- Email: [email protected]
Osteogenic Sarcoma of the Kidney: Report of a Case
Vol. 94, Nov. Printed in U.S.A..
THE JOURNAL OF UROLOGY
Copyright © 1965 by The Williams & Wilkins Co.
OSTEOGENIC SARCOl\/IA OF THE KIDNEY: REPORT OF A CASE PETER J. SOTO, JR.,* EDWARDS. RADER, JAlV[ES M. MARTIN
AND
ANDREW GREGOWICZ
From the Departments of Pathology, Urology and Radiology, St. Louis University School of Medicine, St. Louis, Missouri
Although extraskeletal osteogenic sarcomas have been described in the breast, soft tissues, thyroid gland, uterus and bladder,1 primary osteogenic sarcoma of the kidney is rare. To our knowledge there have been only 4 documented cases previously reported; 2- 5 our report is case 5. CASE REPORT
An 82-year-old woman entered University Hospital with a 4-month history of intermittent gross hematuria, which began with the sudden onset of severe, aching pain in the left flank, radiating to the left hypogastrium and accompanied by nausea and vomiting. She had lost 5 pounds within 2 months. Past history was unremarkable except for a subtotal thyroidectomy in 1937 for hyperthyroidism. Physical examination disclosed an emaciated, pale, white woman with hirsutism of the chin, arms and legs. Her blood pressure was 150/80; pulse rate, 108 per minute; and temperature, 98.8F. Examination of the abdomen disclosed a large, hard, fL'l:ed, slightly tender mass occupying the left side. The liver was not palpable. No lymphadenopathy was found apart from shotty cervical nodes. Laboratory findings included hemoglobin of 8.5 gm. per 100 cc blood, hematocrit value of 35 per cent; and a white blood count (WBC) of 11,600 per cu. mm. with 85 segmented neutrophils, 12 lymphocytes and 3 monocytes. The fasting blood sugar was 130 mg. per cent and the blood urea nitrogen (BUN) was 18 mg. per cent. Accepted for publication February 10, 1965. * Present address: St. Elizabeth's Hospital, Belleville, Illinois. 1 Jernstrom, P., Lindberg, A. L. and Meland, 0. N.: Osteogenic sarcoma of the mammary gland. Amer. J. Clin. Path., 40: 521-526, 1963. 2 Raining, R. B. and Poole, F. E.: Osteoblastoma of the kidney, histologically identical with osteogenic sarcoma. Arch. Path., 21: 44-54, 1936. 3 Hamer, H. G. and Wishard, W. N., Jr.: Osteogenic sarcoma involving the right kidney. J. Urol., 60: 10-17, 1948. 4 Hudson, H. C.: Osteogenic sarcoma involving the left kidney. J. Urol., 75: 21-24, 1956. 5 Fine, G. and Stout, A. P.: Osteogenic sarcoma of the extrask@letal soft tissues. Cancer, 9: 1027-
1043, 1956. 532
The urine sediment disclosed numerous red blood cells and a few amorphous crystals. Radiographic examination of the chest failed to reveal abnormalities of the heart, lungs or pleura. Upper and lower gastrointestinal examinations were negative for intrinsic abnormality, but the stomach and proximal small bowel were displaced to the right and the descending colon somewhat to the left by a large abdominal mass. Excretory urography disclosed no abnormality on the right. The left renal silhouette was obliterated and there was non-visualization of the left renal collecting systein. Multiple calcific densities were observed over the left mid- and lower abdomen. One cluster measuring 6 cm. in diameter was superimposed on the left ilium. Retrograde pyelograrn.s were attempted twice. The first disclosed a medial deviation of the left ureter at the level of the fifth lumbar vertebra, a distorted, dilated renal pelvis that filled only at its periphery, and 2 enlarged and elongated calyces. A second retrograde examination was attempted 7 days later in an effort to fill completely the left renal pelvis but was unsuccessful because of a block encountered in the upper third of the ureter. A renal scan using 150 µc. Hg-203 labeled chlormerodrin revealed a normal kidney on the right side. The left kidney was not demonstrated. Cystoscopic examination disclosed varicosities in the trigone. A 0.5 cn1. papilloma was noted just inferior to the left ureteral orifice. Dark red blood issued from this orifice. The patient received a transfusion of blood and 10 days following hospitalization a transperitoneal exploration and a left nephrectomy and partial ureterectomy were performed. The left kidney weighed 680 gm. and measured 15 by 14 by 9 cm. (fig. 1). The external surface was irregular and covered by a thin, semi-transparent membrane. A segment of ureter, renal artery and vein was also present. Examination of the cut surface revealed an area of recognizable renal parenchyma along one margin. The associated renal pelvis was dilated. The main mass
OSTEOGENIC SARCOMA OF THE KIDNEY
533
FIG. 1. Cut surface of left kidney shows large necrotic mass. White areas represent presence of bone
FIG. 2. X-ray of one half of kidney demonstrates presence of bone
534
SOTO, JR., RADER, MARTIN AND GREGOWICZ
presented a variegated appearance with the col01 varying from yellow to tan. Its consistency likewise varied considerably with areas of necrosis to areas which were bony hard (fig. 2). The mass was very closely associated with the renal pelvis
Fm. 3. Non-osteogenic portion of tumor. Note spindle-cell pattern. H & E X 100.
