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Hemangioendothelioma of the Kidney: A Case Report and Review of the Literature
Vol. 112, December Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1974 by The Williams & Wilkins Co.
HEMANGIOENDOTHELIOMA OF THE KIDNEY: A CASE REPORT AND REVIEW OF THE LITERATURE H.J. PETERS, M. NURI*
AND
R. MUNZEl\iMAIER
From the Departments of Urology and Pathology, Fakultiit fur klinische Medizin Mannheim, University of Heidelberg, Mannheim, Germany
and Ackerley the tumor probably originated in the adrenal and infiltrated into the kidney. 9 To date 3 cases of hemangioendotheliomas with primary localization in the kidney have been published. 10 - 12 f:ase 4 is being reported herein.
Hemangioendotheliomas are the most frequently encountered type of malignant vascular tumor. Synonymous terms for these growths include angiosarcoma, Kupffer's cell sarcoma, hemangioblastoma and angioreticulo-endotheliosarcoma. Hemangioendotheliomas are characterized by a pathological growth of vessels with numerous anastomoses, eugonic formation of atypical endothelial cells within a reticular fiber structure and infiltrative growth with pronounced hematogenous metastasis. The endothelial cells are either in a single row against the basal membrane or proliferate in several rows into the lumen so that the tumor takes on a solid characteristic. All hemangioendotheliomas are malignant. They occur most frequently in the spleen, liver, bone, breast, skin and subcutaneous tissue. To date 43 hemangioendotheliomas of the liver in adults and 38 tumors in children have been reported. 1 • 2 Angiosarcoma in the child is considered less malignant since a more expansive and less infiltrative growth has been observed. 3 Among 4,000 primary bone tumors found at the Mayo Clinic only 9 were hemangioendotheliomas. 4 In 1969 Morgenstern and Westing found only 42 cases of bone hemangioendotheliomas in the literature. 5 In 1965 Steingaszner and associates found 38 cases of primary localization in the breast. 6 Hemangioendotheliomas of the kidney are extremely rare. We found only 4 hemangioendotheliomas of the liver with metastasis to one or both kidneys. 1 • 7 · 8 In the case described by Lorber Accepted for publication April 19, 1974. * Requests for reprints: Facultat fiir Klinische Medizin Mannheim, der Universitat Heidelberg, 68 Mannheim, Germany. 1 Adam, Y. G., Huvos, A. G. and Hadju, S. I.: Malignant vascular tumors of liver. Ann. Surg., 175: 375,
CASE REPORT
A 74-year-old man was seen because of considerable weight loss, tiredness and weakness. In 1960 he had undergone an operation for a ureteral stone on the right side. He had had no other serious illnesses. The patient was in poor general condi · tion. A dark red nodule the size of a pea on the left side of his forehead was initially regarded as a hemangioma. A soft non-displaceable tumor the size of a tomato could be palpated around the area of the 4th right rib in the subcutaneous tissue. The liver was enlarged to 1 fingerbreadth below the costal margin and was slightly tubercular in the of the gallbladder. A large tumor could be on the left side of the central portion of the abdomen, clearly extending from the left The preliminary radiogram of the kidneys revealed an opaque shadow on the left side of the central portion of the abdomen. The psoas shadow was not delineated. The kidney was considerably and the contours could not be defined Excretory urography revealed the kidney to be displaced laterally (fig. 1). The upper calix group did not fill. The central group had been bowed laterally outward by a process occupying space and indicated by a uniform shadow. The ureter was also displaced. A double contour was visible at the lower pole of the kidney. The scintiscan registered the left kidney. The tumor was about the size of a man's head and over the lower pole of the kidney. A vascularly insufficient tumor was nosed angiographically (fig. 2). The upper of the kidney had been destroyed by this tumor. The
1972. 2 Blumenfeld, T. A., Fleming, I. D. and Johnson, W. W.: Juvenile hemangioendothelioma of the liver. Report of a case and review of the literature. Cancer, 24: 853, 1969. 3 Kauffman, S. L. and Stout, A. P.: Malignant hemangioendothelioma in infants and children. Cancer, 14: 1186, 1961. 4 Unni, K. K., Ivins, J.C., Beabout, J. W. and Dahlin, D. C.: Hemangioma, hemangiopericytoma, and hemangioendothelioma (angiosarcoma) of bone. Cancer, 27: 1403, 1971. 5 Morgenstern, P. and Westing, S. W.: Malignant hemangioendothelioma of bone. Fourteen-year follow-up in a case treated with radiation alone. Cancer, 23: 221, 1969. 6 Steingaszner, L. C., Enzinger, F. M. and Taylor, H. B.: Hemangiosarcoma of the breast. Cancer, 18: '.152, 1965. 7 Stout, A. P.: Hemangio-endothelioma; a tumor of blood vessel featuring vascular endothelial cells. Ann. HS: 445, 1943. H., Mizutani, T. and Yamazaki, I.:
