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Malignant mesenchymoma of kidney and inferior vena cava
MALIGNANT MESENCHYMOMA OF KIDNEY AND INFERIOR VENA CAVA HAROLD M. REED, M.D. ROBERT POPPITI, JR., M.D. MANUEL SIVINA, M.D. From the Departments of Urology, Pathology and Cardiovascular Surgery, Mount Sinai Medical Center, Miami Beach, Florida
ABSTRACT--Herein is reported the first known case of malignant mesenchymoma involving the kidney and secondarily the vena cava in an adult. A thirty-nine-year-old woman succumbed to this malignancy within three months after the diagnosis was established, despite multimodal therapy.
A thirty-nine-year-old woman theatrical agent presented with episodic, severe, right flank pain noted for one year and gross hematuria for the previous three months. Her tendency toward procrastination and denial led to a delay in evaluation. An outpatient intravenous pyelogram performed in July, 1981, showed nonfunction of the right kidney with nephrocalcinosis. The left k i d n e y h a d u n d e r g o n e c o m p e n s a t o r y hypertrophy. Parallel to this evaluation, a gynecologic workup was undertaken because of an enlarged uterus with a prolapsed fibroid measuring 7 cm in diameter. At that time, the patient refused any further gynecologic treatm e n t , a l t h o u g h her h e m o g l o b i n was 9.5 Gm/100 ml and hematocrit 27.4. A right retrograde pyelogram showed a sharp cutoff at the ureteropelvic junction with only minimal insinuation of contrast material into the right renal pelvis. CAT scan confirmed a large right renal mass with variable densities secondary to focal calcifications, soft tissue, and cysts. On renal arteriography, tumor encasement of vessels was seen (Fig. 1A). Two small areas of possible neovascularity in the right lobe of the liver were noted, although the liver scan was
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read as negative and the CAT scan of the liver was normal, as were liver function studies. Because of this and a history of ethanol abuse, the likelihood of cirrhotic changes was considered by a gastroenterologist. The involved vena caval segment was delineated from above and below by venogram (Fig. 1B, C). Major hepatic veins were intact as noted in the superior vena caval approach. On surgical exploration, the liver was not grossly involved, but the vena cava in the vicinity of the kidneys was of ligneous consistency. The right kidney shelled out easily. The left renal vein was tied off in view of excellent collateral circulation as seen on cavography. The vena cava, which was originally thought to be unresectable, was cross clamped at the level of the diaphragm, and the involved 5.5-cm segment was removed by the cardiovascular surgeon. Her immediate postoperative course was unremarkable, and she was discharged after receiving doxorubicin (Adriamycin) intravenously (35 mg/Kg body weight). One month later, she embarked on a seven-week course of radiotherapy with a total of 5,400 rad being given to the tumor area (4,500 in broad field and 900 in focused field).
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FIGURE 1. (A) Right renal arteriogram shows encasement of arterial tree with neovascularity. (B) Inferior vena cavogram (from below) showing cutoff of inferior vena cava at L2 and large left gonadal collateral, and (C) from above, patent hepatic veins.
7 em and a segment of the inferior vena cava 5.5 by 2.5 cm . There was nearly total replacement of the renal parenchyma by a variegated, gray-white, firm tumor with focal hemorrhage and necrosis. The remaining renal pelvis was cystically dilated. The tumor encased the right adrenal gland and invaded the right renal vein. A similar tumor was adherent to the wall of the inferior vena cava with obstruction of the lumen. The surgical specimen submitted in November, 1981, included a friable tumor involving the ileum, measuring 9.5 cm in greatest dimension. In November, 1981, she was admitted to the surgical service with an ileal perforation due to extensively necrotic metastatic tumor. She died on the first postoperative day. Gross pathology The specimen submitted in August, 1981, consisted of a right kidney measuring 12 by 8 by
9,98
Microscopic pathology The predominant histologic feature was that of a malignant spindle-cell neoplasm with a storiform pattern, giant cells, "histiocytoid" cells, and a multifocal infiltrate composed of polymorphonuclear leukocytes, lymphocytes, and occasional plasma cells characteristic of a fibrous histiocytoma (Fig. 2A). In other areas
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VOLUME XXII, NUMBER 3
FIGURE 2. (A) Storiforrn pattern with abundant giant cells. (B) Atypical spindle cells' forming vascular slits with extravasated red blood cells. (C) Malignant bone formation.