but did not erode into it. It completely occluded the renal vein. The segment of ureter measured 15 cm. and contained clotted blood but no neoplasm. Histolog;ically, the neoplasm exhibited several patterns. In some areas the neoplastic elements were represented by pleomorphic spindle-shaped cells arranged in irregular bands intermingled with smaller, more plump neoplastic cells (fig. 3). In other areas large collections of eosinophilic staining material with the morphologic and tinctorial qualities of osteoid were noted (fig. 4). The material was arranged in a cribrifonn pattern and in some areas it was calcified. The cells in juxtaposition to the bands of osteoid were larger and more pleomorphic than those apart from the areas of osteogenesis. Extensive necrosis was also seen. Although lymphatic or vascular channel invasion was not evident within the turn.or per se the main renal vein was completely occluded by tumor. The final anatomical diagnosis was primary osteogenic sarcoma of the left kidney with invasion of the renal vein. The residual renal tissue exhibited changes of chronic pyelonephritis and arteriolar nephrosclerosis. The patient was dismissed 16 days after nephrectomy. Before discharge the small papilloma seen cystoscopically was removed. The patient was re-admitted to the hospital
Fm. 4. A, typical field shows osteoblastic activity with formation of osteoid. H & E XlOO. B, area of osteogenesis demonstrated with Koneff's stain. X 100.
535
OSTEOGENIC SARCOMA OF THE KIDNEY
43 days later with a 3-week history of bloody vaginal discharge. Physical examination disclosed an 8 by 5 cm. pedunculated, necrotic, bleeding mass attached to the right side of the vulva near the introitus. Additional findings were those of marked pedal and sacral edema. The liver was palpated 3 cn1. below the right costal margin. The hemoglobin on admission was 7.5 and the hematocrit value, 26. The WBC was 13 150 with 85 segmented neutrophils, 6 lymphocyt~s, and 9 monocytes. Urinalysis disclosed a trace of albumin, specific gravity of 1.013, and pH of 6.0. The urine sediment contained 25 to 30 WBC per high power field and a heavy bacterial component. BUN was 24 mg. per cent. A urine culture grew 10 6 colonies of Aerobacter and Proteus mirabilis. A chest x-ray disclosed scattered nodular densities in both lung fields and left pleural effusion. Calcification was not seen in the metastatic lesions. Calcification was no longer visualized in the abdomen, but the left psoas muscle shadow was obliterated and there was a generalized increase in the density of the left side of the abdomen. Films of the axial and appendicular skeleton revealed demineralization but there was no evidence of primary or metastatic bone neoplasm. The vulvar lesion was excised. Ristologically this neoplasm was identical to that seen in the kidney and was interpreted as metastasis. Purulent pharyngitis developed 14 clays later, and promptly responded to treatment. The remainder of the course was one of progressive deterioration and the patient died in coma 23 days after admission (82 days after nephrectomy). Postmortem examination revealed a cachectic white woman with a large palpable mass in the left upper quadrant of the abdomen. The peritoneal cavity contained 150 cc dark, amber fluid and there were numerous neoplastic implants lying ,vithin the omentum and on the peritoneal surface. A very large mass located in the left upper abdomen extended from the diaphragm to the level of the fourth lumbar vertebra and was attached to the psoas muscle. This mass measured 15 by 10 by 6 cm. and weighed 760 gm.. It incorporated the left adrenal gland and was adherent to the inferior vena cava. The stump of the left renal vein was occluded by residual tumor which extended into the vena cava for a short distance with associated partial
thrombosis inferior to the neoplasm. The right kidney and vessels were not remarkable. Metastases were seen in virtually all lymph node groups, both lungs, the liver and the mesentery. Examination of the central nervous system was not permitted. Histologically the metastases were identical to the neoplasm in the original specimen with variable degrees of bone formation. DISCUSSION
