Arjun Nair MBBS, Presenter: Nothing to Disclose

Michael Gonsalves MBBS, Abstract Co-Author: Nothing to Disclose

Ioannis Vlahos MBBS, Abstract Co-Author: Research grant, Bayer AG Support, Bayer AG Research Consultant, Siemens AG

Sisa Grubnic MD, Abstract Co-Author: Nothing to Disclose

Siobhan Johanna Green MBBS, Abstract Co-Author: Nothing to Disclose

Myrna Cobos Barco Godoy MD, Abstract Co-Author: Research grant, Bayer AG

LAM has long been considered the main pulmonary mainefstation of TSC. Nodular hamartomatous lesions termed MMPH are now well-described in TSC, and are increasingly recognised as having a distinct genetic basis. We aim to: 1) Review the pulmonary manifestations; 2)

Summary of the genetic/ phenotypic concepts underpinning LAM and MMPH in TSC, including: 1)Loss of Heterozygosity (LOH) and point mutations of the TSC2 gene;  2) Immunohistochemical staining; 3) Histological appearances and MMPH mimics eg. AAH Review of the MDCT HRCT findings of LAM and MMPH in TSC, from our international referral centre, specifically: 1)Lesion morphology; 2) Distribution; 3)Ancillary features; 4) Differential diagnoses eg. Langerhans' Cell Histiocytosis (LCH) and metastases

Evolution in MDCT HRCT diagnosis is now helping to develop a radiological appreciation of the different phenotypes of TSC, to parallel the advances in genetic understanding, with potential therapeutic implications. The viewer should be able to: Gain an awareness of the appearances of both cysts and nodules in TSC, and their different clinicopathological significance Appreciate the differential diagnosis to be considered and the implication of histological findings.  

Nair, A, Gonsalves, M, Vlahos, I, Grubnic, S, Green, S, Godoy, M, Understanding the Pulmonary Manifestations of Tuberous Sclerosis Complex (TSC): Lymphangioleiomyomatosis (LAM), Sporadic LAM, Multifocal Micronodular Pneumocyte Hyperplasia (MMPH), and the Clinico-Radiological Spectrum of Cystic and Nodular Disease.  Radiological Society of North America 2008 Scientific Assembly and Annual Meeting, February 18 - February 20, 2008 ,Chicago IL. http://archive.rsna.org/2008/6016747.html