Pleomorphic xanthoastrocytoma: What do we really know about it

Caterina Giannini, Bernd W. Scheithauer, Peter C. Burger, Daniel J. Brat, Peter C. Wollan, Bolek Lach, Brian Patrick O'Neill

Research output: Contribution to journalArticle

250 Citations (Scopus)

Abstract

Background. Pleomorphic xanthoastrocytomas (PXA) may recur and demonstrate aggressive clinical behavior with a mortality rate between 15% and 20%. To the authors' knowledge, no histopathologic features currently are known to reliably predict recurrence or tumor progression. METHODS. The study was based on 71 cases with available information regarding clinical and therapeutic data and follow-up. Diagnostic features included cellular pleomorphism, giant and/or xanthic cells, eosinophilic granular bodies, desmoplasia, and leptomeningeal involvement. The mitotic index (MI), the presence of necrosis, and endothelial proliferation were recorded in all primary resection specimens. RESULTS. The study included 35 females and 36 males, age 26 ± 16 years (mean ± standard deviation). Approximately 98% of tumors were supratentorial, with 49% in the temporal lobe. Seizures were the presenting symptoms in 71% of patients. Extent of tumor removal was macroscopic total resection in 68% of cases and subtotal resection (STR) in 32% of cases. Postoperative radiotherapy, alone or with chemotherapy, was administered in 29% and 12.5% of cases, respectively. The recurrence free survival rates (RFS) were 72% at 5 years and 61% at 10 years, whereas overall survivals rates (OS) were 81% at 5 years and 70% at 10 years. In univariate analysis, the extent of resection was the single factor associated most strongly with RFS (P = 0.003), followed by MI (P = 0.007) and atypical mitoses (P = 0.04). Necrosis was not found to be significant. The extent of resection and MI were confirmed as independent predictors of RFS by multivariate analysis. MI (P = 0.001), atypical mitoses (P = 0.02), and necrosis (P = 0.04) were associated with OS by univariate analysis. In multivariate analysis, only MI was an independent predictor of survival. Information regarding MIB-1 labeling index and the use of adjuvant therapy was too limited to explore their prognostic significance confidently. CONCLUSIONS. The study confirms that PXA is an astrocytic tumor with a relatively favorable prognosis. MI and extent of resection appear to be the main predictors of RFS and OS. Given the slow growth of the tumor, more studied cases and longer periods of follow-up will be essential to confirm our findings regarding prognostic factors affecting this unusual tumor.

Original languageEnglish (US)
Pages (from-to)2033-2045
Number of pages13
JournalCancer
Volume85
Issue number9
DOIs
StatePublished - May 1 1999

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Mitotic Index
Survival Rate
Recurrence
Necrosis
Neoplasms
Mitosis
Multivariate Analysis
Supratentorial Neoplasms
Temporal Lobe
Seizures
Radiotherapy
Drug Therapy
Survival
Mortality
Therapeutics
Growth

Keywords

  • Glioma
  • Natural history
  • Neurooncology
  • Pathology
  • Pleomorphic xanthoastrocytoma
  • Prognostic factors

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Pleomorphic xanthoastrocytoma : What do we really know about it. / Giannini, Caterina; Scheithauer, Bernd W.; Burger, Peter C.; Brat, Daniel J.; Wollan, Peter C.; Lach, Bolek; O'Neill, Brian Patrick.

In: Cancer, Vol. 85, No. 9, 01.05.1999, p. 2033-2045.

Research output: Contribution to journalArticle

Giannini, Caterina ; Scheithauer, Bernd W. ; Burger, Peter C. ; Brat, Daniel J. ; Wollan, Peter C. ; Lach, Bolek ; O'Neill, Brian Patrick. / Pleomorphic xanthoastrocytoma : What do we really know about it. In: Cancer. 1999 ; Vol. 85, No. 9. pp. 2033-2045.
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abstract = "Background. Pleomorphic xanthoastrocytomas (PXA) may recur and demonstrate aggressive clinical behavior with a mortality rate between 15{\%} and 20{\%}. To the authors' knowledge, no histopathologic features currently are known to reliably predict recurrence or tumor progression. METHODS. The study was based on 71 cases with available information regarding clinical and therapeutic data and follow-up. Diagnostic features included cellular pleomorphism, giant and/or xanthic cells, eosinophilic granular bodies, desmoplasia, and leptomeningeal involvement. The mitotic index (MI), the presence of necrosis, and endothelial proliferation were recorded in all primary resection specimens. RESULTS. The study included 35 females and 36 males, age 26 ± 16 years (mean ± standard deviation). Approximately 98{\%} of tumors were supratentorial, with 49{\%} in the temporal lobe. Seizures were the presenting symptoms in 71{\%} of patients. Extent of tumor removal was macroscopic total resection in 68{\%} of cases and subtotal resection (STR) in 32{\%} of cases. Postoperative radiotherapy, alone or with chemotherapy, was administered in 29{\%} and 12.5{\%} of cases, respectively. The recurrence free survival rates (RFS) were 72{\%} at 5 years and 61{\%} at 10 years, whereas overall survivals rates (OS) were 81{\%} at 5 years and 70{\%} at 10 years. In univariate analysis, the extent of resection was the single factor associated most strongly with RFS (P = 0.003), followed by MI (P = 0.007) and atypical mitoses (P = 0.04). Necrosis was not found to be significant. The extent of resection and MI were confirmed as independent predictors of RFS by multivariate analysis. MI (P = 0.001), atypical mitoses (P = 0.02), and necrosis (P = 0.04) were associated with OS by univariate analysis. In multivariate analysis, only MI was an independent predictor of survival. Information regarding MIB-1 labeling index and the use of adjuvant therapy was too limited to explore their prognostic significance confidently. CONCLUSIONS. The study confirms that PXA is an astrocytic tumor with a relatively favorable prognosis. MI and extent of resection appear to be the main predictors of RFS and OS. Given the slow growth of the tumor, more studied cases and longer periods of follow-up will be essential to confirm our findings regarding prognostic factors affecting this unusual tumor.",
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T1 - Pleomorphic xanthoastrocytoma

T2 - What do we really know about it

AU - Giannini, Caterina

AU - Scheithauer, Bernd W.

