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胶质肉瘤( gliosarcoma)是胶质母细胞瘤的种亚型,具有恶性度高、预后差、生存期短等特点,发病率占胶质细胞瘤的2%-8%。1895年Strobe首先将胶质母细胞瘤与肉瘤的混合性肿瘤命名为“胶质肉瘤”,其组织学特点兼有胶质母细胞和恶性间叶成分,间叶成分以恶性纤维组织细胞最多见,也可为骨肉瘤、软骨肉瘤、横纹肌肉瘤等其他肉瘤成分。Gliosarcoma, a subtype of glioblastoma, is characterized by high malignancy, poor prognosis and short survival, accounting for 2%-8% of glioblastomas. In 1895, Strobe first named glioblastoma and sarcoma as "gliosarcoma". The histological characteristics of glioblastoma and malignant mesenchymal components are combined. Most of the mesenchymal components are malignant fibrous tissue cells, and they can also be osteosarcoma, chondrosarcoma, rhabdomyosarcoma and other sarcomas.
胶质肉瘤的年龄分布与胶质母细胞瘤相似,以40~60岁多见(平均年龄52.1岁),少数病例见于儿童,甚至是幼儿。男性发病率高于女性。胶质肉瘤多位于大脑半球,颞叶、额叶、顶叶和枕叶肿瘤的发生频率依次递减,发生于后颅凹和脊髓的肿瘤比较少见。主要为头痛、头晕、恶心、呕吐等颅内压增高症状,可伴有肢体偏瘫、癫痫、意识障碍等。The age distribution of gliosarcoma is similar to that of glioblastoma. It is most common in children aged 40 to 60 (average age 52.1 years), and a few cases are found in children, even young children. The incidence is higher in men than in women. Gliosarcomas are mostly located in the cerebral hemispheres. The frequency of tumors in the temporal lobe, frontal lobe, parietal lobe and occipital lobe decreases successively, and tumors in the posterior fossa and spinal cord are rare. Mainly for headache, dizziness, nausea, vomiting and other increased intracranial pressure symptoms, can be accompanied by limb hemiplegia, epilepsy, consciousness disorders.
其发病机制目前有以下几种解释:①胶质瘤中血管内皮细胞和成纤维细胞高度增生,当这些间胚叶成分呈肿瘤性增生且与胶质瘤混合时,则呈现胶质肉瘤的结构;②胶质瘤侵犯或刺激邻近脑膜,引起脑膜纤维组织增生及肉瘤变(即诱导学说);③脑内原发性肉瘤与其周围由反应性胶质细胞增生并进一步形成的胶质瘤混合而成;④肉瘤与胶质瘤分别发生后混合在一起(碰撞学说);⑤近来有人提出其肉瘤成分可由血管周围间叶组织去分化所致;⑥也有人认为来源于颅内多潜能干细胞,是该细胞向多种方向分化的结果;⑦最新研究表明,患者的部分基因先发生了改变,之后才有肿瘤的进一步发生发展。目前大多数学者认同胶质肉瘤是胶质细胞瘤中血管结缔组织肉瘤变而进一步发展形成所致。CT病灶常较大,形态不规则,分叶状,呈囊实性,囊腔偏向一侧,囊壁明显厚薄不均为胶质肉瘤的特点,病灶周围水肿明显。增强后病灶不均匀强化,部分实质成分明显强化,部分轻度强化,轻中度强化部分为肉瘤成分。病灶内可见多发“条状栅栏样”强化。CT lesions are usually large, irregular, lobulated, and cyst-solid. The lumen is skewed to one side, and the thickness and thickness of the cystic wall are characteristic of gliosarcoma. Edema around the lesion is obvious. After enhancement, the lesion was unevenly enhanced, some parenchymal components were significantly enhanced, some were mildly enhanced, and some were sarcomas. Multiple "strip-like palisade" enhancement were observed in the lesion.MR脑胶质肉瘤表现多种多样,特征性表现是不规则团块状混杂信号,增强后可见病灶不均匀明显强化。其次,MR也可表现为不规则团块状等T稍长T2信号影,但瘤内未见囊变、坏死信号,病灶周边可见环状水肿,增强后病灶明显均匀性强化。另外,还可以表现为等T1、等T2信号影,周围可见大片状长T1、长T2信号水肿区,增强后病灶呈条带状明显强化伴局部脑膜强化,极易误诊为转移瘤和炎性病变。最为少见的MR表现类似脑脓肿,MRI为等T1、长T2类圆形病灶,其内可见更长T2信号,增强后呈厚壁不规则环形强化。胶质肉瘤累及脑膜或大脑镰时,MRI上表现为脑膜明显增厚、强化,当出现“脑膜尾”征时,此征象可作为胶质肉瘤与胶质母细胞瘤的鉴别点。病灶侵犯邻近颅骨可导致邻近骨质破坏。MR gliosarcoma presents a variety of manifestations, characterized by irregular clumps of mixed signals. After enhancement, the lesion can be seen to be inhomogeneous and markedly enhanced. Secondly,MR could also present as an irregular, lumpy, equal-t and slightly longer T2 signal shadow, but there was no cystic degeneration or necrosis signal in the tumor, and circumferential edema could be seen around the lesion, and the enhanced uniformity of the lesion was significantly enhanced. In addition, it can also be manifested as equal T1 and equal T2 signal shadow, with large patchy areas of long T1 and long T2 signal edema around. After enhancement, the