malignant spindle cells with cigar-shaped nuclei and abundant mitotic figures were noted. The cytoplasm of these cells stained yellow with van Gieson stain. Electron microscopy revealed m y o f i l a m e n t s with associated dense bodies characteristic of smooth muscle differentiation. The p r e d o m i n a n t pattern in the inferior vena eava was that of malignant spindle cells with slit-like and dilated ehannels lined by bizarre, atypical endothelial cells characteristic of vascular differentiation (Fig. 2B). T h r o u g h o u t the specimen were numerous foei of osteoid and malignant bone formation (Fig. 2C). The metastatic lesion in the ileum was an undifferentiated spindle-cell sarcoma Comment The differential diagnoses included "sarcomatoid" renal cell carcinoma and adult Wilms tumor, but both were ruled out because of lack of epithelial elements. The diagnoses of malignant fibrous histiocytoma, leiomyosareoma, angiosarcoma, and osteosareoma, all previously reported in the kidney, were considered too uni-
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dimensional to eneompass the heterogeneity of this tumor. ~-4 Malignant mesenehymoma was first described by Gilmour 5 as a neoplasm formed by two or more unrelated mesenehymal components (disregarding the fibrous component) which are rapidly growing, prone to recurrences and oeeasional metastases. This t u m o r is rare in adults although m a n y eases in older children have been reported. ~ We believe that this t u m o r meets the above diagnostie eriteria and is the first such ease reported in the adult kidney and inferior vena eava. Despite initial encouragement by the reseetability of the involved kidney and vena eava with negative perihilar l y m p h nodes, the pathologists, rightfully so, were skeptieal about a hopeful prognosis beeause of the tumor's overw h e l m i n g h e t e r o g e n e i t y , s i g n i f i e a n t pleomorphism, and a b u n d a n t mitotie aetivity. 1688 Meridian Avenue Miami Beach, Florida 33140 (DR. REED) References 1. Tellem M, Shane JJ, and Imbriglia JE: Malignant histioeytoma of left renal pelvis, Am J Clin Pathol 43:450 (1965). 2. Bazaz-Malik G, and Gupta DN: Leiomyosareoma of kidney; J Urol 95:754 (1966). 3. Prince CL: Primary angio-endothelioma of the kidney, ibid. 47:787 (1942). 4. Johnson LA, Ancona VC, Johnson T, and Pineda NB: Primary osteogenie sarcoma of the kidney, ibid. 104:528 (1970). 5. Gihnour JRA: Recurrent tumor of the mesenehyma in an adult, J Pathol Baeteriol 55:495 (1943). 6. Nash A, and Stout AP: Malignant mesenehymomas in children, Cancer 14:524 (1961).
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ABSTRACT--Herein is reported the first known case of malignant mesenchymoma involving the kidney and secondarily the vena cava in an adult. A thirty-nine-year-old woman succumbed to this malignancy within three months after the diagnosis was established, despite multimodal therapy.
A thirty-nine-year-old woman theatrical agent presented with episodic, severe, right flank pain noted for one year and gross hematuria for the previous three months. Her tendency toward procrastination and denial led to a delay in evaluation. An outpatient intravenous pyelogram performed in July, 1981, showed nonfunction of the right kidney with nephrocalcinosis. The left k i d n e y h a d u n d e r g o n e c o m p e n s a t o r y hypertrophy. Parallel to this evaluation, a gynecologic workup was undertaken because of an enlarged uterus with a prolapsed fibroid measuring 7 cm in diameter. At that time, the patient refused any further gynecologic treatm e n t , a l t h o u g h her h e m o g l o b i n was 9.5 Gm/100 ml and hematocrit 27.4. A right retrograde pyelogram showed a sharp cutoff at the ureteropelvic junction with only minimal insinuation of contrast material into the right renal pelvis. CAT scan confirmed a large right renal mass with variable densities secondary to focal calcifications, soft tissue, and cysts. On renal arteriography, tumor encasement of vessels was seen (Fig. 1A). Two small areas of possible neovascularity in the right lobe of the liver were noted, although the liver scan was
UROLOGY
/
S E P T E M B E R 1983
/
read as negative and the CAT scan of the liver was normal, as were liver function studies. Because of this and a history of ethanol abuse, the likelihood of cirrhotic changes was considered by a gastroenterologist. The involved vena caval segment was delineated from above and below by venogram (Fig. 1B, C). Major hepatic veins were intact as noted in the superior vena caval approach. On surgical exploration, the liver was not grossly involved, but the vena cava in the vicinity of the kidneys was of ligneous consistency. The right kidney shelled out easily. The left renal vein was tied off in view of excellent collateral circulation as seen on cavography. The vena cava, which was originally thought to be unresectable, was cross clamped at the level of the diaphragm, and the involved 5.5-cm segment was removed by the cardiovascular surgeon. Her immediate postoperative course was unremarkable, and she was discharged after receiving doxorubicin (Adriamycin) intravenously (35 mg/Kg body weight). One month later, she embarked on a seven-week course of radiotherapy with a total of 5,400 rad being given to the tumor area (4,500 in broad field and 900 in focused field).
