睪丸內平滑肌肉瘤合併腹直肌遠端轉移-罕見案例
徐易廷1、薛又仁1,2、黃建榮1、蕭毅君1、賴昱維1,2、邱文祥1,2
1臺北市立聯合醫院仁愛院區 外科部 泌尿科；2國立陽明交通大學 醫學院 泌尿學科
Primary intratesticular leiomyosarcoma with distant rectus abdominis metastasis – a rare case
Yi-Ting Hsu1, Thomas Y. Hsueh1,2, Andy. C. Huang1, Yi-Chun Hsiao1, Yu-Wei Lai1,2,
Allen W. Chiu1,2
1Division of Urology, Department of Surgery, Taipei City Hospital renai Branch, Taipei, Taiwan;
2Department of Urology, School of Medicine, National Yang-Ming Chiao-Tung University, Taipei, Taiwan
Background: Leiomyosarcoma is a malignant mesenchymal tumor derived from the smooth muscle. Leiomyosarcoma of the scrotum have been classified into the paratesticular and the intratesticular. Primary intratesticular sarcomas are very rare neoplasm with rare potential for distant metastasis. We presented an extremely rare case of a primary testicular leiomyosarcoma with distant metastasis at right rectus abdominis.
Presentation of case: A 59-year-old male patients presented with gradually enlarging right scrotal mass with dull pain and local heat for six months. Physical examination revealed a hard mass measuring 6 x 6 cm. Laboratory investigations revealed normal serum levels of alpha-fetoprotein(AFP), beta human chorionic gonadotropin(beta-HCG) and mildly elevated lactate dehydrogenase(LDH). Computed tomography showed a heterogeneously enhancing right testicular mass measuring 7.4 x 8 cm in size, with solid and cystic components. Also, there is a slightly hypodense heterogeneously enhancing right rectus abdominis mass about 3.3 cm in size. Under clinical diagnosis of testicular malignancy, patient underwent right high inguinal orchidectomy. Grossly, cut surface of the specimen was grey white, solid with foci of hemorrhage, necrosis. The surgical margin was free of malignancy. Immunohistochemical stains of tumor cells are positive for vimentin, desmin, smms-1, focally for smooth muscle actin(SMA) and negative for S100. Besides, echo-guided needle biopsy showed metastatic leiomyosarcoma of abdominal mass. A final diagnosis of primary testicular leiomyosarcoma with distant metastasis at right rectus abdominis was made.
Conclusions: Leiomyosarcoma of the testis is very rare and its clinical and imaging presentation remains similar to other testicular malignancies. It is difficult to define optimal management due to the lack of research and recent recommendations. Effectiveness of adjuvant therapy and systemic therapy is unknown currently.