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Glioblastoma with Both Oligodendroglioma and Primitive Neuroectodermal Tumor-Like Components in a Case with 9-Year Survival.

1. Introduction

Glioblastoma multiforme (GBM) is the most malignant form of astrocytoma and the most frequent primary tumor of the brain in adults. Its prognosis is poor, with a median survival of 12.1-14.6 months despite current multimodal treatment [1]. GBM shows a great degree of histological variability and numerous morphologic subtypes, some of which maybe associated with specific genetic alterations or clinical behaviors [2]. Glioblastoma with primitive neuronal component, which has been referred to as glioblastoma with the primitive neuroectodermal tumor-like component (GBM-PNET) in the literature and which is one of the emerging GBM subtypes, is a rare tumor with the combined features of malignant glioma and PNET. Here, we report a long-term survival patient who presented not only this rare collision tumor but also different histopathological components in the disease course.

2. Case Presentation

A 23-year-old woman presented with progressive headache, nausea, and vomiting for 1 week. Right-side weakness, ptosis, and diplopia were also found. Due to acute onset conscious disturbance (Glasgow Coma Scale of E3VaM5) in the hospital, brain computed tomography was arranged and revealed an enhanced brain tumor with necrotic cystic change. This tumor was located at the left temporal lobe with upward extension to the left basal ganglion and periventricular region, causing perifocal edema and midline shift (Figure 1). We performed emergent craniectomy for tumor removal in December 2006.

Pathology revealed pleomorphic, hyperchromatic cells with glassy, astrocytic cytoplasm, as well as hypercellularity, microvascular proliferation, and necrosis, consistent with the diagnosis of classic GBM (Figure 2).

The patient underwent radiotherapy 1 month later and followed by chemotherapy with temozolomide for 6 months. Her performance status improved to a Karnofsky Grade of 70, and her clinical condition was stable thereafter. However, follow-up brain magnetic resonance imaging (MRI) in June 2014 revealed a new enhanced nodular lesion, approximately 1.1 cm in diameter, at the left temporal base. The brain MRI in October 2014 revealed a progressive change of lesions, maximum 3.0 cm in diameter (Figure 1). Thus, she again received surgery for gross tumor removal.

Histologically, except for the necrosis feature of GBM, the tumors showed the oligodendroglial component. Neoplastic cells also showed isocitrate dehydrogenase 1(IDH1)(+), p53(diffuse +), and [O.sup.6]-methylguanine-DNA methyltransferase (MGMT)(-) as revealed by immunostaining (Figure 3).

During follow-up, signs of increased intracranial pressure were noted in May 2015. Therefore, she received a third debulking surgery. The third pathology revealed both GBM- and PNET-like components. In immunohistochemistry, the PNET-like component exhibited positivity for synaptophysin and CD56 and focal weak positivity for glial fibrillary acidic protein (GFAP) (Figure 4).

One month after the surgery, her condition rapidly deteriorated. The patient and her family chose to pursue hospice care. She passed away with a total 9-year survival since diagnosis (Table 1).

3. Discussion

According to the current 2016 WHO classification system, GBM can be divided into IDH wild type, IDH mutant, and NOS (not otherwise specified). Giant cell glioblastoma, gliosarcoma, and epithelioid glioblastoma are categorized under IDH wild-type glioblastoma [3]. Moreover, based on the described histopathology, diverse glioblastoma variants are still emerging, such as glioblastoma with primitive neuroectodermal tumor-like component (GBM-PNET), GBM with oligodendroglioma component (GBM-O), small cell astrocytoma, and granular cell astrocytoma [2]. Recent studies have suggested that under the stochastic or hierarchical hypothetical model, the presence of a cancer stem cell may account for the substantial diversity in the pathological characteristics of GBM [4].

GBM-Os account for 4% to 27% of all GBMs in previous studies. They feature both astrocytic and oligodendroglial differentiation, with the typical fried-egg appearance. Recent analyses of large tumor databases found a significantly higher IDH1 mutation rate in GBM-Os (31% versus <5%) [5]. Moreover, the EORTC 26981/NCICCE.3 trial found that GBM-Os showed significantly higher levels of IDH1 mutation (19% versus 3%) than GBMs [6]. Thus, GBM-Os exhibit a higher frequency of IDH1 mutations and a lower frequency of PTEN deletions, which is a characteristic distinct from that of classic GBMs. Recent studies have revealed that GBM-Os tend to occur at a younger age and that patients with GBM-Os exhibit longer survival than those with other forms of GBMs [7, 8].

