原發性睪丸旁平滑肌肉瘤併多處轉移
張哲維, 張效駿, 吳維哲, 曾一修, 鐘旭東
亞東紀念醫院外科部 泌尿科
Primary paratesticular leiomyosarcoma with metastasis in seven different organs
Che-Wei Chang, Hsiao-Chun Chang, Wei-Che Wu, Yi-shiou Tseng, Shiu-Dong Chung
Division of Urology, Department of Surgery, Far Eastern Memorial Hospital
Introduction:
Primary paratesticular tumors are rare. More than 75% of these lesions arise from spermatic cord and 20% being leiomyosarcoma. We reported a case of primary paratesticular leiomyosarcoma with metastasis to seven different organs during follow-up.
Case presentation:
A 45 year-old man presented with right scrotal palpable mass for more than one year on August 2009. The mass was located adjacent to right testis and was hard on palpation. The right scrotum was not erythematous nor tender. Sonography revealed a 4x3x3cm right paratesticular tumor. Intra-operative frozen section disclosed spindle cell tumor and right orchiedectomy was performed. Final pathology revealed right paratesticular leiomyosarcoma, grade 3 with margin free of tumor. Serial workup revealed no distant metastases except enlarged lymph nodes over paraaortic lymph node. Adjuvant radiotherapy was applied for paraaortic lymph nodes. Within seven years, patient had multiple metastases including: right middle lobe of lung on July 2011; jejunum on April 2014; left kidney on September 2014; liver on January 2015; left leg soleus muscle on April 2015; T9-10, L1 spine on May 2015; and brain on September 2015. Patient underwent surgery for all metastatic lesions except radiotherapy for spine metastases. All pathology confirmed metastatic leiomyosarcoma. Patient received chemotherapy on November 2015. After first chemotherapy, the patient was hospitalized due to neutropenic fever. Due to severe sepsis and disease progression, the patient expired on December 2015.
Conclusion:
Unlike epididymal leiomyosarcoma, paratesticular leiomyosarcoma has a more aggressive characteristic with most patients presented with metastatic disease or subsequently developed distant sarcoma.