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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT  
Year : 2012  |  Volume : 23  |  Issue : 1  |  Page : 114-116
Primary renal osteosarcoma with systemic dissemination


1 Department of Radiotherapy, All India Institute of Medical Sciences, New Delhi, India
2 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication3-Jan-2012
 

   Abstract 

Primary renal osteosarcoma is an uncommon disease, which, unlike its skeletal counterpart, presents mostly in adults, and is generally diagnosed late due to its non-specific features and intra-abdominal location. Even if the disease is localized at diagnosis, it follows an aggressive course despite radical surgery and adjuvant treatment. We report a case of renal osteosarcoma in a 65-year-old female, who developed regional recurrence, and lung and bone metastases soon after radical nephrectomy for localized disease. Chemotherapy was ineffective in controlling systemic disease.

How to cite this article:
Puri T, Goyal S, Gupta R, Julka PK, Rath GK. Primary renal osteosarcoma with systemic dissemination. Saudi J Kidney Dis Transpl 2012;23:114-6

How to cite this URL:
Puri T, Goyal S, Gupta R, Julka PK, Rath GK. Primary renal osteosarcoma with systemic dissemination. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2019 Nov 12];23:114-6. Available from: http://www.sjkdt.org/text.asp?2012/23/1/114/91314

   Introduction Top


Osteosarcoma arising from the kidney is a rare but intriguing entity, with less than 20 cases reported till date, usually presenting in the adult age group.

Diagnosis may be suggested by calcification present on routine imaging. It follows an aggressive clinical course, with uniformly poor prognosis. A distinction needs to be made from sarcomatoid variant of renal cell carcinoma.


   Case Report Top


A 65-year-old female presented to our institution with a history of left flank pain for three years, two episodes of gross hematuria in one year and occasional dysuria. There was no history of anorexia, weight loss or prior history of renal colic or frequent urinary tract infections. She was a known hypertensive, controlled on medication. General physical examination was unremarkable. Abdominal examination revealed fullness in left lumbar region, but no discrete mass. Complete blood counts, renal and liver function tests, and urine microscopy and culture were normal. Abdominal sonography showed cholelithiasis and renal outflow obstruction with pyonephrosis and a permeative mass involving left kidney. A diethylenetriamine pentaacetic acid (DTPA) renal scan demonstrated a normal right kidney and negligible left renal cortical function. She underwent radical left nephrectomy, considering a preoperative diagnosis of left renal cell carcinoma.

Postoperative histopathology showed a malignant tumor with large areas of necrosis. The tumor consisted of sheets of spindle cells showing scattered mitotic figures. Numerous multi nucleated giant cells, focal areas of osteoid formation and myxoid degeneration were observed [Figure 1] and [Figure 2]. Renal parenchyma was compressed at the periphery. Peri-renal fat was involved in some areas. Renal vein harbored a tumor thrombus. Dissected renal hilar and paraaortic lymph nodes showed tumor deposits with peri-nodal soft tissue extension. Resected ureteric margin was free of tumor. Based on the above features, a diagnosis of primary renal osteosarcoma was considered.
Figure 1: Low-power microphotograph showing the poor demarcation of tumor and renal parenchyma (H&E, 40×).

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Figure 2: Higher power view showing the tumor composed of large cells with vesicular nuclei and prominent nucleoli. The tumor cells are admixed with multinucleated cells. Inset shows deposition of pink osteoid in between tumor cells (H&E, 100×).

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Imaging was repeated a month later, which showed a 3 × 3 cm lesion in the posterior bladder wall. Left renal fossa was normal. Contrast-enhanced CT (CECT) chest showed multiple bilateral pulmonary nodules, the largest being 3 × 2 cm. The patient was asymptomatic at the time of postoperative evaluation. She was offered chemotherapy with adriamycin, ifosfamide and cisplatin for metastatic disease; following five cycles of the same, repeat investigations showed a calcified mass involving posterior wall of urinary bladder and extending along distal left ureteric stump with left para-aortic lymphadenopathy, left psoas infiltration and lumbar ver­tebral extension. A bone scan showed increased uptake in second lumbar vertebra, suggesting early metastatic involvement.

