency or burning may be present. In many of the cases reported, intermittent gross hematuria had been present for many years before the condition became so severe that nephrectomy was done. In most of the cases diagnosis was very difficult before surgery, and most surgeons made the diagnosis of renal tumor or cyst instead of renal hemangioma before operation. Butt and Perry reported one case in which diagnosis was made before surgery. Paletz and Sewell treated a bilateral renal hemangioma by nephrectomy on one side and x-ray therapy on the opposite side. Due to the usual small size of the lesion, the kidney is, as a rule, not palpable. In the differential diagnosis one may rule out renal tumor, cysts, tuberculosis and nonopaque stones as the cause of hematuria. We recently had such a patient who passed 'port wine' colored urine and whose bladder would fill with blood clots, which had to be removed on repeated occasions. Her hematuria was as profuse as any postoperative prostatic bleeding we have seen. CASE REPORT
Mrs. 0. N., a 61-year-old Negress, presented herself with the complaints of gross hematuria, which had begun 7 months prior to admission, but had stopped after 4 months of intermittent hemorrhage, only to begin again 4 weeks prior to admission. The bleeding was painless, present throughout the urinary stream, usually dark 'port wine' in color, and often accompanied by many large clots. Associated symptoms were weakness, fatigue, shortness of breath and apprehens10n. The patient had had typhoid fever in 1910, and within the last 2 years had sustained minor injuries to knees and right shoulder. Abnormal history on system review included occasional frontal headaches, and symptoms of hyperthyroidism with a goiter. Chest, lungs, heart and abdomen revealed no pathologic changes. Pelvic examination revealed no evidence of bleeding or disease. Chest x-ray showed normal bony thorax and diaphragm. The heart was enlarged and there was considerable widening of the aortic shadow. Admission hemoglobin was 8 gm., or 48 per cent. The red blood count was 2,500,000 with a normal white blood count. Prothrombin time was 90 per cent, with coagulation time 1½ minutes and bleeding time 2 minutes. Renal function was normal. Cystoscopic examination on two separate days revealed gross hemorrhage from the right ureter, and retrograde pyelograms revealed a filling defect of the superior calyx (fig. 1, A). A diagnosis of renal tumor or cyst was made. After the patient was prepared by multiple whole blood transfusions and antibiotics a right nephrectomy was done. At the operating table no positive evidence of disease was seen. The postoperative course was uneventful with the
188
J. I. WALLER, M. A. THROCKMORTON AND E. BARBOSA
Fm. 1. A, pyelogram showing filling defect in right kidney. B, cross section of kidney shows brownish red, oval tumor mass on tip of papilla in middle third. In upper part of pelvis, old blood clots are attached to surface of pelvis.
r
~
Fm. 2. A, photomicrograph of hemangioma. Surface is covered by several layers of transitional cells. Tumor is composed mostly of capillaries lined by one layer of endothelium and several cavernous spaces reaching diameter of 300 micron. No renal tubules are seen within tumor. B, high magnification shows surface epithelium composed of transitional cells in two or three layers. Tumor has appearance of capillary hemangioma. No evidence of solid endothelial growth.
RENAL HEMANGIOMA
189
FIG. 3. Photomicrograph of area on margin of tumor showing loss of epithelium on surface and blood-filled spaces protruding over surface. This is apparently source of bleeding.
patient's red blood count rising quickly to normal level. She was fully ambulatory 5 days postoperatively and was discharged without findings or complaint on the fifteenth postoperative day. Pathology report: Grossly the kidney weighed 150 gm. with a finely granular surface. A small hemangioma was present at the papilla of the middle pyramid. There was some coagulated blood attached to the surface of the upper part of the pelvis. Microscopically a tumor in the middle pyramid was composed of thin-walled blood vessels; their walls consisted of one layer of endothelial cells. The spaces varied in size and were filled with red blood cells. In other areas there was infiltration with lymphocytes, eosinophilic leukocytes and plasma cells, and sections of the cortex showed chronic inflammatory changes. The spaces varied in size between 20 micron and 300 micron. Between the blood vessels no tubules could be recognized. There were fibroblasts and few round cells between the blood filled spaces. In one area the transitional epithelium was missing on the surface of the tumor and thin-walled blood filled spaces protruded over the surface. Diagnosis: Hemangioma in renal pyramid and chronic pyelonephritis. See figures 1, B, 2 and 3. DISCUSSION
We were confronted with a patient who had suffered from gross hematuria off and on for many months. There was no doubt that the bleeding came from the right kidney since bloody efflux was seen from the right ureteral orifice. No blood came from the left ureter and the left kidney had good function and was normal from a pyelographic standpoint. There were no casts in the urine and tuberculosis was ruled out. "\Ve made a diagnosis of tumor or cyst of the right kidney. Although the kidney appeared grossly normal at operation we removed it because we felt that such hemorrhage could not arise from a normal kidney.
