Clinicopathologic Characteristics of Intravenous Leiomyomatosis: An Experience of 12 Cases at a Single Medical Center
Ryan J Carr, Pei Hui, Natalia Buza. Yale School of Medicine, New Haven, CT
Background: Intravenous leiomyomatosis (IVL) is a rare neoplasm of smooth muscle origin with the capacity for distant vascular and/or organ extension. The primary lesion is typically found in the uterus, with the most common extrauterine sites being the inferior vena cava, right heart chambers or pulmonary vasculature. Extrauterine involvement can result in direct mass effect or vascular obstruction with associated clinical sequelae.
Design: Twelve cases of IVL were identified in our pathology archives over a twenty-four year period (1988-2012). Patient medical records and gross pathological findings were reviewed. Histologic and related immunohistochemical slides (smooth muscle active, desmin, CD31 and CD34) were re-evaluated by two gynecologic pathologists to confirm the diagnosis using conventional diagnostic criteria.
Results: Clinicopathologic features are summarized in Table 1. Patient age at initial diagnosis ranged from 38 to 64 years (mean: 47.8 years), with a median age of 47 years. Three cases demonstrated extrauterine extension, one case was found to be extrauterine in origin (i.e. arising from the broad ligament) and one case was associated with disease recurrence.
|Case||Age (y)||Presentation||Follow-up||Procedure||Size (cm)||Extrauterine Extension|
|1||52||Chest pain, syncope||AND||TAH-BSO; thrombectomy||1.0-18.0||Y|
|2||50||Abdominal distention/pressure||AND, lost to F/U. Recurrence at 152 months.||Multiple mass excisions; initial - TAH||4.3-17.0||Y|
|5||64||FIB||Dead, unknown cause||TAH-BSO||1.2||N|
|6||47||FIB, ovarian cyst||Lost to F/U||TAH-BSO||1.0||N|
|7||47||FIB, pelvic pain||AND||TAH-BSO||4.0||N|
|10||61||Uterine mass, bleeding||AND||TAH-BSO||2.0||N|
|12||38||MEN, MET||Lost to F/U||TAH-BSO||3.5||N|