Difference between revisions of "Lymphangioleiomyomatosis"

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#redirect [[Medical lung diseases#Lymphangioleiomyomatosis]]
'''Lymphangioleiomyomatosis''', abbreviated '''LAM''', is a rare [[medical lung disease|lung pathology]] that predominantly afflicits women of childbearing age.
 
It is also known as '''lymphangiomyomatosis'''.
 
==General==
*Clinical: [[dyspnea]], recurrent pneumothorax.
*May be an indication for lung transplantation.
*Non-neoplastic muscle proliferation versus tumour that can metastasize.<ref name=pmid20235883>{{Cite journal  | last1 = Taveira-DaSilva | first1 = AM. | last2 = Pacheco-Rodriguez | first2 = G. | last3 = Moss | first3 = J. | title = The natural history of lymphangioleiomyomatosis: markers of severity, rate of progression and prognosis. | journal = Lymphat Res Biol | volume = 8 | issue = 1 | pages = 9-19 | month = Mar | year = 2010 | doi = 10.1089/lrb.2009.0024 | PMID = 20235883 }}</ref>
 
Notes:
*Considered to be a [[PEComa]].
 
===Epidemiology===
*Associated with [[angiomyolipoma]]s.<ref name=emedicine299545>[http://emedicine.medscape.com/article/299545-overview http://emedicine.medscape.com/article/299545-overview]</ref>
*Associated with [[tuberous sclerosis]]<ref name=emedicine299545/> - abnormality in same gene (TSC2).
*Usually affects women - primarily in childbearing years; case reports of LAM in men - usu. with [[TSC]].<ref name=pmid17431222>{{Cite journal  | last1 = Schiavina | first1 = M. | last2 = Di Scioscio | first2 = V. | last3 = Contini | first3 = P. | last4 = Cavazza | first4 = A. | last5 = Fabiani | first5 = A. | last6 = Barberis | first6 = M. | last7 = Bini | first7 = A. | last8 = Altimari | first8 = A. | last9 = Cooke | first9 = RM. | title = Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex. | journal = Am J Respir Crit Care Med | volume = 176 | issue = 1 | pages = 96-8 | month = Jul | year = 2007 | doi = 10.1164/rccm.200610-1408CR | PMID = 17431222 }}</ref>
*Rare.
 
===Radiology===
*Bullae/thin walled cysts - distributed in all lung fields.
*Lymphadenopathy.
 
Radiologic DDx (of cysts):
*[[Eosinophilic granuloma]] - associated with [[smoking]].
*[[Usual interstitial pneumonia]] (UIP).
*[[Emphysema]].
 
==Microscopic==
Features:<ref>[http://emedicine.medscape.com/article/299545-diagnosis http://emedicine.medscape.com/article/299545-diagnosis]</ref>
*Spindle cells with small nuclei + larger epithelioid cells with clear cytoplasm and round nuclei.
*Cyst formation.
*Thick arterial walls.
 
===Images===
<gallery>
Image:Lymphangioleiomyomatosis - very low mag.jpg| LAM - very low mag. (WC)
Image:Lymphangioleiomyomatosis - low mag.jpg| LAM - low mag. (WC)
Image:Lymphangioleiomyomatosis - intermed mag.jpg| LAM - intermed. mag. (WC)
Image:Lymphangioleiomyomatosis - very high mag.jpg| LAM - very high mag. (WC)
</gallery>
www:
*[http://www.nature.com/modpathol/journal/v19/n6/fig_tab/3800610f3.html LAM (nature.com)].
*[http://path.upmc.edu/cases/case111.html LAM - several images (upmc.edu)].
 
==IHC==
*HMB-45 +ve.
*ER +ve.
*PR +ve.
*SMA +ve.
 
==See also==
*[[Medical lung diseases]].
*[[PEComa]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Medical lung diseases]]

Revision as of 05:56, 9 December 2014

Lymphangioleiomyomatosis, abbreviated LAM, is a rare lung pathology that predominantly afflicits women of childbearing age.

It is also known as lymphangiomyomatosis.

General

  • Clinical: dyspnea, recurrent pneumothorax.
  • May be an indication for lung transplantation.
  • Non-neoplastic muscle proliferation versus tumour that can metastasize.[1]

Notes:

Epidemiology

Radiology

  • Bullae/thin walled cysts - distributed in all lung fields.
  • Lymphadenopathy.

Radiologic DDx (of cysts):

Microscopic

Features:[4]

  • Spindle cells with small nuclei + larger epithelioid cells with clear cytoplasm and round nuclei.
  • Cyst formation.
  • Thick arterial walls.

Images

  • LAM - very low mag. (WC)

  • LAM - low mag. (WC)

  • LAM - intermed. mag. (WC)

  • LAM - very high mag. (WC)

www:

IHC

  • HMB-45 +ve.
  • ER +ve.
  • PR +ve.
  • SMA +ve.

See also

References

  1. ↑ Taveira-DaSilva, AM.; Pacheco-Rodriguez, G.; Moss, J. (Mar 2010). "The natural history of lymphangioleiomyomatosis: markers of severity, rate of progression and prognosis.". Lymphat Res Biol 8 (1): 9-19. doi:10.1089/lrb.2009.0024. PMID 20235883.
  2. ↑ 2.0 2.1 http://emedicine.medscape.com/article/299545-overview
  3. ↑ Schiavina, M.; Di Scioscio, V.; Contini, P.; Cavazza, A.; Fabiani, A.; Barberis, M.; Bini, A.; Altimari, A. et al. (Jul 2007). "Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex.". Am J Respir Crit Care Med 176 (1): 96-8. doi:10.1164/rccm.200610-1408CR. PMID 17431222.
  4. ↑ http://emedicine.medscape.com/article/299545-diagnosis
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