190
J. I. WALLER, M. A. THROCKMORTON AND E. BARBOSA SUMMARY
Renal hemangioma is a rare disease. Hematuria, with or without renal colic, is the predominant symptom. The preoperative diagnosis of renal hemangioma is seldom made. Nephrectomy is the treatment of choice in unilateral hemangioma. The seventy-ninth case of renal hemangioma is reported. REFERENCES ANDERSON, J.B., LEE, J. J., HANCOCK, R. A. AND BLACK, S. R.: Hemangioma of the kidney pelvis. J. Urol., 70: 869-873, 1953. BELL, E. T.: Classification of renal tumors, with observations of the frequency of the various types. J. Urol., 39: 238-243, 1938. BUTT, A. J. AND PERRY, J. Q.: Hemangioma of the kidney. J. Urol., 65: 15-19, 1951. DomvIAN, H. N. AND FOWLER, H. A.: Hemangioma of the kidney. J. Urol., 55: 348-357, 1946. DUKES, CUTHBERT: Lecture 2, The pathology of essential hematuria and of renal tuberculosis. Trans. Med. Soc., London, 85: 391, 1947-1949. FENWICK, E. H.: Clinical Cystoscopy. London: p. 392, 1904. HECKEL, N. J. AND PENICK, G.D.: A mixed tumor of the kidney: lipomyohemangioma. J. Urol., 59: 572-576, 1948. JUDD, E. S. AND SIMON, H. E.: Angioma of the kidney. Surg., Gynec. & Obst., 46: 711-713, 1928, LAZARUS, J. A. AND MARKS, M. S.: Renal hemangioma. Urol. & Cutan. Rev., 51: 500-509, 1947. lVIALASHOCK, E. M. AND KoPP, H.J.: Cavernous hemangioma of the kidney. J. Urol., 72: 783-786, 1954. McCREA, L. E.: Hemangioma of the kidney; review of the literature. Urol. & Cutan. Rev., 55: 670-680, 1951. lVlELicow, M. M.: Classification of renal neoplasms. J. Urol., 51: 333-385, 1944. PALETZ, B. E. AND SEWELL, G.: Renal angioma, suspected bilateral involvement. J. Urol., 65: 9-14, 1951. RILEY, A. AND SWAN, W. J.: Angioma of kidney. Urol. & Cutan. Rev., 45: 377-382, 1941. RIVES, H.F. AND PooL, T. L.: Hemangioma of kidney. J.A.M.A., 125: 1187-1188, 1944. VrncHoW, R.: Die krankhaften Geschwulste, Band III, Erste Halfte, Berlin, Hirschwald, 1864. s. 397. WATERFALL, W. B.: Renal angioma causing severe hematuria. Brit. J. Urol., 22: 142-143, 1950. WEYRAUCH, H. M. AND BERGER, M. M.: Case simulating pyeloureteritis cystica; review of the literature. Stanford Med. Bull., 9: 43-46, 1951.
Vol. 74, No. 2, August 1955 Printed in U.S.A.
RENAL HEMANGIOMA J. I. WALLER, M.A. THROCKMORTON
AND
E. BARBOSA
From the Department of Urology, Hertzler Clinic and Hertzler Research Foundation, Halstead, Kansas
While relatively few cases of renal hemangioma have been reported in the literature, the condition is probably much more common than reports would indicate. Many cases of unexplained or so-called 'essential hematuria' are most likely due to renal hemangioma. These lesions may be small and cause only a few red blood cells in the urine from time to time, and in such cases may be clinically ignored. However, in larger lesions causing profuse hemorrhage, one is faced with the problem of controlling bleeding to save life . .Judd and Simon stated that angioma is the rarest of benign tumors of the kidney. They reported a case from the Mayo Clinic and the second case to be reported from that clinic was described by Rives and Pool in 1944. Riley and Swan stated that among 13,219 necropsies at the Boston City Hospital up to .January 1, 19:39, no cases of renal angioma were found. The first renal hemangioma was found at autopsy in 1867 by Virchow. Fenwick reported the first case of a capillary hemangioma successfully removed by nephrectomy. Marks and Lazarus made an exhaustive review of the world literature from 1867 to 1947 and reported 70 cases of renal hemangioma. Since 1947 eight more cases have been found. In 1948 Heckel and Penick reported a mixed tumor described as a lipomyohemangioma. One of us (.J. I. W.) was a resident in urology at Presbyterian Hospital at that time and was privileged to see that patient. Dukes reported a case in 1949 while in 1950 Waterfall reported 1 case treated by nephrectomy. During 1951 Butt and Perry, Paletz and Sewell, Weyrauch and Berger, and l\!IcCrea each reported a case of renal hemangioma. Anderson et al. reported a case in J 95;3 while Malashock and Kopp found another at autopsy and reported it in 1954. This brings the total of reported cases of pure hemangioma to seventy-eight. We wish to add another case to the literature. An analysis of previously reported cases reveals that renal hemangioma occurs about equally in both sexes. If anything, a few n10re cases were found in men than in women. About half the cases occurred in the third or fourth