Pilomyxoid astrocytoma in a patient with neurofibromatosis

Khanani, Muhammad Faisal; Hawkins, Cynthia; Shroff, Manohar; Dirks, Peter B.; Capra, Michael; Burger, Peter C.; Bouffet, Éric

doi:10.1002/pbc.20391

Cited by 26 publications

(17 citation statements)

References 22 publications

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“…9,10 One of these previously reported cases, a 9-year-old girl, is included in our series. A second patient in our series, a 9-month-old girl, also has NF-1.…”

Section: Discussionmentioning

confidence: 99%

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Pilomyxoid Astrocytoma: Expanding the Imaging Spectrum

Linscott¹,

Osborn²,

Blasér³

et al. 2008

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE:Pilomyxoid astrocytoma (PMA) is a recently described variant of pilocytic astrocytoma (PA) with unique clinical and histopathologic characteristics. Because the histopathology of PMA is distinct from that of PA, we hypothesized that PMAs would display distinctive imaging characteristics. We retrospectively reviewed the imaging findings in a large number of patients with PMA to identify these characteristics.

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“…9,10 One of these previously reported cases, a 9-year-old girl, is included in our series. A second patient in our series, a 9-month-old girl, also has NF-1.…”

Section: Discussionmentioning

confidence: 99%

“…Five cases were previously published as case reports. [7][8][9] Contributors searched their respective institutional teaching files, PACS archives, and pathology department data base archives. Pathology reports were available in all cases and documented the diagnosis of PMA.…”

Section: Methodsmentioning

confidence: 99%

Pilomyxoid Astrocytoma: Expanding the Imaging Spectrum

Linscott¹,

Osborn²,

Blasér³

et al. 2008

AJNR Am J Neuroradiol

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“…[3][4][5][6][7] A lack of consistent follow-up poses a challenge for accurate data collection, and the long-term outcomes of adult survivors of PLGG are seldom reported. 5 Determinants of short-term tumor-related outcomes [3][4][5][6][7] include patient-related factors, such as age and an underlying diagnosis of neurofibromatosis type 1 (NF1), 8,9 and tumor-related factors, such as tumor extension and certain pathological subtypes. 6,10,11 Although complete surgical resection is consistently associated with improved survival, the extent of resection highly depends on the tumor location, and resection is feasible only for a minority of tumors.…”

Section: Introductionmentioning

confidence: 99%

Clinical and treatment factors determining long‐term outcomes for adult survivors of childhood low‐grade glioma: A population‐based study

Krishnatry

Zhukova

Stücklin

et al. 2016

Cancer

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134

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BACKGROUND:The determinants of outcomes for adult survivors of pediatric low-grade glioma (PLGG) are largely unknown. METH-ODS: This study collected population-based follow-up information for all PLGG patients diagnosed in Ontario, Canada from 1985 to 2012 (n 5 1202) and determined factors affecting survival. The impact of upfront radiation treatment on overall survival (OS) was determined for a cohort of Ontario patients and an independent reference cohort from the Surveillance, Epidemiology, and End Results database. RESULTS: At a median follow-up of 12.73 years (range, 0.02-33 years), only 93 deaths (7.7%) were recorded, and the 20-year OS rate was 90.1% 6 1.1%. Children with neurofibromatosis type 1 had excellent survival and no tumor-related deaths during adulthood. Adverse risk factors included pleomorphic xanthoastrocytoma (P <.001) and a thalamic location (P <.001). For patients with unresectable tumors surviving more than 5 years after the diagnosis, upfront radiotherapy was associated with an approximately 3-fold increased risk of overall late deaths (hazard ratio [HR], 3.3; 95% confidence interval [CI], 1.6-6.6; P 5.001) and an approximately 4-fold increased risk of tumor-related deaths (HR, 4.4; 95% CI, 1.3-14.6; P 5.013). In a multivariate analysis, radiotherapy was the most significant factor associated with late all-cause deaths (HR, 3.0; 95% CI, 1.3-7.0; P 5.012) and tumor-related deaths (HR, 4.4; 95% CI, 1.3-14.6; P 5 0.014). A similar association between radiotherapy and late deaths was observed in the independent reference cohort (P <.001). In contrast to early deaths, late mortality was associated not with PLGG progression but rather with tumor transformation and non-oncological causes. CONCLUSIONS: The course of PLGG is associated with excellent long-term survival, but this is hampered by increased delayed mortality in patients receiving upfront radiotherapy. These observations should be considered when treatment options are being weighed for these patients. Cancer 2016;122:1261-9.

