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Intravascular Adenomyomatosis: Expanding the Morphologic Spectrum of Intravascular Leiomyomatosis

Hirschowitz, Lynn MBBCh, FRCPath*; Mayall, Frederick G. FRCPath; Ganesan, Raji FRCPath*; McCluggage, W. Glenn FRCPath

The American Journal of Surgical Pathology: September 2013 - Volume 37 - Issue 9 - p 1395–1400
doi: 10.1097/PAS.0b013e31828b2c99
Original Articles

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.

*Department of Cellular Pathology, Birmingham Women’s NHS Foundation Trust, Birmingham

Department of Histopathology, Musgrove Park Hospital, Taunton

Department of Pathology, Royal Group of Hospitals Trust, Belfast, Northern Ireland, UK

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Lynn Hirschowitz, MBBCh, FRCPath, Department of Cellular Pathology, Birmingham Women’s NHS Foundation Trust, Mindelsohn Way, Edgbaston, Birmingham B15 2TG, UK (e-mail:

© 2013 by Lippincott Williams & Wilkins.