decades of life, although the youngest was 4 days old and the oldest reported was 67 years of age. Virchow considered angiomas as originating from imperfectly formed vessels. Two types are encountered, capillary and cavernous. The capillary type is the most common. They may be single or multiple and may be located in the cortex, the medulla, or in the calyx. Lazarus and Marks came to the conclusion that the lesion is usually very small, but that its size may vary from a pinhead to a mass one-third the size of the kidney. Dukes felt that hematuria resulted from rupture of the hemangioma into the pelvis of the kidney. All of the lesions have been Accepted for publication February 23, 1955. 186
RENAL HEMANGIOMA
187
benign and almost all were unilateral. The most predominant symptom is painless, gross hematuria. However, in many cases renal colic or lumbar pain is associated with hematuria due to formation of blood clots in the pelvis of the kidney or in the ureter. Where infection is present, symptoms of nocturia, frequency or burning may be present. In many of the cases reported, intermittent gross hematuria had been present for many years before the condition became so severe that nephrectomy was done. In most of the cases diagnosis was very difficult before surgery, and most surgeons made the diagnosis of renal tumor or cyst instead of renal hemangioma before operation. Butt and Perry reported one case in which diagnosis was made before surgery. Paletz and Sewell treated a bilateral renal hemangioma by nephrectomy on one side and x-ray therapy on the opposite side. Due to the usual small size of the lesion, the kidney is, as a rule, not palpable. In the differential diagnosis one may rule out renal tumor, cysts, tuberculosis and nonopaque stones as the cause of hematuria. We recently had such a patient who passed 'port wine' colored urine and whose bladder would fill with blood clots, which had to be removed on repeated occasions. Her hematuria was as profuse as any postoperative prostatic bleeding we have seen. CASE REPORT
Mrs. 0. N., a 61-year-old Negress, presented herself with the complaints of gross hematuria, which had begun 7 months prior to admission, but had stopped after 4 months of intermittent hemorrhage, only to begin again 4 weeks prior to admission. The bleeding was painless, present throughout the urinary stream, usually dark 'port wine' in color, and often accompanied by many large clots. Associated symptoms were weakness, fatigue, shortness of breath and apprehens10n. The patient had had typhoid fever in 1910, and within the last 2 years had sustained minor injuries to knees and right shoulder. Abnormal history on system review included occasional frontal headaches, and symptoms of hyperthyroidism with a goiter. Chest, lungs, heart and abdomen revealed no pathologic changes. Pelvic examination revealed no evidence of bleeding or disease. Chest x-ray showed normal bony thorax and diaphragm. The heart was enlarged and there was considerable widening of the aortic shadow. Admission hemoglobin was 8 gm., or 48 per cent. The red blood count was 2,500,000 with a normal white blood count. Prothrombin time was 90 per cent, with coagulation time 1½ minutes and bleeding time 2 minutes. Renal function was normal. Cystoscopic examination on two separate days revealed gross hemorrhage from the right ureter, and retrograde pyelograms revealed a filling defect of the superior calyx (fig. 1, A). A diagnosis of renal tumor or cyst was made. After the patient was prepared by multiple whole blood transfusions and antibiotics a right nephrectomy was done. At the operating table no positive evidence of disease was seen. The postoperative course was uneventful with the
188
J. I. WALLER, M. A. THROCKMORTON AND E. BARBOSA
Fm. 1. A, pyelogram showing filling defect in right kidney. B, cross section of kidney shows brownish red, oval tumor mass on tip of papilla in middle third. In upper part of pelvis, old blood clots are attached to surface of pelvis.
r
~
Fm. 2. A, photomicrograph of hemangioma. Surface is covered by several layers of transitional cells. Tumor is composed mostly of capillaries lined by one layer of endothelium and several cavernous spaces reaching diameter of 300 micron. No renal tubules are seen within tumor. B, high magnification shows surface epithelium composed of transitional cells in two or three layers. Tumor has appearance of capillary hemangioma. No evidence of solid endothelial growth.
