精索去分化脂肪肉瘤-文獻綜述及案例報告

黃亮臻,柯智群,王炯珵

恩主公醫院外科部泌尿科

Dedifferentiated Liposarcomas of Spermatic Cord- A Review and Case Report

Liang-Chen Huang, Chih-Chun Ke, Chung-Cheng Wang

Division of Urology, Department of Surgery, En Chu Kong Hospital

Introduction:

    Inguinal hernias are one of the most diagnosis for patients visiting urology department, which definitive treatment is surgical repair. The decision of operation can be oriented by physical examination or scrotal sonography only without other radiological or systemic examinations. Although, tumors of spermatic cord are rare and predominantly benign lesions, the incidence of malignant tumors of spermatic cord is 0.3 cases per million, and the most common type is liposarcoma. This current article aims to report a patient with a lethal disease disguising as a mundane mishap- dedifferentiated liposarcomas of right spermatic cord presenting as right inguinal hernia and conduct a literature review of spermatic cord dedifferentiated liposarcomas.

Case Presentation:

        A 69-year-old male with medical history of prostate enlargement with lower urinary tract symptoms, diabetes mellitus and carrier of HBV suffering from intermittently right lower quadrant abdominal and inguinal dullness with right inguinal slow-growing bulging mass accompanied with intermittently low-grade fever and mild diarrhea for two years since he refused operation for suspicious acute appendicitis was referred to our urology clinic for right inguinal hernia. Physical exam found a 3x3 cm slightly tender bulging mass at right inguinal area. Scrotal sonography found right inguinal hernia with fat-containing component protruding into the scrotum and bilateral varicocele. Right inguinal direct hernia was repaired with a monofilament polypropylene mesh in open-method and multiple tumors over right inguinal area, at least five in numbers were found and excised during operation.

The tumors, with the largest 6x4x2.5 cm, were pathologically confirmed dedifferentiated liposarcomas which composing of well differentiated liposarcoma area and non-lipogenic pleomorphic sarcoma area, pT2, grade II for French Federation of Cancer Centers Sarcoma Group (FNCLCC) with mitotic rate 3/10 high-power fields (HPF) yet without lymphovascular invasion. Further Computed tomography (CT) study revealed residual tumors over right inguinal canal to scrotum. Given the aggressive nature of dedifferentiated liposarcomas, wide excision and right radical orchiectomy were performed with upper margin at anterior superior iliac spine level; base margin at extracted internal inguinal ring, resected superficial fascia and muscle; and with nothing left in right scrotum as the lower margin (Figure 2b). The inguinal fascial defect was repaired with a monofilament polypropylene mesh.

The testis, epididymis and surgical margins were healthy and negative for MDM2 and CDK4 immunohistochemistry stain. Unfortunately, his wound belatedly healed with subcutaneous tunnel and watery discharge more than two months from the operation.