Heteroplastic tissues are occasionally found in the kidney with bone, cartilage and adipose tissue the most corn.man histologic types. They are usually found incidentally and may occur anywhere in the kidney. However, rarely, they may be symptomatic, as in the case recently reported by Pearlman 6 in which a child presented with hematuria. Histological examination revealed a large bony lesion which consisted of trabecular bone containing normal bone marrow. Ossification may also occur in various pathologic renal states including hydronephrosis and chronic pyelonephritis. Sarcorn.as account for only a small proportion of primary malignant renal neoplasms. These include liposarcomas, leiorn.yosarcomas, fibrosarcomas and vVilms tumor. The histogenesis of this neoplasm is a subject of speculation. Perhaps the rn.ost plausible concept is that it represents osseous metaplasia in a malignant mesenchymal neoplasm. This explanation correlates well with the morphologic characteristics of our case in that a spindle-cell sarcomatous pattern was seen in the nonosteogenic areas. All the metastases in our case show osteogenesis while in Haining's 2 case only the metastasis to the opposite kidney contained bone. In Hamer's 3 case the sole metastasis produced bone. No autopsy was performed in Hudson's case. 4 SUMMARY
A rare renal neoplasm is described, an osteogenic sarcoma arising primarily in the kidney. In a review of the literature only four other documented cases of primary osteogenic sarcoma of the kidney were found. One arose in the pararenal area while the others arose within the kidney per se. In our case osteogenic metastases were seen in the liver, lungs, lymph nodes and mesentery. 6 Peadrnan, C. K. and Sc_hrniesing, C. A.: Heterotop1c bone format10n m the kidney: Case report. J. Ural., 91: 223-227, 1964.
THE JOURNAL OF UROLOGY
Copyright © 1965 by The Williams & Wilkins Co.
OSTEOGENIC SARCOl\/IA OF THE KIDNEY: REPORT OF A CASE PETER J. SOTO, JR.,* EDWARDS. RADER, JAlV[ES M. MARTIN
AND
ANDREW GREGOWICZ
From the Departments of Pathology, Urology and Radiology, St. Louis University School of Medicine, St. Louis, Missouri
Although extraskeletal osteogenic sarcomas have been described in the breast, soft tissues, thyroid gland, uterus and bladder,1 primary osteogenic sarcoma of the kidney is rare. To our knowledge there have been only 4 documented cases previously reported; 2- 5 our report is case 5. CASE REPORT
An 82-year-old woman entered University Hospital with a 4-month history of intermittent gross hematuria, which began with the sudden onset of severe, aching pain in the left flank, radiating to the left hypogastrium and accompanied by nausea and vomiting. She had lost 5 pounds within 2 months. Past history was unremarkable except for a subtotal thyroidectomy in 1937 for hyperthyroidism. Physical examination disclosed an emaciated, pale, white woman with hirsutism of the chin, arms and legs. Her blood pressure was 150/80; pulse rate, 108 per minute; and temperature, 98.8F. Examination of the abdomen disclosed a large, hard, fL'l:ed, slightly tender mass occupying the left side. The liver was not palpable. No lymphadenopathy was found apart from shotty cervical nodes. Laboratory findings included hemoglobin of 8.5 gm. per 100 cc blood, hematocrit value of 35 per cent; and a white blood count (WBC) of 11,600 per cu. mm. with 85 segmented neutrophils, 12 lymphocytes and 3 monocytes. The fasting blood sugar was 130 mg. per cent and the blood urea nitrogen (BUN) was 18 mg. per cent. Accepted for publication February 10, 1965. * Present address: St. Elizabeth's Hospital, Belleville, Illinois. 1 Jernstrom, P., Lindberg, A. L. and Meland, 0. N.: Osteogenic sarcoma of the mammary gland. Amer. J. Clin. Path., 40: 521-526, 1963. 2 Raining, R. B. and Poole, F. E.: Osteoblastoma of the kidney, histologically identical with osteogenic sarcoma. Arch. Path., 21: 44-54, 1936. 3 Hamer, H. G. and Wishard, W. N., Jr.: Osteogenic sarcoma involving the right kidney. J. Urol., 60: 10-17, 1948. 4 Hudson, H. C.: Osteogenic sarcoma involving the left kidney. J. Urol., 75: 21-24, 1956. 5 Fine, G. and Stout, A. P.: Osteogenic sarcoma of the extrask@letal soft tissues. Cancer, 9: 1027-
