Lipoastrocytoma: A rare case report and review of literature

Lipoastrocytoma is an extremely rare tumor, with only a few cases described. We report a case of a low‐grade astrocytoma occupying the right cortical lobe in the parafalcine location. The patient was admitted with headache, vomiting and altered sensorium for duration of 1 year. MRI revealed a large heterogeneous enhancing mass in the right fronto‐parieto‐temporal lobe with intratumoral fat along with cystic changes and calcification (correlated with CT) showing mass effect in the third ventricle. A gross total excision of the tumor was performed. Histologically, the tumor showed glial cells that contained lipid droplets coalescing into a single large droplet, similar in appeareance to adipocytes. Immunohistocemically, tumor cells strongly expressed GFAP and S‐100 protein. Ki‐67 labelling index was low. The patient remained in good neurological condition at 3 months follow‐up. This case represents a rare variant of low‐grade astrocytoma that is designated as lipoastrocytoma.     

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.     

小脑发育不良性节细胞瘤

研究背景小脑发育不良性节细胞瘤是小脑良性肿瘤,临床表现不典型,相对罕见。本文报告1例小脑发育不良性节细胞瘤患者,通过复习相关文献,探讨其临床病理学特征,以期提高诊断与鉴别诊断能力。方法与结果女性患者,34岁,头部MRI显示右侧小脑半球类圆形占位性病变。手术全切除病变,术中可见病变表面脑回增宽,呈黄白色,似脑组织样,质地略软,血供丰富,无包膜,与周围脑组织无粘连。组织学形态,颗粒细胞层和浦肯野细胞层被平行排列的异常有髓纤维和结构紊乱的异常神经元取代,伴血管畸形。免疫组织化学染色,异常神经元胞质表达神经元核抗原、突触素、S-100蛋白和神经元特异性烯醇化酶,胶质纤维背景表达胶质纤维酸性蛋白,神经纤维表达神经微丝蛋白,Ki-67抗原标记指数1%。最终病理诊断为小脑发育不良性节细胞瘤。术后仍呈深昏迷,共住院24 d,出院后失访。结论小脑发育不良性节细胞瘤临床相对罕见,组织学形态呈良性,影像学表现具有一定特异性,早期诊断并手术治疗十分必要,应注意与节细胞瘤、节细胞胶质瘤和低级别星形细胞瘤相鉴别。小脑发育不良性节细胞瘤患者及其家属应进行系统检查,关注有无其他器官或系统疾病或肿瘤。     

脂肪样星形细胞瘤简

目的探讨脂肪样星形细胞瘤的临床病理学特征、免疫表型、诊断与鉴别诊断、治疗及预后。方法与结果男性患者,48岁。临床表现为左手无名指及小指麻木、活动不利。MRI显示右顶叶占位性病变,伴囊性变,实性区域明显强化。立体定向下行右顶叶肿瘤全切除术。光学显微镜观察肿瘤呈低级别星形细胞瘤形态,散在一些胞质丰富、核仁明显的神经元样细胞;以脂肪空泡形成为特征,形似成熟的脂肪细胞,呈灶状相互融合,似微囊性变。肿瘤细胞表达胶质纤维酸性蛋白、S-100蛋白、WT-1蛋白和少突胶质细胞转录因子2,以及神经元标志物突触素、微管相关蛋白．2、神经微丝蛋白、神经元特异性烯醇化酶和CD34,不表达异柠檬酸脱氢酶1,P53蛋白呈弱阳性（5％）,Ki-67抗原标记指数约为1％。术后随访20个月,肿瘤无复发。结论脂肪样星形细胞瘤为临床少见肿瘤,组织病理学呈低级别星形细胞瘤形态,伴显著脂肪样细胞分化,亦可伴神经元样分化。明确诊断需结合病理学形态及免疫表型,同时应注意与其他伴脂肪样细胞分化或黄色瘤样变的神经上皮组织肿瘤相鉴别。     

Transformation of intracranial anaplastic astrocytoma associated with neurofibromatosis type I into gliosarcoma: Case report

