Intravenous leiomyomatosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{SI}}
{{SI}}
{{CMG}} {{AE}}<br>
{{CMG}} {{AE}} {{Sonia Sandeep}} {{Sahar}}<br>
{{SK}} Nesidioblastoma, IVLM
{{SK}} Nesidioblastoma, IVLM
==Overview==
==Overview==
Intravenous leiomyomatosis is also refered as [IVL]. 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. Surgery is the mainstay of therapy for intravenous leiomyomatosis. It can grow in to lymphatics /veins.
Intravenous leiomyomatosis is also refered as IVL. Intravenous leiomyomatosis is characterized by the [[extension]] into [[venous]] channels of [[histologically]] [[Leiomyoma|benign smooth muscle tumor]] arising from either the wall of a [[Vessel wall|vessel]] or from a [[uterine leiomyoma]]. The [[etiology]] of intravenous leiomyomatosis is unclear. Intravenous leiomyomatosis must be [[Differentiate|differentiated]] from other diseases such as [[Renal cell cancer|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. Findings on [[CT scan]] [[diagnostic]] of intravenous leiomyomatosis include an irregular [[mass]] exhibiting [[heterogeneous]] enhancement with [[extension]] into adjacent [[veins]]. Findings on an [[ultrasound]] [[diagnostic]] of intravenous Leiomyomatosis include a vascularized [[thrombi]] within the [[pelvic]] [[Vein|veins]] and [[Inferior vena  cava]]. Findings on [[MRI]] [[diagnostic]] of intravenous leiomyomatosis include a leiomyomatous [[lesion]] or a distorted [[uterus]] with projections into [[vasculature]]. [[Surgery]] is the mainstay of therapy for intravenous leiomyomatosis. It can grow into [[lymphatics]] /[[veins]].
==Historical Perspective==
==Historical Perspective==
*Intravenous leiomyomatosis first described in 1896 by German [[Pathologist]] Birch-Hirschfeld.<ref name="pmid11904348">{{cite journal| author=Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case. | journal=Mod Pathol | year= 2002 | volume= 15 | issue= 3 | pages= 351-6 | pmid=11904348 | doi=10.1038/modpathol.3880529 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11904348  }}</ref>
*Intravenous leiomyomatosis first described in 1896 by German [[Pathologist]] Birch-Hirschfeld.<ref name="pmid11904348">{{cite journal| author=Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case. | journal=Mod Pathol | year= 2002 | volume= 15 | issue= 3 | pages= 351-6 | pmid=11904348 | doi=10.1038/modpathol.3880529 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11904348  }}</ref>
*First case of leiomyo-matosis of pelvic origin with [[intravascular]] extension to [[cardiac]] cavities was described in 1907 in Germany by Dürck and Hörmann.<ref><nowiki>https://www.ncbi.nlm.nih.gov/pubmed/23563052</nowiki></ref>
*First case of leiomyo-matosis of pelvic origin with [[intravascular]] extension to [[cardiac]] cavities was described in 1907 in Germany by Dürck and Hörmann.<ref name="pmid10788838">{{cite journal |vauthors=Stolf NA, dos Santos GG, Haddad VL |title=Unusual abdominal tumors with intracardiac extension. Two cases with successful surgical resection |journal=Rev Hosp Clin Fac Med Sao Paulo |volume=54 |issue=5 |pages=159–64 |date=1999 |pmid=10788838 |doi= |url=}}</ref>
==Classification==  
==Classification==  
* There is no established system for the [[classification]] of intravenous leiomyomatosis.  
* There is no established system for the [[classification]] of intravenous leiomyomatosis.  
==Pathophysiology==
==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]].<ref name="pmid240274072">{{cite journal| author=Mariyappa N, Manikyam UK, Krishnamurthy D, Preeti K, Agarwal Y, Prakar U| title=Intravenous leiomyomatosis. | journal=Niger J Surg | year= 2012 | volume= 18 | issue= 2 | pages= 105-6 | pmid=24027407 | doi=10.4103/1117-6806.103122 | pmc=3762011 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24027407  }}</ref>
*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]].<ref name="pmid240274072">{{cite journal| author=Mariyappa N, Manikyam UK, Krishnamurthy D, Preeti K, Agarwal Y, Prakar U| title=Intravenous leiomyomatosis. | journal=Niger J Surg | year= 2012 | volume= 18 | issue= 2 | pages= 105-6 | pmid=24027407 | doi=10.4103/1117-6806.103122 | pmc=3762011 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24027407  }}</ref>
*Approximately 40% of leiomyomata have cytogenetic abnormalities.
*Approximately 40% of leiomyomata have cytogenetic abnormalities.
*They are benign tumors of uterus that extend to veins system but do not invade the surrounding tissues <ref name="pmid12508249">{{cite journal| author=Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24). | journal=Genes Chromosomes Cancer | year= 2003 | volume= 36 | issue= 2 | pages= 205-6 | pmid=12508249 | doi=10.1002/gcc.10159 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12508249  }}</ref>
*They are benign tumors of uterus that extend to veins system but do not invade the surrounding tissues <ref name="pmid12508249">{{cite journal| author=Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24). | journal=Genes Chromosomes Cancer | year= 2003 | volume= 36 | issue= 2 | pages= 205-6 | pmid=12508249 | doi=10.1002/gcc.10159 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12508249  }}</ref>
*They contains receptors to Estrogen and progesterone and hence response to these hormones <ref name="pmid125082492">{{cite journal| author=Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24). | journal=Genes Chromosomes Cancer | year= 2003 | volume= 36 | issue= 2 | pages= 205-6 | pmid=12508249 | doi=10.1002/gcc.10159 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12508249  }}</ref>
*They contains receptors to Estrogen and progesterone and hence response to these hormones <ref name="pmid125082492">{{cite journal| author=Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24). | journal=Genes Chromosomes Cancer | year= 2003 | volume= 36 | issue= 2 | pages= 205-6 | pmid=12508249 | doi=10.1002/gcc.10159 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12508249  }}</ref>
