Lymphangioleiomyomatosis (LAM) is a rare diffuse lung cystic disease (DLCD) that occurs sporadically or in association with Tuberous Sclerosis Complex (TSC). The diagnostic pathway is tracked on the identification of the disease hallmarks on chest High-Resolution Computed Tomography (HRCT). Aim of this review is to discuss the thoracic HRCT pathognomonic features, essential to rule out other DLCD. It also examines the new evidences emerging from Computed Tomography (CT) quantitative studies that, by demonstrating a specific cysts distribution and a pathological aspect of the parenchyma near the cysts, could improve our understanding of this rare disorder and supply pulmonologists with a new tool for a more appropriate long-term management. Finally, the contribution of other image techniques as low dose chest CT, Magnetic Resonance Imaging (MRI) and Ultrasound (US) is discussed.

Role of thoracic imaging in the management of lymphangioleiomyomatosis / P. Crivelli, R.E. Ledda, S. Terraneo, M. Conti, G. Imeri, E. Lesma, F. Di Marco. - In: RESPIRATORY MEDICINE. - ISSN 0954-6111. - 157(2019 Oct), pp. 14-20. [10.1016/j.rmed.2019.08.013]

Role of thoracic imaging in the management of lymphangioleiomyomatosis

S. Terraneo
Secondo
;
G. Imeri;E. Lesma
Penultimo
;
F. Di Marco
Ultimo
2019

Abstract

Lymphangioleiomyomatosis (LAM) is a rare diffuse lung cystic disease (DLCD) that occurs sporadically or in association with Tuberous Sclerosis Complex (TSC). The diagnostic pathway is tracked on the identification of the disease hallmarks on chest High-Resolution Computed Tomography (HRCT). Aim of this review is to discuss the thoracic HRCT pathognomonic features, essential to rule out other DLCD. It also examines the new evidences emerging from Computed Tomography (CT) quantitative studies that, by demonstrating a specific cysts distribution and a pathological aspect of the parenchyma near the cysts, could improve our understanding of this rare disorder and supply pulmonologists with a new tool for a more appropriate long-term management. Finally, the contribution of other image techniques as low dose chest CT, Magnetic Resonance Imaging (MRI) and Ultrasound (US) is discussed.
Lymphangioleiomyomatosis, HRCT, Diagnosis, Tuberous sclerosis complex, Radiological findings
Settore MED/10 - Malattie dell'Apparato Respiratorio
Settore BIO/14 - Farmacologia
Settore MED/36 - Diagnostica per Immagini e Radioterapia
ott-2019
22-ago-2019
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/675975
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