Gliosarcoma is an uncommon malignant brain tumor composed of distinct sarcomatous and malignant glial cell elements. These tumors are defined as a variant of glioblastoma, and it can be developed by progression of the malignant glial cell tumors or primary tumors. We report a rare case with gliosarcomatous recurrence of anaplastic astrocytoma with neurofibromatosis type 1 (NF-1) followed by chemoradiation therapy. A 26-year-old male patient was presented with headache. Five years before admission, he had been diagnosed with NF-1. Magnetic resonance imaging (MRI) showed a well-demarcated, enhanced lesion in the right frontal lobe and multiple enhanced lesions in the scalp, lower cervical, thoracic, and upper lumbar regions. He underwent an osteoplastic craniotomy with total tumor resection. Histopathology of the tumor showed findings corresponding with anaplastic astrocytoma. He was followed by radiotherapy and chemotherapy postoperatively. A month later, his spinal lesion was also resected and confirmed pathologically as plexiform neurofibroma. The subsequent follow-up period of 27 months was uneventful until he developed a generalized tonic-clonic seizure. MRI showed tumor recurrence in the original site of the tumor. Re-exploration was carried out. Pathological examination displayed a biphasic pattern of the glial and sarcomatous components suggesting gliosarcoma.     

Congenital gemistocytic astrocytoma in a fetus

Introduction Congenital brain tumors, especially tumors diagnosed before birth, are very rare. This report presents a case of a congenital gemistocytic astrocytoma diagnosed by antenatal intrauterine ultrasound.Case report An intrauterine MRI revealed hydrocephalus and a mass lesion including massive hemorrhage in the right occipital lobe of a fetus. The patient was delivered by cesarean section and a total excision of the hemorrhagic tumor was carried out on the third day of his life. The histological study revealed gemistocytic astrocytoma (WHO grade II). Neither adjuvant chemotherapy nor radiation was given after the first surgery. Ten months after his birth, a recurrent tumor was depicted on follow-up MRI. The second total excision of the recurrent tumor and chemotherapy using cisplatin and vincristine were performed.Outcome The patient is free of disease at the age of 2 years and 6 months.     

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.     

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.     

Focal cortical dysplasia coexisting with diffuse astrocytoma in childhood: A case report and reappraisal of the glial component in archival FCD cases

We report a rare case of focal cortical dysplasia (FCD) concurring with diffuse astrocytoma and arachnoid cyst, and also re‐evaluate the glial component in archival FCD cases for the differential diagnosis of diffuse gliomas. A 7‐year‐old boy with a 9‐month history of psychomotor seizures disclosed a hyperintense area accompanied by a cystic lesion in the left temporal lobe on MRI. The surgical specimen displayed dyslamination of the cortices and ectopic neurons in the white matter, associated with dysmorphic neurons, indicating FCD type IIA. Additionally, the lesion showed diffuse proliferation and infiltration of glial cells, immunopositive for infiltrating glioma markers (nestin, doublecortin, MAP‐2e) and p53, and MIB‐1 index was 2.0%. These findings indicated coexisting diffuse astrocytoma. Coexistence of diffuse glioma with FCD is unusual, but we often notice increased population of small glial cells in FCD lesions. Re‐evaluation of archival FCD cases with diverse markers revealed that reactive microglia significantly proliferate in the white matter lesions. Therefore, a careful pathological assessment has to be made to define a rare case of diffuse glioma occurring in FCD.     

Glioblastoma occurring at the site of a previous medulloblastoma following a 5‐year remission period

We describe a case of a 14‐year‐old boy who developed a cerebellar and brainstem glioblastoma 5 years after treatment for a medulloblastoma. The patient first presented in 2003 with 9 months of vomiting and a 9‐kg weight loss. A head MRI showed a heterogeneously enhancing posterior fossa mass with hydrocephalus. Gross total resection was performed and the tumor was consistent with a classic medulloblastoma. Postoperative chemotherapy and craniospinal radiation was administered. The patient remained tumor‐free until 2008, at which time he presented with right‐sided weakness and numbness, left eye pain, vomiting and weight loss. Imaging showed abnormalities within the posterior pons, medulla, inferior cerebellar peduncles, cerebellar hemispheres and cervicomedullary junction with expansion of the medulla and cervical spinal cord. Due to the location of the lesion, biopsy was felt to be too risky and was avoided. Despite receiving chemotherapy, his symptoms continued to worsen and he died 4 months later. Post mortem examination limited to the brain and spinal cord confirmed the radiographic extent of the tumor. Microscopic examination showed a highly cellular infiltrative glial neoplasm with extensive palisading necrosis. A diagnosis of glioblastoma was rendered. The question of whether the first and second tumors were related is of potential clinical and academic interest. The first tumor was synaptophysin‐positive and GFAP‐negative, consistent with medulloblastoma. The second tumor was synaptophysin‐negative and focally GFAP‐positive, consistent with glioblastoma. The glioblastoma displayed EGF receptor amplification, and interestingly, it also displayed MYCN amplification; both tumors showed low level PTEN deletion. The medulloblastoma displayed a signal pattern consistent with an isochromosome 17q, while the glioblastoma showed some cells with an isochromosome 17q signal pattern amid a background of cells with abundant chromosomal instability. The relationship between these two tumors, particularly with regard to various molecular events, is discussed.     

