Uncommon presentation of central neurocytoma causing intraventricular hemorrhage: case report

Central neurocytoma was first described by Hassoun et al. in 1982 as a well-differentiated tumor from neuronal origin. This tumor typically occurs in young adults, localized in the ventricular system. It usually presents as intracranial hypertension due to obstructive hydrocephalus. The differential diagnosis should be done with others intraventricular tumors as oligodendroglioma, subependymoma and choroidal plexus papilloma. There are few cases of central neurocytoma presented by intraventricular hemorrhage in the literature. We report a case of 35 year-old woman, who presented with obstructive hydrocephalus due to intraventricular hemorrhage within the tumor. MRI revealed a tumor localized in the right lateral ventricle. Histopathological and immunohystochemical analysis confirmed the diagnosis of central neurocytoma. We review options for the treatment of this entity as well reinforce the inclusion of central neurocytoma as a differential diagnosis for intraventricular hemorrhage.     

中枢神经细胞瘤5例报告及文献复习

目的探讨提高中枢神经细胞瘤诊疗水平的策略和方法。方法回顾分析2004年8月至2006年4月我院收治的5例中枢神经细胞瘤，总结其临床表现、影像学表现、病理学特征及显微外科手术治疗效果。结果肿瘤位于侧脑室前部或室间孔附近，呈宽基底与侧脑室壁或透明隔相连。肿瘤边缘及内部可见多发囊泡，常见钙化，MRI检查T1WI、T2WI相均表现为等或略高信号，增强后呈轻至中度强化。免疫组织化学染色均显示神经突触素阳性。显微外科手术治疗5例，其中全切除4例，次全切除1例，1例死于术后并发症，4例随访至今未复发。结论中枢神经细胞瘤影像学表现具有一定特征，是术前诊断的重要参考。显微手术切除肿瘤是最佳的治疗手段，预后良好。     

Central liponeurocytoma: case report and review of literature

The lipomatous differentiation occurring within neurocytoma has more frequently been reported in neurocytoma of cerebellar origin as case reports and short series. This case report describes the clinical, radiological, histopathological and ultrastructural findings of supratentorial central neurocytoma with lipomatous differentiation in a 45-year-old female. Magnetic resonance imaging (MRI) revealed an intraventricular lesion in the right trigone which was isointense on T1 and hyperintense on T2 weighted images. There were small cystic areas and it enhanced brilliantly and homogenously on contrast. The rare lipomatous differentiation occurring in neurocytoma in the supratentorial location lends it a distinct morphological profile and thus recommends its designation as "central liponeurocytoma".     

Central neurocytoma presenting with intraventricular hemorrhage: case report and review of literature

We report a case of a 25 year old man presenting with acute headache, vomiting and nuchal rigidity. Computed Tomography (CT) scan and MRI without contrast showed a right ventricular hemorrhage surrounding a mass lesion. The tumor and hematoma were completely removed by neurosurgical transcortical-transventricular approach. Anatomopathological analysis revealed a central neurocytoma. Central neurocytoma seldom present with hemorrhage. We review 16 cases of neurocytoma with hemorrhage. It is important to recognize central neurocytoma as a cause of intraventricular hemorrhage, especially in adolescents and young adults. Outcome is often favorable when the tumor is completely removed. In some patients the clinical course is more aggressive and additional treatment such as radiotherapy, radiosurgery or chemotherapy is needed.     

Hemosiderin pigmentation of tumour cells in cerebellar pilocytic astrocytoma associated with post-traumatic hemorrhage in adults

