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Background: Soft tissue tumors with fusions or amplifications of the GLI1 gene have distinctive molecular characteristics and have recently been considered a unique pathological entity, thus named " GLI1 -altered soft tissue tumors." It is a rare mesenchymal neoplasm that involves soft tissues at any site. Case presentation: We report an example of this condition in a 13-year-old Chinese male patient who presented with a mass in the tongue. The tumor was multilobulated; the tumor cells were arranged in nests and sheets, had a rich, delicate fibrovascular network, and were separated by a hyalinized fibrous stroma. The tumor cells were epithelioid to ovoid, with variable eosinophilic to pale vacuolated cytoplasm and round to oval nuclei. Immunostaining revealed that the tumor cells were positive for CDK4 and CD56. Fluorescence in situ hybridization (FISH) for GLI1 translocation was positive, with a high level of amplification of the translocated segment. Literature review: We present a comprehensive literature review of this condition, focusing on its clinical presentation, histological features, immunohistochemical profile, molecular characteristics, and prognosis. [ABSTRACT FROM AUTHOR]

Primary pulmonary myxoid sarcoma is a rare lung sarcoma, mostly involving the central lung and harboring the EWSR1::CREB1 fusion. We report an exceptional case of primary pulmonary myxoid sarcoma arising in the peripheral lung and harboring an EWSR1::ATF1 gene fusion. A 67-year-old man presented with a solid nodule in the right lower lobe, and wedge resection was performed. Microscopically, the tumor consisted of reticular proliferation of uniform mildly atypical spindle cells within abundant myxoid stroma. Immunohistochemically, smooth muscle actin was positive but desmin was negative. Fluorescence in situ hybridization confirmed EWSR1 and ATF1 gene rearrangements. No recurrence was seen for 12 months. Pathological findings and gene rearrangements are important for the diagnosis of primary pulmonary myxoid sarcoma. Complete resection and careful observation are required. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumors (SFTs) are mesenchymal neoplasms associated with the characteristic NAB2::STAT6 gene fusion. They frequently occur in extra-thoracic sites and are not uncommon in the head and neck (HN) region. Myxoid SFT is a rare morphological subtype of SFT, the features of which overlap with those of other myxoid-appearing tumors, making the diagnosis challenging. We describe the distinctive histopathological and immunohistochemical features of myxoid SFT that occurred in a 32-year-old man with a recurrent swelling in the nasal vestibule. Histological examination showed a nodular tumor composed of short spindle-shaped cells in an abundant myxoid stroma. Nuclei were ovoid, with minimal pleomorphism. Occasional intervening slender vascular channels were present; staghorn vasculature was absent. Tumor cells were diffusely immunopositive for STAT6, CD34, and BCL2, while S100, SOX10, EMA, ER, and CD10 were negative, confirming the diagnosis of myxoid SFT. Thus, myxoid SFTs are unusual in the HN, with potential for misdiagnosis. Due to their propensity for local recurrence if incompletely excised, a high index of suspicion is required to include them in differential diagnosis of myxoid mesenchymal neoplasms occurring at this location. STAT6 is a reliable immunohistochemical marker that aids in diagnosis, reducing the necessity for molecular testing. [ABSTRACT FROM AUTHOR]

Examines a case of an angiolipoma involving the thyroid gland of a 74-year-old woman. Presence of an intrathyroidal well-circumscribed nodule that microscopically showed the typical features of an angiolipoma; Lack of evidence of extrathyroidal extension, necrosis, or atypia; Description of angiolipoma.