Pleomorphic xanthoastrocytoma: clinical, imaging and pathological features of four cases

A, Bucciero; De, Marialaura Del Basso; Stefano, V. De; Tedeschi, Enrico; Monticelli, Arianna; Siciliano, Alessandro; Cappabianca, Paolo; Vizioli, L; Cerillo, A

doi:10.1016/s0303-8467(96)00560-4

Cited by 39 publications

(36 citation statements)

References 31 publications

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“…It was described in 1979 by Kepes et al [2] and was added to the World Health Organizations (WHO) classification of tumors of the central nervous system in 1993 [3]. Patients with PXA typically present as children or young adults with seizures as these tumors often involve the temporal lobe [4][5][6]. Most commonly they present as WHO grade II tumors, however, anaplastic transformation can occur in up to 15-20 % of patients [4,7].…”

Section: Introductionmentioning

confidence: 99%

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Patterns of care and outcomes of patients with pleomorphic xanthoastrocytoma: a SEER analysis

et al. 2012

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To study the clinical characteristics, treatment approach and outcome of pleomorphic xanthoastrocytoma (PXA), patients were identified using the National Cancer Intitute's Surveillance, Epidemiology, and End Results (SEER) database. A total of 214 patients were identified with PXA using the November 2010 submission. Patient demographics, tumor characteristics, extent of surgical resection, the use of radiotherapy, and overall survival were evaluated. Overall survival for PXA was then compared to that of pilocytic astrocytoma, oligodendroglioma, ependymom and glioblastoma also using the SEER database. Kaplan-Meier, univariate and multivariate analyses were performed. The majority of patients were found to be young adults with the most common tumor location being temporal lobe. Surgery was performed on most (95 %) patients, while 25 % of patients received radiation therapy. Five and 10 year overall survival rates were 75 and 67 %, respectively. Grade was only available for a limited number of patients but appeared to affect prognosis. Patients with grade IV tumors had a median overall survival of 45 months, whereas median survival was not reached for grade I-III patients. On multivariate analysis, male gender and increasing age were associated with worse overall survival (p values 0.05 and <0.006, respectively). Extent of resection trended towards significance in favor of gross total resection. PXA is a rare diagnosis that affects young adults. Surgical resection is the primary modality of treatment with an overall good prognosis. Elderly patients, those with higher grade disease and patients with incomplete resections may have a worse prognosis. The role of radiation therapy for PXA remains unclear but is more often used for patients with high grade tumors. Compared to other common brain tumors, PXA's appear to fare worse than pilocytic astrocytoma and oligodendroglioma, especially in younger patients. However, even high grade PXA patients have significantly better overall survival compared to glioblastoma.

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Section: Introductionmentioning

confidence: 99%

“…Most commonly they present as WHO grade II tumors, however, anaplastic transformation can occur in up to 15-20 % of patients [4,7]. Due to the rarity of this tumor, the majority of data available are case reports or relatively small case series [2,4,[6][7][8][9][10][11][12][13][14][15][16][17][18][19].…”

Section: Introductionmentioning

confidence: 99%

Patterns of care and outcomes of patients with pleomorphic xanthoastrocytoma: a SEER analysis

et al. 2012

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“…The PXA tumor is revealed in the majority of cases by epileptic seizures, less frequently by neurologic deficits or by increased intracranial pressure. 4 The lesions show distinct borders with the surrounding brain tissue, 5 but not invariable, since deep portions sometimes may exhibit some degree of parenchymal infiltration, and contrast enhancement is a constant finding. 13 The inner table of the skull adjacent to the neoplasm is sometimes eroded 14–16 and attachment to the dura is common.…”

Section: Discussionmentioning

confidence: 99%

“…Spindle cells may be arranged in bundles or in a storiform pattern. 4 A well‐developed reticulin meshwork which is attributed to basal lamina product by tumor cells surrounds individual cells or small nests of cells. 13 It has been suggested that subpial astrocytes represent the cell of origin of PXA.…”

Section: Discussionmentioning

confidence: 99%

Cerebellar pleomorphic xanthoastrocytoma in an infant

Lim¹,

Jang²,

Kim

1999

Pathology International

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Pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor and typically occurs in the superficial cerebral hemispheres of young subjects. We report a case of PXA in the cerebellum of a 3-month-old infant in view of its unusual location and age. The patient presented with a 1-month history of upward eyeball gazing difficulty. To the authors' knowledge, this is the first report of occurrence of this neoplasm in the cerebellum of an infant. We report the morphologic and immunophenotypical features, and literature review with regard to the clinicopathologic aspects of a case of unusual PXA.

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“…7,9 While PXA tends to affect adolescents and young adults, rare cases in infants have been described. 3,9,13 Although originally often misdiagnosed as glioblastoma, PXAs are generally considered to be a more architecturally solid and superficial form of astrocytoma that follows a more favorable prognostic course. Histologically, spindled cells arranged in a fascicular architecture, lymphocytic infiltrates, intercellular reticulin deposition, and often numerous EGBs typify these neoplasms.…”

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confidence: 99%

Possible differentiation of cerebral glioblastoma into pleomorphic xanthoastrocytoma: an unusual case in an infant

Yang

Singhal

Rassekh

et al. 2012

PED

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The authors describe an infant girl who, at 10 months of age, presented with a large right parietooccipital tumor causing increased intracranial pressure, mass effect, and midline shift. The tumor was completely resected, and the entirety of the histology was consistent with glioblastoma. She was subsequently placed on adjuvant high-dose chemotherapy consisting of carboplatin, vincristine, and temozolomide, according to Head Start III, Regimen C. Three months after the complete resection, tumor recurrence was noted on MR imaging, during the third cycle of chemotherapy, and biopsy revealed malignant astrocytoma. Given the recurrence and the patient's intolerance to chemotherapy, a palliative course was pursued. Unexpectedly, the patient was alive and had made significant developmental improvements 18 months into palliation. Subsequently, however, signs of increased intracranial pressure developed and imaging demonstrated a very large new tumor growth at the site of prior resection. The recurrence was again fully resected, but microscopy surprisingly revealed pleomorphic xanthoastrocytoma throughout. The clinicopathological and genetic features of this girl's unusual neoplasm are detailed and potential pathogenic hypotheses are explored in this report.

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Pleomorphic xanthoastrocytoma: clinical, imaging and pathological features of four cases

Cited by 39 publications

References 31 publications

Patterns of care and outcomes of patients with pleomorphic xanthoastrocytoma: a SEER analysis

Patterns of care and outcomes of patients with pleomorphic xanthoastrocytoma: a SEER analysis

Cerebellar pleomorphic xanthoastrocytoma in an infant

Possible differentiation of cerebral glioblastoma into pleomorphic xanthoastrocytoma: an unusual case in an infant

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