RESUMO
We describe a morphologically distinct pattern of tumor infarction and associated sarcoma-like changes, mimicking focal anaplasia, in otherwise WHO grade I meningiomas. The described cases (n = 9) all demonstrated a discrete spindle-cell (pseudosarcomatous) component with brisk mitotic activity (12-14 mitoses/10 HPF), elevated Ki-67 (mean 75.5 ± 25.0%, quantified), absence of PR, SSTR2A, or EMA expression, and potential SMA expression (50%). Despite these high-grade features, all nine patients remained free of progression or recurrence post resection (follow-up mean: 49.8 months). In contrast, among a comparison (control) cohort of consecutive WHO grade II and III meningiomas (n = 16), as expected, progression rate was high (68.8%, P = 0.002, Fisher's exact, average time to progression = 25 months, follow-up mean: 39.8 months). While necrosis was a frequent feature among atypical/anaplastic meningiomas (12/16, 75%), and elevated mitoses and proliferative index were present consistent with histologic grade, a well-defined zonal pattern with pseudosarcomatous component was not present among these tumors. DNA methylation-based analysis readily distinguished meningiomas by copy number profiles and DNA-based methylation meningioma random forest classification analysis (meningioma v2.4 classifier developed at University of Heidelberg); all pseudosarcomatous cases analyzed (4/9) matched with high level calibrated classifier score to "MC benign-1", with isolated loss of chromosome 22q identified as the sole copy number alteration. In contrast, multiple chromosomal losses were detected among the comparison cohort and classifier results demonstrated good concordance with histologic grade. Our findings suggest that pseudosarcomatous alterations represent reactive changes to central meningioma infarction, rather than focal anaplasia, and further support the use of DNA methylation-based analysis as a useful adjunct for predicting meningioma behavior. These indolent tumors should be distinguished from their atypical and anaplastic counterparts.
Assuntos
Infarto/patologia , Neoplasias Meníngeas/patologia , Meningioma/patologia , Idoso , Anaplasia/patologia , Biomarcadores Tumorais/genética , Metilação de DNA , Feminino , Humanos , Masculino , Neoplasias Meníngeas/genética , Meningioma/genética , Pessoa de Meia-IdadeRESUMO
Synovial sarcoma of the extremities represents 7% of all soft-tissue sarcomas. This article presents the case of a patient who was treated for a synovial sarcoma of the lateral aspect of the distal lower leg extending to the ankle and involving the fibular bone. The patient underwent a wide excision of the tumor, including the fibular bone, followed by radiation and chemotherapy, rather than undergo an amputation of the right leg. Consideration is also given to the traumatic etiology of the tumor.