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:
- Considered to be a PEComa.
Treatment:[2]
- Bronchodilators - symptomatic treatment.
- mTOR inhibitors. (???)
- Transplantation.
Epidemiology
- Associated with angiomyolipomas.[3]
- Associated with tuberous sclerosis[3] - abnormality in same gene (TSC2).
- Usually affects women - primarily in childbearing years; case reports of LAM in men - usu. with TSC.[4]
- 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:[5]
- 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
- ↑ 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.
- ↑ 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.
- ↑ 3.0 3.1 http://emedicine.medscape.com/article/299545-overview
- ↑ 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.
- ↑ http://emedicine.medscape.com/article/299545-diagnosis