*It is also referred as quasi-malignant behavior due to its speedy spreading behavior. <ref name="pmid119043482">{{cite journal| author=Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case. | journal=Mod Pathol | year= 2002 | volume= 15 | issue= 3 | pages= 351-6 | pmid=11904348 | doi=10.1038/modpathol.3880529 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11904348 }}</ref>
*It is also referred as quasi-malignant behavior due to its speedy spreading behavior.<ref name="pmid119043482">{{cite journal| author=Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case. | journal=Mod Pathol | year= 2002 | volume= 15 | issue= 3 | pages= 351-6 | pmid=11904348 | doi=10.1038/modpathol.3880529 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11904348  }}</ref>
*Patients are exclusively female, and the majority are white, [[premenopausal]], and multiparous.<ref name="pmid12649198">{{cite journal| author=Quade BJ, Weremowicz S, Neskey DM, Vanni R, Ladd C, Dal Cin P et al.| title=Fusion transcripts involving HMGA2 are not a common molecular mechanism in uterine leiomyomata with rearrangements in 12q15. | journal=Cancer Res | year= 2003 | volume= 63 | issue= 6 | pages= 1351-8 | pmid=12649198 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12649198  }}</ref>
*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.<ref name="pmid125082493">{{cite journal| author=Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC| title=Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24). | journal=Genes Chromosomes Cancer | year= 2003 | volume= 36 | issue= 2 | pages= 205-6 | pmid=12508249 | doi=10.1002/gcc.10159 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12508249 }}</ref>
*On microscopy it is seen as benign, well-differentiated tumor with smooth muscle growing within veins as worm-like projections.
*On microscopy it is seen as benign, well-differentiated tumor with smooth muscle growing within veins as worm-like projections.
* Immunohistochemical studies show presence of  desminand smooth muscle actin, confirming their [[smooth muscle]] nature.  
*Immunohistochemical studies show presence of  desminand smooth muscle actin, confirming their [[smooth muscle]] nature.  
* Tumor cells showed bizarre nuclear morphology with hyperchromatic multilobated nuclei, the mitotic activity was low, with mitotic index of less than 1 per 50 high-power fields
*Tumor cells showed bizarre nuclear morphology with hyperchromatic multilobated nuclei, the mitotic activity was low, with mitotic index of less than 1 per 50 high-power fields
* It has been divided in subtitles based on microscopic appearance as namely, cellular, myxoid and bizarre,however it is not commonly used.
==Causes==
==Causes==
* The etiology of intravenous leiomyomatosis is unclear.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
* The etiology of intravenous leiomyomatosis is unclear.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
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==Differentiating Intravenous Leiomyomatosis from other Diseases==
==Differentiating Intravenous Leiomyomatosis from other Diseases==
*Intravenous leiomyomatosis must be differentiated from other conditions such as intravenous thrombus, leiomyosarcoma, right atrial myxoma and tumor thrombosis with malignant carcinoma, for example, renal carcinoma, hepatocellular carcinoma, adrenal cortical carcinoma.<ref name="LeeKim2011">{{cite journal|last1=Lee|first1=Sak|last2=Kim|first2=Do-Kyun|last3=Narm|first3=Kyoung Shik|last4=Cho|first4=Sang-Ho|title=Pulmonary Artery Embolization of Intravenous Leiomyomatosis Extending into the Right Atrium|journal=The Korean Journal of Thoracic and Cardiovascular Surgery|volume=44|issue=3|year=2011|pages=243–246|issn=2233-601X|doi=10.5090/kjtcs.2011.44.3.243}}</ref><ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
*Intravenous leiomyomatosis must be differentiated from other conditions such as intravenous thrombus, leiomyosarcoma, right atrial myxoma and tumor thrombosis with malignant carcinoma, for example, renal carcinoma, hepatocellular carcinoma, adrenal cortical carcinoma.<ref name="LeeKim2011">{{cite journal|last1=Lee|first1=Sak|last2=Kim|first2=Do-Kyun|last3=Narm|first3=Kyoung Shik|last4=Cho|first4=Sang-Ho|title=Pulmonary Artery Embolization of Intravenous Leiomyomatosis Extending into the Right Atrium|journal=The Korean Journal of Thoracic and Cardiovascular Surgery|volume=44|issue=3|year=2011|pages=243–246|issn=2233-601X|doi=10.5090/kjtcs.2011.44.3.243}}</ref><ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
*For more information on intravenous leiomyomatosis differential diagnosis please [[Leiomyosarcoma differential diagnosis|click here]].
==Epidemiology and Demographics==
==Epidemiology and Demographics==
*There is no available data on the exact incidence of intravenous leiomyomatosis.  
*There is no available data on the exact incidence of intravenous leiomyomatosis.  
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*Intravenous leiomyomatosis is seen more commonly in white female.<ref name="pmid183129832">{{cite journal| author=Poliquin V, Victory R, Vilos GA| title=Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report. | journal=J Minim Invasive Gynecol | year= 2008 | volume= 15 | issue= 2 | pages= 152-60 | pmid=18312983 | doi=10.1016/j.jmig.2007.12.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18312983  }}</ref>
*Intravenous leiomyomatosis is seen more commonly in white female.<ref name="pmid183129832">{{cite journal| author=Poliquin V, Victory R, Vilos GA| title=Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report. | journal=J Minim Invasive Gynecol | year= 2008 | volume= 15 | issue= 2 | pages= 152-60 | pmid=18312983 | doi=10.1016/j.jmig.2007.12.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18312983  }}</ref>
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of intravenous leiomyomatosis are age, cytogenetics , and prior history of this disorder.  
*Common risk factors in the development of intravenous leiomyomatosis are age, cytogenetics , and prior history of uterine leiomyoma, and/or hysterectomy.<ref name="LeitmanKuperstein2008">{{cite journal|last1=Leitman|first1=M.|last2=Kuperstein|first2=R.|last3=Medalion|first3=B.|last4=Stamler|first4=A.|last5=Porat|first5=E.|last6=Rosenblatt|first6=S.|last7=Konen|first7=E.|last8=Krakover|first8=R.|last9=Vered|first9=Z.|title=A highly unusual right atrial mass presented in two women|journal=European Heart Journal - Cardiovascular Imaging|volume=9|issue=6|year=2008|pages=833–834|issn=2047-2412|doi=10.1093/ejechocard/jen173}}</ref>
