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Anaplastic Oligodendroglioma with Transdural Extension
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Anaplastic Oligodendroglioma with Transdural Extension Ai Peng Tan1, Char Loo Tan2, Yin Huei Pang2, Pin Lin Kei3
Key words Anaplastic oligodendroglioma - Transdural extension -
Abbreviations and Acronyms IDH: Isocitrate dehydrogenase OG: Oligodendroglioma WHO: World Health Organization From the Departments of 1Diagnostic Imaging and 2 Pathology, National University Health System; and 3 Department of Diagnostic Radiology, Ng Teng Fong General Hospital, Singapore To whom correspondence should be addressed: Ai Peng Tan, M.D. [E-mail: [email protected]]
Oligodendrogliomas, the third most common primary gliomas, have a strict molecular definition, characterized by the combined presence of isocitrate dehydrogenase mutation and 1p19q codeletion. Herein, we describe an extremely unusual case of molecularly defined anaplastic oligodendroglioma with transdural extension into the frontal and ethmoid sinuses, without prior neurosurgical intervention or radiotherapy. The molecular profile of the tumor is also provided. To the best of our knowledge, this has never been reported before. Most of the previously reported glial tumors with transdural extension were cases of histologically proven glioblastomas and gliosarcomas, typically seen in the context of prior neurosurgical intervention and/or radiotherapy. This case adds to the limited literature on oligodendrogliomas with transdural extension. Further studies are necessary to elucidate the relationship between the incidence of transdural extension and molecular subtypes of oligodendrogliomas.
Citation: World Neurosurg. (2019) 130:10-12. https://doi.org/10.1016/j.wneu.2019.06.146 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
A 30-year-old gentleman presented to our institution with intermittent headaches and altered mental state for 2 months. Computed tomography and magnetic resonance imaging scans of the brain were performed (Figure 1) and revealed a large mass at the left frontal lobe, with transdural extension into the frontal and ethmoidal sinuses. The patient underwent surgical debulking of the tumor and histologic examination revealed sheets of neoplastic cells with small, rounded nuclei and perinuclear halo on a background of “chicken-wire” capillaries, scattered calcifications, high mitotic activity, and areas of necrosis. The tumor was positive for isocitrate dehydrogenase (IDH)1 R132H mutant protein, with retained ATRX nuclear staining and was negative for p53. Fluorescent in-situ hybridization for 1p and 19q showed 1p and 19q codeletion (Figure 2). Overall findings are in keeping with a World Health Organization (WHO) grade III anaplastic oligodendroglioma. No immediate postoperative complication was encountered, and the patient was planned for radiotherapy
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Figure 1. (A and B) Axial and (C) coronal computed tomography images show a heterogeneous mass lesion at the left frontal lobe with internal calcifications. Transdural extension and erosion of the inner wall of the left frontal sinus (black arrows in A and B) are noted. Axial T2-weighted magnetic resonance imaging (MRI) shows tumor extension into the ethmoid sinuses (D) and scalloping of the inner table of the left frontal cranial vault (E). Paramagnetic susceptibilities seen on susceptibility-weighted imaging are in keeping with tumoral calcifications (F). (GLJ) Contrast-enhanced MRI demonstrates a heterogeneously enhancing mass with transdural extension into the left frontal sinus (black arrows) and ethmoid sinuses (white arrows). Prominent tumoral vessels are also noted (dotted white arrows).
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.06.146
CLINICAL IMAGES AI PENG TAN ET AL.
OLIGODENDROGLIOMA WITH TRANSDURAL EXTENSION
been reported before. Two other cases of OGs with transdural extension and skull base invasion occur in patients with prior neurosurgical intervention.7,8 In 2017, Stib et al9 reported a case of anaplastic oligoastrocytoma (IDH-mutant but no evidence of 1p19q codeletion on fluorescence in-situ hybridization analysis) with anterior skull base erosion. Few other reported cases of gliomas with transdural extension were cases of histologically proven glioblastomas and gliosarcomas,7-13 typically seen in the context of prior neurosurgical intervention and/or radiotherapy.8,14,15 REFERENCES 1. Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131:803-820.
Figure 2. (A) Histomicrograph shows a cellular tumor composed of rather uniform round nuclei in fibrillary background. (B) The tumor is positive for IDH1 R132H-mutant protein. Dual-color fluorescent in-situ hybridization for 1p and 19q shows chromosome 1p (C) and 19q (D) codeletion, in a diploid background (1p32 probe ¼ red, 1q42 ¼ green, 19p13 ¼ green, 19q 13 ¼ red, DAPI nuclear counterstain ¼ blue).
