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颅内非典型畸胎瘤样/横纹肌样瘤多见于婴幼儿,呈侵袭性生长,易在中枢神经系统内播散。肿瘤组织中含有横纹肌样细胞、原始神经外胚层、肿瘤性间质成分和上皮组织。免疫组化有助于鉴别原始神经外胚层肿瘤/髓母细胞瘤。细胞遗传学表现为22对染色体呈单体性或22q11.2缺失。临床多采用以手术切除为主的强化性治疗。因继发性囊变坏死或出血,影像学呈多样性表现。
Intracranial atypical teratoma / rhabdomyosarcoma more common in infants and young children, was invasive growth, easy to spread in the central nervous system. Tumor tissue contains striated muscle-like cells, primitive neuroectoderm, tumorous interstitial components and epithelial tissue. Immunohistochemistry helps identify primitive neuroectodermal tumors / medulloblastomas. Cytogenetics showed 22 chromosomes were haplotype or 22q11.2 deletion. More clinical use of surgical excision-based intensive treatment. Due to secondary cystic degeneration or bleeding, imaging showed a variety of performance.