The pilocytic astrocytoma is only rarely associated with gross intratumoral hemorrhage despite rich vasculature and blood vessel changes, accompanied often by perivascular depots of hemosiderin. We report an unusual case of pigmented cerebellar pilocytic astrocytoma presenting with posttraumatic hemorrhage in a 38-year-old man with no history related to the tumor. CT and MRI examination after head injury demonstrated unexpectedly the cystic lesion of 2 cm in diameter in the region of the right cerebellar hemisphere and vermis. The lesion was associated with hematoma and it was surgically removed 3 weeks after trauma. Histopathological examination revealed pilocytic astrocytoma tissue with broad hemorrhagic changes and with an unusual pattern of massive pigmentation of the cytoplasm of pilocytic astrocytes, consistent with hemosiderosis. Positive stains for iron and ferritin and ultrastructural study confirmed deposition of hemosiderin granules in the tumour cells. There was no evidence of melanin or melanosomes. This finding of hemosiderin accumulation in the cytoplasm of neoplastic astroglia seems to be analogous to post-hemorrhagic pigmentation of the normal Bergmann glia and subpial astrocytes. In the literature, the examples of neuroepithelial tumors with hemosiderin pigmentation of tumor cells have been rarely documented. To our knowledge, this is the first reported case of pigmented pilocytic astrocytoma exhibiting extensive intracellular hemosiderin deposition.     

中枢神经细胞瘤的MRI表现

目的探讨中枢神经细胞瘤(central neurocytoma,CNC)的MRI的表现特征。方法回顾性分析11例经手术病理证实的CNC的MRI表现。结果 6例位于右侧脑室,4例位于左侧脑室,2例跨越双侧脑室生长并突入三脑室,位于侧脑室体部前2/3区域10例,侧脑室体部后2/3区域3例,均与透明隔呈宽基底相连。肿瘤呈分叶状,边界较清。呈肥皂泡样或蜂窝状囊实性12例,实性1例,DWI呈花斑状高低混杂信号11例,呈等或稍低信号2例。增强呈丝瓜瓤状不均匀轻中度强化10例,不均匀明显强化2例,呈环状强化1例,病灶边缘与侧脑室壁间见多发条索状影10例,牵拉粘连侧脑室壁。结论CNC具有典型发病部位及MRI特征,MRI对于CNC的术前诊断与鉴别诊断具有重要价值。     

Extraventricular neurocytoma presenting with intratumoral hemorrhage

A case of extraventricular neurocytoma with spontaneous intratumoral hemorrhage is reported. A 47-year-old man presented with sudden left-sided hemiparesis. Magnetic resonance imaging revealed a right parietal subcortical mass with intratumoral hemorrhagic transformation and without contact to the ventricular system. After complete microsurgical removal, the tumor was histologically diagnosed as neurocytoma. Usually, the term "central neurocytoma" is restricted to neurocytic neoplasms arising within the cerebral ventricles. In the majority of the cases, these slow-growing, generally circumscribed lesions become symptomatic by obstructive hydrocephalus. Hemorrhagic onset is sporadically reported in the literature. In contrast to central neurocytomas, neurocytic lesions located within the brain parenchyma, so-called "extraventricular neurocytomas" are very uncommon. To the knowledge of the authors, this is the first case of an extraventricular neurocytoma with histological classic features presenting with intratumoral hemorrhage in adults.     

A case of extraventricular neurocytoma of the spinal cord

Central neurocytoma is a rare neuroectodermal tumor generally found in young adults. It mainly originates from lateral ventricles. Extraventricular location of this kind of tumor, especially spinal cord involvement, is extremely rare. This article is the ninth case of central neurocytoma derived from the spinal region, and includes a review of the literature. The patient in this case is a 49‐year‐old woman presenting with C3–C5 spinal mass with typical histopathologic findings and low MIB?1 index.     

Fourth ventricular neurocytoma: case report and review of the literature

OBJECTIVES: Central neurocytoma is a tumour that typically occurs in young adults in close association with the lateral and third ventricles of the cerebrum. METHODS: We report the unusual case of a central neurocytoma that developed in the fourth ventricle of a 59-year-old woman and metastasized to the upper cervical canal. Subtotal excision and adjuvant radiotherapy were used to treat the lesion. Microscopic evaluation, discussion of the pathologic differential diagnosis and theories of the histogenesis of the tumour are presented. RESULTS AND CONCLUSIONS: Fourth ventricular neurocytoma is rare and has only been reported twice previously. It appears most likely that this tumour arises from subependymal progenitor cell lines.     