RENAL HEMANGIOMA
189
FIG. 3. Photomicrograph of area on margin of tumor showing loss of epithelium on surface and blood-filled spaces protruding over surface. This is apparently source of bleeding.
patient's red blood count rising quickly to normal level. She was fully ambulatory 5 days postoperatively and was discharged without findings or complaint on the fifteenth postoperative day. Pathology report: Grossly the kidney weighed 150 gm. with a finely granular surface. A small hemangioma was present at the papilla of the middle pyramid. There was some coagulated blood attached to the surface of the upper part of the pelvis. Microscopically a tumor in the middle pyramid was composed of thin-walled blood vessels; their walls consisted of one layer of endothelial cells. The spaces varied in size and were filled with red blood cells. In other areas there was infiltration with lymphocytes, eosinophilic leukocytes and plasma cells, and sections of the cortex showed chronic inflammatory changes. The spaces varied in size between 20 micron and 300 micron. Between the blood vessels no tubules could be recognized. There were fibroblasts and few round cells between the blood filled spaces. In one area the transitional epithelium was missing on the surface of the tumor and thin-walled blood filled spaces protruded over the surface. Diagnosis: Hemangioma in renal pyramid and chronic pyelonephritis. See figures 1, B, 2 and 3. DISCUSSION
We were confronted with a patient who had suffered from gross hematuria off and on for many months. There was no doubt that the bleeding came from the right kidney since bloody efflux was seen from the right ureteral orifice. No blood came from the left ureter and the left kidney had good function and was normal from a pyelographic standpoint. There were no casts in the urine and tuberculosis was ruled out. "\Ve made a diagnosis of tumor or cyst of the right kidney. Although the kidney appeared grossly normal at operation we removed it because we felt that such hemorrhage could not arise from a normal kidney.
190
J. I. WALLER, M. A. THROCKMORTON AND E. BARBOSA SUMMARY
Renal hemangioma is a rare disease. Hematuria, with or without renal colic, is the predominant symptom. The preoperative diagnosis of renal hemangioma is seldom made. Nephrectomy is the treatment of choice in unilateral hemangioma. The seventy-ninth case of renal hemangioma is reported. REFERENCES ANDERSON, J.B., LEE, J. J., HANCOCK, R. A. AND BLACK, S. R.: Hemangioma of the kidney pelvis. J. Urol., 70: 869-873, 1953. BELL, E. T.: Classification of renal tumors, with observations of the frequency of the various types. J. Urol., 39: 238-243, 1938. BUTT, A. J. AND PERRY, J. Q.: Hemangioma of the kidney. J. Urol., 65: 15-19, 1951. DomvIAN, H. N. AND FOWLER, H. A.: Hemangioma of the kidney. J. Urol., 55: 348-357, 1946. DUKES, CUTHBERT: Lecture 2, The pathology of essential hematuria and of renal tuberculosis. Trans. Med. Soc., London, 85: 391, 1947-1949. FENWICK, E. H.: Clinical Cystoscopy. London: p. 392, 1904. HECKEL, N. J. AND PENICK, G.D.: A mixed tumor of the kidney: lipomyohemangioma. J. Urol., 59: 572-576, 1948. JUDD, E. S. AND SIMON, H. E.: Angioma of the kidney. Surg., Gynec. & Obst., 46: 711-713, 1928, LAZARUS, J. A. AND MARKS, M. S.: Renal hemangioma. Urol. & Cutan. Rev., 51: 500-509, 1947. lVIALASHOCK, E. M. AND KoPP, H.J.: Cavernous hemangioma of the kidney. J. Urol., 72: 783-786, 1954. McCREA, L. E.: Hemangioma of the kidney; review of the literature. Urol. & Cutan. Rev., 55: 670-680, 1951. lVlELicow, M. M.: Classification of renal neoplasms. J. Urol., 51: 333-385, 1944. PALETZ, B. E. AND SEWELL, G.: Renal angioma, suspected bilateral involvement. J. Urol., 65: 9-14, 1951. RILEY, A. AND SWAN, W. J.: Angioma of kidney. Urol. & Cutan. Rev., 45: 377-382, 1941. RIVES, H.F. AND PooL, T. L.: Hemangioma of kidney. J.A.M.A., 125: 1187-1188, 1944. VrncHoW, R.: Die krankhaften Geschwulste, Band III, Erste Halfte, Berlin, Hirschwald, 1864. s. 397. WATERFALL, W. B.: Renal angioma causing severe hematuria. Brit. J. Urol., 22: 142-143, 1950. WEYRAUCH, H. M. AND BERGER, M. M.: Case simulating pyeloureteritis cystica; review of the literature. Stanford Med. Bull., 9: 43-46, 1951.