In contrast to GBMs, those previously designated as PNETs, incorporated into the term embryonal tumor with multilayered rosettes (ETMR) in the 2016 WHO classification system for CNS tumors, are aggressive neoplasms, with medulloblastoma-like histology, and mostly affect the pediatric population. They are composed of poorly differentiated neuroepithelial cells and are associated with a high risk of metastases through cerebrospinal fluid dissemination. Patients with PNETs exhibit a poor prognosis, requiring craniospinal radiation in addition to platinum-based chemotherapy [3, 9]. GBM-PNETs are a rare pattern in GBMs. They contain the architectures of both traditional GBM and PNET-like areas, with hypercellularity, minimal fibrillary background, high nuclear-tocytoplasmic ratio, oval-round hyperchromatic nuclei, high mitotic-karryorhectic indices, and Homer Wright neuroblastic rosettes. Furthermore, PNET-like areas show lower GFAP expression but positive staining for S-100, synaptophysin, NeuN, and neurofilament protein (NFP) [10-12].

One of the largest series on GBM-PNETs reported 53 cases, with a male:female ratio of 1.3, leptomeningeal metastasis in up to 40% of patients, and the median survival of 9.1 months [11]. However, recent studies have suggested favorable prognostic features for GBM-PNETs. A study of 40 cases of grade III or IV glioma with PNET components, as demonstrated by GFAP and NFP coexpression, reported a low recurrence rate (36%) and a mean survival of 44 months after gross total resection [12]. In another study of 12 patients with GBM-PNETs, IDH1 mutations were observed in 2 patients with an overall survival of 15 and 31 months. The present study also concluded that GBM-PNETs have a higher frequency of IDH1 mutation and might have favorable prognosis [13].

In our case, we observed different pathological variants of glioblastoma in the disease course. Both histopathologic types of GBM-Os and GBM-PNETs tend to contribute to a favorable prognosis. In addition, probably because of initial radical surgical resection, young age at the time of diagnosis, silencing of MGMT, and positive IDH-1 staining, the patient had long-term survival.

4. Conclusion

GBM is associated with substantial diversity in its clinicopathological characteristics. GBM-Os resemble GBMs but also contain areas resembling oligodendroglioma, whereas GBM-PNETs are rare tumors with the combined features of high-grade glioma and primitive neuroectodermal tumors. This report illustrates that the distinct histopathologic variants of glioblastoma can appear throughout the disease course and such tumors are associated with favorable outcomes.

https://doi.org/10.1155/2018/1382680

Consent

The patient's consent was obtained to publish the information and pictures of this case report.

Conflicts of Interest

There are no conflicts of interest.

Authors' Contributions

Ying-Tso Chen wrote the manuscript. Ying-Tso Chen, Chi-Man Yip, Shu-Shong Hsu, and Ping-Hong Lai participated in the diagnosis and treatment of the case. Huai-Pao Lee carried out the pathological analysis. All authors approved the final manuscript.

References

[1] R. Stupp, W. P. Mason, M. J. van den Bent et al., "Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma," The New England Journal of Medicine, vol. 352, no. 10, pp. 987-996, 2005.

[2] M. Karsy, M. Gelbman, P. Shah, O. Balumbu, F. Moy, and E. Arslan, "Established and emerging variants of glioblastoma multiforme: review of morphological and molecular features," Folia Neuropathologica, vol. 50, no. 4, pp. 301-321, 2012.

[3] D. N. Louis, A. Perry, G. Reifenberger et al., "The 2016 World Health Organization classification of tumors of the central nervous system: a summary," Acta Neuropathologica, vol. 131, no. 6, pp. 803-820, 2016.

[4] J. Wang, Y. Ma, and M. K. Cooper, "Cancer stem cells in glioma: challenges and opportunities," Translational Cancer Research, vol. 2, no. 5, pp. 429-441, 2013.

[5] Y. Wang, S. Li, L. Chen et al., "Glioblastoma with an oligodendroglioma component: distinct clinical behavior, genetic alterations, and outcome," Neuro-Oncology, vol. 14, no. 4, pp. 518-525, 2012.

[6] M. E. Hegi, R. C. Janzer, W. L. Lambiv et al., "Presence of an oligodendroglioma-like component in newly diagnosed glioblastoma identifies a pathogenetically heterogeneous subgroup and lacks prognostic value: central pathology review of the EORTC_26981/NCIC_CE.3 trial," Acta Neuropathologica, vol. 123, no. 6, pp. 841-852, 2012.

[7] R. C. Laxton, S. Popov, L. Doey et al., "Primary glioblastoma with oligodendroglial differentiation has better clinical outcome but no difference in common biological markers compared with other types of glioblastoma," Neuro-Oncology, vol. 15, no. 12, pp. 1635-1643, 2013.

[8] J. S. Goda, S. Lewis, A. Agarwal et al., "Impact of oligodendroglial component in glioblastoma (GBM-O): is the outcome favourable than glioblastoma?," Clinical Neurology and Neurosurgery, vol. 135, pp. 46-53, 2015.

[9] D. L. Johnston, D. L. Keene, L. Lafay-Cousin et al., "Supratentorial primitive neuroectodermal tumors: a Canadian pediatric brain tumor consortium report," Journal of Neuro-Oncology, vol. 86, no. 1, pp. 101-108, 2008.

