Dados do Resumo
Título
Malignant Solitary Fibrous Tumor: A Case Report
Introdução
Malignant Solitary Fibrous Tumor (MSFT) is a rare mesenchymal cell neoplasm, classified as a soft tissue neoplasm with an intermediate degree of malignancy (locally aggressive and rarely metastasized). MSFT originates in periendothelial cells (Zimmermann's pericytes) that surround the blood capillaries with the disordered proliferation of these spindle cells in the midst of the collagen matrix. It was first described in the pleura, and may occur in other sites such as the mediastinum, liver, peritoneum, lung, but more rarely in the head and neck region (ethmoid, parapharyngeal space, maxilla, and orbit). The symptoms are nonspecific, varying according to the site of involvement and the volume of the lesion. The following is a case of MSFT in the orbital region, in which, due to its complexity, it took a long time to be diagnosed, which may increase the chances of a worse prognosis.
Objetivo
Case report of a malignant fibrous tumor lodged in the orbit of a patient, a neoplasm very rarely found in head and neck surgery. The study of this case is relevant due to the low incidence of fibrous tumors with malignant pattern and the difficulty in diagnosing the lesion due to the lack of a defined vascularization pattern, sometimes scarce, sometimes exuberant, and the differential diagnosis of other types of lesions are important. The present study can provide an important example of the diagnostic management of a rare entity that had not been diagnosed in previous services, impairing the patient's treatment.
Métodos
The methodology of this report was based on the retrospective analysis of medical records. The data collected guarantees confidentiality, respecting ethical guidelines.
Resultados
EVC, a 41-year-old female, reported a complaint of a nodule in the left eyelid rima since July 2009. The nodule is painless and progressively growing in the medial corner of the left orbit. She underwent resection with biopsy in January 2010, obtaining the result of benign fibrohistiocytic mesenchymal neoplasia. In December 2010, the patient developed local recurrence and sought another specialist, with a revision of the slide: WHO grade 1 meningioma (EMA + focal, Ki67 + 2%, S100: -, Progesterone + focal). Imaging tests were performed in March 2012: dacryocystography showed a lacrimal sac displaced inferolaterally due to an expansive lesion in the left orbit; Computed tomography of the orbit showed an expansive lesion in the inferior medial corner of the left orbit, extraconal, remodeling the nasolacrimal duct, without bone erosion, oval, of 1.7 cm, displacing the eyeball. As a conduct, resection was performed in the operating room with frozen food in the room, and the result of the last biopsy was FST.
Conclusões
Since the first description of Solitary Fibrous Tumor (SFT) in orbit, few cases have been reported in the literature. As in the case reported here, the difficulty in making the correct diagnosis was also found in the literature, however, the patient in question did not present harmful events. Resection is considered the most indicated treatment method, with controversies regarding adjuvant or neoadjuvant methods, given the possible risks of visual impairment.
Palavras Chave
solitary fibrous tumor; Malignancy; orbit;
Área
9.Outros (Temas não mencionados acima, Relatos de Casos e Revisão de literatura)
Autores
GIULIA MALAGONI DE CASTRO GUEDES ARCOS, Bruna Florentino Diniz Silva, Beatriz Westphalen Pomianoski, Victoria Meirelles Honorato, Giulianno Molina Melo