Department of Gynecology with Center for Oncological Surgery, European Competence Center for Ovarian Cancer, Charité - Campus Virchow-Klinikum, University Medicine of Berlin, Berlin, Germany
Institute of Pathology, Charité - Campus Virchow-Klinikum, University Medicine of Berlin, Berlin, Germany.
Anticancer Res. 2020 Oct;40(10):5743-5750. doi: 10.21873/anticanres.14590.
BACKGROUND/AIM: Angiosarcoma of primary gynecologic origin is an extremely rare and highly malignant tumor of endothelial origin with a 5-year survival rate of less than 35%. To date, only 61 cases have been described in the literature. The aim of this study was to present more cases and discuss potential therapy options.
The following case series presents three cases of gynecologic angiosarcomas that were under therapy at the Charité - University medicine of Berlin from June 2014 to February 2018.
Two of the cases deal with primary angiosarcomas of the uterus whereas the third case was diagnosed after the suspicion of a recurrence of a poorly differentiated squamous cell carcinoma of the cervix uteri. In case one a 75-year old patient with initial postmenopausal bleeding and a tumor mass of the uterus is described. After surgery a hemangiosarcoma of the uterus was confirmed. After two months the patient presented with a presacral peritoneal sarcomatosis. Chemotherapy of weekly paclitaxel was administered. Case two deals with a patient presenting with abdominal pain. A uterine sarcoma with infiltration of the parametry and angiosarcomatosis peritonei was diagnosed during an emergency laparotomy because of spontaneous peritoneal bleeding. Moreover, osseous metastasis was found. The patient underwent weekly paclitaxel. Due to tumor progression, chemotherapy was changed to doxorubicin and olaratumab and radiotherapy was induced. The patient died 33 months after initial diagnosis. Case three describes a 34-year old patient with suspected local recurrence of cervical cancer with infiltration of the bladder. During TURB an angiosarcoma was found. Following laparoscopy revealed peritoneal metastasis. The patient underwent weekly paclitaxel followed by a paclitaxel and pazopanib maintainance therapy which showed a regression. Due to progression afterwards, chemotherapy was changed to gemcitabine and docetaxel and gemcitabine monotherapy. The patient died 33 months after initial diagnosis.
Even though there is no evidence on standard treatment of this extremely rare and aggressive tumor entity of the female genital tract the patients showed the longest stability of disease during chemotherapy with weekly paclitaxel.
背景/目的：原发性妇科来源的血管肉瘤是一种极其罕见且高度恶性的内皮来源肿瘤，5 年生存率低于 35%。迄今为止，文献中仅描述了 61 例。本研究旨在介绍更多病例并讨论潜在的治疗选择。
本病例系列介绍了在柏林 Charité 大学医学中心接受治疗的 3 例妇科血管肉瘤病例，时间从 2014 年 6 月至 2018 年 2 月。
其中 2 例为原发性子宫血管肉瘤，第 3 例为宫颈局部低分化鳞癌复发的可疑病例。第 1 例为 75 岁绝经后出血患者，伴有子宫肿瘤。手术后证实为子宫血管肉瘤。2 个月后，患者出现骶前腹膜肉瘤病。给予每周紫杉醇化疗。第 2 例患者表现为腹痛。因自发性腹膜出血行紧急剖腹探查术诊断为子宫肉瘤伴附件浸润和腹膜血管肉瘤病，此外还发现骨转移。患者接受每周紫杉醇化疗。由于肿瘤进展，化疗改为多柔比星和奥拉单抗，并进行放疗。患者在初始诊断后 33 个月死亡。第 3 例患者为 34 岁，疑似宫颈癌局部复发伴膀胱浸润。行 TURB 时发现血管肉瘤。腹腔镜检查显示腹膜转移。患者接受每周紫杉醇治疗，随后进行紫杉醇和帕唑帕尼维持治疗，显示肿瘤缩小。随后进展，化疗改为吉西他滨和多西他赛，吉西他滨单药治疗。患者在初始诊断后 33 个月死亡。
尽管对于这种女性生殖道极其罕见且侵袭性的肿瘤实体，尚无标准治疗的证据，但患者在接受每周紫杉醇化疗时疾病稳定时间最长。
