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Intraventricular Pleomorphic Xanthoastrocytoma: A Case Report
Abstract and Figures
Pleomorphic xanthoastrocytoma (PXA), an uncommon glial neoplasm, typically affects adolescents and young adults and accounts for less than 1% of all astrocytic neoplasms. The authors present a case of PXA located entirely in the third ventricle in a 24-year-old male patient. The patient presented with a 6-month history of headaches, progressive decline in cognitive function and profound behavioral disturbances. He was admitted to the hospital with signs of increased intracranial pressure. Magnetic resonance imaging showed a well-enhanced solid tumor, located entirely inside the third ventricle. The tumor was totally removed via a right fronto-pterional trans-lamina terminalis approach and neuropathology report confirmed PXA diagnosis. No further treatment was indicated. To the best of our knowledge, this is the first case report of a solid PXA confined to the third ventricle.
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... Rare cases of PXA have been reported to have occurred sporadically in the suprasellar region, pineal region, cerebellum, and spine [4,[6][7][8]. In a few publications, this rare tumor has been described in rare intraventricular and periventricular locations [9][10][11][12][13][14][15][16][17]. We report a case of PXA in an atypical periventricular location. ...
... The intraventricular and periventricular locations of PXA are extremely rare. To date, only nine cases of intraventricular and periventricular PXAs (grades 2 and 3) have been found described in the English literature, and only two of these cases are located in the periventricular region (Table 1) [9][10][11][12][13][14][15][16][17]. We report a rare atypical periventricular location of PXA. ...
... It is, however, difficult to conclude whether there is an age or gender predilection based on the few PXA cases described in the literature in these rare locations. Similar to our case, five of the reported cases were grade 2 [10,[12][13][14]17], while four were grade 3 PXAs [9,11,15,16]. An association with neurofibromatosis 1 was reported with one case of periventricular PXA grade 2 [12]. ...
Background
Pleomorphic xanthoastrocytoma is a rare astrocytic tumor often diagnosed at a young age. Typically, they appear as supratentorial cortical tumors, frequently involving the temporal lobe with few reported rare locations. The prognosis is favorable following surgical excision; however, recurrence, dissemination, and anaplastic transformation occurred in some cases.
Case presentation
A 50-year-old female presented with convulsions and an altered consciousness. Imaging showed a periventricular mixed solid and cystic lesion. Histopathological examination revealed features of pleomorphic xanthoastrocytoma WHO grade 2 without necrosis or mitotic activity.
Conclusions
This report highlights the classic imaging findings of pleomorphic xanthoastrocytoma but in an atypical periventricular location. Although rare, pleomorphic xanthoastrocytoma should be considered in the differential diagnosis of mixed solid and cystic periventricular lesions.
... Typically, PXAs usually affected children as well as young adults, and were predominantly located superficially in the cerebral hemispheres [3]. Very rarely, PXA could arise in other locations including sella [4], spinal cord [5], retina [6] and ventricle [7][8][9][10][11]. To our knowledge, so far there was only two cases of PXA occurring in lateral ventricle was reported in the English literature [7,9]. ...
... In astrocytic tumors, PXA is relatively rare, which most commonly involved the superficial cerebral hemispheres of children or young adults. Rarely, PXA could involve the sites including sella [4], spinal cord [5], retina [6] and ventricle [7][8][9][10][11]. To our knowledge, so far there were only two cases of PXA occurring in lateral ventricle reported in English literature [7,9]. ...
... Immunohistochemically, PXA showed consistent reactivity for S-100, GFAP and oligo-2, suggesting its nature of astrocytes [7][8][9][10][11]. However, PXA occasionally showed neuronal differentiation and expressed neuronal markers including synaptophysin and neurofilament [5]. ...