Malignant hemangioendothelioma of the liver with metastases to the kidneys and adrenals. Mie Med. J., 16: 221, 1967. 9 Lorber, J. and Ackerley, A.G.: Malignant haemangioendotheliomata simulating miliary tuberculosis and neuroblastoma of the adrenal. Proc. Roy. Soc. Med., 5l:
288, 1958.
723
1 °Kurii, M. F.: Gemangioendothelioma potschki. Vrach. Delo, 5: 48, 1973. 11 Prince, C. L.: Primary angio-endothelioma of the kidney: report of a case and brief review. J. Urol., 47: 787,
1942.
"Vigo, N. J., Schaposnik, F. and Laguens, R.: Hemangioendothelioma renal. Rev. Argent. Urol. Nefrol., 33: 107, 1963.
'I
724
PETERS, NURI AND MVNZENMAIER
FIG. 1. Urogram 15 minutes after injection of contrast medium revealed both kidneys adequately. Left kidney and ureter are displaced laterally, being indicated by uniform ray transmission. Left upper calix group did not fill. Psoas shadow cannot be clearly defined. Double contour is present at lower pole of kidney. Kidney shadow is not delineated in cranial direction.
kidney vessels were elongated and broken open. Minor contrast medium pools were detectable. Capsule vessels passed in an arc cranial and caudal. The renal vein was not filling. An x-ray of the thorax revealed multiple foci in both lower lung regions, which were regarded as metastases. The liver scintiscan showed a minor parenchymal defect in the left lobe of the liver. Gastroscopy revealed no evidence of a morbid condition. The hemoglobin was 8 gm. per cent, blood sedimentation rate 45/78, alkaline phosphatase 410 mU. per ml. and lactate dehydrogenase 800 mU. per ml. All other laboratory chemical tests were normal. Since hypemephroma with lung and liver metastasis was suspected, the patient underwent nephrectomy on March 19, 1973. After resection of the 11th rib the large tumor was found to have infiltrated the lateral abdominal wall. The upper pole of the kidney was completely destroyed. The tumor and fatty capsule were removed completely. The specimen weighed 2,320 gm. and contained a 19 by 15 by 14 cm. tumor, which was surrounded by a membraniform capsule. The tumor had almost completely destroyed the upper pole of the kidney. The tumor tissue was not clearly delineated in relation to the kidney parenchyma. A
section of the tumor comprised grayish-red soft tissue, with striped markings in places, and was penetrated by extensive bleeding and fresh necrosis. The ureter and pelvis exhibited no morbid changes. Microscopic examination of the tumor revealed divisions filled with blood and capillary in nature, formed from endothelial cells (figs. 3 and 4). The elongated or rounded cells exhibited distinct polymorphic chromatin-rich nuclei. Mitoses were identified in isolated cases and several cell rows were superimposed in some areas. The hollow spaces were surrounded by a reticular fibrous network. Solid rows of cells could also be identified. In the edge zones groups of capillaries with normal configuration were encountered. The kidney parenchyma had signs of compression in the vicinity of the tumor, with largely fibroid sections of tissue. In the cortical area a small trabecular adenoma was found. Because of the histological pattern diagnosis was hemangioendothelioma. Initial convalescence was satisfactory. However, 2 days postoperatively the patient suddenly went into shock and blood pressure could not be measured. The hemoglobin dropped from 10.8 to 2 gm. per cent. The patient became moribund within 15 minutes. Despite massive infusions and transfusions it was impossible to hold the blood pressure above 50 mm. Hg. During infusion therapy an increased swelling of the trunk was noticed. The abdomen reacted only slightly to pressure. Retroperitoneal drainage yielded no blood. Because we assumed that there was intra-abdominal bleeding laparotomy was done 1 hour later. We found 5 L of blood in the abdominal cavity. The cause of
FIG. 2. Aortography reveals lateral displacement of left kidney. Renal vessels are stretched and spread. There is slight coloration of large tumor with isolated arteriovenous anastomoses. Capsule vessels run in arc in cranial and caudal directions.
