Case report: Leiomyosarcoma of the renal pelvis

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a childhood malignancy, typically occurring in the bone and rarely in any other part of the body. We herein present a case of ES/PNET of the renal pelvis. A 37-year-old male patient presented with a chief complaint of pain in the left flank and gross hematuria. The tumor had caused moderate hydronephrosis, and ureteroscopic biopsy findings were highly suspicious of sarcoma. Subsequently, radical nephroureterectomy was performed. On the basis of the pathological and cytogenetic findings, a final diagnosis of primary ES/PNET of left renal pelvis was made. Adjuvant chemotherapy with adriamycin and ifosfamide was initiated as ES/PNET often exhibits aggressive biological behavior. The patient was disease-free at his last regular follow-up visit 18 months after the surgery. To our knowledge, this is the first reported case of primary ES/PNET of the renal pelvis.

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Primary renal leiomyosarcoma in adult patients: a systematic review and individual patient data analysis

Periasamy,

Dey,

Goyal

et al. 2024

Afr J Urol

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Purpose The optimal management of primary renal leiomyosarcomas is unknown owing to its rarity and minimal available information about their primary, adjuvant treatment and clinical outcomes. This study systematically reviews treatment evidence and effects in terms of survival for leiomyosarcomas arising primarily from kidney, renal pelvis and renal vessels. Method PubMed and Embase databases were searched from inception to March 2023, with manual searches of reference lists. Two investigators independently reviewed the studies reporting management and survival outcomes of renal leiomyosarcomas. Results A total of 85 publications met inclusion criteria, reporting on 188 cases. The median age was 55.5 years, predominantly female [52.7%]. Pain was the most common presenting symptom [41.5%], and most tumors were high grade [45.8%]. Complete surgical resection with negative margins forms definitive treatment. The median disease-free survival and overall survival (OS) for all reviewed patients were 24 months [95%CI 4.1–43.9] and 42 months [95%CI 32.5–51.4], respectively. The OS of 1 year, 2 year, 3 year and 5 year was 78.8%, 64.4%, 53.8% and 38.9%, respectively. On univariate analysis, favorable factors for OS included tumor size ≤ 5 cm, low-grade histology, tumors of renal vascular origin and non-metastatic disease at presentation. Neoadjuvant or adjuvant treatment with either radiotherapy or chemotherapy has been shown to improve OS (NR vs. 36 months, p < 0.001), especially for high-grade tumors > 5 cm in size. Conclusion Radical nephrectomy with en bloc tumor resection with negative margins forms the mainstay of treatment for renal leiomyosarcomas. Adjuvant radiotherapy or chemotherapy appears to improve OS. To validate this treatment strategy, prospective multicentric efforts are required to acquire reliable data from randomized trials.

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Case report: Leiomyosarcoma of the renal pelvis

Cited by 5 publications

References 3 publications

Computed Tomography and Magnetic Resonance Imaging Features of Lesions of the Renal Medulla and Sinus

Computed Tomography and Magnetic Resonance Imaging Features of Lesions of the Renal Medulla and Sinus

Primary Ewing sarcoma/primitive neuroectodermal tumor of the renal pelvis: a case report

Primary renal leiomyosarcoma in adult patients: a systematic review and individual patient data analysis

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