==Screening==
==Screening==
*There is insufficient evidence to recommend routine screening for intravenous leiomyomatosis.
*There is insufficient evidence to recommend routine [[screening]] for intravenous leiomyomatosis.
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*If left untreated, patients with intravenous leiomyomatosis may progress to develop congestive heart failure, venous obstruction, and sudden death.<ref name="pmid284149953">{{cite journal| author=Schäfer HM, Isaak A, Gürke L| title=Case report of an intracaval leiomyomatosis 10 months after complete hysterectomy. | journal=Int J Surg Case Rep | year= 2017 | volume= 35 | issue=  | pages= 1-3 | pmid=28414995 | doi=10.1016/j.ijscr.2017.03.031 | pmc=5394212 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28414995  }}</ref><ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref><ref name="KocicaVranes2005">{{cite journal|last1=Kocica|first1=Mladen J.|last2=Vranes|first2=Mile R.|last3=Kostic|first3=Dusan|last4=Kostic|first4=Natasa Kovacevic-|last5=Lackovic|first5=Vesna|last6=Mihajlovic|first6=Vesna Bozic-|last7=Velinovic|first7=Milos M.|last8=Mikic|first8=Aleksandar Dj.|last9=Kalezic|first9=Nevena Dimitrijevic-|title=Intravenous leiomyomatosis with extension to the heart: Rare or underestimated?|journal=The Journal of Thoracic and Cardiovascular Surgery|volume=130|issue=6|year=2005|pages=1724–1726|issn=00225223|doi=10.1016/j.jtcvs.2005.08.021}}</ref>
*If left untreated, [[patients]] with intravenous leiomyomatosis may progress to develop [[congestive heart failure]], [[venous]] obstruction, and sudden death.<ref name="pmid284149953">{{cite journal| author=Schäfer HM, Isaak A, Gürke L| title=Case report of an intracaval leiomyomatosis 10 months after complete hysterectomy. | journal=Int J Surg Case Rep | year= 2017 | volume= 35 | issue=  | pages= 1-3 | pmid=28414995 | doi=10.1016/j.ijscr.2017.03.031 | pmc=5394212 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28414995  }}</ref><ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref><ref name="KocicaVranes2005">{{cite journal|last1=Kocica|first1=Mladen J.|last2=Vranes|first2=Mile R.|last3=Kostic|first3=Dusan|last4=Kostic|first4=Natasa Kovacevic-|last5=Lackovic|first5=Vesna|last6=Mihajlovic|first6=Vesna Bozic-|last7=Velinovic|first7=Milos M.|last8=Mikic|first8=Aleksandar Dj.|last9=Kalezic|first9=Nevena Dimitrijevic-|title=Intravenous leiomyomatosis with extension to the heart: Rare or underestimated?|journal=The Journal of Thoracic and Cardiovascular Surgery|volume=130|issue=6|year=2005|pages=1724–1726|issn=00225223|doi=10.1016/j.jtcvs.2005.08.021}}</ref>
*Other possible [[complications]] of intravenous leiomyomatosis include [[tumor]] [[recurrence]], and [[metastasis]].<ref name="pmid7465135">{{cite journal |vauthors=Evans AT, Symmonds RE, Gaffey TA |title=Recurrent pelvic intravenous leiomyomatosis |journal=Obstet Gynecol |volume=57 |issue=2 |pages=260–4 |date=February 1981 |pmid=7465135 |doi= |url=}}</ref><ref name="AwonugaRotas2008">{{cite journal|last1=Awonuga|first1=Awoniyi O.|last2=Rotas|first2=Michael|last3=Imudia|first3=Anthony N.|last4=Choi|first4=Christine|last5=Khulpateea|first5=Neekianund|title=Recurrent benign metastasizing leiomyoma after hysterectomy and bilateral salpingo-oophorectomy|journal=Archives of Gynecology and Obstetrics|volume=278|issue=4|year=2008|pages=373–376|issn=0932-0067|doi=10.1007/s00404-008-0581-z}}</ref>
*Other possible [[complications]] of intravenous leiomyomatosis include [[tumor]] [[recurrence]], and [[metastasis]].<ref name="pmid7465135">{{cite journal |vauthors=Evans AT, Symmonds RE, Gaffey TA |title=Recurrent pelvic intravenous leiomyomatosis |journal=Obstet Gynecol |volume=57 |issue=2 |pages=260–4 |date=February 1981 |pmid=7465135 |doi= |url=}}</ref><ref name="AwonugaRotas2008">{{cite journal|last1=Awonuga|first1=Awoniyi O.|last2=Rotas|first2=Michael|last3=Imudia|first3=Anthony N.|last4=Choi|first4=Christine|last5=Khulpateea|first5=Neekianund|title=Recurrent benign metastasizing leiomyoma after hysterectomy and bilateral salpingo-oophorectomy|journal=Archives of Gynecology and Obstetrics|volume=278|issue=4|year=2008|pages=373–376|issn=0932-0067|doi=10.1007/s00404-008-0581-z}}</ref>
*Prognosis is generally good.
*[[Prognosis]] is generally good.
== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Criteria===
*There is no specific diagnostic Criteria for intravenous leiomyomatosis.
*There is no specific [[diagnostic]] Criteria for intravenous leiomyomatosis.
*Diagnosis may be challenging. However, a history of  A history of hysterectomy or the presence of uterine leiomyomas may be helpful.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
*[[Diagnosis]] may be challenging. However, a history of  A history of [[hysterectomy]] or the presence of [[uterine leiomyomas]] may be helpful.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
=== History and Symptoms ===
=== History and Symptoms ===
*[[Patients]] may be asymptomatic or have [[symptoms]] related to uterine leiomyomas.<ref name="pmid7820530">{{cite journal| author=Nakayama Y, Kitamura S, Kawachi K, Kawata T, Fukutomi M, Hasegawa J et al.| title=Intravenous leiomyomatosis extending into the right atrium. | journal=Cardiovasc Surg | year= 1994 | volume= 2 | issue= 5 | pages= 642-5 | pmid=7820530 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7820530  }}</ref><ref name="pmid15985146">{{cite journal| author=Moorjani N, Kuo J, Ashley S, Hughes G| title=Intravenous uterine leiomyosarcomatosis with intracardial extension. | journal=J Card Surg | year= 2005 | volume= 20 | issue= 4 | pages= 382-5 | pmid=15985146 | doi=10.1111/j.1540-8191.2005.200476.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15985146  }}</ref><ref name="pmid183129833">{{cite journal| author=Poliquin V, Victory R, Vilos GA| title=Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report. | journal=J Minim Invasive Gynecol | year= 2008 | volume= 15 | issue= 2 | pages= 152-60 | pmid=18312983 | doi=10.1016/j.jmig.2007.12.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18312983  }}</ref>