725
HEMANGIOENDOTHELIOMA OF KIDNEY
FIG. 3. Hemangioendotheiioma-note hollow spaces of capillary nature filled with blood and with cover formed by flattened cells with polymorphic nuclei. Some solid tumor cell chains are present. H & E, reduced from x250.
bleeding proved to be a ruptured vascular tumor in the left lobe of the liver. The tumor was approximately the size of a hen's egg and corresponded to the parenchymal defect recorded the liver scintiscan. Because of the poor condition of the patient a partial liver resection was impossible and a strip of lyophilized dura was secured to the rupture with tissue adhesive (histoacryl). Postoperatively the required controlled breathing for several - to shortage of breath. Six ~Ao>,mn a tar-like stool was noted. The source of bleeding was not detectable gastroscopically. H,m"0 " ' " the examiner found a healing round ulcer which had not been noted during preliminary The patient recovered slowly from the second operation and tar-like stools were noted Nodule formation above the 4th rib, lung metastasis and changes in the liver scintiscan increased significantly. venJJLl!llLL numerous skin metastases appeared. Because of metastases we decided not to use radiation The patient died on 19, 1973, 2 months - nephrectomy. Postmortem examination massive lung metastases, heart metastases with hemopericardium, and tumors in the bladder, adrenal, peritoneum, skin and subcutaneous tissue. The tumor in the left lobe of the liver had increased 3 times in size since the second However, the rupture point was still sealed by the dura patch. Metastases 'Nere detectable in the right lobe of the liver. The cause of the tar-like stool was a metastasis in mucous membrane, which had heen identified as 2_ D!SCUSS!O,'J
therefore,
FIG. 4. Argentaffin network in v1cm1ty of vascular tubes. A, Gomori, reduced from x 160. B, H & E, reduced from x400.
degree of probability as hemangioendotheliomas 1 ' Including our case, only 4 hemangioendotheliomss with primary localization in the kidney have been described. Metastasis to the kidneys also occurs rarely. In our patient the initial diagnosis and the course of treatment indicated that the tumors were localized in the left The thesis of a illness with multicent,ic occurrence of tumors is contradicted the numerous metastases in almost all organs which became evident rapidly after liver rupture. Differential diagnosis of le; achieved with the aid of silver coloration of reticu lar fibers. Endothelial cells can be identified as such if they are within the reticular fiber structure. whereas the are found outside the basal membrane. Diagnosis of a requires, in addition, a endothelial cells into the vascular lumen, so that rn the impression of a solid tumor arises, On other hand, the at another resemble a cavernous hemangioma. Broad vascular Iumina combining in anastomosis with one another are covered a endothelial Metas, tases were found m cases of such tumors which as cavernous hemai,gi omas. many such cases and ,a-u~,oJ,J'O'
