Intravenous leiomyomatosis

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[edit] Discussion of Intravenous leiomyomatosis

  • 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.

  • 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.

  • 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

  • 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.

  • 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.

[edit] Imaging Findings for Intravenous leiomyomatosis

[edit] Images

Patient #1: Patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus

[edit] See Also

[edit] External Links

[edit] References for Intravenous leiomyomatosis