Primitive neuroectodermal tumors of the central nervous system

Primitive neuroectodermal tumors are morphologically similar malignant tumors arising in intracranial and peripheral sites of the nervous system, showing varying degrees of cellular differentiation with a tendency to disseminate along cerebrospinal fluid pathways. They occur primarily in children and young adults. Under the designation primitive neuroectodermal tumors are included medulloblastomas and tumors that may differentiate in other directions, such as medulloepithelioma, neuroblastoma, polar spongioblastoma, pineoblastoma, ependymoblastoma, retinoblastoma, and olfactory neuroblastoma. From a practical, histologic point of view, these tumors are often indistinguishable from one another and are best thought of as primitive neuroectodermal tumors with or without differentiating features.     

Lipocortin-1 immunoreactivity in central and peripheral nervous system glial tumors

We examined the cellular distribution of lipocortin-1 (L-1), a major physiologic substrate for the epidermal growth factor receptor/kinase, in 122 central nervous system (CNS) and peripheral nervous system (PNS) neoplasms using the peroxidase-antiperoxidase technique with a polyclonal antibody specific for L-1. Extensive L-1 immunoreactivity was demonstrated in many CNS tumors; in 11 of 21 glioblastoma multiformes, in five of 12 anaplastic astrocytomas, and in five of 14 astrocytomas. Significant numbers of immunoreactive ependymocytes or astrocytes were also seen in six of 13 ependymomas. In contrast, no immunostaining was detected in the oligodendrocytes in any of ten oligodendrogliomas. PNS tumors, found in two of five malignant nerve sheath tumors, 13 of 15 schwannomas, 13 of 17 neurofibromas, and 14 of 15 traumatic neuromas, also contained considerable L-1 immunoreactivity in Schwann cells or mast cells. These findings raise the possibility that L-1 may participate in the proliferation or subsequent differentiation of neoplastic astrocytes, ependymocytes, and Schwann cells.     

具有梭形细胞的神经系统肿瘤

中枢神经系统很多肿瘤都以梭形细胞为表型,了解这些肿瘤的病变特点并结合免疫组织化学染色,有助于正确判断肿瘤来源和良恶性.     

Clinicopathological and immunohistochemical features of primary central nervous system germ cell tumors: a 24-years experience

Primary central nervous system (CNS) germ cell tumors (GCTs) are a rare heterogeneous group of lesions, which the clinicopathological features have a marked degree of heterogeneity comparing with that of gonadal GCTs. Accurately diagnosing CNS GCTs might be extremely difficult and requires immunohistochemical verification. This study was to investigate the biological feature of CNS GCTs and diagnostic value of immunohistochemical markers OCT3/4, C-kit, PLAP, and CD30 in CNS GCTs. A retrospective study was performed on 34 patients with CNS germ cell tumors between 1990 and 2014. 34 CNS GCTs account for 9.2% of all primary CNS neoplasms. The sellar region (35.3%) and pineal gland (17.6%) were the most common sites of intracranial GCTs. Hydrocephalus (82.4%) and diplopia (46.9%) were the two most common clinical presentations. The most common histological subtypes were germinoma (67.6%). PLAP, c-kit, OCT3/4 were highly expressed in gernimomas. CD30 and CK AE1/3 stainings were positive in embryonal carcinoma. Yolk sac tumor component showed positive staining for AFP and CK AE1/3. β-HCG staining was positive in choriocarcinoma and STGC. Patients with mature teratomas and germinomas had a better prognosis (a 5-year survival rate) than those with embryonal carcinoma and choriocarcinoma (a 5-year survival rates were 0). Our finding suggest that the incidences of primary CNS GCTs are higher in South China than in the West, but mixed GCTs are uncommon in our study. The judicious use of a panel of selected markers is helpful in diagnosing and predicting the prognosis for CNS GCTs.     

Review: Glial lineages and myelination in the central nervous system

Oligodendrocytes, derived from stem cell precursors which arise in subventricular zones of the developing central nervous system, have as their specialist role the synthesis and maintenance of myelin. Astrocytes contribute to the cellular architecture of the central nervous system and act as a source of growth factors and cytokines; microglia are bone-marrow derived macrophages which function as primary immunocompetent cells in the central nervous system. Myelination depends on the establishment of stable relationships between each differentiated oligodendrocyte and short segments of several neighbouring axons. There is growing evidence, especially from studies of glial cell implantation, that oligodendrocyte precursors persist in the adult nervous system and provide a limited capacity for the restoration of structure and function in myelinated pathways damaged by injury or disease.     

中枢神经系统血管周细胞瘤临床病理学观察

目的探讨中枢性血管周细胞瘤(haemangiopericytoma,HPC)的临床特点、影像学特征、免疫表型。方法对4例中枢性HPC患者临床病理资料进行总结分析,随访3～24个月,并复习相关文献。结果男性2例,女性2例,平均年龄50岁。4例患者均先行肿瘤切除术,其中2例术后行放疗。免疫表型:3例vimentin、CD34、CD99均(+),3例EMA(-)性,1例EMA灶状(+)。例3和例4的Ki-67增殖指数分别为灶状5%和10%。随访3～24个月,肿瘤无复发及颅外转移。结论中枢性HPC发病率极低,其影像学表现与脑膜瘤相似,确诊均需免疫组化标记来证实,但其与脑膜瘤在生物学行为、组织学及治疗预后方面均不同,故中枢性HPC患者术后需进行长期随访,及早发现复发或转移并给予及时处理,可降低患者病死率。     

FIP1L1-PDGFRA positive chronic eosinophilic leukaemia and associated central nervous system involvement

Interstitial deletion involving chromosome 4q12 generates the novel tyrosine kinase fusion protein encoded by FIP1L1-PDGFRA, which is present in many patients previously labelled as having hypereosinophilic syndrome, initially reported in 2003. Reports in recent literature document excellent clinical and molecular response to the tyrosine kinase inhibitor imatinib (Glivec). This report describes the case of a 58-year-old lady, diagnosed with FIP1L1-PDGFRA positive hypereosinophilic disorder, who subsequently developed symptoms related to an intracranial lesion. Biopsy and molecular genetic studies confirmed a diffuse infiltrative lesion, with evidence of FIP1L1-PDGFRA gene fusion. Initiation of imatinib treatment led to impressive clinical and radiological response.     

