Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG‐921: A phase III combined modality study

Hong, Theodore S.; Mehta, Minesh P.; Boyett, James M.; Donahue, Bernadine R.; Rorke, Lucy B.; Zeltzer, Paul M.

doi:10.1002/pbc.20184

Cited by 18 publications

(5 citation statements)

References 33 publications

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“…At last follow up, 123 (69%) were dead and 55 (31%) were alive. The resection cavity was the most common site of disease recurrence, followed by the spine, which is consistent with the patterns of failure of PNETs [99] , [100] .…”

Section: Resultssupporting

confidence: 81%

Radiation for ETMR: Literature review and case series of patients treated with proton therapy

Jaramillo

Grosshans

Philip

et al. 2019

Clinical and Translational Radiation Oncology

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Section: Resultssupporting

confidence: 81%

Radiation for ETMR: Literature review and case series of patients treated with proton therapy

Jaramillo

Grosshans

Philip

et al. 2019

Clinical and Translational Radiation Oncology

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“…Even the latter figure contrasts sharply with the 1.1% frequency of CSF spread seen in one large series of 267 adult GBMs ( P < 0.001; chi‐square) (38). Our numbers are much closer to the 37% frequency of meningeal metastases in medulloblastomas and CNS PNETs (14). Additionally, three patients who switched to platinum‐based chemotherapy after failing radiation therapy and temozolomide subsequently showed radiographic responses.…”

Section: Discussioncontrasting

confidence: 55%

Malignant Gliomas with Primitive Neuroectodermal Tumor‐like Components: A Clinicopathologic and Genetic Study of 53 Cases

Perry¹,

Miller²,

et al. 2008

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Central nervous system neoplasms with combined features of malignant glioma and primitive neuroectodermal tumor (MG-PNET) are rare, poorly characterized, and pose diagnostic as well as treatment dilemmas. We studied 53 MG-PNETs in patients from 12 to 80 years of age (median = 54 years). The PNET-like component consisted of sharply demarcated hypercellular nodules with evidence of neuronal differentiation. Anaplasia, as seen in medulloblastomas, was noted in 70%. Within the primitive element, N-myc or c-myc gene amplifications were seen in 43%. In contrast, glioma-associated alterations involved both components, 10q loss (50%) being most common. Therapy included radiation (78%), temozolomide (63%) and platinum-based chemotherapy (31%). Cerebrospinal fluid (CSF) dissemination developed in eight patients, with response to PNET-like therapy occurring in at least three. At last follow-up, 27 patients died, their median survival being 9.1 months. We conclude that the primitive component of the MG-PNET: (i) arises within a pre-existing MG, most often a secondary glioblastoma; (ii) may represent a metaplastic process or expansion of a tumor stem/progenitor cell clone; (iii) often shows histologic anaplasia and N-myc (or c-myc) amplification; (iv) has the capacity to seed the CSF; and (v) may respond to platinum-based chemotherapy regimens.

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“…Any metastatic disease in stPNETs, either intra‐ or extraneurally, is a poor prognostic factor. It is estimated that the 3‐year progression free survival (PFS) for stPNETs is 14% for patients with metastatic disease compared to 53% for those with localized disease 16. Studies involving a relatively small number of patients have shown that extraneural metastases in CNS PNETs, including medulloblastomas, are a significantly worse prognostic factor.…”

Section: Discussionmentioning

confidence: 99%

Long‐term survival in a pediatric patient with supratentorial primitive neuro‐ectodermal tumor and extraneural metastasis at diagnosis

Rubens

Gosiengfiao

Tomita

et al. 2011

Pediatric Blood & Cancer

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Extraneural metastases of central nervous system (CNS) tumors are rare occurrences most commonly observed in medulloblastomas. Survival outcomes are generally dismal. Supratentorial primitive neuroectodermal tumors (stPNET) are rare childhood tumors with few documented cases of extraneural metastases. We present a rare occurrence of a 23-month-old patient with long-term survival after diagnosis of stPNET with metastases to the lungs. This patient was treated with surgical resection, induction chemotherapy, tandem autologous hematopoietic cell rescues, and focal radiotherapy. We report long-term survival for a patient with a stPNET and extraneural metastases at diagnosis following an intensive approach to treatment.

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Patterns of treatment failure in infants with primitive neuroectodermal tumors who were treated on CCG‐921: A phase III combined modality study

Abstract: Despite aggressive chemotherapy, younger children with PNETs have high rates of treatment failure and fare worse than high-risk, older patients with PF-PNETs, indicating the need to maximize local, regional, and systemic therapies.

Cited by 18 publications

References 33 publications

Radiation for ETMR: Literature review and case series of patients treated with proton therapy

Radiation for ETMR: Literature review and case series of patients treated with proton therapy

Malignant Gliomas with Primitive Neuroectodermal Tumor‐like Components: A Clinicopathologic and Genetic Study of 53 Cases

Long‐term survival in a pediatric patient with supratentorial primitive neuro‐ectodermal tumor and extraneural metastasis at diagnosis

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