Solitary Fibrous Tumor of the Pleura: Histology, CT Scan Images and Review of Literature over the Last Twenty Years
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 1, Issue 1
Abstract
Solitary fibrous tumor of the pleura is a rare neoplasm. In Literature up to 800 cases [1-3] have been reported, and these numbers show its rarity, despite of mesotheliomas, the most pleural tumors represented. Males and females are equal distributed and the same is true for age. No correlation with exposure to asbestos, tobacco or others environmental agents, were found for its development. Solitary fibrous tumor of the pleura occurs as localized neoplasms of the pleura and was initially classified as “localized mesothelioma”. Recently, with the aid of the electronic microscope and immunohistochemistry, has been possible emphasize that their origin is mesenchymal and not mesothelial, so the term “localized mesothelioma” has been replaced with “solitary fibrous pleural tumor” STFP [4]. In the past STFP were described only in the pleura but recently it has been found located also in other sites [5,6] such as abdomen, liver, peritoneum, retroperitoneal spaces, meninges, orbit, thyroid, salivary glands and soft tissues including the breast [7-10]. The STFP can be associated to other synchronous or metacron neoplasms like prostate, lung, breast, endometrial carcinoma and thyroid. Although most of STFP are benign neoplasms, a part of these could have a malignant behavior. The clinical behavior is unpredictable and probably is due to their histological and morphological features. STFP may remain silent for many years before turning into malignant form [11]. Often SFTPs are accidentally discovered by radiological investigation like X-Ray [12,13]. Most patients with SFTP become symptomatic when these tumors reach large size [2,10,14]. About 54-67% of patients with benign SFTP are symptomatic, while 75% of cases of malignant STFP, show symptoms [15]. These symptoms are cough, chest pain and dyspnoea. If there is obstruction of the airway, also hemoptysis and pneumonia [2,10] could be seen. Paraneoplastic syndromes, represented by digital hypocratism and Pierre-Marie-Bamberg syndrome, are observed in 10-20% of cases and specially with large size SFTPs. Symptoms usually vanish after the neoplasm has been removed, but may reappear in case of recurrence [10,16,17]. In less than 5% of patients with SFTP an increase of insulin-like factor II type occur and this causes refractory to therapy hypoglycaemia (Doege-Potter syndrome) [10,18,19]. The incidence of Doege-Potter syndrome in SFPT is similar in both sexes and there no differences in both benign and malignant forms. Some patients may also present gynecomastia or galactorrhoea [1]. Sometimes large size SFTPs may appear with an unusual clinical presentation, as the two cases reported by Santambrogio et al. [20] and Shaker et al. [21]. The first described a patient with large size SFTP manifested with syncope episodes of coughing. The second author reported the case of a woman with lower limbs edema and dyspnea caused by a bulky SFTP compression to the right atrium and the inferior vena cava.
Authors and Affiliations
Giulia Bora, Flavio Colaut, Gianni Segato, Luisa Delsedime, Alberto Oliaro
Keywords
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- EP ID EP567575
- DOI 10.26717/BJSTR.2017.01.000150
- Views 184
- Downloads 0
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