Cytologic diagnosis of glial tumors of the central nervous system

The authors analysed retrospectively 476 histologic and cytologic preparations made from the biopsy specimens of neuroectodermal CNS tumours. The identity of histologic and cytologic diagnoses was determined in 89.5% of cases. Cytologic diagnostic criteria for tumours with different histogenesis, such as astrocytomas, oligodendrogliomas, ependymomas, glioblastomas, medulloblastomas, are presented. Anaplasia features of these tumours are identified according to IV degrees of malignancy.     

Possibilities of cytological diagnosis of central nervous system tumors

The cytological and pathohistological pictures of 384 glial, meningeal, embryonic, neuronal, metastatic tumors and tumors growing into the cranial cavity and spinal canal (the impression smears of removed tumor fragments and the aspirates on fine needle puncture) were studied in parallel. The common supporting cellular and structural signs required for the diagnosis of the major groups of central nervous system (CNS) tumors, for the verification of some histological types of a neoplasm, and for the estimation of the stage of their malignancy were revealed. An algorithm of cytological diagnosis of some CNS tumors and an adapted working classification are proposed.     

Primary tumors of the central nervous system: histogenetic classification, grade of malignity, clinico-pathologic features, and therapeutic criteria

We review the current classification of the central nervous system neoplasms focusing on grading and new clinicopathological entities. Moreover a brief synopsis on up to date treatments in different groups of neoplasms is given.     

原发性中枢神经系统平滑肌肉瘤的临床病理分析

目的探讨罕见的原发性中枢神经系统平滑肌肉瘤(primary leiomyosarcoma of the central nervous system,PLCNS)的临床病理特点、诊断及鉴别诊断、治疗及预后。方法对1例PLCNS进行免疫组化EnVision两步法染色及原位杂交技术检测,搜索国外24例PLCNS,对25例PLCNS临床病理特点、治疗及预后进行分析讨论。结果患儿男性,1岁8个月,血清学HIV阴性,肿瘤位于小脑靠近天幕。镜下显示短梭形肿瘤细胞呈短条索状排列于黏液性间质中,细胞明显异型,核分裂多见。免疫标记SMA阳性,Ki-67增殖指数约80%,EBV原位杂交阴性,患儿行肿瘤全切除术后5个月因肿瘤复发死亡。25例PLCNS中免疫缺陷及放疗无关者(简称:无关者)8例,免疫缺陷合并EBV感染者13例(简称:免疫缺陷者),长期放疗者1例。结论PLCNS临床特点:无关者可发生于儿童和中年,免疫缺陷者均为中青年;PLCNS多为单发肿块,常位于幕上,多与脑膜相关,免疫缺陷者可发生于脊髓,可多发,可合并多系统平滑肌肉瘤;无关者病程较免疫缺陷者短。PLCNS常体积巨大伴轻度瘤周水肿,可侵蚀颅骨。组织学上免疫缺陷者分化较好;无关组多为差分化平滑肌肉瘤(包括黏液性平滑肌肉瘤)。PLCNS预后差,治疗以手术切除为主,放、化疗疗效尚不清楚。     

Granular cell tumors of the central nervous system of rats.

On the basis of our study of 12 cases of granular cell tumors in the brains of rats of different inbred strains, it seems that granular cell tumors are not rate in the central nervous system of untreated, aging BN/Bi rats. In none were metastases found. Awareness of the occurrence of these tumors in the central nervous system of these rats may stimulate further studies into the cellular origin of granular cell tumors that form in the central nervous system of the rat and might help to elucidate the origin of these tumors in general.     

Selected biomarkers in the primary tumors of the central nervous system: short review

Classification, grading and treatment of central nervous system tumors is currently based on morphology. Advances in molecular biology help to clarify pathogenesis, refine prognosis and detect potential targets for targeted therapy in a wide spectrum of CNS tumors. In this short review we present our view on selected diagnostic, prognostic and predictive biomarkers of primary CNS tumors, with an emphasis on application in daily praxis.     

Germ cell tumors of the central nervous system: A brief review and site-specific considerations

Germ cell tumors of the central nervous system (GCT-CNS) arise predominantly in midline locations of the CNS and affect young patients in their first to third decades of life. Involvement of the CNS is thought to be a sequelae of residual primordial germ cells with incomplete embryologic migration. Clinically, GCT-CNS present with symptoms of ventricular obstruction or compression of affected brain structures. Histologically, these tumors are analogous to their gonadal and extra-gonadal counterparts. Diagnosis relies heavily on morphology and immunohistochemical findings, and can be complicated by limited tumor sampling. There is currently only a limited role for molecular studies. Treatment of these lesions is made difficult by their involvement of deep and vital brain structures and accurate pathologic diagnosis is essential for appropriate therapy. Diagnosis should involve review of the clinical history, imaging studies, and assessment of serum and cerebrospinal fluid tumor markers. Current therapeutic strategies involving radiation therapy with or without chemotherapy are quite effective, in spite of the locational difficulties that often prevent gross total resection.