Pleomorphic xanthoastrocytoma (PXA) is a relatively rare, low grade astrocytic tumor that usually affects children as well as young adults. The reported cases were predominantly located superficially in the temporal lobe. To our knowledge, so far only two cases of PXA occurring in lateral ventricle were reported in English literature. Herein, we present the third case of PXA intra-lateral ventricle in a 28-year-old Chinese male. Histologically, the tumor was relatively well circumscribed and consisted of spindle-shaped, ovoid, and multinuclear giant cells admixed with scattered eosinophilic granular bodies, inflammatory cells, and xanthomatous cells. Immunohistochemically, the tumor cells were strongly positive for S-100, GFAP, oligo-2 and vimentin, focally positive for synaptophysin and CD34, and negative for cytokeratin, EMA, NeuN and IDH1. Ki-67 proliferation index was approximately 2%. A BRAF V600E mutation was then identified in the tumor. Based on morphologic features, the immunohistochemical staining and BRAF V600E mutation, the tumor was diagnosed as a PXA. Because of the presence of the bizarre multinuclear giant cells and xanthomatous cells and the unusual location, PXA was easily misdiagnosed as a high-grade tumor. It should be noted that PXA was also an important differential diagnosis for intraventricular tumors.
... [10][11][12][13][14][15][16][17][18][19]. ...
We present a unique case of a 45-year-old male with cerebral palsy, who experienced walking difficulties and altered consciousness. The initial MRI revealed an intraventricular mass that rapidly enlarged over a month, consisting of two distinct components with different characteristics on CT and MRI, and was associated with agenesis of the corpus callosum. Despite initial treatment, surgical intervention was necessary, where preoperative imaging suggested an exophytically growing glioblastoma. However, postsurgical pathological examination identified the mass as pleomorphic xanthoastrocytoma (PXA), World Health Organization (WHO) Classification of Tumours of the Central Nervous System (CNS) grade 3. This study is notable for its rarity and complexity, challenging standard diagnostic approaches.
PXA is an uncommon astrocytic tumor, and its occurrence intraventricularly is extremely rare. This study highlights its unique imaging features and the critical role of MRI in preoperative assessment, underlining the tumor's unusual intraventricular location, and its relationship with corpus callosum agenesis. Our comprehensive review of PXA's history and imaging spectrum offers valuable insights for neuroradiologists and neurosurgeons, emphasizing the diagnostic challenges of such rare tumor locations and the importance of meticulous MRI analysis for accurate diagnosis.
... Two patients had leptomeningeal dissemination at diagnosis. Median MIB-1 labeling index was found to be 2 [Range: [1][2][3][4][5][6][7][8][9][10]. BRAF mutational analyses were performed for 14 cases, and mutations were identified in 10/14 (71.4%.) ...
Pleomorphic Xanthoastrocytoma [PXA] is a rare low grade glial tumor commonly affecting young adults. We did this systematic review and meta-analysis to identify prognostic factors and optimal treatment in these patients. A thorough search of the PubMed, Google scholar was made to find all possible publications related to grade II PXA. A total of 167 patients from 89 articles were included in the analysis. Median age of the entire cohort was 20 years. Headache was the most common presentation in 49.1% of the patients followed by seizure in 27.9%. Temporal lobe was the most common location of the tumor. 63% patents underwent a gross total resection [GTR] and 26.7% underwent a sub total excision [STR]. Adjuvant radiation was given to 17.6% of patients. Median follow-up for the entire cohort was 33 months. Estimated median overall survival [OS] for the entire cohort was 209.0 months [96% CI: 149.7-268.3]. Estimated median progression free survival [PFS] was 48 months [95% CI: 31.9-64.0]. In univariate and multivariate analysis younger patients and patients who underwent a GTR had a significantly better survival outcome. Use of adjuvant therapy was not found to be a significant factor affecting PFS or OS. Radiotherapy was used in salvage treatment in 76.1% of the patients. Younger patients and patients who undergo a GTR, have better survival outcomes. There is inadequate evidence to recommend routine adjuvant radiation or chemotherapy in all patients with grade II PXA.
... It usually develops in the superficial cortex, especially in the temporal lobes, and meningeal involvement is common [2,3]. Uncommon sites of PXA include the cerebellum [4,5], ventricle [6,7], spinal cord [8,9], sella [10], retina [9,11] and pineal gland [11][12][13][14]. Patients usually present with a prolonged history of seizure. ...
