Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30–40% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases.
侵袭性血管黏液瘤是一种罕见的、具有局部侵袭性的黏液样间叶组织肿瘤，主要发生于青年女性的盆腔和会阴区域。该肿瘤生长缓慢且具有局部浸润性。由于此类肿瘤罕见且缺乏特征性体征与症状，术前诊断较为困难。主要治疗方式为肿瘤切除术。因其浸润性生长特性且缺乏明确包膜，不完全切除的情况较为常见。临床上也采用其他非手术疗法，如放射治疗、栓塞术、促性腺激素释放激素类似物或其他抗雌激素药物。局部复发率约为30%-40%，且常在首次切除多年（有时甚至数十年）后出现。偶有远处转移的病例报道。目前文献中报道的病例数量有限，多为小型病例系列或个案报告。因此，由MITO罕见肿瘤专家组撰写的本文旨在综述该罕见疾病的临床表现、病理特征及患者预后，以期为临床诊疗提供最新指导。
Aggressive Angiomyxoma of the Lower Female Genital Tract: A Review of the MITO Rare Tumors Group
肿瘤(癌症)患者之家