移性副睪橫紋肌肉瘤(肺泡型)之案例報告

梁柏崧、蔡佳穆、林仁泰

高雄榮民總醫院 外科部 泌尿外科

Alveolar rhabdomyosarcoma of epididymis with multiple metastases: A case report

Po-Sung Liang, Chia-Mu Tsai, Jen-Tai Lin

Division of Urology, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan

Introduction: Rhabdomyosarcoma in epididymis is a rare disease. We presented a case of 59-year-old man with rhabdomyosarcoma in epididymis treated with radical orchiectomy, followed by chemotherapy.  

Case presentation: A 59-year-old male with history of chronic obstructive pulmonary disease (COPD) presented to urologic clinic with right scrotal mass for 1 month. He denied lower urinary tract symptoms, fever, nor abdominal pain. The scrotal sonography showed enlarged right epididymal body and tail with increased vascularity. There was no focal lesion at the right testis. The patient preferred observation initially. He came back to urologic clinic 3 months later due to gradual enlargement of the mass. Then, he received resection and tissue proof of the mass. During the operation, the right epididymal mass was palpated, along with firm to hard testis and multiple enlarged swelling nodules over the spermatic cord. Intraoperative sonography showed heterogenous right testis, favoring invasion of the right epididymal mass. Right radical orchiectomy with high ligation of the spermatic cord was subsequently performed. Pathological report showed alveolar rhabdomyosarcoma. Serum alpha-fetoprotein (AFP), lactate dehydrogenase (LDH) and beta-human chorionic gonadotropin (hCG) were checked for testicular cancer survey, and serum LDH level up to 1322 U/L was noted. The computed tomography (CT) of the abdomen and chest revealed multiple retroperitoneal lymph node metastasis, two peritoneal seeding tumors over pelvic cavity, and one small nodule near right diaphragm, 1.3 cm in size, suspicious of another tumor seeding. Three weeks later, subcutaneous tumors were palpated over the right chest wall and the bilateral abdomen. Excisional biopsy was conducted, and the pathologic report showed metastatic rhabdomyosarcoma, consistent with right epididymis in origin. ¹⁸F-fluoro-2-deoxy-D-glucose (FDG) positron emission tomography (PET) was arranged for comprehensive tumor survey and revealed metastases involving lungs, mediastinal/retroperitoneal lymph nodes and mesentery. Generalized increased bone marrow activity was noticed, and bone marrow infiltration was suspected. Bone marrow puncture was performed, and the pathological report also showed metastatic rhabdomyosarcoma. He was referred to medical oncologist, and chemotherapy with VDC regimen (Vincristine + Dactinomycin + Cyclophosphamide) was arranged. There was no tumor progression in the 4-month follow-up.

Conclusions: Rhabdomyosarcoma in epididymis is rare but aggressive. As its non-specific clinical manifestation, it is often confused with epididymitis or other epididymal tumor, which may lead to misdiagnosis and delay treatment. Urologists should pay attention to the possibility of the malignant epididymal tumors.