Background
Pleomorphic xanthoastrocytoma is rare, accounting for <1 % of all central nervous system (CNS) neoplasms. Angiomatous pleomorphic xanthoastrocytoma is an extremely rare variant of pleomorphic xanthoastrocytoma, with only six cases reported thus far. Case presentationA 24-year-old Chinese female patient who presented with seizure and loss of consciousness for 15 min underwent computed tomography and magnetic resonance imaging, which revealed a mass in the left parietal lobe. Histologically, the tumor was characterized by pleomorphic tumor cells and prominent vascularity. The angiomatous region varied, ranging from a sinusoidal pattern to a venous malformation. Focal fibrinoid necrosis, hyalinization, and a moderate infiltration by lymphocytes and plasma cells were visible in the vessel wall. The tumor cells were in close proximity with adjacent small vessels. Capillaries adjacent to or extending between tumor cells were focally observed. Most tumor cells were positive for glial fibrillary acidic protein and oligodendrocyte lineage transcription factor 2. The Ki-67 index was low. Based on the patient’s history, clinical data, and pathological findings, she was diagnosed with angiomatous pleomorphic xanthoastrocytoma (WHO grade II). Conclusions
This case serves as a reminder to pathologists of the need to be aware of this rare variant of pleomorphic xanthoastrocytoma to avoid a misdiagnosis of this indolent CNS tumor and therefore inappropriate treatment.
Objectives Xanthoastrocytoma (XA) is a low-grade glial tumor seen in young adults and there is lack of robust data on treatment of this rare tumor. In this systematic review and individual patient's data analysis, we aimed to look into the demography, pattern of care, survival outcomes, and prognostic factors in patients with both Grade II and III XA.
Methods A comprehensive search was conducted with the Medical Subject Heading terms: “Xanthoastrocytoma; Pleomorphic Xanthoastrocytoma; Anaplastic Xanthoastrocytoma; Xanthoastrocytoma AND treatment; and Anaplastic Xanthoastrocytoma AND survival” to find all possible publications.
Results A total of 325 individual patients from a total of 138 publications pertaining to XA were retrieved. Median age of the entire cohort was 19 years. About 56.1% of the patients underwent a gross total resection (GTR) and 31.4% underwent a subtotal resection. Nearly, 76.6% of the patients had a Grade II tumor and adjuvant radiation was delivered in 27.4% of the patients. Estimated 2- and 5-year progression-free survival (PFS) were 68.5 and 51.2%, respectively. Age, grade, and extent of surgery were significant factors affecting PFS. Estimated 2- and 5-year overall survival (OS) was 88.8 and 78%, respectively. The median OS for Grade II and Grade III tumors were 209 and 49 months, respectively. Age and extent of surgery were significant factors affecting OS.
Conclusion XA is a disease of young adults with favorable prognosis. Younger patients (<20 years), patients who undergo a GTR, and patients with a lower grade tumor have a better treatment outcome.
Background:
Pleomorphic xanthoastrocytomas (PXAs) are a rare type of astrocytoma, which, similar to other gliomas, can rarely arise from glial nests in the meninges, manifesting as an extra-axial mass. We describe a solitary extra-axial intracranial primary meningeal PXA in the pediatric age group, which was masquerading as a tentorial meningioma.
Case description:
A 9-year-old girl presented with features of raised intracranial pressure. Imaging revealed a dural-based mass in the tentorial region suggestive of a meningioma. This suspicion was further strengthened by intraoperative visualization of an extra-axial tumor with wide tentorial attachment. Near-total excision was achieved. Histopathologic examination established the diagnosis of PXA. Given the tumor's apparent meningeal origin and lack of connection with brain parenchyma in imaging and intraoperative findings, primary meningeal PXA was diagnosed. The absence of coexisting tumor foci on spinal magnetic resonance imaging further refined the diagnosis as solitary extra-axial intracranial primary meningeal PXA. The patient received radiotherapy for the residual tumor and was doing well at 6 months after presentation; however, she was lost to follow-up after that.
