Journal of Clinical Oncology & Advanced Therapy

Abstract

Introduction: Paratesticular leiomyosarcoma (PLMS) is an exceptionally rare malignant mesenchymal tu mour, accounting for only 20% of paratesticular neoplasms and <1% of all genitourinary malignancies [1].Originating from the spermatic cord (60%), epididymis (30%), or tunica vaginalis (10%), these tumours typically present as painless scrotal masses, with peak incidence in the sixth to seventh decades of life [2]. Diagnostic challenges arise from their nonspecific clinical and radiological features, leading to frequent mis diagnosis as benign lesions (lipomas, adenomatoid tumours) in up to 40% of cases [3].

Case Presentation: A 65-year-old immunocompetent male presented to our urology clinic with a 3-year histo ry of a progressively enlarging (5.2 cm to 7.6 cm), painless left scrotal mass. Physical examination revealed a f irm, mobile, nontender mass distinct from the testis. Scrotal ultrasound demonstrated a well-circumscribed, heterogeneous hypoechoic mass (7.6 × 2.0 × 5.2 cm) with internal vascularity (Doppler RI: 0.62). Staging CT (chest/abdomen/pelvis) showed no lymphadenopathy or distant metastases (clinical stage T2aN0M0). The patient underwent radical left inguinal orchiectomy with en bloc resection of the spermatic cord and adjacent soft tissues (surgical margins: 1.5 cm).

Discussion: Histopathological analysis revealed a well-differentiated leiomyosarcoma (FNCLCC Grade 2: 5/10 HPF mitoses, moderate nuclear atypia, no necrosis) originating from the spermatic cord. Immunohis tochemistry showed diffuse positivity for SMA (3+), desmin (2+), and vimentin (3+), with negative S100, CD34, and cytokeratin stains, confirming smooth muscle lineage. The Ki-67 proliferation index was 15-20%. Postoperative recovery was uneventful, and at 6-month follow-up, surveillance imaging showed no evidence of recurrence.

Conclusion: This case highlights three critical management principles for PLMS: (1) the diagnostic triad of clinical suspicion (painless growing mass in elderly), radiologic correlation (heterogeneous vascularized mass on ultrasound), and pathologic confirmation (spindle cells with smooth muscle markers); (2) the gold standard treatment of wide local excision via radical orchidectomy with R0 resection (≥1 cm margins); and (3) the importance of multidisciplinary management involving urologic oncologists, pathologists, and ra diologists (4). While adjuvant therapy remains controversial for localized disease, our case reinforces that complete surgical resection provides excellent local control, with 5-year survival rates exceeding 80% for low-grade tumours (5). These findings underscore the need for heightened clinical awareness to prevent diag nostic delays in this rare malignancy.