*[[Patients]] may be asymptomatic or have [[symptoms]] related to uterine leiomyomas.<ref name="pmid7820530">{{cite journal| author=Nakayama Y, Kitamura S, Kawachi K, Kawata T, Fukutomi M, Hasegawa J et al.| title=Intravenous leiomyomatosis extending into the right atrium. | journal=Cardiovasc Surg | year= 1994 | volume= 2 | issue= 5 | pages= 642-5 | pmid=7820530 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7820530  }}</ref><ref name="pmid15985146">{{cite journal| author=Moorjani N, Kuo J, Ashley S, Hughes G| title=Intravenous uterine leiomyosarcomatosis with intracardial extension. | journal=J Card Surg | year= 2005 | volume= 20 | issue= 4 | pages= 382-5 | pmid=15985146 | doi=10.1111/j.1540-8191.2005.200476.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15985146  }}</ref><ref name="pmid183129833">{{cite journal| author=Poliquin V, Victory R, Vilos GA| title=Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report. | journal=J Minim Invasive Gynecol | year= 2008 | volume= 15 | issue= 2 | pages= 152-60 | pmid=18312983 | doi=10.1016/j.jmig.2007.12.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18312983  }}</ref>
Line 53: Line 51:
*There are no specific laboratory findings associated with intravenous Leiomyomatosis.  
*There are no specific laboratory findings associated with intravenous Leiomyomatosis.  
=== Electrocardiogram ===
=== Electrocardiogram ===
*There are no ECG findings associated with intravenous Leiomyomatosis.
*There are no [[ECG]] findings associated with intravenous Leiomyomatosis.
=== X-ray ===
=== X-ray ===
*There are no x-ray findings associated with intravenous Leiomyomatosis.
*There are no [[x-ray]] findings associated with intravenous Leiomyomatosis.
=== Echocardiography or Ultrasound ===
=== Echocardiography or Ultrasound ===
*Echocardiography/ultrasound may be helpful in the diagnosis of intravenous Leiomyomatosis.  
*[[Echocardiography]]/[[ultrasound]] may be helpful in the [[diagnosis]] of intravenous Leiomyomatosis.  
*Findings on an echocardiography/ultrasound suggestive of/diagnostic of intravenous Leiomyomatosis include a vascularized thrombi within the pelvic veins and IVC.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
*Findings on an echocardiography/ultrasound suggestive of/diagnostic of intravenous Leiomyomatosis include a vascularized thrombi within the pelvic veins and IVC.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
*[[Echocardiography]] may show findings consistent with [[right atrial]] involvement of the [[tumor]].
*[[Echocardiography]] may show findings consistent with [[right atrial]] involvement of the [[tumor]].
*Possible findings of IVL on [[echocardiography]] include hyperechoic elongated mobile [[mass]] extending from the [[inferior vena cava]] and an irregular [[lesion]] in the [[right atrium]] and sometimes with protrusion into the [[right ventricle]].<ref name="pmid22340462">{{cite journal |vauthors=Kang LQ, Zhang B, Liu BG, Liu FH |title=Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging |journal=Chin. Med. J. |volume=125 |issue=1 |pages=33–7 |date=January 2012 |pmid=22340462 |doi= |url=}}</ref>
=== CT scan ===
=== CT scan ===
*[[Pelvic]] [[CT scan]] may be helpful in the [[diagnosis]] of intravenous leiomyomatosis.<ref name="Xu2013">{{cite journal|last1=Xu|first1=Zhi-Feng|title=Uterine intravenous leiomyomatosis with cardiac extension: Imaging characteristics and literature review|journal=World Journal of Clinical Oncology|volume=4|issue=1|year=2013|pages=25|issn=2218-4333|doi=10.5306/wjco.v4.i1.25}}</ref>
*[[Pelvic]] [[CT scan]] may be helpful in the [[diagnosis]] of intravenous leiomyomatosis.<ref name="Xu2013">{{cite journal|last1=Xu|first1=Zhi-Feng|title=Uterine intravenous leiomyomatosis with cardiac extension: Imaging characteristics and literature review|journal=World Journal of Clinical Oncology|volume=4|issue=1|year=2013|pages=25|issn=2218-4333|doi=10.5306/wjco.v4.i1.25}}</ref>
*Findings on [[CT scan]] suggestive of/[[diagnostic]] of intravenous leiomyomatosis include an irregular mass exhibiting heterogeneous enhancement with extension into adjacent [[veins]].
*Findings on [[CT scan]] suggestive of/[[diagnostic]] of intravenous leiomyomatosis include an irregular [[mass]] exhibiting [[heterogeneous]] enhancement with [[extension]] into adjacent [[veins]].
=== MRI ===
=== MRI ===
*[[Pelvic]] [[MRI]] may be helpful in the [[diagnosis]] of intravenous leiomyomatosis.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
*[[Pelvic]] [[MRI]] may be helpful in the [[diagnosis]] of intravenous leiomyomatosis.<ref name="FasihPrasad Shanbhogue2008">{{cite journal|last1=Fasih|first1=Najla|last2=Prasad Shanbhogue|first2=Alampady K.|last3=Macdonald|first3=David B.|last4=Fraser-Hill|first4=Margaret A.|last5=Papadatos|first5=Demetrios|last6=Kielar|first6=Ania Z.|last7=Doherty|first7=Geoffrey P.|last8=Walsh|first8=Cynthia|last9=McInnes|first9=Matthew|last10=Atri|first10=Mostafa|title=Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations|journal=RadioGraphics|volume=28|issue=7|year=2008|pages=1931–1948|issn=0271-5333|doi=10.1148/rg.287085095}}</ref>
*Findings on MRI suggestive of/diagnostic of intravenous leiomyomatosis include a leiomyomatous lesion or a distorted uterus with projections into vasculature.
*Findings on [[MRI]] suggestive of/[[diagnostic]] of intravenous leiomyomatosis include a leiomyomatous [[lesion]] or a distorted [[uterus]] with projections into [[vasculature]].
*The [[tumor]] usually shows low signal intensity similar to that of [[smooth muscle]] on T2-weighted images and low to intermediate signal intensity on T1-weighted images.
*The [[tumor]] usually shows low signal intensity similar to that of [[smooth muscle]] on T2-weighted images and low to intermediate signal intensity on T1-weighted images.
*The appearanceof the lesion varies according to its smooth muscle and fibrous tissue content.
*The appearanceof the [[lesion]] varies according to its [[smooth muscle]] and [[fibrous]] [[tissue]] content.
 