Conclusions:
Solitary extra-axial intracranial primary meningeal PXA is an extremely rare entity with only 3 reported cases in the literature including the present case. This is the first report of such a tumor in a pediatric patient. This report also highlights that primary meningeal PXA can manifest as an extra-axial mass lesion and may warrant inclusion in the differential diagnosis of extra-axial mass lesions.
To describe the clinical features, histologic characteristics, and management of patients with pleomorphic xanthoastrocytoma (PXA), we reviewed data on 13 children who had histologically confirmed PXA and were referred to the neuro-oncology service between 1985 and 1999. Neuro-imaging with CT and/or MRI documented the anatomic location, tumor extent, and degree of resection. There were 3 males and 10 females; median age was 12.9 years (range, 8.2-17.2 years). The most frequent presentations included seizures (n = 8) and headache (n = 5). Tumor sites included temporal (n = 5), parietal (n = 3), frontal (n = 1), frontoparietal (n = 1), parieto-occipital (n = 1), and temporoparietal (n = 1) lobes and the spinal cord (n = 1). CT/MRI revealed a cystic component in 6 patients, with cyst wall enhancement in 3 patients. The solid component was uniformly enhancing in 11 patients. Vasogenic edema was present in 9 patients, and calcification was noted in 4 patients. Histopathologic findings included meningeal invasion in 12 patients, calcifications in 4, and necrosis in 2. Mitotic figures (1-12 per high-power field) were seen in 8 patients. Gross total resection was achieved in 8 patients, near total resection in 1, and subtotal resection in 4. Ten patients were alive with a median follow-up of 41 months at this writing. Two patients died of progressive disease, and 1 died of an unrelated cause. In conclusion, pleomorphic xanthoastrocytoma is a rare neoplasm in childhood, commonly presenting with seizures. Gross total resection without adjuvant therapy provides prolonged disease control, as seen in 6 of 7 patients (85%) in our series.
Pleomorphic xanthoastrocytomas (PXA) with malignant transformation are reported in two adult men with a long history of seizures, recent onset of neurological symptoms and superficially located, temporal lobe lesions. Although PXA is generally described as having relatively benign behaviour, this report adds two further cases of malignant transformation to the literature.
In 1979, researchers described a series of young patients with clinically and histologically distinctive supratentorial gliomas which were designated pleomorphic (meningocerebral) xanthoastrocytomas (PXA). Significantly, patients with these neoplasms were reported to have a relatively favorable prognosis. The authors present a new case of PXA in a 32-year-old man. This case is unique for two reasons: (1) a relatively rapid fatal outcome with death 21 months after diagnosis; and (2) the presence, at autopsy, of extensive recurrent tumor with features of a malignant astrocytoma. Detailed electron microscopic and immunohistochemical studies, supporting the proposed subpial astrocytic origin of PXA, are presented. Literature pertaining to PXA is reviewed. This report illustrates the unique features of PXA and demonstrates its potential for aggressive behavior. Cancer 52:2055-2063, 1983.
This review deals with one of the newly adopted entities in the second edition of the histologic typing of CNS tumours by the World Health Organization: pleomorphic xanthoastrocytoma and reviews the clinical features, gross and microscopic characteristics as well as the common and some of the unusual variants of this tumor. The steps and events leading to the recognition of the basic character of this neoplasm and its designation as an independent entity are recollected and a few characteristic MRI scans and photomicrographs of pleomorphic xanthoastrocytomas are provided.