lesion presented obvious banded enhancement with local meningeal enhancement, which was easily misdiagnosed as metastatic tumor and inflammatory lesions. The most rare MR appearance was similar to that of brain abscess. MRI showed round lesions of equal T1 and long T2 type, with longer T2 signal and thick-walled irregular annular enhancement. When gliosarcoma involves the meninges or falx cerebri, the meninges are significantly thickened and strengthened on MRI. When the "meningeal tail" sign is present, this sign can be used as a distinguishing point between gliosarcoma and glioblastoma. Invasion of adjacent skull lesions can lead to adjacent bone destruction.典型的胶质肉瘤发病年龄较大,MRI表现复杂多样,常为囊实性,囊壁明显厚薄不均为胶质肉瘤的特点,局部可见壁结节,多数可见多发条状栅栏样强化。胶质肉瘤更容易发生颅内外转移颅外转移多位于肺、肝、肾上腺等处。Typical gliosarcomas are older in onset, and MRI findings are complex and varied, often with cystic consolidation. The characteristic of gliosarcomas is that the cystic walls are obviously uneven in thickness, with local wall nodules and multiple striated palisades in most cases. Gliosarcoma is more likely to have extracranial metastases. Extracranial metastases are usually located in the lungs, liver, adrenal gland, etc.胶质肉瘤主要与以下疾病相鉴别1.脑膜瘤，发生于大脑凸面或大脑镰旁的胶质肉瘤,若局部与硬脑膜或大脑镰关系密切、且表现缺乏特异性时,易与脑膜瘤相混淆。此时脑膜尾征有一定鉴别诊断意义。Meningioma, a gliosarcoma occurring on the convex surface of the brain or near the falx of the brain, is easily confused with meningioma if it is closely related to the dura or falx of the brain and has no specificity. At this point, the meningeal tail sign has some differential diagnosis significance.2.淋巴瘤属小圆细胞类肿瘤,未经治疗很少坏死,边界尚清,增强后明显均匀强化;而胶质肉瘤多有不同程度坏死,增强扫描不均匀强化，环状强化，瘤周水肿程度轻重不等。Lymphoma is a small round cell tumor with little necrosis without treatment, with clear boundary and obvious uniform enhancement after enhancement. However, most gliosarcomas show different degrees of necrosis, with uneven enhancement, annular enhancement, and varying degrees of peritumor edema.3.室管膜瘤多呈良性过程,病程长,有两个发病高峰,分别为5岁前和40岁左右;儿童好发于脑室内,尤以四脑室好发;T1WI呈稍低信号, T2WI呈高信号,坏死囊变常见,增强扫描呈不均匀强化、环状强化。根据发病年龄、病程及发病部位可加以鉴别。Most ependymomas are benign, with a long course and two peaks, respectively before the age of 5 and about the age of 40. Children tend to occur in the ventricle, especially in the fourth ventricle; T1WI showed slightly low signal, T2WI showed high signal, necrotic cystic degeneration was common, and the enhancement scan showed uneven enhancement and annular enhancement. According to the age of onset, the course of disease and the location of onset can be identified.4.毛细胞型星形细胞瘤常见于儿童和青少年、以小脑最多,而成年人多见于大脑半球,呈良性生物学行为。MR多表现为囊实性,可见壁结节,增强后壁结节及实性部分明显强化,囊壁强化或不强化,且囊壁强化者少见,瘤周一般无水肿.pilocytic astrocytoma are most common in children and adolescents and are most common in the cerebellum. In adults, they are most common in the cerebral hemisphere and present benign biological behavior. MR usually presents as cystic consolidation, with wall nodules visible, posterior wall nodules and solid parts significantly enhanced, and cystic wall enhancement or non-enhancement, with rare cystic wall enhancement, and generally no edema around the tumor.PS:预后有证据表明胶质肉瘤较普通的胶质母细胞瘤预后稍好,但大量的临床研究显示二者的预后没有显著性差异。
There is evidence that gliosarcoma has a better prognosis than normal glioblastoma, but a large number of clinical studies have shown no significant difference in prognosis between gliosarcoma and glioblastoma.
