[1116] 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.

Table 1. Clinical and pathologic findings.
CaseAge (y)PresentationFollow-upProcedureSize (cm)Extrauterine Extension
152Chest pain, syncopeANDTAH-BSO; thrombectomy1.0-18.0Y
250Abdominal distention/pressureAND, lost to F/U. Recurrence at 152 months.Multiple mass excisions; initial - TAH4.3-17.0Y
349UnknownANDTAH, thrombectomy0.5Y
441FIBANDSupracervical hysterectomy2.5N
564FIBDead, unknown causeTAH-BSO1.2N
647FIB, ovarian cystLost to F/UTAH-BSO1.0N
747FIB, pelvic painANDTAH-BSO4.0N
843MENANDTAH-BSO4.3N
942FIB, MENANDTAH2.5N
1061Uterine mass, bleedingANDTAH-BSO2.0N
1140FIB, MENANDTAH3.5N
1238MEN, METLost to F/UTAH-BSO3.5N
Abbreviations: AND, alive with no evidence of disease; F/U, Follow-up; FIB, fibroid uterus; MEN, menorrhagia; MET, metrorrhagia; TAH-BSO, total abdominal hysterectomy-bilateral salpingo-oophorectomy.


Conclusions: Our study represents the fourth largest IVL cohort in medical literature to date, and the third largest from a single institution. IVL is a rare and potentially underdiagnosed neoplastic condition of smooth muscle origin that may result in significant morbidity when extrauterine vasculature or organs are involved. A high index of suspicion must be maintained. Prompt diagnosis combined with surgical excision has been shown to portend an increased survival rate and good overall prognosis in such cases.
Category: Gynecologic & Obstetrics

Tuesday, March 5, 2013 1:00 PM

Poster Session IV # 240, Tuesday Afternoon

 

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