Fibrous tumour of the pleura (SFTP): a proteiform disease. Clinical, histological and atypical radiological patterns selected among our cases
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Abstract
First described by Klemperer and Rabin in 1931, solitary fibrous tumour of the pleura (SFTP) is a mesenchymal tumour that tends to involve the pleura, although it has also been described in other thoracic areas (mediastinum, pericardium and pulmonary parenchyma) and in extrathoracic sites (meninges, epiglottis, salivary glands, thyroid, kidneys and breast). SFTP usually presents as a peripheral mass abutting the pleural surface, to which it is attached by a broad base or, more frequently, by a pedicle that allows it to be mobile within the pleural cavity. A precise preoperative diagnosis can be arrived at with a cutting-needle biopsy, although most cases are diagnosed with postoperative histology and immunohistochemical analysis of the dissected sample. SFTP, owing to its large size or unusual locations (paraspinal, paramediastinal, intrafissural and intraparenchymal), can pose interpretation problems or, indeed, point towards a diagnosis of diseases of a totally different nature. We present some unusual radiographic and computed tomography (CT) images of large SFTP or SFTP located in atypical thoracic locations in patients who underwent surgical resection.
Keywords
Mesenchymal neoplasms Pleura Computed tomographyIl tumore fibroso solitario della pleura (TFSP): patologia dai mille volti. Clinica, istologia e rappresentazione degli aspetti radiologici insoliti selezionati dalla nostra casistica
Riassunto
Il tumore fibroso solitario della pleura (TFSP), descritto per la prima volta da Klemperer e Rabin nel 1931, é una neoplasia mesenchimale che solitamente coinvolge la pleura, ma che viene descritta anche in altre sedi sia toraciche, quali il mediastino, il pericardio ed il parenchima polmonare, sia extratoraciche, quali le meningi, l’epiglottide, le ghiandole salivari, la tiroide, i reni e la mammella. Di solito si presenta come massa periferica a contatto con la superficie pleurica alla quale è unito da una larga base d’impianto o più spesso da un peduncolo che lo puè rendere mobile all’interno del cavo pleurico. La diagnosi preoperatoria di certezza puè essere ottenuta mediante biopsia con ago tranciante, ma nella maggior parte dei casi è post-chirurgica, frutto dell’esame istologico e dell’analisi immuno-istochimica sul pezzo operatorio. Il tumore fibroso solitario della pleura tuttavia, spesso a causa delle notevoli dimensioni o dello sviluppo in sedi inconsuete (paraspinali, paramediastiniche, intrascissurali ed intraparenchimali) può creare numerose difficoltà di interpretazione o addirittura orientare la diagnosi verso patologie di tutt’altra natura. In questo lavoro presentiamo gli aspetti radiologici inusuali di TFSP di grosse dimensioni o localizzati in sedi toraciche atipiche in pazienti sottoposti ad intervento chirurgico di exeresi.
Parole chiave
Tumori mesenchimali Pleura Tomografia computerizzataPreview
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