An elderly case of malignant small cell glioma with hemorrhage coexistent with a calcified pilocytic astrocytoma component in the cerebellar hemisphere

Pilocytic astrocytoma is a less aggressive form of glial tumor that commonly occurs in the pediatric population, and its malignant transformation is extremely rare. Here, we report an elderly case of malignant small cell glioma with hemorrhage coexistent with a calcified pilocytic astrocytoma component. An 80‐year‐old male was found to have a right cerebellar non‐enhanced tumor with hematoma adjoining a calcified nodule. The lesion was surgically removed, and a histological examination verified that the tumor was a malignant small cell glioma with hemorrhagic change and the calcified nodule showed features of pilocytic astrocytoma. Genetic analyses revealed no glioma‐relevant genetic alterations such as IDH and BRAF mutations. Although calcification is generally observed in slowly growing gliomas, the aggressive clinical course of calcified cerebellar pilocytic astrocytoma has been previously reported. Our extremely rare case shows that careful follow‐up is necessary even for calcified pilocytic astrocytomas.     

儿童小脑毛细胞型星形细胞瘤的诊治(附15例报告)

目的总结儿童小脑毛细胞型星形细胞瘤的临床资料，探讨其诊治特点。方法分析儿童小脑毛细胞型星形细胞瘤15例的影像学、病理学特点及术中所见和预后情况。结果肿瘤全切除13例，次全切或大部切除2例。术后发热2例，均于术后3d内消失；头痛2例，随病情好转而逐渐消失。余病例未出现新的症状和体征。术后随访未见到复发。结论儿童小脑毛细胞型星形细胞瘤有其独特的临床特点，在条件允许的情况下应尽可能切除肿瘤。     

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.     

Late malignant recurrence of childhood cerebellar astrocytoma

Juvenile pilocytic astrocytoma of the cerebellum has a benign course and a good prognosis. We report a case of juvenile cerebellar astrocytoma in a 6-year-old girl that underwent surgical resection of the tumor and had two recurrences, 13 and 35 years after first removal. After surgery the patient did not receive any radiation therapy. The last relapse showed histological features of an anaplastic astrocytoma. Six months later the patient died with a diffuse leptomeningeal dissemination. Late malignant transformation of a benign cerebellar astrocytoma is very rare and it is thought to be favored by postsurgical irradiation. The possible pathogenetic mechanisms of this evolution are discussed. This case and the few others reported in the literature emphasize the risk of an unpredictable outcome with the low-grade cerebellar astrocytomas of childhood.     

A case of juvenile pilocytic astrocytoma with unusual neuroimaging features]

We present a case of cerebellar juvenile pilocytic astrocytoma(JPA) with unusual neuroimaging features. The patient was a 14-year-old male who suffered from chronic headaches for a couple of weeks. Plain craniogram showed a decalcification and bulging of the occipital bone. Computed tomography(CT) scans demonstrated low density multiple components with small calcifications in the right cerebellar hemisphere extending to the left. These calcifications were found at the margin of these multi-lobular components. Magnetic resonance imaging(MRI) revealed iso or low intensity on T1 weighted image, and slightly high intensity on T2 weighted image. The lesion indicated more heterogeneous and higher intensity than brain parenchyma on FLAIR imaging, and remarkable higher intensity than brain parenchyma with some small low intensity areas on diffusion weighted imaging. He underwent the complete resection except for the cranial tissue surrounding the pons. Histologic diagnosis was juvenile pilocytic astrocytoma, because of biphasic pattern of bipolar cells and a number of Rosenthal fibers. Generally JPA has sharp and smooth demarcated cysts with well-enhanced mural nodule. It was difficult to diagnose the presented tumor as JPA before operation, since its unusual neuroimaging resembled both dermoid and high grade gliomas.     

Co‐existent pilocytic astrocytoma with acute B‐cell leukemia within the cerebellum

Pilocytic astrocytomas are the commonest childhood brain tumor. They are typically benign and usually are solitary neoplasms. To our knowledge, only one report of a pilocytic astrocytoma with leukemia has been previously issued. We herein describe the first case with documented histopathology of a 2‐year‐old boy who had a cerebellar pilocytic astrocytoma co‐localized with an acute B‐lymphoblastic leukemia. We speculate that chemotactic migration of leukemic cells to the pilocytic astrocytoma may be partly mediated through vascular endothelial growth factor (VEGF) and VEGF receptors.     

