Dash R C, Provenzale J M, McComb R D, Perry D A, Longee D C, McLendon R E
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA.
Arch Pathol Lab Med. 1999 Apr;123(4):342-5. doi: 10.5858/1999-123-0342-MSGGCG.
From both epidemiologic and pathologic viewpoints, gangliogliomas exhibiting components of giant cell glioblastomas are extraordinary neoplasms. We report herein the case of a 6-year-old girl who presented initially with a World Health Organization grade IV anaplastic ganglioglioma (a mixed ganglion cell tumor-giant cell glioblastoma). Despite aggressive management, the patient died of disease in a relatively short period.
Formalin-fixed, paraffin-embedded tissue blocks were sectioned at 5 microm for histochemical and immunohistochemical analyses. Hematoxylin-eosin-stained sections and immunohistochemically stained sections from the primary and secondary resections were reviewed. Reactivity for glial fibrillary acidic protein, neurofilament protein, synaptophysin, and Ki67 nuclear antigen was evaluated.
Histologically, 2 distinct cell populations were noted on both the primary and secondary resections. The primary resection revealed a neoplasm having a predominant glial component consistent with a glioblastoma. Interspersed were dysmorphic ganglion cells supporting a diagnosis of ganglioglioma. The second resection (following therapy) demonstrated a much more prominent dysmorphic ganglion cell component and a subdued glial component.
Although immunohistochemical analysis clearly distinguished the 2 tumor cell populations, the identification of Nissl substance in neurons proved to be equally helpful. Although other cases of grade III gangliogliomas and rare cases of grade IV gangliogliomas have been reported, the present case is exceptional in that, to our knowledge, it is the only report of a patient who presented initially with a composite grade IV ganglioglioma and who was clinically followed up to the time of death. This case allows direct comparison between the histologic findings in a giant cell glioblastoma and a ganglioglioma and documents the aggressive biologic behavior of this complex neoplasm.
从流行病学和病理学角度来看，具有巨细胞胶质母细胞瘤成分的节细胞胶质瘤是一种特殊的肿瘤。我们在此报告一例6岁女孩，最初表现为世界卫生组织IV级间变性节细胞胶质瘤（混合性神经节细胞瘤 - 巨细胞胶质母细胞瘤）。尽管采取了积极的治疗措施，但患者在相对较短的时间内死于该疾病。
将福尔马林固定、石蜡包埋的组织块切成5微米厚进行组织化学和免疫组织化学分析。对原发和二次切除标本的苏木精 - 伊红染色切片和免疫组织化学染色切片进行复查。评估胶质纤维酸性蛋白、神经丝蛋白、突触素和Ki67核抗原的反应性。
组织学上，在原发和二次切除标本中均发现了2种不同的细胞群。原发切除标本显示肿瘤以胶质成分占主导，与胶质母细胞瘤一致。其间散在着异形神经节细胞，支持节细胞胶质瘤的诊断。二次切除标本（治疗后）显示异形神经节细胞成分更为突出，胶质成分减弱。
尽管免疫组织化学分析清楚地区分了2种肿瘤细胞群，但神经元中尼氏体的鉴定同样有帮助。虽然已有III级节细胞胶质瘤的其他病例和IV级节细胞胶质瘤的罕见病例报道，但本病例具有特殊性，据我们所知，这是唯一一例最初表现为IV级复合节细胞胶质瘤且临床随访至死亡的病例报告。该病例使得能够直接比较巨细胞胶质母细胞瘤和节细胞胶质瘤的组织学发现，并记录了这种复杂肿瘤的侵袭性生物学行为。