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“…1,[7][8][9][10] Pilomyxoid astrocytoma and pilocytic astrocytoma have even been encountered in patients with neurofibromatosis type I. 3,11 In terms of the nature of pilomyxoid astrocytoma neoplastic cells, it has been postulated that pilomyxoid astrocytoma might be derived from astrocytic cells or radial glia existing in the embryonic optic chiasm. 5 However, some researchers have found ependymal differentiation using electron microscopy and have suggested an ependymal derivation of the tumor.…”

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confidence: 99%

Clinicopathological features and global genomic copy number alterations of pilomyxoid astrocytoma in the hypothalamus/optic pathway: comparative analysis with pilocytic astrocytoma using array-based comparative genomic hybridization

et al. 2008

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Pilomyxoid astrocytoma is a recently identified variant of pilocytic astrocytoma. We studied 11 circumscribed astrocytomas with focal (n ¼ 5) or diffuse (n ¼ 6) pilomyxoid features and compared them with 17 pilocytic astrocytomas from the hypothalamic/chiasmatic region in children. In one patient, a tumor that recurred after initial surgery had changed from pure-form pilomyxoid astrocytoma to the mixed form. The presence of a pilomyxoid area was associated with shorter survival. Next, we compared the comprehensive genome copy number changes in the pilomyxoid astrocytoma (n ¼ 4) with those in pilocytic astrocytoma (n ¼ 6) cases by array-based comparative genomic hybridization. The number of lost clones was larger in pilomyxoid astrocytoma than in pilocytic astrocytoma. Clones located in chromosome 8q24.3 were frequently gained in pilocytic astrocytoma (four of six) and in pilomyxoid astrocytoma (one of four). Clones located in 9p24.3 and 15q26.3 were lost in all of the pilomyxoid astrocytomas and in five of the pilocytic astrocytomas. Those in 8p23.3 showed a copy number loss in three of the pilomyxoid astrocytomas and four of the pilocytic astrocytomas. The frequency of copy number changes was significantly different between pilomyxoid astrocytoma and pilocytic astrocytoma in 47 (3.6%) clones, 20 of them having been located in 2p, 10 in 2q, and 11 in 3q. An unsupervised hierarchical clustering analysis classified the cases into three clusters: one pilomyxoid astrocytoma patient into one cluster, two pilomyxoid astrocytoma patients into another cluster, and six pilocytic astrocytoma patients and one pilomyxoid astrocytoma patient into the third cluster. In conclusion, the presence of mixed-form pilomyxoid astrocytoma, the acquisition of pilocytic astrocytoma features in a recurrent tumor in pure-form pilomyxoid astrocytoma, and the above results of the genome-wide gene copy number analysis suggest that pilomyxoid astrocytoma might be a pathologically and genetically related, aggressive variant of pilocytic astrocytoma with partially different genetic alterations.

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Pilomyxoid astrocytoma in a patient with neurofibromatosis

Cited by 26 publications

References 22 publications

Pilomyxoid Astrocytoma: Expanding the Imaging Spectrum

Pilomyxoid Astrocytoma: Expanding the Imaging Spectrum

Clinical and treatment factors determining long‐term outcomes for adult survivors of childhood low‐grade glioma: A population‐based study

Clinicopathological features and global genomic copy number alterations of pilomyxoid astrocytoma in the hypothalamus/optic pathway: comparative analysis with pilocytic astrocytoma using array-based comparative genomic hybridization

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