Report of a case of solitary fibrous tumour of the orbit
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CASE REPORT
Report of a case of solitary fibrous tumour of the orbit Tufi Neder Meyer & Bruno Henrique Figueiredo Matos & Lucinei Roberto Oliveira & Alexandre Tourino Mendonça
Received: 30 April 2012 / Accepted: 3 October 2012 / Published online: 16 October 2012 # Springer-Verlag Berlin Heidelberg 2012
Abstract Background Solitary fibrous tumors of the orbit are uncommon lesions, only one relatively large series having been published. Neoplasms, formerly considered as separate entities, including hemangiopericytoma, are presently encompassed as solitary fibrous tumors, a unifying designation. There is a tendency towards recurrence and some cases are malignant. Their rarity justifies the publication of new cases, in order to increase the amount of information about this pathological entity. Case report A 40-year-old female patient developed swelling in the left lower lid and after slow progression of the symptom during 2 years, presented herself to our elective surgery service. Image exams showed a 20-mm encapsulated tumor. Surgical treatment was performed: complete excision, made difficult by the growth of the lesion amid the inferior rectus and inferior oblique muscles. Histopathological and immunochemical examinations with CD34 positivity, diagnosed a solitary fibrous tumor, without suggestive signs of malignancy. After a follow-up of 40 months, no recurrence has been detected. Discussion Solitary fibrous tumors, though uncommon, should be considered in the differential diagnosis of orbital expansive lesions. The key point to diagnosis is finding CD34 positivity in immunohistochemical examination. Such tumors have a tendency for recurrence, even after more than 5 years. Keywords Hemangiopericytoma . Solitary fibrous tumour . Orbital neoplasms . Angiofibroma T. N. Meyer (*) : B. H. F. Matos : L. R. Oliveira : A. T. Mendonça UNINCOR, Três Corações, Minas Gerais, Brazil e-mail: [email protected]
Background Orbital and periorbital tumours, which are infrequent, may be challenging in terms of both diagnosis and treatment. Among them, a single pathological entity, the solitary fibrous tumour, encompasses different neoplasms, including hemangiopericytomas, which were once considered separately [1]. These are found in approximately 1 % of orbital biopsies [2]. Solitary fibrous tumours are rare in the orbit, being more common in the pleura, but also occurring in the peritoneum, mediastinum, liver, pericardium, retroperitoneum and other anatomical sites [3]. Symptoms and signs most commonly include, swelling, asymmetry, pain, slowly progressive proptosis, loss of eyeball mobility, diplopia and visual loss [4]. This growth usually arises between 30 and 60 years; although some cases have been reported from 5 months to 16 years [5–7]. The gold standard of treatment is complete surgical excision, which can be difficult due to friability and high vascularity. This increases the risks of incomplete excision and explains why local recurrence is common [8]. Pre- or post-operative radiotherapy can be used to reduce the tumour size,
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