V O L U M E XXII, N U M B E R 3
297
FIGURE 1. (A) Right renal arteriogram shows encasement of arterial tree with neovascularity. (B) Inferior vena cavogram (from below) showing cutoff of inferior vena cava at L2 and large left gonadal collateral, and (C) from above, patent hepatic veins.
7 em and a segment of the inferior vena cava 5.5 by 2.5 cm . There was nearly total replacement of the renal parenchyma by a variegated, gray-white, firm tumor with focal hemorrhage and necrosis. The remaining renal pelvis was cystically dilated. The tumor encased the right adrenal gland and invaded the right renal vein. A similar tumor was adherent to the wall of the inferior vena cava with obstruction of the lumen. The surgical specimen submitted in November, 1981, included a friable tumor involving the ileum, measuring 9.5 cm in greatest dimension. In November, 1981, she was admitted to the surgical service with an ileal perforation due to extensively necrotic metastatic tumor. She died on the first postoperative day. Gross pathology The specimen submitted in August, 1981, consisted of a right kidney measuring 12 by 8 by
9,98
Microscopic pathology The predominant histologic feature was that of a malignant spindle-cell neoplasm with a storiform pattern, giant cells, "histiocytoid" cells, and a multifocal infiltrate composed of polymorphonuclear leukocytes, lymphocytes, and occasional plasma cells characteristic of a fibrous histiocytoma (Fig. 2A). In other areas
UROLOGY
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SEPTEMBER 1983
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VOLUME XXII, NUMBER 3
FIGURE 2. (A) Storiforrn pattern with abundant giant cells. (B) Atypical spindle cells' forming vascular slits with extravasated red blood cells. (C) Malignant bone formation.
malignant spindle cells with cigar-shaped nuclei and abundant mitotic figures were noted. The cytoplasm of these cells stained yellow with van Gieson stain. Electron microscopy revealed m y o f i l a m e n t s with associated dense bodies characteristic of smooth muscle differentiation. The p r e d o m i n a n t pattern in the inferior vena eava was that of malignant spindle cells with slit-like and dilated ehannels lined by bizarre, atypical endothelial cells characteristic of vascular differentiation (Fig. 2B). T h r o u g h o u t the specimen were numerous foei of osteoid and malignant bone formation (Fig. 2C). The metastatic lesion in the ileum was an undifferentiated spindle-cell sarcoma Comment The differential diagnoses included "sarcomatoid" renal cell carcinoma and adult Wilms tumor, but both were ruled out because of lack of epithelial elements. The diagnoses of malignant fibrous histiocytoma, leiomyosareoma, angiosarcoma, and osteosareoma, all previously reported in the kidney, were considered too uni-
UROLOGY
/
SEPTEMBER 1983
/
dimensional to eneompass the heterogeneity of this tumor. ~-4 Malignant mesenehymoma was first described by Gilmour 5 as a neoplasm formed by two or more unrelated mesenehymal components (disregarding the fibrous component) which are rapidly growing, prone to recurrences and oeeasional metastases. This t u m o r is rare in adults although m a n y eases in older children have been reported. ~ We believe that this t u m o r meets the above diagnostie eriteria and is the first such ease reported in the adult kidney and inferior vena eava. Despite initial encouragement by the reseetability of the involved kidney and vena eava with negative perihilar l y m p h nodes, the pathologists, rightfully so, were skeptieal about a hopeful prognosis beeause of the tumor's overw h e l m i n g h e t e r o g e n e i t y , s i g n i f i e a n t pleomorphism, and a b u n d a n t mitotie aetivity. 1688 Meridian Avenue Miami Beach, Florida 33140 (DR. REED) References 1. Tellem M, Shane JJ, and Imbriglia JE: Malignant histioeytoma of left renal pelvis, Am J Clin Pathol 43:450 (1965). 2. Bazaz-Malik G, and Gupta DN: Leiomyosareoma of kidney; J Urol 95:754 (1966). 3. Prince CL: Primary angio-endothelioma of the kidney, ibid. 47:787 (1942). 4. Johnson LA, Ancona VC, Johnson T, and Pineda NB: Primary osteogenie sarcoma of the kidney, ibid. 104:528 (1970). 5. Gihnour JRA: Recurrent tumor of the mesenehyma in an adult, J Pathol Baeteriol 55:495 (1943). 6. Nash A, and Stout AP: Malignant mesenehymomas in children, Cancer 14:524 (1961).
VOLUME XXII, NUMBER 3
299