Intravenous leiomyomatosis: A case report and narrative literature review

In the present study, we report a case of intravenous leiomyomatosis with intracardiac extension. A subsequent extensive literature review was initiated in order to characterize this entity in terms of clinical manifestations, diagnosis and treatment strategies. We performed a literature search in PubMed, Webscience and Scopus using the MeSH term "intravenous leiomyomatosis" and included case reports, case series and retrospective studies published in the last 10 years. A total of 74 articles, with a total of 672 cases of intravenous leiomyomatosis were included. The average age at diagnosis was 45.5 years and 92% of the cases were reported in Asian countries. A total of 21% of the patients were asymptomatic. The most frequent symptoms included perception of pelvic mass, dyspnea, abnormal uterine bleeding and palpitations. In 55% of cases there was a preoperative suspicion of the diagnosis. In 61%, the lesion was confined to the pelvic cavity and in 35% there was extension beyond the renal veins (including intracardiac extension). When there was a preoperative diagnosis, the surgical team usually included elements from gynecology, vascular surgery and/or cardiac surgery. The treatment consisted of one-stage surgery in 86% of cases, with complete resection in 90%. The recurrence rate was 12%. Intravenous leiomyomatosis can have a nonspecific presentation which, combined with its rarity, requires a high degree of suspicion. The challenges in its treatment and follow-up arise from its surgical complexity and the absence of guidelines. The objective of this review was to compile the most recent cases reported to better characterize this rare entity thereby achieving optimal management.