Introduction: Primary LMS of the kidney is a rare tumor with an aggressive behaviour. It constituent 1 – 2 % of all malignant renal tumors. It does not have any characteristic diagnostic feature clinically and radiologically, frequently diagnosed on histopathology. Tumors with spindle cell morphology in the kidney should not always be taken for a sarcomatoid variant of renal cell carcinoma and should be investigated thoroughly. According to the clinical characteristics, prognosis, survival and diagnosis of primary renal Leiomyosarcoma (LMS). We analysed clinical manifestations, treatment and prognosis of our group and correlated to the literature.

Case presentation: We report a case of 61 Yrs old female presented with complaints of right flank pain since 2 months. CECT findings shows a fairly defined large mass in mid and lower pole region of kidney, completely encasing renal pelvis and PUJ region with moderate hydronephrosis; superomedially it is compressing over 2nd part of duodenum with suboptimal intervening fat planes; medially it is compressing IVC with suboptimal intervening fat planes; left renal vein is poorly delineated, possibly encased by mass lesion; no obvious involvement of ipsilateral adrenal gland- likely renal cell neoplasm- Primary. Open radical nephrectomy was done. Intra- op findings shows right renal mass occupying whole of right renal tissue along with lower pole of right kidney adherent to omentum. In Histopathology we received right renal mass, hilar lymph node, margin from inferior pole of kidney at the site of attachment with mesentery. Histopathology along with IHC technique confirmed the diagnosis of LMS kidney. According to TNM we gave pT4 N0, Residual margin also show tumor tissue.

Conclusion: Prognosis of LMS is poor so early diagnosis and radical nephrectomy can prolong patient life. Life expectancy of high grade primary LMS is poor as compared to low grade LMS. Prognostic factors for soft tissue sarcomas include stage, histological subtype, grade, age and gender of patient. Surgery is the main treatment modality. However chemotherapy and radiotherapy are also recommended considering the aggressive behaviour of neoplasm.

To be updated soon.