NAB2 (NGFI-A Binding Protein 2)

This study provides a comprehensive genomic overview of AM, highlighting recurrent alterations in the MAPK and cell cycle pathways, and potential demographic-specific molecular signatures. These findings support the need for expanded molecular profiling to improve prognostic accuracy and identify targets for future therapy.

Despite advances in molecular diagnostics and therapeutic strategies, the biological complexity and unpredictable clinical behavior of SFTs present significant challenges. This review provides an updated overview of SFT, with a focus on its molecular genetics, histopathological features, and diagnostic considerations.

This case underscores the aggressive potential of dedifferentiated SFTs and highlights the diagnostic role of NAB2::STAT6 fusion. It emphasizes the importance of multidisciplinary care in managing rare, high-risk tumors of the bone.

Stains for MDM2, CDK4, and caldesmon are negative. The case is notable for its atypical progression, as most dedifferentiated SFTs transition directly from a benign-appearing SFT to a high-grade component without signs of malignancy in the conventional SFT region.

To our knowledge, this case represents the second known example of an SFT originating in the nasal vestibule and highlights that even in anatomically constrained regions where en bloc excision is difficult, meticulous piecemeal endoscopic resection can achieve complete tumor clearance and favorable early outcomes. Long-term surveillance remains essential due to the potential for late recurrence.

We report a new case of malignant SFT with NAB2 ex6::STAT6 ex16 Fusion managed with surgery and adjuvant radiotherapy with no recurrence after 2 months. This case highlights the diagnostic challenges of SFT and the importance of continued case documentation to guide recognition of this rare entity.

The pathogenesis is linked to the centromeric inversion of chromosome 12q, resulting in the fusion of the NAB2 and STAT6 genes. Most of these tumors exhibit favorable prognosis.

Recognizing and including this entity in the differential is necessary before the final diagnosis may be achieved through proper immunohistochemical and molecular workup. In this paper, we present two cases of extrapleural SFT with unusual locations: the first is a primary SFT present in a parotid gland and the second is a metastatic SFT present as two solid pancreatic masses.

The patient's abdominal pain resolved completely following resection. This case highlights the diagnostic challenges of mesenteric SFTs and underscores the importance of complete surgical resection with intact borders and immunohistochemical analysis in securing an accurate diagnosis and favorable outcome.

Consequently, combining BET inhibitors with PARP (Poly (ADP-ribose) polymerase) inhibitors or with ATR inhibitors significantly enhanced anti-proliferative effects in SFT cells. Taken together, this study establishes BET inhibitors Mivebresib and BMS-986158 as promising anti-SFT agents.

Conventional chemotherapy may be used, with doxorubicin and dacarbazine being active though yielding poor responses. Other active drugs with currently ongoing studies in SFT are eribulin and trabectedin...An area of recent investigation is immunotherapy, with an ongoing randomized trial comparing nivolumab + ipilimumab versus pazopanib in advanced rare soft tissue sarcomas, including SFTs. Despite its rarity and therefore the difficulty in performing prospective randomized trials with a large number of patients, many promising results in perioperative (radiotherapy) or in the metastatic (medical therapy) setting have been obtained. This review overviews the main characteristics and provides the current knowledge on standard therapies.