(delivered using intensity-modulated radiation therapy technique) and adjuvant chemotherapy (procarbazine, lomustine, and vincristine). The patient was discharged in good health with no focal neurologic deficit, 2 weeks post surgery. Patient remained well at 6-month follow up. The current WHO classification of central nervous system tumors has made a radical leap from past editions by incorporating molecular criteria to the preexisting histologic diagnostic criteria.1 OGs have a strict molecular definition, characterized by the combined presence of IDH mutation and 1p19q codeletion, which carries both diagnostic and therapeutic implications. The signature whole chromosomal arm 1p and 19q loss is associated with prolonged survival and a favorable response to procarbazine, lomustine, and vincristine or temozolomide chemotherapy and/or radiation therapy. On histologic
examination, oligodendroglial features include uniform round-to-oval nuclei, perinuclear cytoplasmic clearing, and delicate branching “chicken-wire” vessels.2 Findings of high cellularity, cytologic atypia, necrosis, vascular proliferation, and significant mitotic activity are features of anaplastic OG.3 Surgical resection remains the mainstay of treatment for anaplastic OG, followed by adjuvant radiotherapy and chemotherapy.4 Just recently, Iwadate et al5 proposed that OG can be successfully treated with upfront chemotherapy alone, without compromising overall survival, irrespective of WHO grading, age, and extent of surgery. To avoid the potential risk of radiotherapy, many have opted to omit radiotherapy until tumor progression for anaplastic OGs using upfront chemotherapy.6 To the best of our knowledge, transdural extension of OG in the absence of prior surgery or radiotherapy had never
WORLD NEUROSURGERY 130: 10-12, OCTOBER 2019
2. Wood MD, Halfpenny AM, Moore SR. Applications of molecular neuro-oncology—a review of diffuse glioma integrated diagnosis and emerging molecular entities. Diagn Pathol. 2019;14:29. 3. Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114:97-109. 4. Reifenberger G, Wirsching H-G, KnobbeThomsen CB, Weller M. Advances in the molecular genetics of gliomas—implications for classification and therapy. Nat Rev Clin Oncol. 2017; 14:434-452. 5. Iwadate Y, Matsutani T, Hara A, et al. Eighty percent survival rate at 15 years for 1p/19q codeleted oligodendroglioma treated with upfront chemotherapy irrespective of tumor grade. J Neurooncol. 2019;141:205-211. 6. Panageas KS, Iwamoto FM, Cloughesy TF, et al. Initial treatment patterns over time for anaplastic oligodendroglial tumors. Neuro Oncol. 2012;14: 761-767. 7. Thakar S, Mohan D, Srinivasa R, Ghosal N, Hegde AS. Intracranial high grade glioma masquerading as a skull base lesion: report of two unusual cases. J Cancer Res Ther. 2015;11:1044. 8. Pompili A, Calvosa F, Caroli F, et al. The transdural extension of gliomas. J Neurooncol. 1993;15: 67-74. 9. Stib MT, Johnson M, Siu A, et al. High-grade glioma with anterior skull base erosion and intranasal extension: case report. J Neurosurg. 2017; 126:1484-1487. 10. Nguyen Q-BD, Perry A, Graffeo CS, et al. Gliosarcoma with primary skull base invasion. Case Rep Radiol. 2016;2016:1762195.
www.journals.elsevier.com/world-neurosurgery
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CLINICAL IMAGES AI PENG TAN ET AL.
OLIGODENDROGLIOMA WITH TRANSDURAL EXTENSION
11. Orita T, Nishizaki T, Furutani Y, Aoki H. Extradural nasal and orbital extension of malignant glioma. Surg Neurol. 1989;31:395-399.
14. Horiuchi T, Osawa M, Itoh N, et al. Extradural extension of glioblastoma multiforme into the oral cavity: case report. Surg Neurol. 1996;46:42-46.
commercial or financial relationships that could be construed as a potential conflict of interest.
12. D’Elia A, Fazzolari B, Arcovio E, et al. Transdural spread of glioblastoma with endonasal growth in a long-term survivor patient: case report and literature review. Turk Neurosurg. 2016;26:799-804.
15. Orita T, Nishizaki T, Furutani Y, Aoki H. Extradural nasal and orbital extension of malignant glioma. Case report. Surg Neurol. 1989;31: 395-399.
Citation: World Neurosurg. (2019) 130:10-12. https://doi.org/10.1016/j.wneu.2019.06.146
13. Aoyama I, Makita Y, Nabeshima S, Motomochi M, Masuda A. Extradural nasal and orbital extension of glioblastoma multiforme without previous surgical intervention. Surg Neurol. 1980;14:343-347.
