成人攝護腺橫紋肌肉瘤－罕見病例報告與文獻回顧

吳冠賢^1,3, 李經家^1,2,3*

¹高雄醫學大學附設中和醫院 泌尿部、²高雄醫學大學醫學系 泌尿學科、^３高雄醫學大學 臨床醫學研究所

Prostate embryonal rhabdomyosarcoma in adults: Case report and review of literature

Kuan-Hsien Wu^1,3, Ching-Chia Li^1,2,3*

¹ Department of Urology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan

² Department of Urology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan

³ Graduate Institute of Clinical Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan

Introduction: Prostate embryonal rhabdomyosarcoma (ERMS) is a common tumor in infants and children, but it is rarely seen in adults. It is characterized by a high degree of malignancy, both local formation of rapid growth large pelvic masses and systemic spread. The combined modality has improved outcome in children, whereas several therapeutic approaches have failed to gain a significant survival benefit in adult patients.

Case report: We reported a 32-year-old man who presented abdominal fullness with occasional thin stool shape during defecation for 1 month. Prostate-specific antigen (PSA) was within normal levels. The histology report of prostate transrectal biopsy showed spindle cell neoplasm, in favor of sarcoma. Pathologic diagnosis of radical prostatectomy was prostate ERMS, FNCLCC grade 2, pT3N0. The patient received combined-modality treatment, with the administration of 4 courses of doxorubicin, ifosfamide and 2-mercaptoethane sulfonate sodium (mesna), and subsequent radiotherapy to the prostatic bed (60 Gy/30 fxs) and pelvic lymph node (46 Gy/23 fxs). The patient remained free of progression of disease for about 1 year to finally experience a relapse with diffusion restriction at the right aspect of anterior abdominal wall. The pathologic report of tumor excision revealed recurrent ERMS. The patient received another episode combined-modality treatment, with the administration of 4 courses of VAC (vincristine, doxorubicin, and cyclophosphamide) and IE (ifosphamide, etoposide), and radiotherapy to the abdomen wall (46 Gy/15 fxs). Currently, the patient was under regular follow up at our out-patient department about two years after following the initial diagnosis.

Conclusion: Prostate ERMS in adults is a very rare and aggressive disease. Even though there is no well-established treatment suggestion, our report demonstrates that early diagnosis and complete surgical resection with subsequent chemo-radiation combined treatment can control the prostate disease, reducing the symptoms and improving the quality of life of these patients.