A biphasic tumor consisting of pilocytic astrocytoma with an anaplastic solitary fibrous tumor component in the pineal region: A case report and literature review

Here we report a case of a biphasic tumor consisting of pilocytic astrocytoma with anaplastic solitary fibrous tumor component in the pineal region. The majority of the tumor showed typical histologic features of pilocytic astrocytoma. A minor part of the tumor showed marked proliferation of short spindle cells around vessels. These spindle cells showed CD34 and CD99 immunoreactivity. From a review of the literature, we found that only one similar case has been reported. Contrary to the reported case, our case showed anaplastic features of solitary fibrous tumor histology.     

Cerebellar pilocytic astrocytoma presenting with intratumor bleeding,subarachnoid hemorrhage,and subdural hematoma

Introduction  Massive intracranial hemorrhage is a very rare initial presentation of cerebellar pilocytic astrocytomas. There are no reports in the medical literature on a cerebellar pilocytic astrocytoma presenting with intratumor bleeding (ITB), subarachnoid hemorrhage (SAH), and subdural hematoma (SDH). Case report  A 15-month-old boy presented with lethargy and nausea to our hospital. Magnetic resonance imaging showed a mass with ITB at the left cerebellar hemisphere in addition to SDH in the posterior fossa and SAH at the interpeduncular cistern. The patient underwent emergency surgery. On incising the dura, we found SDH, the tumor was visible at the cerebellar cortex, and near total removal followed. Microscopic examination of tissue sections revealed a pilocytic astrocytoma. Discussion  The authors’ case is the first report with a presentation including ITB, SAH, and SDH. The presumed mechanism of the SAH and SDH was leaking of the ITB into subarachnoid and subdural spaces.     

Spinal metastasis of pilocytic astrocytoma of the chiasma opticum

We report a case of spinal seeding of a pilocytic astrocytoma of the chiasma opticum. Microscopic examination of the optic nerve tumor and the spinal tumor showed the same pathological patterns, without any signs of anaplastic transformation. The clinical course demonstrates the low growth rate of this kind of tumor, but spinal metastasis has not yet been described. We discuss different pathophysiological explanations of this atypical biological behavior with reference to the literature.Presented at the XIth Meeting of the European Society for Paediatric Neurosurgery, Naples, 1988     

Epilepsy and neuropsychologic deficit in a child with cerebellar astrocytoma

We report the case of a 32-month-old female patient presenting with cerebellar pilocytic astrocytoma with epileptic seizures, psychomotor delay, and severe language delay. Usually, the typical onset of cerebellar tumor is characterized by raised intracranial pressure and cerebellar incoordination. A review of the few cases reported in the literature evidencing epileptic seizures symptomatic of a focal, nondegenerative mass limited to the cerebellum is included. Moreover, a discussion about the cerebellar contribution to nonmotor functions in children is presented, in particular following tumor resection.     

脑毛细胞型星形细胞瘤的MRI诊断特点(附32例报告)

目的探讨脑毛细胞型星形细胞瘤的MRI表现,以提高诊断正确率。方法回顾性分析32例经手术病理证实的毛细胞型星形细胞瘤的临床及MRI特点。结果32例中,71.9%发生于20岁以下青少年人,其中小脑蚓部10例(31.3%),小脑半球9例(28.1%),鞍区7例(21.9%),基底节区和脑干各2例(6.3%),第三脑室内1例(3.1%),右侧颞叶1例(3.1%)。MRI上分四型:单囊型14例(43.8%)、多囊型12例(37.5%);实性型5例(15.6%);完全囊变型1例(3.1%)。肿瘤实性部分平扫T1WI为等或稍低信号,T2WI为等或稍高信号;囊性部分T2WI信号多比脑脊液信号高。增强扫描实性部分明显强化,呈蜂窝状。灶周无水肿28例(87.5%);4例灶周轻微水肿。结论毛细胞型星形细胞瘤的MRI表现有一定的特征性,结合患者的年龄及临床表现可以提高术前诊断率。     

Juvenile pilocytic astrocytoma 'pilomyxoid variant' with spinal metastases.

