Affiliation:
1. Department of Pathology, University Hospital of Cologne, Medical Faculty of Cologne University, Cologne, Germany
2. Department of Pathology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA
Abstract
Dedifferentiated solitary fibrous tumor (DDSFT) is a rare and clinically aggressive malignancy with a poor prognosis. It represents the progression of solitary fibrous tumor to a high-grade, morphologically nondistinctive sarcoma. This study characterizes the clinicopathologic and molecular features of 25 DDSFT. The study cohort comprised 13 males and 12 females with a median age of 63 years (range 31 to 84). Tumors were most common in the pelvic cavity (8/25), thoracic cavity (6/25), and trunk (4/25). Histologically, DDSFT demonstrated remarkably variable morphology, including pleomorphic, epithelioid, spindle cell, and round cell features. Heterologous elements were present in 4/25 (16%). Immunohistochemical expression of STAT6 was completely lost in 8/22 (36%) tumors. Targeted DNA sequencing demonstrated that in most tumors (10/13; 77%), the NAB2::STAT6 fusion variant resulted in a truncated STAT6 (STAT6-TAD) in the fusion protein. Recurrent secondary alterations involved TP53 (10/14; 71%), TERT (8/14; 57%), and RB1 (3/14; 21%). Statistical analysis of the study cohort and 55 cases reported in the literature demonstrated that complete loss of STAT6 in DDSFT is associated with shorter disease-specific survival (HR 12.69, P=0.023).
Publisher
Ovid Technologies (Wolters Kluwer Health)