Malignant Glioma with Primitive Neuroectodermal Components: Clinical and Pathologic Features with Treatment Modalities of Five Cases

Kimbason, Tara; Turner, Scott; Kazmi, Syed A. Jaffar; Fourgas, Edward; Gergel, Thomas; Belles, Lynn; Whitmire, Angela; Lacroix, Michel; Toms, Steven

doi:10.4172/2161-0681.1000255

Cited by 7 publications

(12 citation statements)

References 10 publications

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“…In other more recent studies, the possibility of associating cisplatin or carboplatin with standard treatment with temozolomide is suggested to reduce the risk of cerebrospinal fluid spread from PNC derived tumor cells. [4] As with "conventional" GBs, the response rate to treatment is higher in tumors with the presence of MGMT promoter methylation; however, in GB/PNC, there is no standard therapeutic protocol. Despite the correct treatment, the prognosis of these tumors remains unfavorable and, as with glioblastomas, it represents a therapeutic challenge for all professionals.…”

Section: Discussionmentioning

confidence: 99%

Glioblastoma with primitive neuronal component: A case report and considerations of fluorescence-guided surgery

Ortega

Aguas-Valiente

Sota-Ochoa

et al. 2020

Surgical Neurology International

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Background: Glioblastoma with primitive neuronal components (GB/PNC) is an extremely rare type of glioblastoma characterized by presenting histological and cytogenetic features of both entities. The mixed nature of these tumors limits the imaging diagnosis and supposes a therapeutic dilemma. Case Description: We present the case of a 77-year-old female with a GB/PNC who is treated with surgery and adjuvant radiochemotherapy according to the STUPP protocol, where an abnormal uptake of 5-aminolevulinic acid (5-ALA) is evident during surgery in probable relation to the mixed nature of GB/PNC. Conclusion: GB/PNC is extremely rare tumors. Given its low prevalence, there are no studies that refer to the macroscopic characteristics of the tumor as well as evidence of the effectiveness of adjuvant treatment. Fluorescence-guided resection with 5-ALA is the surgical treatment of choice in surgery for high-grade gliomas; however, in GB/PNC, it may not be as useful since PNC may have less fluorescent marker uptake and be more dimly visualized when excited by light using the surgical microscope.

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Section: Discussionmentioning

confidence: 99%

Glioblastoma with primitive neuronal component: A case report and considerations of fluorescence-guided surgery

Ortega

Aguas-Valiente

Sota-Ochoa

et al. 2020

Surgical Neurology International

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“…According to the predominance of GBM or PNET component, radiotherapy could consist of focal irradiation, as Stupp protocol, or CSI with concomitant and adjuvant TMZ, respectively (4,11). An early introduction of 'PNET-like' platinum-based chemotherapy should be planned in order to prevent CSF dissemination or in case of disease recurrence (7,8,14,18,20).…”

Section: Discussionmentioning

confidence: 99%

“…IDH1 mutation have been observed also in a small percentage of adult PNETs (15)(16)(17). The PNET components are explained by two main hypotheses (8): PNET-like foci arise from pre-existing gliomas, most often a secondary GBM (neuroblastic or neuronal metaplasia) and the clonal expansion of tumour stem cells or progenitor cells resulting in PNET-like nodules (7,15,18).…”

Section: Therapeutic Approach In Glioblastoma Multiforme With Primitimentioning

confidence: 99%

“…Since most of the data concerning the management of GBM/PNETs are based on single case reports or case series (2,(7)(8)(9)11,12,15,(18)(19)(20) and despite therapeutic strategies have been identified for GMBs and PNETs, a common therapeutic protocol for GBM/PNETs has not yet been defined. In addition no evidence on treatment survival outcomes are published (11).…”

Section: Therapeutic Approach In Glioblastoma Multiforme With Primitimentioning

confidence: 99%

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Therapeutic approach in glioblastoma multiforme with primitive neuroectodermal tumor components: Case report and review of the literature

et al. 2018

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Abstract. Glioblastoma multiforme (GBM) is the most common and aggressive malignant glioma that is treated with first-line therapy, using surgical resection followed by local radiotherapy and concomitant/adjuvant temozolomide (TMZ) treatment. GBM is characterised by a high local recurrence rate and a low response to therapy. Primitive neuroectodermal tumour (PNET) of the brain revealed a low local recurrence rate; however, it also exhibited a high risk of cerebrospinal fluid (CSF) dissemination. PNET is treated with surgery followed by craniospinal irradiation (CSI) and platinum-based chemotherapy in order to prevent CSF dissemination. GBM with PNET-like components (GBM/PNET) is an emerging variant of GBM, characterised by a PNET-like clinical behaviour with an increased risk of CSF dissemination; it also may benefit from platinum-based chemotherapy upfront or following failure of GBM therapy. The results presented regarding the management of GBM/PNET are based on case reports or case series, so a standard therapeutic approach for GBM/PNET is not defined, constituing a challenging diagnostic and therapeutic dilemma. In this report, a case of a recurrent GBM/PNET treated with surgical resection and radiochemotherapy as Stupp protocol, and successive platinum-based chemotherapy due to the development of leptomeningeal dissemintation and an extracranial metastasis, is discussed. A review of the main papers regarding this rare GBM variant and its therapeutic approach are also reported. In conclusion, GBM/PNET should be treated with a multimodal approach including surgery, chemoradiotherapy, and/or the early introduction of CSI and platinum-based chemotherapy upfront or at recurrence.

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“…Originally described as glioblastoma with a primitive neuroectodermal tumour (PNET)‐like component, this rare variant accounts for 0.5% of all glioblastomas and occurs mostly in adults with a median age of 54 y. Only a few additional cases have been reported since the original description of this tumor . The primitive neuronal component, which is hypothesised to arise either as a metaplastic process or through the expansion of a glioblastoma stem cell or progenitor cell clone .…”

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confidence: 99%

Glioblastoma with primitive neuronal component: Cytologic findings in intraoperative squash preparations

Borys

Prabhu

Pambuccian

2018

Diagnostic Cytopathology

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Malignant Glioma with Primitive Neuroectodermal Components: Clinical and Pathologic Features with Treatment Modalities of Five Cases

Cited by 7 publications

References 10 publications

Glioblastoma with primitive neuronal component: A case report and considerations of fluorescence-guided surgery

Glioblastoma with primitive neuronal component: A case report and considerations of fluorescence-guided surgery

Therapeutic approach in glioblastoma multiforme with primitive neuroectodermal tumor components: Case report and review of the literature

Glioblastoma with primitive neuronal component: Cytologic findings in intraoperative squash preparations

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