===Other Imaging Findings===
*There are no other [[imaging]] findings associated with intravenous leiomyomatosis.
===Other Diagnostic Studies===
*There are no other [[diagnostic]] studies associated with intravenous leiomyomatosis.
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
Line 74: Line 76:
=== Surgery ===
=== Surgery ===
*[[Surgery]] is the treatment of choice, and complete removal of the [[tumor]] is mandatory.
*[[Surgery]] is the treatment of choice, and complete removal of the [[tumor]] is mandatory.
*Recurrences up to 15 years after the primary occurrence in the patients with Incomplete resection of the tumor.<ref name="pmid25743551">{{cite journal| author=Yanagiya A, Yamada O, Nanbu T, Hamada H, Takada J, Matsuura M et al.| title=[One-stage resection of intravenous leiomyomatosis extending into the right atrium]. | journal=Kyobu Geka | year= 2015 | volume= 68 | issue= 3 | pages= 188-91 | pmid=25743551 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25743551  }}</ref>
*Recurrences up to 15 years after the primary occurrence in the [[patients]] with Incomplete resection of the [[tumor]].<ref name="pmid25743551">{{cite journal| author=Yanagiya A, Yamada O, Nanbu T, Hamada H, Takada J, Matsuura M et al.| title=[One-stage resection of intravenous leiomyomatosis extending into the right atrium]. | journal=Kyobu Geka | year= 2015 | volume= 68 | issue= 3 | pages= 188-91 | pmid=25743551 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25743551  }}</ref>
*Bilateral oophorectomy causes shutdown of hormonal stimulation hence helps preventing recurrence.<ref name="pmid16583249">{{cite journal| author=Castelli P, Caronno R, Piffaretti G, Tozzi M| title=Intravenous uterine leiomyomatosis with right heart extension: successful two-stage surgical removal. | journal=Ann Vasc Surg | year= 2006 | volume= 20 | issue= 3 | pages= 405-7 | pmid=16583249 | doi=10.1007/s10016-006-9024-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16583249  }}</ref>
*[[Bilateral]] [[oophorectomy]] causes shutdown of hormonal stimulation hence helps preventing recurrence.<ref name="pmid16583249">{{cite journal| author=Castelli P, Caronno R, Piffaretti G, Tozzi M| title=Intravenous uterine leiomyomatosis with right heart extension: successful two-stage surgical removal. | journal=Ann Vasc Surg | year= 2006 | volume= 20 | issue= 3 | pages= 405-7 | pmid=16583249 | doi=10.1007/s10016-006-9024-0 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16583249  }}</ref>
*Surgical removal can be done in single or staged procedures,<ref name="pmid12694674">{{cite journal| author=Nam MS, Jeon MJ, Kim YT, Kim JW, Park KH, Hong YS| title=Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. | journal=Gynecol Oncol | year= 2003 | volume= 89 | issue= 1 | pages= 175-80 | pmid=12694674 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12694674  }}</ref>
*[[Surgical]] removal can be done in single or staged procedures,<ref name="pmid12694674">{{cite journal| author=Nam MS, Jeon MJ, Kim YT, Kim JW, Park KH, Hong YS| title=Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature. | journal=Gynecol Oncol | year= 2003 | volume= 89 | issue= 1 | pages= 175-80 | pmid=12694674 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12694674  }}</ref>
*If staged procedure is chosen than it is done with abdominopelvic and intrathoracic components in two separate operations within a short time interval. <ref name="pmid20802437">{{cite journal| author=Tielliu IF, Otterman ML, Meuzelaar JJ, Zeebregts CJ, Peeters PM| title=Intravenous leiomyomatosis: report of two cases and strategy for surgical resection. | journal=Minerva Chir | year= 2010 | volume= 65 | issue= 4 | pages= 489-93 | pmid=20802437 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20802437  }}</ref>
*If staged procedure is chosen than it is done with abdominopelvic and [[intrathoracic]] components in two separate operations within a short time interval. <ref name="pmid20802437">{{cite journal| author=Tielliu IF, Otterman ML, Meuzelaar JJ, Zeebregts CJ, Peeters PM| title=Intravenous leiomyomatosis: report of two cases and strategy for surgical resection. | journal=Minerva Chir | year= 2010 | volume= 65 | issue= 4 | pages= 489-93 | pmid=20802437 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20802437  }}</ref>
*In literature it is seen the Complete excision with one-stage operation was used in 19 (27.9%)  and Complete excision with two-stage operation in 29 (42.7%) <br />
=== Primary Prevention ===
=== Primary Prevention ===
*There are no primary preventive measures available for [IVL].
*There are no [[Primary prevention|primary preventive]] measures available for IVL.
=== Secondary Prevention ===
=== Secondary Prevention ===
*There are no secondary preventive measures available for [IVL].
*There are no [[Secondary prevention|secondary preventive]] measures available for IVL.
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Revision as of 20:02, 23 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Template:Sonia Sandeep Sahar Memar Montazerin, M.D.[2]
Synonyms and keywords: Nesidioblastoma, IVLM

