Solitary Extrapleural Fibrous Tumor in Salivary Glands: Our Experience—Case Series and Literature Review

Abstract

(1) Background: Extrapleural solitary fibrous tumors (ESFTs) are rare oncological entities occurring in the head and neck, and even more so in the salivary glands. The clinical presentation and histologic features are usually unspecific, resulting in frequent misclassification. As an unusual tumor, ESTFs have an unpredictable clinical behavior. (2) Methods: We present two clinical cases referred to our Maxillofacial Surgery Unit for the onset of a symptomless mass involving, in one case, the parotid gland, and in the other case, the sublingual gland. (3) Results: Solitary fibrous tumors could be considered as neoplasms with intermediate biological behavior that are not entirely predictable on the basis of morphological features, as these are mostly still unknown. However, a few histologic, immunohistochemical, and imaging features, such as a hypodense signal at the T1 sequence in an MRI, or positivity for CD34, bcl2, and CD99, and the NAB2-STATS6 fusion gene, could be useful for an early differential diagnosis of ESTFs. (4) Conclusions: All patients were alive at follow-up with no evidence of disease. Surgical management should always be considered as the first choice for oncological radicality, and clinical behavior should always be defined with the help of the study of radiological and anatomopathological features.