Leiomyosarcoma of kidney – a case report with long term result after radiotherapy and chemotherapy

Abstract

Leiomyosarcoma of the kidney is a rare entity with poor prognosis. Among renal sarcomas, it is the most common histologic subtype amounting to 50-60% of all cases. The tumors usually arise from either the renal capsule or smooth muscle tissue in the renal pelvic wall. We describe a case of renal leiomyosarcoma that presented with history of left flank pain. Physical examination was normal. Abdominal ultrasound showed a solid lesion of 38 x 36 mm arising from the left kidney. Intravenous urography showed cut off of both major and minor calyces of the left kidney from its pelvis with a normal right kidney. Exploratory laparotomy revealed a well-encapsulated mass confined to the renal parenchyma for which radical nephrectomy was performed. Histopathology showed a Grade I leiomyosarcoma arising from the renal capsule. Adjuvant chemotherapy and sandwich radiotherapy was given and he is disease free after six and half years of follow up. The rarity of this disease, its severity and poor prognosis, uncommon form of presentation, resulting delay in diagnosis and difficulty in management is highlighted and reported.

Primary renal leiomyosarcoma: A diagnostic challenge

“…[1] Amongst the renal sarcomas, leiomyosarcoma is the most common histological subtype, accounting for 50-60% of all cases. [23] The most common symptoms and signs are like those of renal cell carcinoma, namely pain, palpable mass, and hematuria, all of which are indicators of an extensive local disease. They present as solid or cystic masses.…”

Renal Leiomyosarcoma: Case Report and Review of the Literature

“…Clinical presentations tend to be non-specific as patients typically complain of flank pain or abdominal pain and hematuria. One additional challenge in making the correct diagnosis is that conventional imaging is limited as the tumor characteristics seen on imaging may not be sufficient to reliably differentiate leiomyosarcoma from RCC [4]. Therefore, it is not surprising that renal leiomyosarcoma is often mistaken for RCC prior to surgery and subsequent tissue analysis [5, 6].…”

“…Sharma et al . describes a low-grade leiomyosarcoma case where the patient received post-operative chemotherapy with Mesna, Adriamicin, Ifosfamide, and Dacarbazine regimen and sandwich radiotherapy with a dose of 44 Gy in fractions to the renal bed and adjoining lymphatic area [4]. We advised our patient about the potential benefits of adjuvant chemotherapy despite his negative margin status and the absence of metastasis.…”

Successful management of renal leiomyosarcoma using radiofrequency ablation: Case report and brief review of literature

“…Moreover, some epithelial markers can be present in pure smooth muscle sarcomas, while some smooth muscle markers are positive in carcinomas. Since the prognosis for a renal sarcoma is particularly poor, differentiation from sarcomatoid renal cell carcinoma is necessary [14][15][16] . Renal LMS show high grade morphology and aggressive biological behavior with poor prognosis [13,15] .…”