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.     

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.     

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.     

Astrocytic differentiation in medulloblastoma.

A case of cerebellar medulloblastoma with transitional features towards malignant fibrillary astrocytoma is described. In the cerebellum the tumour is characterised by extensive subpial infiltration with cells of undifferentiated type, and the astrocytic component could only be identified by a positive glial fibrillary acidic protein reaction. In the brainstem the character of the growth transforms to that of diffuse astrocytoma. This demonstrates the potential for differentiation existing in a "primitive" neuroepithelial neoplasm.     

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。     

Astrocytomas of the cerebellum in children: treatment and results]

Between 1980 and 1987, 113 children with astrocytoma of the cerebellum were treated. The range of the age was 11 months to 15 years. 103 children were treated by surgery only and 10 received surgery and postoperative irradiation. Shunting was necessary in 22% patient with cerebellar astrocytoma. One-year survival for patients with cerebellar astrocytoma was 97%, five-year 89%.     

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.     

Radiologically typical pilocytic astrocytoma with histopathological signs of atypia

Pilocytic astrocytoma (PCA) is the most common CNS tumor in primary school-aged children. Herein, we report the case of a 7-month-old female child with a large cerebellar hypodense tumor on computer tomography occupying nearly the whole cerebellar hemisphere. Magnetic resonance imaging revealed characteristic features of a PCA, depicting a mass with hyperintense solid and cystic areas on T2-weighted images and with marked solid and ring-like enhancement pattern. Histopathological and immunohistochemical analysis showed partially typical features of a PCA, but also atypical features such as a high proliferation rate, hypercellularity, and focally diffuse infiltration pattern were present. However, definite signs of transformation to an anaplastic PCA were not observed. We therefore classified the tumor as pilocytic astrocytoma with signs of atypia instead of simple WHO grade I pilocytic astrocytoma. The case illustrates that neuroimaging features may give very important clues for the definite diagnosis in histopathologically atypical PCA.     

Intracranial Metastases of Cervical Intramedullary Low-Grade Astrocytoma without Malignant Transformation in Adult

The first case of intracranial metastases of a cervical intramedullary low-grade astrocytoma without malignant transformation in adult is presented in this report. Seven years ago, a 45 year-old male patient underwent biopsy to confirm pathologic characteristics and received craniocervical radiation and chemotherapy for a grade II astrocytoma in the cervical spinal cord. Two years later, posterior fusion was necessary for progressive kyphosis in the cervical spine. He was well for approximately 7 years after the primary surgery. Two months ago, he presented with partial weakness and incoordination with gait difficulty. MRI Scan demonstrated multiple small lesions in the cerebellar vermis and left hemisphere. After suboccipital craniectomy and posterior cervical exposure, the small masses in the cerebellar vermis and hemispheres were excised to a large extent by guidance of an intraoperative navigation system. The tumor at the cervical and brain lesions was classified as an astrocytoma (WHO grade II). When a patient with low-grade astrocytoma in the spinal cord has new cranial symptoms after surgery, radiaton, and chemotherapy, the possibility of its metastasis should be suspected because it can spread to the intracranial cavity even without malignant transformation as shown in this case.     

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.     

Lipoastrocytoma: Case report and review of the literature

Lipoastrocytoma is an extremely rare tumor, with only six cases described. We report the case of an astrocytoma involving the upper part of the cerebellar‐pontine angle and the right portion of the clivus starting from the brainstem with a diffuse lipomatous component in a 39 year‐old man. The patient was admitted with headache of 1 year's duration and diplopia over the previous 3 months. MRI revealed a ponto‐cerebellar lesion that showed irregular enhancement after contrast administration. Subtotal excision of the tumor was accomplished. Adjuvant chemotherapy and radiation therapy were not administered. Histologically the tumor showed the classical histology of low‐grade astrocytoma and a portion of the lesion was composed of lipid‐laden cells. Immunohistochemistry for glial fibrillary acid and S‐100 proteins clearly demonstrated the glial nature of these cells. Ki‐67/Mib‐1 labeling index was low (2%). The patient remains in good neurological conditions after 10 months. Our case has a benign postoperative behavior, also after subtotal excision, with restrictions due to the short follow‐up. It is important to record each new case of this rare tumor to produce a better characterization of this lesion.     

A case of an epithelioid glioblastoma with the BRAF V600E mutation colocalized with BRAF intact low‐grade diffuse astrocytoma

Epithelioid glioblastomas are one of the rarest histological variants of glioblastomas, which are not formally recognized by the World Health Organization (WHO) classification. Epithelioid glioblastomas usually occur as primary lesions, but there have been several reports of secondary epithelioid glioblastomas or epithelioid glioblastomas with pre‐ or co‐existing lesions to date. The serine/threonine‐protein kinase B‐Raf (BRAF) V600E mutation has been found at a high frequency of 54% in epithelioid glioblastomas. We present a case of a 26‐year‐old female patient with an epithelioid glioblastoma with the BRAF V600E mutation in her right frontal lobe. In the present case, a low‐grade diffuse astrocytoma component had colocalized with the epithelioid glioblastoma. The component presented prominent calcification on neuroimages as well as by histology, and low‐grade diffuse astrocytoma was considered to be a precursor lesion of an epithelioid glioblastoma. However, the BRAF V600E mutation was detected only in epithelioid glioblastoma but not in low‐grade diffuse astrocytoma. To the best of our knowledge, this is the first report demonstrating a discrepancy in the BRAF V600E mutation states between epithelioid glioblastoma and colocalized low‐grade astrocytoma.     