We report a case of juvenile pilocytic astrocytoma of the hypothalamic/chiasmatic region with cerebrospinal fluid dissemination in a 16-month old girl. The tumour in this case had unusual histological features including the abundance of myxoid background, the absence of Rosenthal fibres and the presence of an angiocentric pattern. These features are consistent with the recently described "variant" named pilomyxoid astrocytoma. It remains unclear whether pilomyxoid astrocytoma represents an aggressive variant of classical juvenile pilocytic astrocytoma, or an entirely distinct clinico-pathological entity. Larger series and new molecular techniques may answer this question in the future.     

Cortical ependymoma or monomorphous angiocentric glioma?

Ependymoma is the third most common childhood intracranial tumor after medulloblastoma and pilocytic astrocytoma. Most ependymomas occur in the posterior fossa and spinal cord but only five cases confined to the cerebral cortex have been reported. The current case is a 5‐year‐old boy with a somewhat ill‐defined cortical tumor diagnosed as pilocytic astrocytoma on biopsy, and treated with radiotherapy. Nine years later, resection of the essentially unaltered tumor was performed for treatment of intractable seizures. Histologically, the tumor had some areas with the typical appearance of ependymoma as well other areas which contained piloid cells. There was also evidence of focal infiltrative growth. These findings bore resemblance to a recently described entity monomorphous angiocentric glioma/angiocentric neuroepithelial tumor, which combines features of ependymoma with pilocytic and diffuse astrocytomas. Both cortical ependymomas and angiocentric monomorphous glioma/angiocentric neuroepithelial tumor appear to be low‐grade tumors although their rarity makes accurate prognosis problematic. The current case has features of both entities, suggesting they may be closely related.     

Cerebellar Pilocytic Astrocytomas with Spontaneous Intratumoral Hemorrhage in Adult

Cerebellar pilocytic astrocytomas (PAs) are benign gliomas predominantly found in the pediatric population. Intracranial hemorrhages are extremely rare in initial presentations of cerebellar PAs. There are no reports in the medical literature of adult cerebellar PA cases presenting with intratumoral hemorrhage. We report 2 cases of adult cerebellar pilocytic astrocytomas with intratumoral hemorrhage. The first case is a 37-year-old woman presenting with severe headache, nausea, and vomitting. Computed tomography demonstrated an acute hemorrhage adjacent to the right cerebellar hemisphere and hydrocephalus. Magnetic resonance imaging (MRI) revealed a cerebellar vermian tumor with the hemorrhage as a mixed isointense area in the T2-weighted image, and as a mixed hyperintense area in the contrast-enhanced T1-weighted image. The second case is a 53-year-old man presenting with headache for 3 weeks. MRI revealed a cerebellar hemispheric tumor with the hemorrhage as a mixed hyperintense area. It had a cystic mass with a heterogeneous enhanced mural nodule in the gadolinium-enhanced T1-weighted image and a fluid-fluid level within the cyst in the T2-weighted image. Both of them underwent radical resections of their respective lesions. Histological examination of the specimens revealed typical astrocytoma, including a hemorrhagic portion. Both patients recovered postoperatively and continue to do well at present. The medical literature on hemorrhagic cerebellar PAs is also reviewed.     

Pilocytic astrocytoma of neurohypophysis

A case of pilocytic astrocytoma of neurohypophysis is presented. The clinical, pathological and MRI features of a rare tumor of the neurohypophysis are described. A 5‐year‐old girl presented with a 3 month history of lethargy, im‐balance and visual disturbances. A MRI revealed a large suprasellar mass. Histopathological examination demonstrated a pilocytic astrocytoma. Its astrocytic nature was confirmed by positive immunostaining for GFAP and the findings of an electron microscopy.     