中枢神经细胞瘤的磁共振影像学分析

目的探讨中枢神经细胞瘤（CNC）的MRI影像学特点，提高对该病的认识。方法回顾性分析经手术病理证实的6例CNC的MRI资料。结果本组病例均位于侧脑室前中部及Monro孔附近，5例与透明隔呈宽基底相接，1例仅有粘连；MRI信号不均，实性部分T1WI呈等或稍低信号，周围可见多发囊变及等信号条索样结构与脑室壁及透明隔粘连；增强后轻度至明显不均匀强化。1例肿瘤出血。结论对于年轻患者，MBI显示位于侧脑室前中部及Monro孔附近占位性病变，可提示为CNC。     

中枢神经细胞瘤的诊断和治疗

目的探讨中枢神经细胞瘤的诊断和治疗。方法回顾性分析1992年12月至2005年12月我科收治的7例中枢神经细胞瘤病人的临床资料。结果本组7例中枢神经细胞瘤中,4例位于左侧脑室,2例位于右侧脑室,1例位于胼胝体;5例全切,1例次全切,1例部分切除,术后辅以放疗。随访无复发。结论中枢神经细胞瘤手术后辅以放疗可获得良好的预后。     

脑室内少突胶质细胞瘤与中枢神经细胞瘤的临床病理分析

目的 总结发生于脑室内的少突胶质细胞瘤与中枢神经细胞瘤的临床病理特点。方法 选取同期发生于侧脑室内的6例少突胶质细胞瘤和5例中枢神经细胞瘤进行回顾性研究,对各自的临床病理特点进行分析,探讨二者的鉴别诊断要点。结果。发生于脑室内的少突胶质细胞瘤与中枢神经细胞瘤在临床表现、神经影像学和光镜下的病理学表现均十分相似。结论 目前认为光镜下出现无核原纤维岛、免疫组织化学显示突触素阳性表达、电镜超微结构检查证实肿瘤细胞有神经元分化证据为诊断中枢神经细胞瘤的金标准,也是鉴别少突胶质细胞瘤与中枢神经细胞瘤的重要依据。     

中枢神经细胞瘤的影像学分析与鉴别诊断

目的探讨中枢神经细胞瘤（central neurocytoma,CNC）的影像学特征,提高术前诊断准确率。方法回顾性分析21例经手术病理证实的中枢神经细胞瘤的CT及MRI表现,分析中枢神细胞瘤的影像特点。结果肿瘤多位于侧脑室前中部室间孔区,常跨透明隔生长,CT平扫多呈等密度及较高密度,常散在钙化灶,MRI表现为T1WI上呈不均匀等信号或稍低信号,T2WI上呈不均匀稍高信号,病灶边缘可见多发小囊状影及病灶和侧脑室边缘相连的丝网状影,病灶内较常见血管流空信号,增强后病灶不均匀明显强化。结论中枢神经细胞瘤影像学表现具有一定特异性,其特征性有助于提高术前诊断正确率。     

Cystic ganglioneurocytoma outside the ventricular region

Recently cases of ganglioneurocytoma and cerebral neurocytoma, very rare variants of central neurocytoma, have been reported. The former is characterized by differentiation toward ganglion cells and the latter by extraventricular origin in the cerebrum, but their existence as distinct clinicopathological entities, is controversial. We report an unusual case of neurocytoma, which arose extraventricularly from the frontal lobe, formed a large cystic lesion and showed ganglioid differentiation, in a 11-year-old girl. Following subtotal tumor resection, she showed a satisfactory clinical course and no evidence of recurrence. This is a very rare case of central neurocytoma-like tumor outside the ventricular system and also of ganglioneurocytoma. This case may provide some insight into the tumorigenesis and widen the clinicopathological concept of neurocytoma. Received: 1 October 1996 / Revised, accepted: 9 January 1997     

中枢神经细胞瘤影像学特点及病理分析(附10例报告)

目的提高对中枢神经细胞瘤(CNC)的影像、病理及免疫组化特征的认识。方法回顾性分析10例CNC的影像学特点,采用HE染色及Envision免疫组织化学染色观察其病理组织学特点和免疫表型特征。结果肿瘤与透明隔关系密切,钙化、囊变常见,MRI表现为蜂窝样混杂信号,斑片状、条状增强。组织学形态类似少突胶质细胞。免疫组化10例Syn(+),8例NSE(+),7例GFAP(+)。结论 CNC主要发生在脑室前部或靠近室间孔区附近,其影像学有一定的特点,当此区发生肿瘤时要考虑CNC可能,需行免疫组化检查。肿瘤具有双向分化特征,免疫组化标记Syn、NSE阳性对诊断CNC起重要作用。     