12
www.SCIENCEDIRECT.com
Received 10 May 2019; accepted 19 June 2019
Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com
Conflict of interest statement: The authors declare that the article content was composed in the absence of any
1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.06.146
Anaplastic Oligodendroglioma with Transdural Extension Ai Peng Tan1, Char Loo Tan2, Yin Huei Pang2, Pin Lin Kei3
Key words Anaplastic oligodendroglioma - Transdural extension -
Abbreviations and Acronyms IDH: Isocitrate dehydrogenase OG: Oligodendroglioma WHO: World Health Organization From the Departments of 1Diagnostic Imaging and 2 Pathology, National University Health System; and 3 Department of Diagnostic Radiology, Ng Teng Fong General Hospital, Singapore To whom correspondence should be addressed: Ai Peng Tan, M.D. [E-mail: [email protected]]
Oligodendrogliomas, the third most common primary gliomas, have a strict molecular definition, characterized by the combined presence of isocitrate dehydrogenase mutation and 1p19q codeletion. Herein, we describe an extremely unusual case of molecularly defined anaplastic oligodendroglioma with transdural extension into the frontal and ethmoid sinuses, without prior neurosurgical intervention or radiotherapy. The molecular profile of the tumor is also provided. To the best of our knowledge, this has never been reported before. Most of the previously reported glial tumors with transdural extension were cases of histologically proven glioblastomas and gliosarcomas, typically seen in the context of prior neurosurgical intervention and/or radiotherapy. This case adds to the limited literature on oligodendrogliomas with transdural extension. Further studies are necessary to elucidate the relationship between the incidence of transdural extension and molecular subtypes of oligodendrogliomas.
Citation: World Neurosurg. (2019) 130:10-12. https://doi.org/10.1016/j.wneu.2019.06.146 Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
A 30-year-old gentleman presented to our institution with intermittent headaches and altered mental state for 2 months. Computed tomography and magnetic resonance imaging scans of the brain were performed (Figure 1) and revealed a large mass at the left frontal lobe, with transdural extension into the frontal and ethmoidal sinuses. The patient underwent surgical debulking of the tumor and histologic examination revealed sheets of neoplastic cells with small, rounded nuclei and perinuclear halo on a background of “chicken-wire” capillaries, scattered calcifications, high mitotic activity, and areas of necrosis. The tumor was positive for isocitrate dehydrogenase (IDH)1 R132H mutant protein, with retained ATRX nuclear staining and was negative for p53. Fluorescent in-situ hybridization for 1p and 19q showed 1p and 19q codeletion (Figure 2). Overall findings are in keeping with a World Health Organization (WHO) grade III anaplastic oligodendroglioma. No immediate postoperative complication was encountered, and the patient was planned for radiotherapy
10
www.SCIENCEDIRECT.com
Figure 1. (A and B) Axial and (C) coronal computed tomography images show a heterogeneous mass lesion at the left frontal lobe with internal calcifications. Transdural extension and erosion of the inner wall of the left frontal sinus (black arrows in A and B) are noted. Axial T2-weighted magnetic resonance imaging (MRI) shows tumor extension into the ethmoid sinuses (D) and scalloping of the inner table of the left frontal cranial vault (E). Paramagnetic susceptibilities seen on susceptibility-weighted imaging are in keeping with tumoral calcifications (F). (GLJ) Contrast-enhanced MRI demonstrates a heterogeneously enhancing mass with transdural extension into the left frontal sinus (black arrows) and ethmoid sinuses (white arrows). Prominent tumoral vessels are also noted (dotted white arrows).
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.06.146
CLINICAL IMAGES AI PENG TAN ET AL.
OLIGODENDROGLIOMA WITH TRANSDURAL EXTENSION
been reported before. Two other cases of OGs with transdural extension and skull base invasion occur in patients with prior neurosurgical intervention.7,8 In 2017, Stib et al9 reported a case of anaplastic oligoastrocytoma (IDH-mutant but no evidence of 1p19q codeletion on fluorescence in-situ hybridization analysis) with anterior skull base erosion. Few other reported cases of gliomas with transdural extension were cases of histologically proven glioblastomas and gliosarcomas,7-13 typically seen in the context of prior neurosurgical intervention and/or radiotherapy.8,14,15 REFERENCES 1. Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131:803-820.
Figure 2. (A) Histomicrograph shows a cellular tumor composed of rather uniform round nuclei in fibrillary background. (B) The tumor is positive for IDH1 R132H-mutant protein. Dual-color fluorescent in-situ hybridization for 1p and 19q shows chromosome 1p (C) and 19q (D) codeletion, in a diploid background (1p32 probe ¼ red, 1q42 ¼ green, 19p13 ¼ green, 19q 13 ¼ red, DAPI nuclear counterstain ¼ blue).
