Surgical histopathologic findings of 232 Chinese children cases with drug-resistant seizures.

OBJECTIVES: The drug-resistant seizures are characterized by frequent and severe onset of seizures in childhood. There is only little literature had extensively explored the types of pathological brain damage in Chinese children cases. The present study aims to investigate the histopathologic findings and seizure outcomes of drug-resistant seizures in cases of 0-14-year-old children. MATERIALS AND METHODS: About 232 children cases were retrospectively who underwent epilepsy surgery. The medical records, onset age, age of surgery, disease course, seizure type, pathological reports, and seizure outcomes of these patients were retrospectively analyzed. RESULTS: The most common categories were malformations of cortical development (focal cortical dysplasia (FCD) was the most common type (94.36%, 67/71), found in 30.60% of the patients, tumors in 18.11%, glial scar in 12.50%, and encephalitis in 11.63%). It was found that the effective seizure outcome of FCD cases with shorter duration of epilepsy (<2 years, 87.23%) was better than that with longer duration (≥2 years, 60.00%) and the difference was statistically significant (χ2  = 4.76, p < .05). Patients with FCD I, FCD II, and tumor showed the relatively better postsurgical seizure outcome than patients with other pathological types. CONCLUSION: The malformations of cortical development (MCD) (most FCD) were the most common pathological type for children cases in China with drug-resistant seizures. It was speculated further that the FCD patients with shorter duration of epilepsy before surgery seem to have a higher ratio of being seizure-free after surgery.

Proximal-Type Epithelioid Sarcoma in Skull Base: a Pathological Diagnosis Challenge with Other Intracranial Tumors.

Proximal-type ES (PES) is a rare and aggressive sarcoma originated from soft tissues with uncertain differentiation. It mainly affects middle-aged patients and often locates in proximal extremity and deep-seated tissues. Only one case of PES located in the skull base has been reported to date. Herein, we report two cases of PES occurred in the middle cranial fossa in two middle-aged Chinese women. Microscopically, the tumors were consisted of epithelial-like cells with or without rhabdoid cells. And frequent mitotic activity and coagulation necrosis were present. Immunohistochemically, tumor cells in the two cases were positive for vimentin, AE1/AE3, epithelial membrane antigen (EMA), CD34, and synaptophysin. A few number of tumor cells expressed CD56. They were completely negative for integrase interactor-1 (INI1). Besides, TP53 positive cells were observed (>50%) in the two cases. The MIB-1 proliferation index was high up to 50-70%. Fluorescence in situ hybridization showed the monoallelic deletions of INI1. Intracranial PES is needed to identify with other mimic tumors, especially rhabdoid meningioma, epithelioid MPNST and adult AT/RT. The prognosis of the two patients was very poor. They died respectively less than a month and half a month after surgery. Tumor grew rapidly and was easy to infiltrate into the surrounding tissues. It may suggest that the prognosis of PES occurred at the base of skull was worse than in other sites.