Giuliani Antonio, Romano Lucia, Romanzi Federica, Giubbolini Giacomo, Coletti Gino, Di Stefano Nicoletta, Le'Clerc Jean Nicolas, Schietroma Mario, Carlei Francesco, Di Stefano Leonardo
Ann Ital Chir. 2020 Jan 19;9:S2239253X20031527.
Uterine sarcomas are mesenchymal tumors; they are rare, representing less than 2-3% of all uterine malignancies. Among them, we can define four types: leiomyosarcoma (LMS), endometrial stromal sarcoma (ESS), Adenosarcoma and Carcinosarcoma. This last type was recently reclassified by FIGO as a Mullerian type of the endometrial adenocarcinoma. Therefore, today only the first three types are histologically considered.
In this paper, we reported a case of simultaneous presence of three different rare neoplasms in the same surgical specimen, resulting from a hysterectomy of a premenopausal woman. The woman presented to the ED with a six-months history of vaginal bleeding. Given the complexity of the clinical picture, we suggested hospitalization in our Department of Gynecology, to perform appropriate diagnostic tests. Because of the persistent hemorrhage and the absence of required fertility preservation, a laparotomic hysterectomy with bilateral annessiectomy was performed.
The postoperative histology of the specimen described the myoma at the fundus as a leiomyosarcoma. The myoma of the uterine anterior wall appeared as an endometrial stromal sarcoma of low-grade. Moreover, an intramural cavernous hemangioma of 3 cm in diameter was reported at the uterine corpus.
All these described pathologies have no specific clinic characteristics; the most common symptom is abnormal uterine bleeding. To date, hysterectomy and bilateral salpingo-oophorectomy are the standards of care in the management of all early stage uterine sarcomas. To our knowledge, cases of LMS, ESS and cavernous haemangioma coexisting in the same patient have not been reported in literature to date. The pathogenesis of this combination remains to be elucidated. Key words: Cavernous hemangioma, Endometrial stromal sarcoma, Leiomyosarcoma, Uterine sarcomas.
子宫肉瘤是间叶组织肿瘤;它们很罕见,占所有子宫恶性肿瘤的比例不到2%-3%。其中,我们可以定义四种类型:平滑肌肉瘤(LMS)、子宫内膜间质肉瘤(ESS)、腺肉瘤和癌肉瘤。最近,国际妇产科联盟(FIGO)将最后一种类型重新分类为子宫内膜腺癌的苗勒氏类型。因此,如今在组织学上仅考虑前三种类型。
在本文中,我们报告了一例在同一手术标本中同时存在三种不同罕见肿瘤的病例,该标本取自一名绝经前女性的子宫切除术。该女性因阴道出血六个月就诊于急诊科。鉴于临床表现复杂,我们建议她入住我们的妇科进行适当的诊断检查。由于持续出血且无需保留生育功能,遂行剖腹子宫切除术及双侧附件切除术。
标本的术后组织学检查显示,子宫底部的肌瘤为平滑肌肉瘤。子宫前壁的肌瘤表现为低级别子宫内膜间质肉瘤。此外,子宫体部报告有一个直径3厘米的壁内海绵状血管瘤。
所有这些所描述的病理情况均无特异性临床特征;最常见的症状是子宫异常出血。迄今为止,子宫切除术和双侧输卵管卵巢切除术是所有早期子宫肉瘤治疗的标准方法。据我们所知,文献中迄今尚未报道同一患者同时存在平滑肌肉瘤、子宫内膜间质肉瘤和海绵状血管瘤的病例。这种组合的发病机制仍有待阐明。关键词:海绵状血管瘤、子宫内膜间质肉瘤、平滑肌肉瘤、子宫肉瘤