Cerebellar glioblastoma genetically defined as a secondary one

We report here the case of a 29-year-old woman with cerebellar glioblastma. In the present case, tumor lesions were observed in each cerebellar hemisphere. The left-side lesion was diagnosed as glioblastoma, and the right-side lesion as malignant astrocytoma by histopathology. Immunohistochemistry revealed that the tumor cells of the left-side lesion was positive for p53, whereas epidermal growth factor receptors (EGFR) were negative in tumor cells from both sides. Genetic alterations were investigated using a genome DNA microarray (GenoSensor Array 300), which has led us to define this tumor as a secondary glioblastoma. The clinical presentation and genetic findings of this relatively rare entity are discussed.     

Cerebellar Liponeurocytoma with an Unusually Aggressive Histopathology : Case Report and Review of the Literature

We report a rare case of cerebellar liponeurocytoma with an unusually aggressive histopathology. A 49-year-old man presented with a four-month history of headache, vertigo, and progressive swaying gait. Magnetic resonance imaging showed a 3×3.5 cm sized relatively well-demarcated round mass lesion in the fourth ventricle, characterized by high signal intensity on T2-weighted images. Postcontrast images revealed strong enhancement of the solid portion and the cyst wall. The patient underwent suboccipital craniectomy and tumor removal. The pathologic diagnosis was cerebellar liponeurocytoma. Adjuvant radiotherapy was offered due to concerns related to the high proliferative index (Ki-67, 13.68%) of the tumor. At the last routine postoperative follow-up visit (12 months), the patient complained of no specific symptom and there was no evidence of tumor recurrence. However, long-term follow-up and the analysis of similar cases are necessary because of the low number of reports and the short follow-up of cases.     

Pleomorphic granular cell astrocytoma in the pineal gland: Case report

Pleomorphic granular cell astrocytoma in the pineal region is exceedingly rare, and its clinicopathological features are distinctive. A 67‐year‐old woman was admitted with a staggering gait. Magnetic resonance imaging revealed a mass lesion at the pineal gland accompanied by obstructive hydrocephalus. Following surgery, pathological examinations demonstrated a pleomorphic granular cell astrocytoma. The patient has been free from recurrence for 24 months after surgery without adjuvant therapy. The specimen exhibited nuclear and cytoplasmic pleomorphism. The nuclei varied in size, shape and coarseness. Variability was also observed in the eosinophilic granular bodies, Rosenthal fibers and spindle‐shaped tumor cells. GFAP, S‐100 and vimentin were immunohistochemically positive. Reticulin network was absent between the tumor cells, and granular cells with ballooned cytoplasm showing positive staining for PAS. Pleomorphic granular cell astrocytoma is believed to be a form of astrocytoma originating from the pineal gland. Its clinicopathological features resemble those of pleomorphic xanthoastrocytoma. However, it can be differentiated from the latter by the absence of reticulin fibers, absence of basement membrane between adjacent cells, and presence of large numbers of mitochondria.     

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.     

Cerebellar astrocytoma with extensive lipidization mimicking adipose tissue

We report the case of an elderly woman with a history of headache, vomiting and dizziness while walking. On CT scans a mass was identified in the right cerebellar hemisphere exhibiting radiological characteristics of lipomatous tissue. Surgery revealed a compact lesion consisting of whitish-yellow tissue with a fatty aspect and texture. Smears of tissue samples and paraffin sections showed features suggestive of tissue mainly composed of fully differentiated lipocytes. Lipid-specific stainings on fresh frozen material confirmed univacuolar intracytoplasmic fat accumulation. However, immunohistochemistry for glial fibrillary acidic protein and electron microscopy clearly demonstrated the glial lineage of these lipid-laden cells. Therefore, the tumor was diagnosed as a highly lipidized astrocytoma. In our view, this case represents a variant of lipidized gliomas that has not been described previously and that differs phenotypically from the entities documented earlier.     