1043, 1956. 532
The urine sediment disclosed numerous red blood cells and a few amorphous crystals. Radiographic examination of the chest failed to reveal abnormalities of the heart, lungs or pleura. Upper and lower gastrointestinal examinations were negative for intrinsic abnormality, but the stomach and proximal small bowel were displaced to the right and the descending colon somewhat to the left by a large abdominal mass. Excretory urography disclosed no abnormality on the right. The left renal silhouette was obliterated and there was non-visualization of the left renal collecting systein. Multiple calcific densities were observed over the left mid- and lower abdomen. One cluster measuring 6 cm. in diameter was superimposed on the left ilium. Retrograde pyelograrn.s were attempted twice. The first disclosed a medial deviation of the left ureter at the level of the fifth lumbar vertebra, a distorted, dilated renal pelvis that filled only at its periphery, and 2 enlarged and elongated calyces. A second retrograde examination was attempted 7 days later in an effort to fill completely the left renal pelvis but was unsuccessful because of a block encountered in the upper third of the ureter. A renal scan using 150 µc. Hg-203 labeled chlormerodrin revealed a normal kidney on the right side. The left kidney was not demonstrated. Cystoscopic examination disclosed varicosities in the trigone. A 0.5 cn1. papilloma was noted just inferior to the left ureteral orifice. Dark red blood issued from this orifice. The patient received a transfusion of blood and 10 days following hospitalization a transperitoneal exploration and a left nephrectomy and partial ureterectomy were performed. The left kidney weighed 680 gm. and measured 15 by 14 by 9 cm. (fig. 1). The external surface was irregular and covered by a thin, semi-transparent membrane. A segment of ureter, renal artery and vein was also present. Examination of the cut surface revealed an area of recognizable renal parenchyma along one margin. The associated renal pelvis was dilated. The main mass
OSTEOGENIC SARCOMA OF THE KIDNEY
533
FIG. 1. Cut surface of left kidney shows large necrotic mass. White areas represent presence of bone
FIG. 2. X-ray of one half of kidney demonstrates presence of bone
534
SOTO, JR., RADER, MARTIN AND GREGOWICZ
presented a variegated appearance with the col01 varying from yellow to tan. Its consistency likewise varied considerably with areas of necrosis to areas which were bony hard (fig. 2). The mass was very closely associated with the renal pelvis
Fm. 3. Non-osteogenic portion of tumor. Note spindle-cell pattern. H & E X 100.
but did not erode into it. It completely occluded the renal vein. The segment of ureter measured 15 cm. and contained clotted blood but no neoplasm. Histolog;ically, the neoplasm exhibited several patterns. In some areas the neoplastic elements were represented by pleomorphic spindle-shaped cells arranged in irregular bands intermingled with smaller, more plump neoplastic cells (fig. 3). In other areas large collections of eosinophilic staining material with the morphologic and tinctorial qualities of osteoid were noted (fig. 4). The material was arranged in a cribrifonn pattern and in some areas it was calcified. The cells in juxtaposition to the bands of osteoid were larger and more pleomorphic than those apart from the areas of osteogenesis. Extensive necrosis was also seen. Although lymphatic or vascular channel invasion was not evident within the turn.or per se the main renal vein was completely occluded by tumor. The final anatomical diagnosis was primary osteogenic sarcoma of the left kidney with invasion of the renal vein. The residual renal tissue exhibited changes of chronic pyelonephritis and arteriolar nephrosclerosis. The patient was dismissed 16 days after nephrectomy. Before discharge the small papilloma seen cystoscopically was removed. The patient was re-admitted to the hospital
Fm. 4. A, typical field shows osteoblastic activity with formation of osteoid. H & E XlOO. B, area of osteogenesis demonstrated with Koneff's stain. X 100.