[10] N. O. Kandemir, B. Bahadir, S. Gul, N. Karadayi, and S. O. Ozdamar, "Glioblastoma with primitive neuroectodermal tumor-like features: case report," Turkish neurosurgery, vol. 19, no. 3, pp. 260-264, 2009.

[11] A. Perry, C. R. Miller, M. Gujrati et al., "Malignant gliomas with primitive neuroectodermal tumor-like components: a clinicopathologic and genetic study of 53 cases," Brain Pathology, vol. 19, no. 1, pp. 81-90, 2009.

[12] P. Varlet, D. Soni, C. Miquel et al., "New variants of malignant glioneuronal tumors: a clinicopathological study of 40 cases," Neurosurgery, vol. 55, no. 6, pp. 1377-1392, 2004.

[13] X. Song, R. Andrew Allen, S. Terence Dunn et al., "Glioblastoma with PNET-like components has a higher frequency of isocitrate dehydrogenase 1 (IDH1) mutation and likely a better prognosis than primary glioblastoma," International Journal of Clinical and Experimental Pathology, vol. 4, no. 7, pp. 651-660, 2011.

Ying-Tso Chen (ID), (1) Shu-Shong Hsu, (2,3) Chi-Man Yip, (1) Ping-Hong Lai, (4) and Huai-Pao Lee (5,6)

(1) Division of Neurosurgery, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan

(2) Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan

(3) Department of Surgery, National Defense Medical Center, Taiwan

(4) Department of Radiology, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan

(5) Department of Pathology, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan

(6) Department of Nursing, Meiho University, Pingtung, Taiwan

Correspondence should be addressed to Ying-Tso Chen; a7513366@vghks.gov.tw

Received 26 February 2018; Accepted 23 May 2018; Published 11 June 2018

Academic Editor: Jochen Tuttenberg

Caption: Figure 1: (a) Brain computed tomography in December 2006 showed an enhanced brain tumor over the left temporal region with mass effect; (b) no obvious recurrent tumor was found by brain MRI in 2013; (c) recurrent tumor was found by MRI in 2014, before the second surgery; and (d) brain MRI in May 2015, before the third surgery, revealed tumor progression.

Caption: Figure 2: (a) Markedly increased cellularity of neoplastic astrocytes with nuclear pleomorphism and hyperchromasia (200x), (b) microvascular proliferation (200x), (c) necrosis (200x), and (d) increased proliferation index of tumor cells as revealed by Ki-67 immunostaining (16.3%) (100x).

Caption: Figure 3: (a) Foci of oligodendroglial component featuring clear cytoplasm, round nuclei (200x). The neoplastic cells showed (b) necrosis (100x), (c) GFAP(+) (100x), (d) p53(+) (100x), and (e) IDH1(+) (100x).

Caption: Figure 4: (a) Neoplastic cells with nuclear pleomorphism, hyperchromatism, abundant mitosis, and apoptosis (200x). Immunohistochemistry revealed (b) focal weak positivity for GFAP (200x), (c) synaptophysin(+) (400x), and (d) CD56(+) (400x).
Table 1: Timeline.

Dates           Relevant past medical history and interventions

December 2006       23 y/o female with progressive headache,
                        nausea, and vomiting for one week

Date                 Summaries from         Diagnostic testing
                  initial and follow-       (including dates)
                       up visits

December 20,      Right-side weakness,           Brain CT
2006              ptosis and diplopia,
                  conscious drowsiness      Pathology: classic
                                                   GBM

January 18,
2007~March
07, 2007

March 2007~
January 2008

June 09, 2011

October 10,                                  MRI--progressive
2014                                        change of several
                                           tumors over the left
                                              temporal base

December 02,                                   Pathology--
2014                                           glioblastoma
                                             multiforme, with
                                             oligodendroglial
                                                component

May 07, 2015       Headache, nausea,         MRI--tumor local
                      and vomiting           recurrence, with
                                            ventricle seeding

May 13, 2015                                   Pathology--
                                               glioblastoma
                                             multiforme with
                                           PNET-like component

June 2015          Signs of increased        Brain CT: tumor
                      intracranial             progression
                        pressure

July 16, 2015           Expired

Dates                Relevant past
                  medical history and
                     interventions

December 2006      23 y/o female with
                      progressive
                   headache,  nausea,
                  and vomiting for one
                          week

Date                 Interventions

December 20,      Emergent craniectomy
2006              for removal of tumor

January 18,                RT
2007~March
07, 2007

March 2007~          6 courses of
January 2008            Temadol

June 09, 2011         Cranioplasty

October 10,
2014

December 02,      Craniotomy for tumor
2014                    removal

May 07, 2015

May 13, 2015      Craniotomy for tumor
                        removal

June 2015             Conservative
                       treatment

July 16, 2015
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Article Details
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Title Annotation:Case Report
Author:Chen, Ying-Tso; Hsu, Shu-Shong; Yip, Chi-Man; Lai, Ping-Hong; Lee, Huai-Pao
Publication:Case Reports in Surgery
Geographic Code:9TAIW
Date:Jan 1, 2018
Words:2063
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