Metastatic Primary Intratesticular Rhabdomyosarcoma in an Adolescent with Rapid Early Response to VAC Chemotherapy: A Case Report and Literature Review of 99 Cases.
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BACKGROUND: Primary intratesticular rhabdomyosarcoma (PTRMS) is an exceptionally rare malignancy and may be clinically indistinguishable from more common scrotal conditions or germ cell tumors prior to orchiectomy. Evidence to guide management is limited, and treatment is typically extrapolated from pediatric and adolescent/young adult rhabdomyosarcoma protocols. CASE PRESENTATION: A 17-year-old male presented with progressive, painless enlargement of the right testis. Cross-sectional imaging demonstrated a large intratesticular mass with extensive retroperitoneal and pelvic lymphadenopathy causing grade II hydronephrosis due to ureteral compression, bilateral pulmonary nodules, and axillary lymphadenopathy. He underwent radical inguinal orchiectomy. Histopathology showed a high-grade malignant neoplasm involving the testis, epididymis, and spermatic cord with focal vascular invasion; immunohistochemistry was strongly positive for SMA, desmin, and MyoD1, supporting rhabdomyosarcoma. A double-J ureteral stent was placed, and systemic therapy with vincristine, actinomycin D, and cyclophosphamide (VAC) was initiated. After three cycles, follow-up imaging demonstrated complete radiologic resolution of pulmonary metastases, marked regression of nodal disease (including axillary involvement), and resolution of hydronephrosis with ongoing clinical improvement. LITERATURE REVIEW: In a PubMed-based review of 87 eligible articles comprising 99 individual cases of primary testicular or paratesticular rhabdomyosarcoma, the mean age at presentation was 19.76 years (SD 14.44). Tumors were predominantly paratesticular (80.8%) rather than intratesticular (19.2%). Among cases reporting staging data, nodal involvement at presentation was observed in 31.5% and distant metastasis in 25.6%. Treatment most commonly included surgery plus chemotherapy (54.5%) or trimodality therapy with surgery, chemotherapy, and radiotherapy (24.2%). CONCLUSION: PTRMS can present with advanced nodal and distant metastases yet remain chemosensitive. This case highlights a brisk response of disseminated intratesticular rhabdomyosarcoma to VAC chemotherapy and supports comprehensive staging and response-adapted multidisciplinary management. The complete case-level extraction and full citations are provided in the Supplementary material.