Novel case of recurrent intraventricular atypical central neurocytoma with prominent gangliogliomatous differentiation in a 10‐year‐old boy with 10 years of follow up

Central neurocytoma is a rare neuronal tumor that typically occurs in young adults. Infrequently, these tumors exhibit advanced neuronal maturation and glial differentiation, giving rise to a histologically diverse tumor, in contrast to a typical central neurocytoma. We present a novel case of intraventricular central neurocytoma with prominent gangliogliomatous differentiation that developed atypical features upon recurrence after 10 years of follow up in a 10‐year‐old boy. Our case provides insight into the divergent differentiation capability of a neurocytic tumor and illustrates the diverse histological features of this rare entity.     

Central neurocytoma: long-term follow-up of a paediatric case

In order to gain a better understanding of the clinical and histological features of central neurocytoma and the role of radiotherapy in the treatment of these tumours, we present an unusual paediatric case and review the pertinent literature.Most patients present in the second and third decade of life, rarely in the teenage years and exceptionally in children. Our patient was treated by a combined surgical and radiation therapy treatment for a central neurocytoma at the age of 7 years, but the tumour had been diagnosed when she was 3 years old. At a 38-year follow-up, she is well with no evidence of tumour progression.This patient seems to be the youngest histologically proven case of central neurocytoma and with the longest recorded survival.     

Pseudopapillary neurocytoma of temporal lobe with glial differentiation

We present a case of cerebral neurocytoma with unusual pseudopapillary pattern, which was a predominant feature in the tumor and was characterized histologically by hyalinized vascular cores surrounded by a single or multilayered small round cells. Vascular hyalinization was also evident in the linear arborizing capillary networks in the cellular mass of the tumor. Immunohistochemically, the tumor cells were strongly positive for synaptophysin and neuron-specific enolase except some cells lining the pseudopapillae, which showed immunoreactivity for glial fibrillary acidic protein, vimentin and S-100 protein. Ultrastructural examination revealed neuritic process of the tumor cells with occasional synaptic structures and neurosecretory granules. This report suggests that neurocytoma should be included in the differential diagnosis of papillary tumors in the central nervous system. Received: 11 September 1996 / Revised, accepted: 28 January 1997     

Patterns of differentiation in central neurocytoma

Summary Central neurocytoma has been characterised by its intraventricular localisation, predominant occurrence in young adults, oligodendroglioma-like histology, benign course and ultrastructural evidence for neuronal differentiation. Eleven intraventricular central neurocytomas were studied histopathologically, employing cell type-specific immunocytochemical markers and electron microscopic analysis. In the past, these lesions have caused diagnostic problems since central neurocytomas share basic histopathological features with other periventricular neoplasms. Accordingly, several tumours of this series had previously been classified as ependymomas of the foramen of Monro or oligodendrogliomas. Although generally regarded as benign lesions, two central neurocytomas of this series showed histopathological evidence of anaplasia, with focal necrosis, mitotic activity and vascular proliferation. All central neurocytomas exhibited immunoreactivity for neuronspecific enolase and synaptophysin, indicating consistent neuronal differentiation. Three tumours were studied by electron microscopy and contained synaptic vesicles, neuritic processes and neurosecretory granules. In addition, one tumour contained ganglioid cells and this was associated with focal immunoreactivity for neurofilament protein, suggesting that some central neurocytomas may, at least focally, continue to differentiate towards the formation of mature neurons. Two of the tumours expressed glial fibrillary acidic protein in a considerable percentage of neoplastic cells which demonstrates a capacity for bipotential, i.e. glial and neuronal differentiation. We conclude that the central neurocytoma can be reliably diagnosed using antibodies to neuron-specific enolase and synaptophysin, and that histogenetically, this neoplasm is derived from a neuroectodermal precursor cell capable of both, neuronal and glial differentiation. The hypothesis is proposed that the central neurocytoma originates from the subependymal plate of the lateral ventricles, an embryonal matrix cell layer which postnatally maintains a limited proliferative potential.