Overview

Intravenous leiomyomatosis is also refered as IVL. 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. Findings on CT scan diagnostic of intravenous leiomyomatosis include an irregular mass exhibiting heterogeneous enhancement with extension into adjacent veins. Findings on an ultrasound diagnostic of intravenous Leiomyomatosis include a vascularized thrombi within the pelvic veins and Inferior vena cava. Findings on MRI diagnostic of intravenous leiomyomatosis include a leiomyomatous lesion or a distorted uterus with projections into vasculature. Surgery is the mainstay of therapy for intravenous leiomyomatosis. It can grow into lymphatics /veins.

Historical Perspective

  • Intravenous leiomyomatosis first described in 1896 by German Pathologist Birch-Hirschfeld.[1]
  • First case of leiomyo-matosis of pelvic origin with intravascular extension to cardiac cavities was described in 1907 in Germany by Dürck and Hörmann.[2]

Classification

  • There is no established system for the classification of 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.[3]
  • Approximately 40% of leiomyomata have cytogenetic abnormalities.
  • They are benign tumors of uterus that extend to veins system but do not invade the surrounding tissues [4]
  • They contains receptors to Estrogen and progesterone and hence response to these hormones [5]
  • It is also referred as quasi-malignant behavior due to its speedy spreading behavior.[6]
  • On microscopy it is seen as benign, well-differentiated tumor with smooth muscle growing within veins as worm-like projections.
  • Immunohistochemical studies show presence of desminand smooth muscle actin, confirming their smooth muscle nature.
  • Tumor cells showed bizarre nuclear morphology with hyperchromatic multilobated nuclei, the mitotic activity was low, with mitotic index of less than 1 per 50 high-power fields

Causes

  • The etiology of intravenous leiomyomatosis is unclear.[7]
  • Only two cytogenetic reports in IVL and both exhibited a karyotype with a der(14)t(12;14)(q15;q24) and two normal copies of chromosome 12 which has close association to uterine leiomyoma genetics

Differentiating Intravenous Leiomyomatosis from other Diseases

  • Intravenous leiomyomatosis must be differentiated from other conditions such as intravenous thrombus, leiomyosarcoma, right atrial myxoma and tumor thrombosis with malignant carcinoma, for example, renal carcinoma, hepatocellular carcinoma, adrenal cortical carcinoma.[8][7]
  • For more information on intravenous leiomyomatosis differential diagnosis please click here.