13 Largiader, F.: Morphologie, Histogenese und K!a,,sil'1kation der Nierentumoren. Urol. Int., 6: 272, l 958.
726
PETERS, NURI AND MCTNZENMAIER
found, in addition to extreme differentiated tissue, multi-row atypical endothelial cells, such as are typical of the hemangioendotheliomas. He, therefore, doubts the existence of benign metastatic hemangiomas. 7 The differential diagnosis in respect to angioleiomyoliposarcoma, hamartoblastomas of the kidney is achieved by proving the presence of other mesenchymal tumor components. All hemangioendotheliomas are malignant. However, in children the tumors usually grow slowly and expansively. In most cases children die of pulmonary complications resulting from displacement or from heart failure, since the tumor causes a massive arteriovenous shunt. This slow process is not observed in adults. Unlike in the infantile type of tumor the proliferation of endothelial cells into the lumen leads to reduced blood circulation. In our case we found an avascular tumor on angiography, which aroused attention because of its uniform radiation transmission. A renal tumor of this type had not been noted previously on angiography. The rupture of the liver metastasis greatly worsened the prognosis. Before the planned irradiation of the metastases could be started rapid metastases to almost all organs were
noted and the patient died. Autopsy showed that the lyophilized dura patch attached as a desperation measure with histoacryl fulfilled its purpose and completely sealed the rupture point. The prognosis of hemangioendotheliomas can only be improved if the tumor is identified early. In our case preoperative metastases were already evident. Skin metastases were incorrectly identified as hemangiomas. Except for cachexia serious clinical symptoms were not present. The patient had never received thorotrast injections. An association in this respect assumed by several authors remains in dispute. SUMMARY
A case of malignant hemangioendothelioma of the kidney in a 74-year-old man is described. Progress of the illness was complicated by spontaneous perforation of liver metastasis with severe intra-abdominal bleeding. Later, multiple metastases occurred in almost all organs. Four cases of hemangioendotheliomas with primary localization in the kidney have been reported and ours is the first tumor of its kind to have been established by angiography.
THE JOURNAL OF UROLOGY
Copyright© 1974 by The Williams & Wilkins Co.
HEMANGIOENDOTHELIOMA OF THE KIDNEY: A CASE REPORT AND REVIEW OF THE LITERATURE H.J. PETERS, M. NURI*
AND
R. MUNZEl\iMAIER
From the Departments of Urology and Pathology, Fakultiit fur klinische Medizin Mannheim, University of Heidelberg, Mannheim, Germany
and Ackerley the tumor probably originated in the adrenal and infiltrated into the kidney. 9 To date 3 cases of hemangioendotheliomas with primary localization in the kidney have been published. 10 - 12 f:ase 4 is being reported herein.
Hemangioendotheliomas are the most frequently encountered type of malignant vascular tumor. Synonymous terms for these growths include angiosarcoma, Kupffer's cell sarcoma, hemangioblastoma and angioreticulo-endotheliosarcoma. Hemangioendotheliomas are characterized by a pathological growth of vessels with numerous anastomoses, eugonic formation of atypical endothelial cells within a reticular fiber structure and infiltrative growth with pronounced hematogenous metastasis. The endothelial cells are either in a single row against the basal membrane or proliferate in several rows into the lumen so that the tumor takes on a solid characteristic. All hemangioendotheliomas are malignant. They occur most frequently in the spleen, liver, bone, breast, skin and subcutaneous tissue. To date 43 hemangioendotheliomas of the liver in adults and 38 tumors in children have been reported. 