Intravenous leiomyomatosis: Difference between revisions
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'''Associate Editor-In-Chief:''' {{CZ}} | '''Associate Editor-In-Chief:''' {{CZ}} | ||
{{SK}} Nesidioblastoma | |||
==Overview== | ==Overview== | ||
Revision as of 17:30, 12 September 2012
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]
Synonyms and keywords: Nesidioblastoma
Overview
- Intravenous leiomyomatosis (IVLM) is characterized by the extension into venous channels of histologically benign smooth muscle tumor arising from either the wall of a vessel or from a uterine leiomyoma.
- Fewer than 100 cases have been reported in all, and only 14 cases involved intracardiac extension from the IVC.
- In one reported case, this slowly growing invasive neoplasm extended not only into the heart but into both pulmonary arteries as well. [1]
Pathophysiology
- The etiology of IVLM is unclear. All described patients are female, and most are white, premenopausal, and parous.
- The median age is 45 years, with patients ranging from 26 to 70 years old.
- The patients may be asymptomatic or have symptoms of uterine leiomyomas.
- Patients with obstruction of the right atrium may present with syncopal episodes, dyspnea on exertion, shortness of breath, etc.
- The tumor is slow growing, and the prognosis is favorable.
Differentiating Intravenous Leiomyomatosis from other Diseases
- When the IVC is involved the differential diagnosis should include renal malignancies and primary leiomyoma or sarcoma, as well as thrombosis of the IVC.
- IVLM should be considered in young women with cardiac symptoms who have a right atrial mass as well as a pelvic mass or who have previously undergone hysterectomy for leiomyoma uterus with intravenous involvement.
Complications
- Although embolization of the tumor represents a theoretical risk, this has not been reported.
- The tumor can recur, and repeat operation may be necessary.
- Most reported deaths involved extension of the tumor into the heart, with death due to mechanical obstruction rather than the neoplastic process per se
- Intravenous leiomyomatosis should not be confused with benign metastasizing leiomyoma, in which a benign uterine leiomyoma is associated with a benign smooth muscle tumor located in the parenchyma of a distant organ, such as lung.
- Intravenous leiomyomatosis is confined to vessels, whereas benign metastasizing leiomyoma shows no relation to vascular channels.
Images
Example #1
Patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus (Images shown below are courtesy of RadsWiki and copylefted}
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CT in Intravenous leiomyomatosis
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Related Chapters
- Uterine leiomyoma
- Benign metastasizing leiomyoma
References
- ↑ DJ Kaszar-Seibert, GP Gauvin, PA Rogoff, FJ Vittimberga, S Margolis, AD Hilgenberg, DK Saal, and GO Goldsmith. Intracardiac extension of intravenous leiomyomatosis. Radiology 1988 168: 409-410.