Idegami Daiki, Amano Tsukuru, Torii Hiroko, Tsuji Shunichiro, Urabe Mamoru, Murakami Takashi
Department of Obstetrics and Gynecology, Saiseikai Shigaken Hospital, Ritto, Japan.
Department of Obstetrics and Gynecology, Shiga University of Medical Science, Otsu, Japan.
Case Rep Oncol. 2024 Jun 19;17(1):666-672. doi: 10.1159/000539428. eCollection 2024 Jan-Dec.
Epithelioid endothelial tumor (ETT) is an extremely rare tumor that typically occurs in women of reproductive age. The diagnosis tends to be delayed because it often necessitates a total hysterectomy. Therefore, it is important to understand ETT macroscopic and imaging findings. Here, we report a case of ETT with detailed macroscopic and imaging findings.
A 39-year-old woman with positive pregnancy test results was admitted to a nearby hospital. No gestational sac was found in the uterus, and magnetic resonance imaging (MRI) revealed a cystic mass of approximately 7 cm that extended continuously from the anterior wall of the lower uterine segment into the pelvic cavity. She underwent laparoscopic and hysteroscopic surgeries for a ruptured cervical pregnancy. Pathology of the specimens obtained from this surgery did not allow for the diagnosis of ETT. Two months after the surgery, as the serum human chorionic gonadotropin β subunit (β-HCG) level did not decrease, she was diagnosed with low-grade gestational trophoblastic neoplasia, leading to the administration of chemotherapy. After three regimens of chemotherapy over 9 months, her β-HCG level decreased but did not reach normal levels. Ultimately, a total hysterectomy was performed. The pathological diagnosis was mixed ETT and choriocarcinoma. A literature review revealed several cases similar to ours.
ETT in the lower uterus often perforates the myometrium and forms cystic lesions in the retroperitoneal space or subserosa. The MRI and laparoscopic/hysteroscopic findings in this case may have contributed to the early diagnosis of ETT.
上皮样内皮瘤（ETT）是一种极其罕见的肿瘤，通常发生于育龄女性。由于其诊断往往需要进行全子宫切除术，故诊断往往会延迟。因此，了解ETT的宏观和影像学表现很重要。在此，我们报告一例具有详细宏观和影像学表现的ETT病例。
一名妊娠试验结果呈阳性的39岁女性入住附近医院。子宫内未发现妊娠囊，磁共振成像（MRI）显示一个约7 cm的囊性肿块，从子宫下段前壁连续延伸至盆腔。她因宫颈妊娠破裂接受了腹腔镜和宫腔镜手术。此次手术获取标本的病理检查无法诊断为ETT。手术后两个月，由于血清人绒毛膜促性腺激素β亚基（β-HCG）水平未下降，她被诊断为低级别妊娠滋养细胞肿瘤，从而接受化疗。在9个月内进行了三个疗程的化疗后，她的β-HCG水平有所下降，但未恢复至正常水平。最终，她接受了全子宫切除术。病理诊断为混合型ETT和绒毛膜癌。文献回顾显示了几例与我们的病例相似的情况。
子宫下段的ETT常穿透肌层，并在腹膜后间隙或浆膜下形成囊性病变。该病例的MRI及腹腔镜/宫腔镜检查结果可能有助于ETT的早期诊断。
