Almefty Rami O, Tyree Tammy L, Fusco David J, Coons Stephen W, Nakaji Peter
Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona, USA.
J Neurosurg Pediatr. 2013 Sep;12(3):251-7. doi: 10.3171/2013.6.PEDS12533. Epub 2013 Jul 26.
Histiocytic sarcoma is a rare malignancy with only 10 reports confirmed primarily involving the CNS. The diagnosis is dependent on the finding of malignant cells with histiocytic morphology and immunophenotype. The authors report a case of pathologically proven HS of the CNS. A 16-year-old boy presented with headaches, emesis, and altered sensorium. Noncontrast head CT scanning demonstrated a left parietal mass consistent with a tumor. Surgery was undertaken. Intraoperative findings revealed green-yellow exudates consistent with an abscess. Cultures were obtained and broad-spectrum antibiotics were started. The patient subsequently underwent multiple surgical procedures, including drainage and debulking of abscesses and hemicraniectomy. Two months after initial presentation, the patient's diagnosis of histiocytic sarcoma was confirmed. Pathological examination demonstrated necrotizing inflammation with preponderant neutrophil infiltration, variably atypical mononuclear and multinucleate histiocytes, and numerous mitoses. Additional immunohistochemistry studies confirmed immunoreactivity for CD68, CD45, CD45RO, and CD15 and were negative for CD3, CD20, melanoma cocktail, CD30, CD1a, CD34, HMB-45, and melan-A. Once the diagnosis of histiocytic sarcoma was confirmed, antibiotics were stopped and radiation therapy was undertaken. Despite treatment, the patient's neurological status continued to decline and the patient died 126 days after initial presentation. This case represents a rare confirmed example of CNS histiocytic sarcoma. A profound inflammatory infiltrate seen on pathology and green exudates seen intraoperatively make the condition difficult to distinguish from an abscess. Immunohistochemistry showing a histiocytic origin and negative for myeloid, dendritic, or other lymphoid markers is essential for the diagnosis. Further research is needed to establish consensus on treatment.
组织细胞肉瘤是一种罕见的恶性肿瘤，仅有10篇主要涉及中枢神经系统的确诊报告。诊断依赖于发现具有组织细胞形态和免疫表型的恶性细胞。作者报告了一例经病理证实的中枢神经系统组织细胞肉瘤病例。一名16岁男孩出现头痛、呕吐和意识改变。非增强头部CT扫描显示左侧顶叶有一个与肿瘤相符的肿块。进行了手术。术中发现黄绿色渗出物，与脓肿一致。进行了培养并开始使用广谱抗生素。患者随后接受了多次外科手术，包括脓肿引流、减瘤和颅骨切除术。初次就诊两个月后，患者组织细胞肉瘤的诊断得到证实。病理检查显示坏死性炎症，有大量中性粒细胞浸润、不同程度的非典型单核和多核组织细胞以及大量有丝分裂。额外的免疫组织化学研究证实对CD68、CD45、CD45RO和CD15呈免疫反应，而对CD3、CD20、黑色素瘤组合、CD30、CD1a、CD34、HMB - 45和黑色素A呈阴性。一旦组织细胞肉瘤的诊断得到证实，停用抗生素并进行放射治疗。尽管进行了治疗，患者的神经状态仍持续恶化，初次就诊126天后死亡。该病例是中枢神经系统组织细胞肉瘤罕见的确诊实例。病理上可见的严重炎症浸润和术中所见的绿色渗出物使得该病症难以与脓肿区分开来。免疫组织化学显示组织细胞起源且对髓系、树突状或其他淋巴标记物呈阴性对于诊断至关重要。需要进一步研究以建立关于治疗的共识。