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.     

Late growth of supratentorial meningioma after radiotherapy of cerebellar tumor

The authors report a case of supratentorial meningioma of the brain developing after radiotherapy for cerebellar astrocytoma. On the basis of a survey of the literature and own speculations the authors try to demonstrate a cause-and-effect relationship between radiotherapy and meningioma development.     

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.     

Heterologous transplantation of cerebral and cerebellar astrocytomas

Summary Twenty two cases of cerebral and so-called cerebellar astrocytomas were heterologously transplanted to guinea pigs. Although the fifteen cerebral astrocytomas were selected according to optimal conditions for transplantation, all failed to grow heterologously. The cerebral astrocytomas represent varieties of fibrillary, protoplasmic and pilocytic types, and all originated in adults. Of the seven randomly selected so-called cerebellar astrocytomas, all of which occurred in children, only one grew when transplanted intracerebrally to guinea pigs. This tumor before transplantation had all the histological characteristics of a so-called cerebellar astrocytoma. The prevailing cells were elongated, spongioblast-like elements; however, other cell types, e.g., astrocytes occurred in varying numbers. The heterotransplant was dominated by elongated, mostly bipolar cells with a very characteristic cytoarchitectural arrangement. The controversy regarding classification of cerebellar astrocytomas versus cerebellar spongioblastomas was discussed. Although the name spongioblastoma is not favored, the concept of classifying the so-called cerebellar astrocytoma together with other slow growing midline gliomas as proposed by Zülch has definite merits. Heterotransplantation studies clearly demonstrate that the so-called cerebellar astrocytoma has different biological properties than cerebral astrocytomas.Supported by United States Public Health Service Research Grant No. NB-07849-04.     

Cerebellar hemangioblastoma and subependymoma: a case report of an unprecedented association

We report the case of a 71-year-old man who suffered from both a cerebellar hemangioblastoma and a subependymoma (glomerate subependymal astrocytoma) of the base of the fourth ventricle. His symptoms included episodes of loss of consciousness, cerebellar ataxia, and postural vertigo. The clinical diagnosis presented considerable difficulties. The simultaneous occurrence of both tumors in one patient has not been reported previously. After reviewing the literature, we conclude that this unusual association was a result of chance rather than common oncogenic factors.     

Intracranial bilateral symmetrical calcification on CT-scanning. A case report and a review of the literature

The case of a 57-year-old woman with idiopathic hypoparathyroidism is presented. A CT-scan showed extensive bilateral symmetrical calcification in the region of the basal ganglia, nuclei of the cerebellum and the cerebral and cerebellar white matter. A review of the literature showed that bilateral symmetrical calcification detected by CT is usually small in extent and is most often confined to the globus pallidus. It is most commonly found in patients older than 50 years, who only rarely have symptoms associated with it. The finding is, though, non-specific and may occur in a variety of pathological conditions both with and without an aetiological relationship. Further study of the cerebral parathormone responsive adenylate cyclase enzyme proves hopeful to elucidate the aetiology of idiopathic bilateral symmetrical calcification.     

Cerebellopontine calcification: a new entity of idiopathic intracranial calcification?

We report the autopsy case of a 40-year-old woman with severe intellectual and motor disabilities, who showed calcification in the cerebellum and pons but not in the basal ganglia on CT scan, and died of intracranial hemorrhage due to intractable hypertension. At autopsy, numerous calcium deposits were noted in the cerebellar cortex, the dentate nucleus, the cerebellar white matter and the ventral pons. These deposits were distributed both in the neuropil and the white matter, but rarely within the arterial walls or in contact with capillaries. This weak relationship between calcification and the blood vessels, in addition to the paucity of basal ganglia calcification, is in contrast to the findings with other disorders involving intracranial calcification, including Fahrs disease and calcium metabolism disorders. Immunohistochemistry revealed intense staining of calbindin-D28K and parvalbumin at sites of calcium deposits both in the present case and in a case of pseudohypoparathyroidism, whereas these proteins were not localized to calcium deposits in the cerebellum of a Fahrs disease brain. We propose that the present case may represent a distinct entity among diseases characterized by idiopathic intracranial calcification. In addition, calcium-binding proteins may be involved in the calcification process in some cases with intracranial calcification.     

Precentral cerebellar vein in cystic astrocytomas of the vermis.

A peculiar abnormality of the precentral cerebellar vein seen only in cystic astrocytoma of the vermis is reported. Implications of this angiographic finding are discussed. The importance of posterior fossa angiography (especially venography) in the diagnosis of posterior fossa mass lesions is emphasized.