1 • 2 Angiosarcoma in the child is considered less malignant since a more expansive and less infiltrative growth has been observed. 3 Among 4,000 primary bone tumors found at the Mayo Clinic only 9 were hemangioendotheliomas. 4 In 1969 Morgenstern and Westing found only 42 cases of bone hemangioendotheliomas in the literature. 5 In 1965 Steingaszner and associates found 38 cases of primary localization in the breast. 6 Hemangioendotheliomas of the kidney are extremely rare. We found only 4 hemangioendotheliomas of the liver with metastasis to one or both kidneys. 1 • 7 · 8 In the case described by Lorber Accepted for publication April 19, 1974. * Requests for reprints: Facultat fiir Klinische Medizin Mannheim, der Universitat Heidelberg, 68 Mannheim, Germany. 1 Adam, Y. G., Huvos, A. G. and Hadju, S. I.: Malignant vascular tumors of liver. Ann. Surg., 175: 375,
CASE REPORT
A 74-year-old man was seen because of considerable weight loss, tiredness and weakness. In 1960 he had undergone an operation for a ureteral stone on the right side. He had had no other serious illnesses. The patient was in poor general condi · tion. A dark red nodule the size of a pea on the left side of his forehead was initially regarded as a hemangioma. A soft non-displaceable tumor the size of a tomato could be palpated around the area of the 4th right rib in the subcutaneous tissue. The liver was enlarged to 1 fingerbreadth below the costal margin and was slightly tubercular in the of the gallbladder. A large tumor could be on the left side of the central portion of the abdomen, clearly extending from the left The preliminary radiogram of the kidneys revealed an opaque shadow on the left side of the central portion of the abdomen. The psoas shadow was not delineated. The kidney was considerably and the contours could not be defined Excretory urography revealed the kidney to be displaced laterally (fig. 1). The upper calix group did not fill. The central group had been bowed laterally outward by a process occupying space and indicated by a uniform shadow. The ureter was also displaced. A double contour was visible at the lower pole of the kidney. The scintiscan registered the left kidney. The tumor was about the size of a man's head and over the lower pole of the kidney. A vascularly insufficient tumor was nosed angiographically (fig. 2). The upper of the kidney had been destroyed by this tumor. The
1972. 2 Blumenfeld, T. A., Fleming, I. D. and Johnson, W. W.: Juvenile hemangioendothelioma of the liver. Report of a case and review of the literature. Cancer, 24: 853, 1969. 3 Kauffman, S. L. and Stout, A. P.: Malignant hemangioendothelioma in infants and children. Cancer, 14: 1186, 1961. 4 Unni, K. K., Ivins, J.C., Beabout, J. W. and Dahlin, D. C.: Hemangioma, hemangiopericytoma, and hemangioendothelioma (angiosarcoma) of bone. Cancer, 27: 1403, 1971. 5 Morgenstern, P. and Westing, S. W.: Malignant hemangioendothelioma of bone. Fourteen-year follow-up in a case treated with radiation alone. Cancer, 23: 221, 1969. 6 Steingaszner, L. C., Enzinger, F. M. and Taylor, H. B.: Hemangiosarcoma of the breast. Cancer, 18: '.152, 1965. 7 Stout, A. P.: Hemangio-endothelioma; a tumor of blood vessel featuring vascular endothelial cells. Ann. HS: 445, 1943. H., Mizutani, T. and Yamazaki, I.:
Malignant hemangioendothelioma of the liver with metastases to the kidneys and adrenals. Mie Med. J., 16: 221, 1967. 9 Lorber, J. and Ackerley, A.G.: Malignant haemangioendotheliomata simulating miliary tuberculosis and neuroblastoma of the adrenal. Proc. Roy. Soc. Med., 5l:
288, 1958.
723
1 °Kurii, M. F.: Gemangioendothelioma potschki. Vrach. Delo, 5: 48, 1973. 11 Prince, C. L.: Primary angio-endothelioma of the kidney: report of a case and brief review. J. Urol., 47: 787,
1942.
"Vigo, N. J., Schaposnik, F. and Laguens, R.: Hemangioendothelioma renal. Rev. Argent. Urol. Nefrol., 33: 107, 1963.