Epidemiology and Demographics

  • There is no available data on the exact incidence of intravenous leiomyomatosis.
  • The median age at the time of diagnosis of IVL is 45 years, with patients ranging from 26 to 70 years old. [9]
  • Female are exclusively affected with intravenous leiomyomatosis.[10]
  • Intravenous leiomyomatosis is seen more commonly in white female.[11]

Risk Factors

  • Common risk factors in the development of intravenous leiomyomatosis are age, cytogenetics , and prior history of uterine leiomyoma, and/or hysterectomy.[12]

Screening

  • There is insufficient evidence to recommend routine screening for intravenous leiomyomatosis.

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

  • Patients with intravenous Leiomyomatosis usually depends on location and extension of the tumor which may include jugular venous distention, increased pulse rate, fluid shift in abdominal exam, and lower extremity edema.[21]

Laboratory Findings

  • There are no specific laboratory findings associated with intravenous Leiomyomatosis.

Electrocardiogram

  • There are no ECG findings associated with intravenous Leiomyomatosis.

X-ray

  • There are no x-ray findings associated with intravenous Leiomyomatosis.

Echocardiography or Ultrasound

CT scan

MRI

Other Imaging Findings

  • There are no other imaging findings associated with intravenous leiomyomatosis.

Other Diagnostic Studies

  • There are no other diagnostic studies associated with intravenous leiomyomatosis.

Treatment

Medical Therapy

  • There is no treatment for intravenous leiomyomatosis.

Surgery

  • Surgery is the treatment of choice, and complete removal of the tumor is mandatory.
  • Recurrences up to 15 years after the primary occurrence in the patients with Incomplete resection of the tumor.[24]
  • Bilateral oophorectomy causes shutdown of hormonal stimulation hence helps preventing recurrence.[25]
  • Surgical removal can be done in single or staged procedures,[26]
  • If staged procedure is chosen than it is done with abdominopelvic and intrathoracic components in two separate operations within a short time interval. [27]

