背景:上皮样滋养细胞肿瘤(ETT)是妊娠滋养细胞肿瘤(GTNs)的一种极其罕见的变体。ETT的生物学行为和治疗方案仍有待定义,其经常提出诊断和治疗挑战。虽然ETT是一种相对惰性的恶性肿瘤,当出现转移时,治疗效果和生存率显著下降.肺是ETT转移的最常见部位。
方法:一名39岁女性患者出现不规则阴道出血和小腹轻度胀痛。
方法:患者经手术及免疫组化染色后确诊为肺转移瘤。
方法:行全腹子宫切除术加双侧输卵管切除术和组织病理学检查。患者接受了3个周期的依托泊苷,甲氨蝶呤,放线菌素-D/依托泊苷,顺铂(EMA/EP)方案术后化疗。由于肺转移的存在,她接受了肺病灶切除术和另一个周期的术后化疗。
结果:患者最初对治疗表现出良好的反应。然而,患者因家庭原因未完成全部初始治疗,2.5个月后出现复发迹象.血清β-hCG水平逐渐升高,肺部影像学显示病灶面积逐渐扩大。经过15个月的随访,由于没有出现症状,患者拒绝进一步治疗.
结论:异常阴道出血和β-hCG水平低的患者应考虑ETT的诊断。转移性疾病患者应进行完整的手术切除和强化联合化疗,以最大限度地提高治愈机会。靶向生物制剂可能是化疗耐药或复发患者的潜在治疗策略。
BACKGROUND: Epithelioid trophoblastic tumor (ETT) is an extremely rare variant of gestational trophoblastic neoplasms (GTNs). The biological behavior and therapeutic schedule of ETT remains to be defined which frequently poses diagnostic and therapeutic challenges. Although ETT is a relatively indolent malignancy tumor, the therapeutic efficacy and survival rate decrease significantly when presented with metastases. The lung is the most common site of ETT metastasis.
METHODS: A 39-year-old female patient presented with irregular vaginal bleeding and slight distention pain in lower abdomen.
METHODS: The patient was diagnosed ETT with lung metastasis after surgery and immunohistochemical staining.
METHODS: A total abdominal hysterectomy plus bilateral salpingectomy and histopathology were performed. The patient received 3 cycles of etoposide, methotrexate, actinomycin-D/etoposide, cisplatin (EMA/EP) regimen chemotherapy after surgery. Due to the presence of lung metastasis, she received pulmonary lesion resection and another cycle of postoperative chemotherapy.
RESULTS: The patients showed a good response to treatment initially. However, the patient did not complete the full initial treatment for family reasons and had signs of recurrence after 2.5 months. The serum β-hCG level gradually elevated and the lung imaging showed that the lesion area gradually expanded. After 15 months of follow-up, the patient declined further treatment due to a lack of presenting symptoms.
CONCLUSIONS: The diagnosis of ETT should be taken into consideration in patients with abnormal vaginal bleeding and low levels of β-hCG. Patients with metastatic disease should be treated with complete surgical resection and intensive combination chemotherapy to maximize the opportunity for cure. Targeted biological agents might be potential therapeutic strategies for chemotherapy-resistant or recurrent patients.