Intravenous leiomyomatosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Ammu Susheela, M.D. [3]

Synonyms and Keywords: Nesidioblastoma, IVLM

Overview

Intravenous leiomyomatosis 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. The etiology of intravenous leiomyomatosis is unclear. Intravenous leiomyomatosis must be differentiated from other diseases such as renal malignancies and sarcoma. The median age is 45 years, with patients ranging from 26 to 70 years old. Only females may develop intravenous leiomyomatosis. Common complications of intravenous leiomyomatosis include embolization, recurrence of the tumor, and metastasis. Surgery is the mainstay of therapy for intravenous leiomyomatosis.

Pathophysiology

  • Intravenous leiomyomatosis 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.
  • Patients are exclusively female, and the majority are white, premenopausal, and parous.
  • Intravenous leiomyomatosis 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.

Causes

  • The etiology of intravenous leiomyomatosis is unclear.

Differentiating Intravenous Leiomyomatosis from other Diseases

  • Intravenous leiomyomatosis must be differentiated from other diseases such as:
  • Renal malignancies
  • Sarcoma
  • Thrombosis of the intravenous catheter

Epidemiology and Demographics

  • The median age is 45 years, with patients ranging from 26 to 70 years old.
  • Female are exclusively affected with intravenous leiomyomatosis.

Natural History, Complications and Prognosis

  • Common complications of intravenous leiomyomatosis include embolization, recurrence of the tumor, and metastasis.
  • The tumor is slow growing, and the prognosis is favorable.

Diagnosis

Symptoms

Images

Example #1

The patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus.

Treatment

  • Surgery is the mainstay of therapy for intravenous leiomyomatosis.

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