Primary Prevention

Secondary Prevention

References

  1. ↑ Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC (2002). "Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case". Mod Pathol. 15 (3): 351–6. doi:10.1038/modpathol.3880529. PMID 11904348.
  2. ↑ Stolf NA, dos Santos GG, Haddad VL (1999). "Unusual abdominal tumors with intracardiac extension. Two cases with successful surgical resection". Rev Hosp Clin Fac Med Sao Paulo. 54 (5): 159–64. PMID 10788838.
  3. ↑ Mariyappa N, Manikyam UK, Krishnamurthy D, Preeti K, Agarwal Y, Prakar U (2012). "Intravenous leiomyomatosis". Niger J Surg. 18 (2): 105–6. doi:10.4103/1117-6806.103122. PMC 3762011. PMID 24027407.
  4. ↑ Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC (2003). "Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24)". Genes Chromosomes Cancer. 36 (2): 205–6. doi:10.1002/gcc.10159. PMID 12508249.
  5. ↑ Dal Cin P, Quade BJ, Neskey DM, Kleinman MS, Weremowicz S, Morton CC (2003). "Intravenous leiomyomatosis is characterized by a der(14)t(12;14)(q15;q24)". Genes Chromosomes Cancer. 36 (2): 205–6. doi:10.1002/gcc.10159. PMID 12508249.
  6. ↑ Quade BJ, Dal Cin P, Neskey DM, Weremowicz S, Morton CC (2002). "Intravenous leiomyomatosis: molecular and cytogenetic analysis of a case". Mod Pathol. 15 (3): 351–6. doi:10.1038/modpathol.3880529. PMID 11904348.
  7. ↑ 7.0 7.1 7.2 7.3 7.4 7.5 Fasih, Najla; Prasad Shanbhogue, Alampady K.; Macdonald, David B.; Fraser-Hill, Margaret A.; Papadatos, Demetrios; Kielar, Ania Z.; Doherty, Geoffrey P.; Walsh, Cynthia; McInnes, Matthew; Atri, Mostafa (2008). "Leiomyomas beyond the Uterus: Unusual Locations, Rare Manifestations". RadioGraphics. 28 (7): 1931–1948. doi:10.1148/rg.287085095. ISSN 0271-5333.
  8. ↑ Lee, Sak; Kim, Do-Kyun; Narm, Kyoung Shik; Cho, Sang-Ho (2011). "Pulmonary Artery Embolization of Intravenous Leiomyomatosis Extending into the Right Atrium". The Korean Journal of Thoracic and Cardiovascular Surgery. 44 (3): 243–246. doi:10.5090/kjtcs.2011.44.3.243. ISSN 2233-601X.
  9. ↑ Canzonieri V, D'Amore ES, Bartoloni G, Piazza M, Blandamura S, Carbone A (1994). "Leiomyomatosis with vascular invasion. A unified pathogenesis regarding leiomyoma with vascular microinvasion, benign metastasizing leiomyoma and intravenous leiomyomatosis". Virchows Arch. 425 (5): 541–5. PMID 7850080.
  10. ↑ Poliquin V, Victory R, Vilos GA (2008). "Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report". J Minim Invasive Gynecol. 15 (2): 152–60. doi:10.1016/j.jmig.2007.12.009. PMID 18312983.
  11. ↑ Poliquin V, Victory R, Vilos GA (2008). "Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report". J Minim Invasive Gynecol. 15 (2): 152–60. doi:10.1016/j.jmig.2007.12.009. PMID 18312983.
  12. ↑ Leitman, M.; Kuperstein, R.; Medalion, B.; Stamler, A.; Porat, E.; Rosenblatt, S.; Konen, E.; Krakover, R.; Vered, Z. (2008). "A highly unusual right atrial mass presented in two women". European Heart Journal - Cardiovascular Imaging. 9 (6): 833–834. doi:10.1093/ejechocard/jen173. ISSN 2047-2412.
  13. ↑ Schäfer HM, Isaak A, Gürke L (2017). "Case report of an intracaval leiomyomatosis 10 months after complete hysterectomy". Int J Surg Case Rep. 35: 1–3. doi:10.1016/j.ijscr.2017.03.031. PMC 5394212. PMID 28414995.
  14. ↑ Kocica, Mladen J.; Vranes, Mile R.; Kostic, Dusan; Kostic, Natasa Kovacevic-; Lackovic, Vesna; Mihajlovic, Vesna Bozic-; Velinovic, Milos M.; Mikic, Aleksandar Dj.; Kalezic, Nevena Dimitrijevic- (2005). "Intravenous leiomyomatosis with extension to the heart: Rare or underestimated?". The Journal of Thoracic and Cardiovascular Surgery. 130 (6): 1724–1726. doi:10.1016/j.jtcvs.2005.08.021. ISSN 0022-5223.
  15. ↑ Evans AT, Symmonds RE, Gaffey TA (February 1981). "Recurrent pelvic intravenous leiomyomatosis". Obstet Gynecol. 57 (2): 260–4. PMID 7465135.
  16. ↑ Awonuga, Awoniyi O.; Rotas, Michael; Imudia, Anthony N.; Choi, Christine; Khulpateea, Neekianund (2008). "Recurrent benign metastasizing leiomyoma after hysterectomy and bilateral salpingo-oophorectomy". Archives of Gynecology and Obstetrics. 278 (4): 373–376. doi:10.1007/s00404-008-0581-z. ISSN 0932-0067.
  17. ↑ Nakayama Y, Kitamura S, Kawachi K, Kawata T, Fukutomi M, Hasegawa J; et al. (1994). "Intravenous leiomyomatosis extending into the right atrium". Cardiovasc Surg. 2 (5): 642–5. PMID 7820530.
  18. ↑ Moorjani N, Kuo J, Ashley S, Hughes G (2005). "Intravenous uterine leiomyosarcomatosis with intracardial extension". J Card Surg. 20 (4): 382–5. doi:10.1111/j.1540-8191.2005.200476.x. PMID 15985146.
  19. ↑ Poliquin V, Victory R, Vilos GA (2008). "Epidemiology, presentation, and management of retroperitoneal leiomyomata: systematic literature review and case report". J Minim Invasive Gynecol. 15 (2): 152–60. doi:10.1016/j.jmig.2007.12.009. PMID 18312983.
  20. ↑ Vaquero ME, Magrina JF, Leslie KO (2009). "Uterine smooth-muscle tumors with unusual growth patterns". J Minim Invasive Gynecol. 16 (3): 263–8. doi:10.1016/j.jmig.2009.01.013. PMID 19423058.
  21. ↑ Andrade LA, Torresan RZ, Sales JF, Vicentini R, De Souza GA (1998). "Intravenous leiomyomatosis of the uterus. A report of three cases". Pathol. Oncol. Res. 4 (1): 44–7. PMID 9555121.
  22. ↑ Kang LQ, Zhang B, Liu BG, Liu FH (January 2012). "Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging". Chin. Med. J. 125 (1): 33–7. PMID 22340462.
  23. ↑ Xu, Zhi-Feng (2013). "Uterine intravenous leiomyomatosis with cardiac extension: Imaging characteristics and literature review". World Journal of Clinical Oncology. 4 (1): 25. doi:10.5306/wjco.v4.i1.25. ISSN 2218-4333.
  24. ↑ Yanagiya A, Yamada O, Nanbu T, Hamada H, Takada J, Matsuura M; et al. (2015). "[One-stage resection of intravenous leiomyomatosis extending into the right atrium]". Kyobu Geka. 68 (3): 188–91. PMID 25743551.
  25. ↑ Castelli P, Caronno R, Piffaretti G, Tozzi M (2006). "Intravenous uterine leiomyomatosis with right heart extension: successful two-stage surgical removal". Ann Vasc Surg. 20 (3): 405–7. doi:10.1007/s10016-006-9024-0. PMID 16583249.
  26. ↑ Nam MS, Jeon MJ, Kim YT, Kim JW, Park KH, Hong YS (2003). "Pelvic leiomyomatosis with intracaval and intracardiac extension: a case report and review of the literature". Gynecol Oncol. 89 (1): 175–80. PMID 12694674.
  27. ↑ Tielliu IF, Otterman ML, Meuzelaar JJ, Zeebregts CJ, Peeters PM (2010). "Intravenous leiomyomatosis: report of two cases and strategy for surgical resection". Minerva Chir. 65 (4): 489–93. PMID 20802437.

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