Multiple epitheloid plexiform tumourlet leiomyoma of the uterus with focal vascular invasion

Department of Gynaecology, Erasme Hospital, Free University Hospital (ULB), Brussels, Belgium.
European journal of gynaecological oncology (Impact Factor: 0.61). 01/2010; 31(4):443-5.
Source: PubMed


We report the first case of multiple plexiform tumourlets showing focal vascular invasion suggesting that, unlike what the common and accepted opinion would advocate, these tumours may have a more aggressive behaviour. However, the prognosis of this variant of epithelioid leiomyoma remains unknown, due to its rarity. The main differential diagnosis of this entity are discussed, including endometrial stromal sarcoma exhibiting epitheloid cytomorphology, intravenous leiomyomatosis of epitheloid variant.

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    ABSTRACT: Intravascular leiomyomatosis (IVL) is characterized by the presence of smooth muscle in venous and lymphatic spaces within the myometrium. Although the intravascular component usually consists solely of typical smooth muscle or variants of smooth muscle differentiation, we report 5 cases in which the intravascular component also included endometrioid glandular and stromal elements. We propose the term "intravenous adenomyomatosis" to describe this unusual variant of IVL. The mean age of the patients in this series was 50.2 years, slightly older than that of patients with conventional IVL. In addition to intravenous adenomyomatosis, both adenomyosis and leiomyomas were identified in all of our cases, supporting the hypothesis that the intravascular smooth muscle component in IVL is derived from associated myometrial pathology rather than from vessel walls. In our series, intravenous adenomyomatosis had a similar benign clinical behavior to most cases of IVL with no metastatic or recurrent disease identified at follow-up in 4 cases for which follow-up information was available. The main differential diagnoses are adenomyosis with vascular involvement, low-grade endometrial stromal sarcoma (ESS), including ESS with smooth muscle and glandular differentiation, and adenosarcoma with lymphovascular invasion. The possibility of intravenous adenomyomatosis should be borne in mind when considering these diagnoses, particularly ESS and adenosarcoma, which have different implications for patient management and prognosis.
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