535
OSTEOGENIC SARCOMA OF THE KIDNEY
43 days later with a 3-week history of bloody vaginal discharge. Physical examination disclosed an 8 by 5 cm. pedunculated, necrotic, bleeding mass attached to the right side of the vulva near the introitus. Additional findings were those of marked pedal and sacral edema. The liver was palpated 3 cn1. below the right costal margin. The hemoglobin on admission was 7.5 and the hematocrit value, 26. The WBC was 13 150 with 85 segmented neutrophils, 6 lymphocyt~s, and 9 monocytes. Urinalysis disclosed a trace of albumin, specific gravity of 1.013, and pH of 6.0. The urine sediment contained 25 to 30 WBC per high power field and a heavy bacterial component. BUN was 24 mg. per cent. A urine culture grew 10 6 colonies of Aerobacter and Proteus mirabilis. A chest x-ray disclosed scattered nodular densities in both lung fields and left pleural effusion. Calcification was not seen in the metastatic lesions. Calcification was no longer visualized in the abdomen, but the left psoas muscle shadow was obliterated and there was a generalized increase in the density of the left side of the abdomen. Films of the axial and appendicular skeleton revealed demineralization but there was no evidence of primary or metastatic bone neoplasm. The vulvar lesion was excised. Ristologically this neoplasm was identical to that seen in the kidney and was interpreted as metastasis. Purulent pharyngitis developed 14 clays later, and promptly responded to treatment. The remainder of the course was one of progressive deterioration and the patient died in coma 23 days after admission (82 days after nephrectomy). Postmortem examination revealed a cachectic white woman with a large palpable mass in the left upper quadrant of the abdomen. The peritoneal cavity contained 150 cc dark, amber fluid and there were numerous neoplastic implants lying ,vithin the omentum and on the peritoneal surface. A very large mass located in the left upper abdomen extended from the diaphragm to the level of the fourth lumbar vertebra and was attached to the psoas muscle. This mass measured 15 by 10 by 6 cm. and weighed 760 gm.. It incorporated the left adrenal gland and was adherent to the inferior vena cava. The stump of the left renal vein was occluded by residual tumor which extended into the vena cava for a short distance with associated partial
thrombosis inferior to the neoplasm. The right kidney and vessels were not remarkable. Metastases were seen in virtually all lymph node groups, both lungs, the liver and the mesentery. Examination of the central nervous system was not permitted. Histologically the metastases were identical to the neoplasm in the original specimen with variable degrees of bone formation. DISCUSSION
Heteroplastic tissues are occasionally found in the kidney with bone, cartilage and adipose tissue the most corn.man histologic types. They are usually found incidentally and may occur anywhere in the kidney. However, rarely, they may be symptomatic, as in the case recently reported by Pearlman 6 in which a child presented with hematuria. Histological examination revealed a large bony lesion which consisted of trabecular bone containing normal bone marrow. Ossification may also occur in various pathologic renal states including hydronephrosis and chronic pyelonephritis. Sarcorn.as account for only a small proportion of primary malignant renal neoplasms. These include liposarcomas, leiorn.yosarcomas, fibrosarcomas and vVilms tumor. The histogenesis of this neoplasm is a subject of speculation. Perhaps the rn.ost plausible concept is that it represents osseous metaplasia in a malignant mesenchymal neoplasm. This explanation correlates well with the morphologic characteristics of our case in that a spindle-cell sarcomatous pattern was seen in the nonosteogenic areas. All the metastases in our case show osteogenesis while in Haining's 2 case only the metastasis to the opposite kidney contained bone. In Hamer's 3 case the sole metastasis produced bone. No autopsy was performed in Hudson's case. 4 SUMMARY
A rare renal neoplasm is described, an osteogenic sarcoma arising primarily in the kidney. In a review of the literature only four other documented cases of primary osteogenic sarcoma of the kidney were found. One arose in the pararenal area while the others arose within the kidney per se. In our case osteogenic metastases were seen in the liver, lungs, lymph nodes and mesentery. 6 Peadrnan, C. K. and Sc_hrniesing, C. A.: Heterotop1c bone format10n m the kidney: Case report. J. Ural., 91: 223-227, 1964.