Ependymosarcoma with eosinophilic granular cells

Ependymosarcoma is a new entity of malignant gliomas composed of ependymal and sarcomatous components. We report a rare case of ependymosarcoma with eosinophlic cells which occurred to the right trigon of the lateral ventricle. A 62‐year‐old man complained of headaches over a 2‐month period. A hard, gray mass was found in the right trigon of the lateral ventricle during the operation. Although he received radiation and chemotherapy, the patient died due to tumor disseminating through the whole brain within 7 months after the operation. The histological examination revealed that the anaplastic glial components intermingled with the sarcomatous components. Immunohistochemically, sarcomatous cells were positive for α smooth muscle actin and desmin. However, anaplastic glial cells were not positive for these markers. In addition, Masson trichrome stain showed a plethora of collagen fibers between sarcomatous cells, but no collagen fibers were produced by the glial tumor cells. Solid focal papillary lesions of the glial tumor showed dot‐like epithelial membrane antigen and diffuse cytoplasmic D2‐40 immunoreactivity. Based on the above findings, these anaplastic glial tumor cells should show focal ependymal differentiation, and sarcomatous cells show myofibroblastic differentiation. In addition, almost 10% of the tumor cells in the neoplasm showed bright eosinophilic granules in the cytoplasm. These cytoplasmic eosinophilic granules and bundles were negative on PAS staining. Intracytoplasmic eosinophilic granules of tumor cells were strongly positive for αB‐crystallin, HSP 27 and GFAP, respectively. These findings suggest that the clinicopathological characteristics of the present case should be consistent with the criterion of ependymosarcoma by Rodriguez et al.     

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.     

Desmoplastic non‐infantile ganglioglioma

Desmoplastic ganglioglioma is a rare, markedly desmoplastic variant of ganglioglioma that usually presents in the first year of life. It is a mixed glial and neuronal cerebral tumor. A few cases of desmoplastic ganglioglioma have been reported in non‐infantile patients. We report a case of desmoplastic ganglioglioma in a 14‐year‐old boy. The patient presented with a large solid cystic mass with mild peritumoral edema in the right posterior parieto‐occipital region with direct contact to the falx cerebri. Histopathological examination revealed a low‐grade glial tumor with prominent desmoplasia including hypocellular collagenous areas. The tumor was well demarcated with respect to the surrounding brain. Ganglion cells with dysplastic features were present in the tumor and clustered in some areas. Focal lymphocytic infiltration was also observed. Mitotic activity was very low. Immunohistochemistry revealed glial fibrillary acidic protein positivity in the astrocytic cells hidden in the desmoplastic tissue. Synaptophysin and neuron specific enolase were positive in ganglion‐like neuronal cells. The MIB‐1 labeling index was less than 1%. This present case confirms that desmoplastic ganglioglioma can be present in young adult patients with its characteristic radiologic features.     

Multimodal target point assessment for stereotactic biopsy in children with diffuse bithalamic astrocytomas

INTRODUCTION: Diffuse glial tumors with bithalamic involvement are rare in children. Diagnostic assessment can be difficult as the radiological findings can be unspecific. MATERIALS AND METHODS: In order to enhance the diagnostic yield metabolic imaging with MRS and PET using FET ( O-(2-[(18)F]fluoroethyl)- L-tyrosine) was performed in two children (2 and 10 years of age). Co-registered images were used for image-guided biopsy, which was planned with neuronavigation and stereotaxy simultaneously. RESULTS: Biopsies from the right thalamus were planned, but locations were changed in both cases after metabolic imaging was available. MRS (thalamic voxel) was typical for a glial tumor in one child. In the older girl FET-PET revealed an unexpected lesion in the left cerebellar hemisphere, with a tumor-to-cortex ratio of 3.8, as against 1.7 in the thalamus. Accordingly, a stereotactic biopsy specimen was taken from the left cerebellar hemisphere, and a final diagnosis of anaplastic astrocytoma was made. The other patient showed a higher uptake (tumor-to-cortex ratio 1.6) in the left dorsal thalamus, compared with bilateral homogeneous hyperintensity of the thalamus structures on MRI. Stereotactic biopsy revealed a low-grade diffuse astrocytoma. CONCLUSION: Stereotactic biopsy using metabolic imaging and image fusion can enhance the diagnostic yield in cases of diffuse pediatric gliomas disclosing unexpected 'hot spots'.     

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.