Intravenous leiomyomatosis: Difference between revisions

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{{CMG}} '''Associate Editor-In-Chief:''' {{CZ}}; {{Ammu}}


'''Associate Editor-In-Chief:''' {{CZ}}; {{Ammu}}
{{SK}} Nesidioblastoma, IVLM
 
{{SK}} Nesidioblastoma


==Overview==
==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. Female are affected with Intravenous leiomyomatosis. Common complications of Intravenous leiomyomatosis include embolization, recurrence of the tumor, and metastasis.
*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. <ref>DJ Kaszar-Seibert, GP Gauvin, PA Rogoff, FJ Vittimberga, S Margolis, AD Hilgenberg, DK Saal, and GO Goldsmith. [http://radiology.rsnajnls.org/cgi/content/abstract/168/2/409 Intracardiac extension of intravenous leiomyomatosis.] Radiology 1988 168: 409-410.</ref>


==Pathophysiology==
==Pathophysiology==
*The etiology of intravenous leiomyomatosis is unclear. All described patients are female, and most are white, premenopausal, and parous.  
* 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]].  
* All described patients are female, and most 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.
*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==
==Differentiating Intravenous Leiomyomatosis from other Diseases==
*Intravenous leiomyomatosis must be differentiated from other diseases such as:
*Intravenous leiomyomatosis must be differentiated from other diseases such as:
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*Female are affected with Intravenous leiomyomatosis.
*Female are affected with Intravenous leiomyomatosis.
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*Common complications of Intravenous leiomyomatosis include embolization, recurrence of tumor, and metastasis.
*Common complications of Intravenous leiomyomatosis include embolization, recurrence of the tumor, and metastasis.
*The tumor is slow growing, and the prognosis is favorable.  
*The tumor is slow growing, and the prognosis is favorable.  
== Diagnosis ==
== Diagnosis ==
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==Images==
==Images==
===Example #1===
===Example #1===
Patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus.
The patient presented with S.O.B. one year after hysterectomy for a leiomyomatous uterus.
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==Treatment==
*Surgery is the mainstay of therapy for intravenous leiomyomatosis.


==Related Chapters==
==Related Chapters==

Revision as of 20:19, 14 April 2016

WikiDoc Resources for 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. Female are affected with Intravenous leiomyomatosis. Common complications of Intravenous leiomyomatosis include embolization, recurrence of the tumor, and metastasis.

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.
  • All described patients are female, and most 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 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

  • The patients may be asymptomatic or have symptoms of uterine leiomyomas, syncopal episodes, dyspnea on exertion, shortness of breath

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.

Related Chapters

References


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