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Histological and molecular features of solitary fibrous tumor of the extremities: Clinical correlation

Virchows Archiv Sep 05, 2019

Bianchi G, Sambri A, Pedrini E, et al. - Through a cohort of 41 patients with solitary fibrous tumor of the extremities, researchers assessed the prognostic role of clinical and histological characteristics, presence of C228T and C250T mutations at the TERT promoter region, and NAB2–STAT6 fusion variants. The two patients with metastasis at presentation were classified in the high-risk group whereas the one with metastasis following surgery was listed in the intermediate-risk group. In nine out of 38 DNA available, TERT promoter mutations were identified. All patients with metastasis were identified by a TERT promoter mutation. TERT promoter mutation was related to mitoses > 4 per high-power field, necrosis, and size > 10 cm. In 27 out of 41 cases without any prognostic value, NAB2–STAT6 fusion variants were recognized. In conclusion, the patients with solitary fibrous tumor of the limbs have a better prognosis in comparison with other solitary fibrous tumors, with a very low percentage of metastatic events. Moreover, a correlation between TERT promoter mutations and histologically malignant characteristics was suggestive, implying a possible molecular role in stratifying patients into intermediate- to a high-risk tumor.
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