Unit of Obstetrics and Gynecology, IRCCS- Azienda Unità Sanitaria Locale, Viale Risorgimento n 80, Reggio Emilia, Italy.
Laboratory of Translational Research, Azienda Unità Sanitaria Locale, IRCCS, Reggio Emilia, Italy.
BMC Cancer. 2018 Feb 5;18(1):134. doi: 10.1186/s12885-018-4037-y.
Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours' histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes.
A 79-year-old woman was referred to our Department with an abdominal mass resembling a fibroid with a haemorrhage. Her gynaecological history was negative. A transvaginal and transabdominal ultrasound examination revealed a multicystic mass resembling an ovarian tumour arising from the pelvis and extending up to the abdomen. At laparotomy a peritoneal mass arising from Douglas peritoneum was resected. The uterus and adnexa appeared normal, and a supra-cervical hysterectomy with bilateral salpingo-oophorectomy was performed. No macroscopic residual disease was present. Final pathology diagnosed a malignant peripheral nerve sheath tumors with divergent differentiation. Four weeks later a new, multicystic mass was found. Due to the progressive poor condition, the patient died four months after diagnosis. Histological slides were reviewed by external expert pathologists and the final diagnosis was of extra-genital adenosarcoma with sarcomatous overgrowth. Furthermore, we also collected and analysed articles written in English regarding extra-uterine and extra-ovarian adenosarcomas published between January 1974 and October 2016. PubMed was used as a database for this search. Clinical and pathological characteristics, treatments and outcomes were assessed.
Only 41 cases has been reported in literature. Previous endometriosis and sarcomatous overgrowth showed an inverse effect on prognosis. Endometriosis was confirmed to have a positive effect on disease free survival Complete surgical resection is the mainstay of treatment. A worldwide registry is urgently required to collect data to standardize treatment and to obtain reliable data on prognosis.
子宫外的苗勒管混合性腺肉瘤因其是否存在子宫内膜异位症或肉瘤性过度生长而具有不同的生物学行为。这些行为表现取决于肿瘤的组织学特征和起源部位。由于文献中仅描述了少数子宫外和卵巢外的苗勒管混合性腺肉瘤病例，因此最佳治疗和肿瘤学结果尚未阐明。在这里，我们报告了一例原发性腹膜腺肉瘤伴肉瘤性过度生长，并回顾了所有报道的发生在子宫和卵巢外的腺肉瘤病例，以确定最佳治疗方案并阐明结果。
一名 79 岁女性因类似出血性纤维瘤的腹部肿块就诊于我院。她的妇科病史为阴性。经阴道和经腹超声检查显示，来自骨盆并延伸至腹部的多房囊性肿块，类似于卵巢肿瘤。剖腹探查时，切除了来自道格拉斯腹膜的腹膜肿块。子宫和附件外观正常，行经宫颈子宫切除术和双侧输卵管卵巢切除术。无肉眼残留病变。最终病理诊断为恶性外周神经鞘肿瘤伴分化。四周后，发现了一个新的多房性肿块。由于病情逐渐恶化，患者在诊断后四个月死亡。外部专家病理学家对组织学切片进行了复查，最终诊断为生殖器官外腺肉瘤伴肉瘤性过度生长。此外，我们还收集并分析了 1974 年 1 月至 2016 年 10 月期间以英文发表的关于子宫外和卵巢外腺肉瘤的文章。使用 PubMed 作为该搜索的数据库。评估了临床和病理特征、治疗方法和结果。
文献中仅报道了 41 例病例。既往子宫内膜异位症和肉瘤性过度生长对预后有相反的影响。子宫内膜异位症被证实对无病生存有积极影响。完全手术切除是主要的治疗方法。迫切需要建立一个全球登记处，以收集数据来规范治疗，并获得关于预后的可靠数据。
