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首页> 外文期刊>Gynecologic Oncology Reports >Poorly differentiated, ovarian Sertoli-Leydig cell tumor with heterologous rhabdomyosarcoma and glandular elements: Diagnosis and management of a rare neoplasm
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Poorly differentiated, ovarian Sertoli-Leydig cell tumor with heterologous rhabdomyosarcoma and glandular elements: Diagnosis and management of a rare neoplasm

机译：异质性横纹肌肉瘤和腺体成分低分化的卵巢Sertoli-Leydig细胞肿瘤：罕见肿瘤的诊断和处理

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摘要

Ovarian Sertoli-Leydig cell tumors (SLCT) represent <2% of primary ovarian tumors, which range from benign to malignant; majority of the latter are low-grade. We present the case of a 12-year-old pre-pubertal female with poorly differentiated SLCT and heterologous rhabdomyosarcoma (FIGO stage-IA). She presented with worsening abdominal pain, pelvic mass, and elevated pre-operative serum Alpha-fetoprotein (AFP, 77.1?ng/mL). She underwent right salpingo-oophorectomy, omentectomy, and lymphadenectomy. The high-risk histology warranted 4-cycles of adjuvant BEP chemotherapy. There was no clinical evidence of recurrence at the 20-month follow-up. High-grade sex cord-stromal tumors are rare, present with low-stage disease, and have good progression-free survival following chemotherapy. Rarity of these tumors poses challenges in their diagnosis and treatment. Review of literature suggests that the presented case is youngest patient with dedifferentiated SLCT. Highlights ? SLCTs are uncommon; mostly well-differentiated; dedifferentiation is rare. ? AFP can be elevated; caveat is not to misdiagnose as yolk-sac tumor. ? Presented case is youngest reported, had low-stage disease, high-risk histology. ? High-risk histology, rupture & high-stage need treatment and confer poor prognosis.
机译：卵巢Sertoli-Leydig细胞肿瘤（SLCT）占原发性卵巢肿瘤的<2％，范围从良性到恶性。后者多数是低级的。我们介绍了一个12岁的青春期前女性，其SLCT和异源性横纹肌肉瘤（FIGO-IA期）分化差。她表现出腹痛加剧，骨盆肿块和术前血清甲胎蛋白（AFP，77.1？ng / mL）升高。她接受了右输卵管卵巢切除术，网膜切除术和淋巴结清扫术。高危组织学证明辅助BEP化疗需要4个周期。在20个月的随访中没有复发的临床证据。高等级性索-间质肿瘤罕见，现存低期疾病，化疗后具有良好的无进展生存期。这些肿瘤的稀有性对其诊断和治疗提出了挑战。文献综述表明，本病例是最年轻的SLCT患者。强调 ？ SLCT不常见;差异很大;去分化是罕见的。 ？ AFP可以升高;注意不要误诊为卵黄囊肿瘤。 ？本病例报告最年轻，有低期疾病，高危组织学。 ？高危组织学，破裂和高危需要治疗，预后不良。

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