Lymphangioleiomyomatosis

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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:

Clinical DDx:[3]

Treatment:[4]

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

Epidemiology

Gross/Radiology

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

Radiologic DDx (of cysts):

Microscopic

Features:[6]

  • 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. 2.0 2.1 2.2 Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA (November 2006). "Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors". Kidney Int. 70 (10): 1777–82. doi:10.1038/sj.ki.5001853. PMID 17003820.
  3. Xu, KF.; Lo, BH. (2014). "Lymphangioleiomyomatosis: differential diagnosis and optimal management.". Ther Clin Risk Manag 10: 691-700. doi:10.2147/TCRM.S50784. PMID 25187723.
  4. 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.
  5. 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.
  6. http://emedicine.medscape.com/article/299545-diagnosis