(delivered using intensity-modulated radiation therapy technique) and adjuvant chemotherapy (procarbazine, lomustine, and vincristine). The patient was discharged in good health with no focal neurologic deficit, 2 weeks post surgery. Patient remained well at 6-month follow up. The current WHO classification of central nervous system tumors has made a radical leap from past editions by incorporating molecular criteria to the preexisting histologic diagnostic criteria.1 OGs have a strict molecular definition, characterized by the combined presence of IDH mutation and 1p19q codeletion, which carries both diagnostic and therapeutic implications. The signature whole chromosomal arm 1p and 19q loss is associated with prolonged survival and a favorable response to procarbazine, lomustine, and vincristine or temozolomide chemotherapy and/or radiation therapy. On histologic
examination, oligodendroglial features include uniform round-to-oval nuclei, perinuclear cytoplasmic clearing, and delicate branching “chicken-wire” vessels.2 Findings of high cellularity, cytologic atypia, necrosis, vascular proliferation, and significant mitotic activity are features of anaplastic OG.3 Surgical resection remains the mainstay of treatment for anaplastic OG, followed by adjuvant radiotherapy and chemotherapy.4 Just recently, Iwadate et al5 proposed that OG can be successfully treated with upfront chemotherapy alone, without compromising overall survival, irrespective of WHO grading, age, and extent of surgery. To avoid the potential risk of radiotherapy, many have opted to omit radiotherapy until tumor progression for anaplastic OGs using upfront chemotherapy.6 To the best of our knowledge, transdural extension of OG in the absence of prior surgery or radiotherapy had never
WORLD NEUROSURGERY 130: 10-12, OCTOBER 2019
2. Wood MD, Halfpenny AM, Moore SR. Applications of molecular neuro-oncology—a review of diffuse glioma integrated diagnosis and emerging molecular entities. Diagn Pathol. 2019;14:29. 3. Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114:97-109. 4. Reifenberger G, Wirsching H-G, KnobbeThomsen CB, Weller M. Advances in the molecular genetics of gliomas—implications for classification and therapy. Nat Rev Clin Oncol. 2017; 14:434-452. 5. Iwadate Y, Matsutani T, Hara A, et al. Eighty percent survival rate at 15 years for 1p/19q codeleted oligodendroglioma treated with upfront chemotherapy irrespective of tumor grade. J Neurooncol. 2019;141:205-211. 6. Panageas KS, Iwamoto FM, Cloughesy TF, et al. Initial treatment patterns over time for anaplastic oligodendroglial tumors. Neuro Oncol. 2012;14: 761-767. 7. Thakar S, Mohan D, Srinivasa R, Ghosal N, Hegde AS. Intracranial high grade glioma masquerading as a skull base lesion: report of two unusual cases. J Cancer Res Ther. 2015;11:1044. 8. Pompili A, Calvosa F, Caroli F, et al. The transdural extension of gliomas. J Neurooncol. 1993;15: 67-74. 9. Stib MT, Johnson M, Siu A, et al. High-grade glioma with anterior skull base erosion and intranasal extension: case report. J Neurosurg. 2017; 126:1484-1487. 10. Nguyen Q-BD, Perry A, Graffeo CS, et al. Gliosarcoma with primary skull base invasion. Case Rep Radiol. 2016;2016:1762195.
www.journals.elsevier.com/world-neurosurgery
11
CLINICAL IMAGES AI PENG TAN ET AL.
OLIGODENDROGLIOMA WITH TRANSDURAL EXTENSION
11. Orita T, Nishizaki T, Furutani Y, Aoki H. Extradural nasal and orbital extension of malignant glioma. Surg Neurol. 1989;31:395-399.
14. Horiuchi T, Osawa M, Itoh N, et al. Extradural extension of glioblastoma multiforme into the oral cavity: case report. Surg Neurol. 1996;46:42-46.
commercial or financial relationships that could be construed as a potential conflict of interest.
12. D’Elia A, Fazzolari B, Arcovio E, et al. Transdural spread of glioblastoma with endonasal growth in a long-term survivor patient: case report and literature review. Turk Neurosurg. 2016;26:799-804.
15. Orita T, Nishizaki T, Furutani Y, Aoki H. Extradural nasal and orbital extension of malignant glioma. Case report. Surg Neurol. 1989;31: 395-399.
Citation: World Neurosurg. (2019) 130:10-12. https://doi.org/10.1016/j.wneu.2019.06.146
13. Aoyama I, Makita Y, Nabeshima S, Motomochi M, Masuda A. Extradural nasal and orbital extension of glioblastoma multiforme without previous surgical intervention. Surg Neurol. 1980;14:343-347.
12
www.SCIENCEDIRECT.com
Received 10 May 2019; accepted 19 June 2019
Journal homepage: www.journals.elsevier.com/worldneurosurgery Available online: www.sciencedirect.com
Conflict of interest statement: The authors declare that the article content was composed in the absence of any
1878-8750/$ - see front matter ª 2019 Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2019.06.146