• Jing Li, Zhenwu Du, Tianmin Xu, Chenhong Li, Shumin Ba, and He Zhu.
• Department of Obstetrics and Gynecology, The Second Hospital of Jilin University, Changchun, Jilin, China.
• Medicine (Baltimore). 2024 Jul 5; 103 (27): e38108e38108.

RationaleEpithelioid 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.Patient ConcernsA 39-year-old female patient presented with irregular vaginal bleeding and slight distention pain in lower abdomen.DiagnosesThe patient was diagnosed ETT with lung metastasis after surgery and immunohistochemical staining.InterventionsA 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.OutcomesThe 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.LessonsThe 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.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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