毛细胞型星形细胞瘤的诊断与治疗

目的分析毛细胞型星形细胞瘤的临床特点及诊治方法。方法回顾性分析解放军总医院1997年1月～2007年1月手术治疗的37例毛细胞型星形细胞瘤患者的临床资料。结果病理显示典型毛细胞型星形细胞瘤30例（81.2%）,黏液型毛细胞型星形细胞瘤7例（18.8%）;28例肿瘤全切,31例平均随访45个月。结论毛细胞型星形细胞瘤的诊断主要依赖MRI检查,显微手术全切肿瘤是本病的主要治疗方法,应在保留功能的前提下尽量切除肿瘤,术后放化疗存在争议,复发的肿瘤可再次手术;应加强对黏液型毛细胞型星形细胞瘤的认识。     

Congenital anaplastic astrocytoma differentiated into pilocytic astrocytoma: An autopsy case

We report an autopsy case of congenital astrocytoma and its histopathological changes during 5 years of the patient's development from birth to death. At birth, a right exophthalmic tumor was observed, and MRI revealed that the tumor occupied the right orbital space and had also affected the suprasellar diencephalic structures. The right orbital tumor, which was enucleated at 2 months of age, was a highly cellular tumor with moderate pleomorphism resembling anaplastic astrocytoma. On the other hand, at autopsy, a brain tumor was found in the right diencephalic region with features of pilocytic astrocytoma, accompanied by leptomeningeal dissemination. A biopsy specimen, which was obtained from the chiasmatic part of the tumor at 4 months of age, showed an intermediate appearance between the orbital tumor and the brain tumor obtained at autopsy. Immunohistochemical examination confirmed that all three phases of the tumors showed an astrocytic lineage, and the Ki‐67 labeling index decreased rapidly after 2 months of age. We believe that this congenital anaplastic astrocytoma differentiated into a pilocytic astrocytoma during the 5 years of the patient's development. The transformation of the congenital astrocytoma from anaplastic to pilocytic forms can be attributed to the nature of the tumor, namely postmitotic neoplastic cells are characterized by their ability to undergo self‐differentiation, along with the organotropism of the developing brain.     

Rosette‐forming glioneuronal tumor of the fourth ventricle with advanced microvascular proliferation – a case report

Rosette‐forming glioneuronal tumor (RGNT) of the fourth ventricle is a recently described novel type of primary brain tumor that was included into the current WHO classification of CNS tumors. It is a very rare, slowly growing, mixed neoplasm at cerebellar localization with distinctive morphological pattern. We present an unusual case of a 20‐year‐old patient with RNGT of the fourth ventricle with advanced microvascular proliferation. MRI revealed the solid‐cystic tumor mass largely involving the cerebellar vermis and left hemisphere with compression of the fourth ventricle. Microscopically, the tumor showed classical architectural pattern with two distinctive components. The main component consisted of neurocytic rosettes formed by round, isomorphic nuclei arranged around eosinophilic, fibrillar cores with strong synaptophysin expression. The perivascular rosettes with cell arrangement along blood vessels were observed only sporadically. The second neoplastic component consisted of spindle or stellate astroglial cells with piloid process and Rosenthal fibers, strongly resembling pilocytic astrocytoma. Focally, the astroglial cells showed increased cellularity but without marked nuclear atypia. The glial part of the tumor revealed advanced proliferation of microvessels. The vessels of glomeruloid type exhibited multilayered endothelial proliferation and marked mitotic activity. MIB1 labelling index was generally low; however, in areas exhibiting microvascular proliferation its expression was significantly increased up to 20%. This report demonstrates the unique case of RGNT with conspicuous microvascular proliferation of glomeruloid type and extensive endothelial proliferation. As there is still limited clinical experience with RGNT, further studies are necessary to evaluate the biology of this type of tumor.     

毛细胞型星形细胞瘤的诊断与治疗

目的 探讨毛细胞型星形细胞瘤的临床诊断和最佳治疗方案。方法 通过头颅CT、MRI和手术探讨此类肿瘤的发病率,常见部位,临床特点,手术疗效,预后与手术切除肿瘤程度以及与术后放疗的关系。结果 毛细胞型星形细胞瘤以20岁以下发病率最高77．4％,以小脑居首92%。临床表现以颅内压增高为主、其次共济失调。影像学CT和MRI没有特征性征象,诊断率50％以上,最后确诊主要依靠病理学。预后与手术切除程度有关,肿瘤全切除术后10年内存活率达95％、部分切除术后达72．7％。结论 (1)毛细胞型星形细胞瘤多发病于青少年,以小脑居首。(2)尽可能全切除肿瘤、术后无需放疗其预后良好,可视为“良性肿瘤”;若不能伞切除的病例,术后可给予放疗亦能达到理想的预后。