AU - Burger, Peter C.

AU - Brat, Daniel J.

AU - Wollan, Peter C.

AU - Lach, Bolek

AU - O'Neill, Brian Patrick

PY - 1999/5/1

Y1 - 1999/5/1

N2 - Background. Pleomorphic xanthoastrocytomas (PXA) may recur and demonstrate aggressive clinical behavior with a mortality rate between 15% and 20%. To the authors' knowledge, no histopathologic features currently are known to reliably predict recurrence or tumor progression. METHODS. The study was based on 71 cases with available information regarding clinical and therapeutic data and follow-up. Diagnostic features included cellular pleomorphism, giant and/or xanthic cells, eosinophilic granular bodies, desmoplasia, and leptomeningeal involvement. The mitotic index (MI), the presence of necrosis, and endothelial proliferation were recorded in all primary resection specimens. RESULTS. The study included 35 females and 36 males, age 26 ± 16 years (mean ± standard deviation). Approximately 98% of tumors were supratentorial, with 49% in the temporal lobe. Seizures were the presenting symptoms in 71% of patients. Extent of tumor removal was macroscopic total resection in 68% of cases and subtotal resection (STR) in 32% of cases. Postoperative radiotherapy, alone or with chemotherapy, was administered in 29% and 12.5% of cases, respectively. The recurrence free survival rates (RFS) were 72% at 5 years and 61% at 10 years, whereas overall survivals rates (OS) were 81% at 5 years and 70% at 10 years. In univariate analysis, the extent of resection was the single factor associated most strongly with RFS (P = 0.003), followed by MI (P = 0.007) and atypical mitoses (P = 0.04). Necrosis was not found to be significant. The extent of resection and MI were confirmed as independent predictors of RFS by multivariate analysis. MI (P = 0.001), atypical mitoses (P = 0.02), and necrosis (P = 0.04) were associated with OS by univariate analysis. In multivariate analysis, only MI was an independent predictor of survival. Information regarding MIB-1 labeling index and the use of adjuvant therapy was too limited to explore their prognostic significance confidently. CONCLUSIONS. The study confirms that PXA is an astrocytic tumor with a relatively favorable prognosis. MI and extent of resection appear to be the main predictors of RFS and OS. Given the slow growth of the tumor, more studied cases and longer periods of follow-up will be essential to confirm our findings regarding prognostic factors affecting this unusual tumor.

AB - Background. Pleomorphic xanthoastrocytomas (PXA) may recur and demonstrate aggressive clinical behavior with a mortality rate between 15% and 20%. To the authors' knowledge, no histopathologic features currently are known to reliably predict recurrence or tumor progression. METHODS. The study was based on 71 cases with available information regarding clinical and therapeutic data and follow-up. Diagnostic features included cellular pleomorphism, giant and/or xanthic cells, eosinophilic granular bodies, desmoplasia, and leptomeningeal involvement. The mitotic index (MI), the presence of necrosis, and endothelial proliferation were recorded in all primary resection specimens. RESULTS. The study included 35 females and 36 males, age 26 ± 16 years (mean ± standard deviation). Approximately 98% of tumors were supratentorial, with 49% in the temporal lobe. Seizures were the presenting symptoms in 71% of patients. Extent of tumor removal was macroscopic total resection in 68% of cases and subtotal resection (STR) in 32% of cases. Postoperative radiotherapy, alone or with chemotherapy, was administered in 29% and 12.5% of cases, respectively. The recurrence free survival rates (RFS) were 72% at 5 years and 61% at 10 years, whereas overall survivals rates (OS) were 81% at 5 years and 70% at 10 years. In univariate analysis, the extent of resection was the single factor associated most strongly with RFS (P = 0.003), followed by MI (P = 0.007) and atypical mitoses (P = 0.04). Necrosis was not found to be significant. The extent of resection and MI were confirmed as independent predictors of RFS by multivariate analysis. MI (P = 0.001), atypical mitoses (P = 0.02), and necrosis (P = 0.04) were associated with OS by univariate analysis. In multivariate analysis, only MI was an independent predictor of survival. Information regarding MIB-1 labeling index and the use of adjuvant therapy was too limited to explore their prognostic significance confidently. CONCLUSIONS. The study confirms that PXA is an astrocytic tumor with a relatively favorable prognosis. MI and extent of resection appear to be the main predictors of RFS and OS. Given the slow growth of the tumor, more studied cases and longer periods of follow-up will be essential to confirm our findings regarding prognostic factors affecting this unusual tumor.

KW - Glioma

KW - Natural history

KW - Neurooncology

KW - Pathology

KW - Pleomorphic xanthoastrocytoma

KW - Prognostic factors

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