Lenhard Miriam, Kuemper Caroline, Ditsch Nina, Diebold Joachim, Stieber Petra, Friese Klaus, Burges Alexander
Department of Obstetrics and Gynaecology, Campus Grosshadern, Ludwig-Maximilians-University, Munich, Germany.
Clin Chem Lab Med. 2007;45(5):657-61. doi: 10.1515/CCLM.2007.120.
Sertoli-Leydig cell tumours of the ovary account for only 0.2% of malignant ovarian tumours. Two-thirds of all patients become apparent due to the tumour's hormone production.
A 41-year-old patient (gravida 4, para 4) presented with dyspnoea, enlarged abdominal girth and melaena. Diagnostic imaging was suspicious for an ovarian cancer. The standard tumour marker for ovarian cancer (CA 125) was elevated to 984 U/mL.
Surgical exploration of the abdomen revealed a mouldering tumour of both adnexes extending to the level of the navel. Frozen sections showed an undifferentiated carcinoma of unknown origin. Radical surgery was performed. The final histological report described a malignant sex-cord stroma tumour, a Sertoli-Leydig cell tumour, emanating from both ovaries. Analysis of preoperative blood serum showed elevated levels of CYFRA 21-1 (10.4 ng/mL), neuron-specific enolase (36.2 ng/mL), oestradiol (485 pg/mL) and CA-125 (984 U/mL). Adjuvant chemotherapy and regional hyperthermia were performed due to the malignant potential and incomplete resection of the tumour.
Undifferentiated Sertoli-Leydig cell tumours show a poor clinical course. As only two-thirds of patients with this rare disease present with elevated hormone levels, new markers deserve further investigation to offer more specific, individualised tumour monitoring.
卵巢支持-间质细胞瘤仅占卵巢恶性肿瘤的0.2%。所有患者中有三分之二因肿瘤产生激素而出现症状。
一名41岁患者(孕4产4)出现呼吸困难、腹围增大和黑便。诊断性影像学检查怀疑为卵巢癌。卵巢癌的标准肿瘤标志物(CA 125)升高至984 U/mL。
腹部手术探查发现双侧附件有一个腐烂的肿瘤,延伸至肚脐水平。冰冻切片显示为来源不明的未分化癌。进行了根治性手术。最终组织学报告描述为一种恶性性索间质肿瘤,即支持-间质细胞瘤,起源于双侧卵巢。术前血清分析显示CYFRA 21-1(10.4 ng/mL)、神经元特异性烯醇化酶(36.2 ng/mL)、雌二醇(485 pg/mL)和CA-125(984 U/mL)水平升高。由于肿瘤具有恶性潜能且切除不完全,进行了辅助化疗和区域热疗。
未分化支持-间质细胞瘤临床病程较差。由于这种罕见疾病只有三分之二的患者激素水平升高,因此新的标志物值得进一步研究,以提供更具特异性的个体化肿瘤监测。