'I
724
PETERS, NURI AND MVNZENMAIER
FIG. 1. Urogram 15 minutes after injection of contrast medium revealed both kidneys adequately. Left kidney and ureter are displaced laterally, being indicated by uniform ray transmission. Left upper calix group did not fill. Psoas shadow cannot be clearly defined. Double contour is present at lower pole of kidney. Kidney shadow is not delineated in cranial direction.
kidney vessels were elongated and broken open. Minor contrast medium pools were detectable. Capsule vessels passed in an arc cranial and caudal. The renal vein was not filling. An x-ray of the thorax revealed multiple foci in both lower lung regions, which were regarded as metastases. The liver scintiscan showed a minor parenchymal defect in the left lobe of the liver. Gastroscopy revealed no evidence of a morbid condition. The hemoglobin was 8 gm. per cent, blood sedimentation rate 45/78, alkaline phosphatase 410 mU. per ml. and lactate dehydrogenase 800 mU. per ml. All other laboratory chemical tests were normal. Since hypemephroma with lung and liver metastasis was suspected, the patient underwent nephrectomy on March 19, 1973. After resection of the 11th rib the large tumor was found to have infiltrated the lateral abdominal wall. The upper pole of the kidney was completely destroyed. The tumor and fatty capsule were removed completely. The specimen weighed 2,320 gm. and contained a 19 by 15 by 14 cm. tumor, which was surrounded by a membraniform capsule. The tumor had almost completely destroyed the upper pole of the kidney. The tumor tissue was not clearly delineated in relation to the kidney parenchyma. A
section of the tumor comprised grayish-red soft tissue, with striped markings in places, and was penetrated by extensive bleeding and fresh necrosis. The ureter and pelvis exhibited no morbid changes. Microscopic examination of the tumor revealed divisions filled with blood and capillary in nature, formed from endothelial cells (figs. 3 and 4). The elongated or rounded cells exhibited distinct polymorphic chromatin-rich nuclei. Mitoses were identified in isolated cases and several cell rows were superimposed in some areas. The hollow spaces were surrounded by a reticular fibrous network. Solid rows of cells could also be identified. In the edge zones groups of capillaries with normal configuration were encountered. The kidney parenchyma had signs of compression in the vicinity of the tumor, with largely fibroid sections of tissue. In the cortical area a small trabecular adenoma was found. Because of the histological pattern diagnosis was hemangioendothelioma. Initial convalescence was satisfactory. However, 2 days postoperatively the patient suddenly went into shock and blood pressure could not be measured. The hemoglobin dropped from 10.8 to 2 gm. per cent. The patient became moribund within 15 minutes. Despite massive infusions and transfusions it was impossible to hold the blood pressure above 50 mm. Hg. During infusion therapy an increased swelling of the trunk was noticed. The abdomen reacted only slightly to pressure. Retroperitoneal drainage yielded no blood. Because we assumed that there was intra-abdominal bleeding laparotomy was done 1 hour later. We found 5 L of blood in the abdominal cavity. The cause of
FIG. 2. Aortography reveals lateral displacement of left kidney. Renal vessels are stretched and spread. There is slight coloration of large tumor with isolated arteriovenous anastomoses. Capsule vessels run in arc in cranial and caudal directions.
725
HEMANGIOENDOTHELIOMA OF KIDNEY
FIG. 3. Hemangioendotheiioma-note hollow spaces of capillary nature filled with blood and with cover formed by flattened cells with polymorphic nuclei. Some solid tumor cell chains are present. H & E, reduced from x250.