She was planned for second-line chemotherapy with ifosfamide, carboplatin and etoposide, which she declined.


   Discussion Top


Primary renal osteosarcoma is a rare entity, first described by Haining and Poole in 1936. [1] No predilection for side or sex has been seen, with patients' age ranging from 29 to 82 years and most patients being middle-aged.

Diagnosis is first suggested on a plain X-ray abdomen by a renal mass with dense or rim-like calcification. [2] The other radiologic characteristics include a non-functioning kidney on renal scan and a sunburst pattern of calcification on intravenous urogram. Serum alkaline phosphatase may be elevated in the absence of demonstrable bone disease. Osteoid formation is a universal feature on microscopy; Axelrod et al have described a case where the entire postmortem renal tumor specimen was composed of bone. [3]

Diagnosis is generally delayed, with the tumor showing a rapid growth pattern with a tendency to infiltrate contiguous structures, such as the present case which developed recurrence in the urinary bladder wall despite negative resection margins, and a high propensity for distal metastases most commonly to the lungs and bones. Metastases may also demonstrate characteristic dense calcification. [4] The involvement of regional hilar and para-aortic nodes by the primary tumor as in this case has not been described earlier and contradicts the primarily hematogenous spread of osteosarcoma.

The major differential diagnosis includes a renal cell carcinoma with metaplastic change, i.e. sarcomatoid and osteogenic differentiation of malignant epithelial cells.

Electron microscopy confirms such a diagnosis with demonstration of pentalaminar desmosomes, basement membrane formation and intracytoplasmic lumina with an occasional atenuated brush border formation, implying epithelial origin. [5],[6]

Most such cases usually demonstrate at least some areas of epithelial differentiation.

The aggressive nature of the disease warrants an equally aggressive approach, with radical surgery in localized tumors and adjuvant chemotherapy. A survival advantage has been suggested with high-dose methotrexate and ifosfamide in skeletal osteosarcomas, but such information is sparse in tumors of renal origin. [7],[8] The present case progressed with extensive distal dissemination even though adjuvant combination chemotherapy was given, but maintained a good performance status with minimal symptoms despite the disease extent, suggesting that chemotherapy may at least contribute to the quality of life even if no survival benefit is seen.

 
   References Top

1.Haining RB, Poole FE. Osteoblastoma of the kidney histologically identical with osteosarcoma. Arch Pathol 1936;21:44-54.  Back to cited text no. 1
    
2.Mortensen PH. Primary osteogenic sarcoma of the kidney. Br J Urol 1989;63:101-2.  Back to cited text no. 2
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3.Axelrod R, Naidech H, Myers J, et al. Primary osteosarcoma of the kidney. Cancer 1978;41: 724-7.  Back to cited text no. 3
    
4.Watson R, Kanowski P, Ades C. Primary osteogenic sarcoma of the kidney. Aust N Z J Surg 1995;65:622-3.  Back to cited text no. 4
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5.Tannenbaum M. Ultrastructural pathology of human renal cell tumors. Pathol Ann 1971; 6249-77.  Back to cited text no. 5
    
6.Macke RA, Hussain MB, Imray TJ, et al. Osteogenic and sarcomatoid differentiation of a renal cell carcinoma. Cancer 1985;56:2452-7.  Back to cited text no. 6
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7.Rosen G. An opinion supporting the role of high-dose methotrexate in the treatment of osteosarcoma. Cancer Treat Res 1993;62:49-54.  Back to cited text no. 7
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8.Bacci G, Briccoli A, Ferrari S, et al. Neoadjuvant chemotherapy for osteosarcoma of the extremities with synchronous lung metastases: treatment with cisplatin, adriamycin and high dose of methotrexate and ifosfamide. Oncol Rep 2000;7:339-46.  Back to cited text no. 8
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Correspondence Address:
Shikha Goyal
Department of Radiotherapy, All India Institute of Medical Sciences, New Delhi-110029
India
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PMID: 22237231

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    Figures

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    Abstract
   Introduction
   Case Report
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    References
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