惡性睪丸間質細胞瘤合併多處腹腔內轉移
簡宏祐1陳順郎1, 2 陳文榮1 謝佐宜1王紹全1, 2
1中山醫學大學附設醫院泌尿科 2 中山醫學大學醫學系
Malignant Leydig Cell Tumor with Intraperitoneal Carcinomatosis
Horn-yo Chien1, Sung-Lang Chen1,2, Wen-Jung Chen1, Tzuo-Yi Hsieh 1, Shao-Chuan Wang 1,2
1 Department of Urology, Chung Shan Medical University Hospital. 2 School of medicine, Chung Shan Medical University
Introduction:
Leydig cell tumor is a rare benign testicular non-germ cell tumor and accounts for 1-3% of testicular tumor, of which malignant transformation is even rare. We report a case of malignant Leydig cell tumor with intraperitoneal carcinomatosis, retroperitoneal lymphadenopathy and abdominal wall metastases.
Case Report:
A 64-year-old male patient was previously healthy. He visited our urology clinic due to progressive enlarged, palpable mass over left scrotum for 6 months. On exam, a 2 cm in size, indurated mass within left testis was noted. Scrotal sonography showed a 2.2*1.5 cm in size nodular lesion at left testis. Serum tumor marker including AFP & bHCG were within normal limit, only elevated LDH was noted. After left radical orchiectomy, pathology revealed malignant Leydig cell tumor. Diffused metastasis of peritoneum, retroperitoneum and abdominal wall were noted by CT. He underwent tumor excision for abdominal wall tumor which proved metastatic Leydig cell tumor. He received adjuvant chemotherapy with BEP. Currently, the clinical course was uneventful.
Discussion:
Leydig cell tumor is derived from the interstitial Leydig cells. About 10% of the tumors show malignant behavior. As Leydig cells share the same mesodermal origin and the same phenotype with adrenocortical cells, this tumor may be related to various endocrine symptoms. Diagnosis is made according to histopathology examination. Metastasis frequently involves retroperitoneal lymph nodes, liver, lungs and bone. Malignant Leydig cell tumors are treated by orchiectomy with retroperitoneal lymphadenectomy. Unfortunately, in metastasis setting, it does not respond well to chemotherapy and radiation. Survival time has ranged from 2 - 17 years (median, 2 years)
Conclusion:
Leydig cell tumors are rare tumors of the male genitalia. Malignant Leydig cell tumors are even rare. There were no reliable clinical criteria for diagnosis except endocrine manifestations and metastasis. Accurate preoperative diagnosis can be challenging. Most patient require surgical intervention - orchiectomy for final diagnosis. The prognosis is poor!