bleeding proved to be a ruptured vascular tumor in the left lobe of the liver. The tumor was approximately the size of a hen's egg and corresponded to the parenchymal defect recorded the liver scintiscan. Because of the poor condition of the patient a partial liver resection was impossible and a strip of lyophilized dura was secured to the rupture with tissue adhesive (histoacryl). Postoperatively the required controlled breathing for several - to shortage of breath. Six ~Ao>,mn a tar-like stool was noted. The source of bleeding was not detectable gastroscopically. H,m"0 " ' " the examiner found a healing round ulcer which had not been noted during preliminary The patient recovered slowly from the second operation and tar-like stools were noted Nodule formation above the 4th rib, lung metastasis and changes in the liver scintiscan increased significantly. venJJLl!llLL numerous skin metastases appeared. Because of metastases we decided not to use radiation The patient died on 19, 1973, 2 months - nephrectomy. Postmortem examination massive lung metastases, heart metastases with hemopericardium, and tumors in the bladder, adrenal, peritoneum, skin and subcutaneous tissue. The tumor in the left lobe of the liver had increased 3 times in size since the second However, the rupture point was still sealed by the dura patch. Metastases 'Nere detectable in the right lobe of the liver. The cause of the tar-like stool was a metastasis in mucous membrane, which had heen identified as 2_ D!SCUSS!O,'J
therefore,
FIG. 4. Argentaffin network in v1cm1ty of vascular tubes. A, Gomori, reduced from x 160. B, H & E, reduced from x400.
degree of probability as hemangioendotheliomas 1 ' Including our case, only 4 hemangioendotheliomss with primary localization in the kidney have been described. Metastasis to the kidneys also occurs rarely. In our patient the initial diagnosis and the course of treatment indicated that the tumors were localized in the left The thesis of a illness with multicent,ic occurrence of tumors is contradicted the numerous metastases in almost all organs which became evident rapidly after liver rupture. Differential diagnosis of le; achieved with the aid of silver coloration of reticu lar fibers. Endothelial cells can be identified as such if they are within the reticular fiber structure. whereas the are found outside the basal membrane. Diagnosis of a requires, in addition, a endothelial cells into the vascular lumen, so that rn the impression of a solid tumor arises, On other hand, the at another resemble a cavernous hemangioma. Broad vascular Iumina combining in anastomosis with one another are covered a endothelial Metas, tases were found m cases of such tumors which as cavernous hemai,gi omas. many such cases and ,a-u~,oJ,J'O'
13 Largiader, F.: Morphologie, Histogenese und K!a,,sil'1kation der Nierentumoren. Urol. Int., 6: 272, l 958.
726
PETERS, NURI AND MCTNZENMAIER
found, in addition to extreme differentiated tissue, multi-row atypical endothelial cells, such as are typical of the hemangioendotheliomas. He, therefore, doubts the existence of benign metastatic hemangiomas. 7 The differential diagnosis in respect to angioleiomyoliposarcoma, hamartoblastomas of the kidney is achieved by proving the presence of other mesenchymal tumor components. All hemangioendotheliomas are malignant. However, in children the tumors usually grow slowly and expansively. In most cases children die of pulmonary complications resulting from displacement or from heart failure, since the tumor causes a massive arteriovenous shunt. This slow process is not observed in adults. Unlike in the infantile type of tumor the proliferation of endothelial cells into the lumen leads to reduced blood circulation. In our case we found an avascular tumor on angiography, which aroused attention because of its uniform radiation transmission. A renal tumor of this type had not been noted previously on angiography. The rupture of the liver metastasis greatly worsened the prognosis. Before the planned irradiation of the metastases could be started rapid metastases to almost all organs were
noted and the patient died. Autopsy showed that the lyophilized dura patch attached as a desperation measure with histoacryl fulfilled its purpose and completely sealed the rupture point. The prognosis of hemangioendotheliomas can only be improved if the tumor is identified early. In our case preoperative metastases were already evident. Skin metastases were incorrectly identified as hemangiomas. Except for cachexia serious clinical symptoms were not present. The patient had never received thorotrast injections. An association in this respect assumed by several authors remains in dispute. SUMMARY
A case of malignant hemangioendothelioma of the kidney in a 74-year-old man is described. Progress of the illness was complicated by spontaneous perforation of liver metastasis with severe intra-abdominal bleeding. Later, multiple metastases occurred in almost all organs. Four cases of hemangioendotheliomas with primary localization in the kidney have been reported and ours is the first tumor of its kind to have been established by angiography.