Twelve cases of a distinctive form of supratentorial astrocytoma occurring in young subjects (ages 7 to 25) are reported. The tumors were superficial and involved the leptomeninges extensively. The tumor cells display marked pleomorphism, including bizarre giant cells and a number of mitotic figures, but no necrosis. Many contain large amounts of lipid in their cytoplasm and are surrounded by reticulin fibers, thus simulating a mesenchymal tumor. For these reasons, some examples of this tumor have been previously interpreted to represent meningocerebral fibrous xanthomas. Immunoperoxidase technique performed in nine of the twelve cases has, however, established the presence of glial fibrillary acidic protein in the tumor cells, which are therefore considered to be astrocytic. By electron microscopy many tumor cells are surrounded by basal laminae, accounting for the abundant reticulin network demonstrable in silver preparations. Since subpial astrocytes are known to be partly covered by a basal lamina, it is likely that they are the cells of origin for this neoplasm. In contrast to its pleomorphic cytology, the biological behavior of this tumor appears to be relatively favorable, and long survival times (up to 25 years) have been recorded in some cases. (These tumors are distinct from intracranial fibrous xanthomas of mesenchymal derivation. Cells of the latter are negative on GFAP stain.)
The role of histologic features and of tumor grade in predicting biological behavior has received less attention, although increased mitotic activity and necrosis have been associated with increased aggressiveness.62 For that reason, we studied a large group of patients with PXA and undertook an analysis of previously published cases in which adequate information regarding morphology and outcome had been provided. From the analysis of the data, it became apparent that these two patient populations, one mainly representing the combination of two large North American consult practices and the other a compilation of case reports and small series from the international neuropathology community, were remarkably similar in their characteristics and closely mirrored the original experience of Kepes et al.2 Numerous similarities, including age distribution, with a high incidence in children and young adults, equal frequency of occurrence in both males and females, seizures as the most common presenting sign, predilection for the temporal lobe, and a high frequency of cystic lesions, all contribute to making these two groups of patients comparable. Furthermore, frequencies of GTR as well as of radiation therapy were quite similar. Thus, it was not surprising that both recurrence free and overall survival curves were essentially superimposable. Our study confirmed that PXA, with its 70% 10-year survival rate, behaves significantly better than those lesions with which it was and continues to be mistaken. Nonetheless, unlike other astrocytic tumors of favorable prognostic type e.g., pilocytic astrocytoma and subependymal giant cell astrocytoma, PXA is associated with a higher frequency of recurrence, anaplastic transformation, and death.
Pleomorphic xanthoastrocytoma (PXA) is a rare benign tumor that is usually located in the superficial cerebral hemisphere. Most reports of PXAs have included only a single case or small series. Therefore, the data with respect to the natural history of this tumor are fragmentary. We report a case of a PXA in the unusual location of the right lateral ventricle with extensive subarachnoid dissemination. To our knowledge, this is a rare case of PXA in the lateral ventricle. In addition, extensive subarachnoid space dissemination of this distinctly benign type of glioma is exceedingly rare. In our case, there was meningeal dissemination and metastases to the bilateral trigeminal nerves and oculomotor nerves. The neuroradiographic features, tumor location, and dissemination were reviewed.
Pleomorphic xanthoastrocytoma (PXA) is a rare astrocytic tumor that usually occurs in the superficial cerebral hemispheres of children and young adults and has a relatively favorable prognosis. We report an unusual case of supratentorial, intraventricular tumor in a 52-year-old man. The tumor was composed of pleomorphic cells, including giant cells, most of which were multinucleated, and small cells. In addition, frequent xanthic changes in the cytoplasm of the tumor cells, and widespread reticulin deposits and lymphocytic infiltrates in the stroma were characteristic features. Large areas of necrosis were also evident. However, mitotic figures were rare (1-2 mitoses per 10 high-power fields). Many tumor cells were positive for GFAP, and a number were positive for neurofilament protein and synaptophysin, indicating their neuronal differentiation. In addition, occasional tumor cells were positive for CD34. p53 protein was entirely negative in the tumor cells. In diagnosing this tumor histopathologically, differentiation between PXA and giant cell glioblastoma (GCG), a rare variant of glioblastoma, was problematic. However, considering the overall histopathological picture, a final diagnosis of PXA with anaplastic features was made. The present case indicates that PXA can occur as an intraventricular tumor, and suggests that in some instances, it would be very difficult to differentiate PXA and GCG histopathologically.