Difference between revisions of "Lymphangioleiomyomatosis"

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| Other      =
| Other      =
| ClinDDx    = [[eosinophilic granuloma]], [[usual interstitial pneumonia]], [[emphysema]]
| ClinDDx    = [[eosinophilic granuloma]], [[usual interstitial pneumonia]], [[emphysema]]
| Tx        = lung transplantation
| Tx        = medical therapy, lung transplantation
}}
}}
'''Lymphangioleiomyomatosis''', abbreviated '''LAM''', is a rare [[medical lung disease|lung pathology]] that predominantly afflicits women of childbearing age.  
'''Lymphangioleiomyomatosis''', abbreviated '''LAM''', is a rare [[medical lung disease|lung pathology]] that predominantly afflicits women of childbearing age.  
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Notes:
Notes:
*Considered to be a [[PEComa]].
*Considered to be a [[PEComa]].
Treatment:<ref name=pmid25478388>{{Cite journal  | last1 = Vlachostergios | first1 = PJ. | last2 = Rad | first2 = BS. | last3 = Karimi | first3 = K. | last4 = Apergis | first4 = G. | title = Angiomyolipomas, Renal Cell Carcinomas and Pulmonary Lymphangioleiomyomatosis. | journal = J Clin Diagn Res | volume = 8 | issue = 10 | pages = MJ01 | month = Oct | year = 2014 | doi = 10.7860/JCDR/2014/9733.5021 | PMID = 25478388 }}</ref>
*Bronchodilators - symptomatic treatment.
*mTOR inhibitors. (???)
*Transplantation.


===Epidemiology===
===Epidemiology===

Revision as of 06:07, 9 December 2014

Lymphangioleiomyomatosis
Diagnosis in short

Lymphangioleiomyomatosis. H&E stain.

Synonyms lymphangiomyomatosis

LM spindle cells with small nuclei + larger epithelioid cells with clear cytoplasm and round nuclei, cyst formation, thick arterial walls
IHC HMB-45 +ve, ER +ve, PR +ve, SMA +ve
Site lung - see medical lung diseases

Syndromes tuberous sclerosis

Clinical history almost always women of childbearing age, recurrent pneumothorax
Symptoms dyspnea
Prevalence very rare
Radiology bullae/thin walled cysts distributed in all lung fields, lymphadenopathy
Clin. DDx eosinophilic granuloma, usual interstitial pneumonia, emphysema
Treatment medical therapy, lung transplantation

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:

Treatment:[2]

  • Bronchodilators - symptomatic treatment.
  • mTOR inhibitors. (???)
  • Transplantation.

Epidemiology

Radiology

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

Radiologic DDx (of cysts):

Microscopic

Features:[5]

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

Images

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. Vlachostergios, PJ.; Rad, BS.; Karimi, K.; Apergis, G. (Oct 2014). "Angiomyolipomas, Renal Cell Carcinomas and Pulmonary Lymphangioleiomyomatosis.". J Clin Diagn Res 8 (10): MJ01. doi:10.7860/JCDR/2014/9733.5021. PMID 25478388.
  3. 3.0 3.1 http://emedicine.medscape.com/article/299545-overview
  4. 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.
  5. http://emedicine.medscape.com/article/299545-diagnosis