Surgically benign recovery and histopathologically malignant transformation of a gliomatosis cerebri after radiotherapy: natural course or an unpredictable desired effect?

Ihsan Dogan; Atilla Erdem; Eyyub S. M. Al-Beyati; Onur Ozgural

doi:10.18203/2349-2902.isj20174902

Authors

Ihsan Dogan Department of Neurosurgery, Ankara University, Ankara, Turkey
Atilla Erdem Department of Neurosurgery, Ankara University, Ankara, Turkey
Eyyub S. M. Al-Beyati Department of Neurosurgery, Ankara University, Ankara, Turkey
Onur Ozgural Department of Neurosurgery, Ankara University, Ankara, Turkey

DOI:

https://doi.org/10.18203/2349-2902.isj20174902

Keywords:

Gliomatosis cerebri, Malignant transformation, Radiotherapy, Total removal

Abstract

Gliomatosis cerebri is a rare primary glial neoplasm of the central nervous system characterized by excessive involvement of the brain tissue. Dispersion and infiltrative growth pattern of this insidious pathology limit the utility of total surgical removal and are underlying causes of its poor prognosis. Furthermore, unresponsiveness of gliomatosis cerebri to radiotherapy and its classification as high grade or type 2 is reportedly associated with poor long-term survival. Contrary to our current knowledge, we report the case of a 54-year-old male who was diagnosed with gliomatosis cerebri. He presented with an exceptional clinical and pathological course and poor prognostic factors but was successfully treated with total surgical resection after radiotherapy. The present case findings indicate the potential utility of surgery, usually underestimated, as a curative treatment for gliomatosis cerebri. Clinicians should be aware of negative predictive factors that may help identify a small subset of patients suitable for total surgical tumor removal, as in the present case.

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References

Fuller GN, Kros JM. Gliomatosis cerebri. In: Lois DN, Ohgaki H, Wiestler OD, Cavenee WK, eds. WHO classification of tumours of the central nervous system. Lyon: IARC Press. 2007;1:50-2.

Lantos PL. Gliomatosis cerebri in tumors of the nervous system. Lyon: IARC Press. 2000:92-3.

Elshaikh MA, Stevens GH, Peereboom DM, Cohen BH, Prayson RA, Lee SY, et al. Gliomatosis cerebri: treatment results with radiotherapy alone. Cancer. 2002;1;95(9):2027-31.

Weinberg JS, Rhines LD, Cohen ZR, Langford L, Levin VA. Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases. Neurosurgery. 2003;52(1):216-23.

Louis DN, Ohgaki H, Wiestler OD, Cavenee WK. WHO Classification of Tumors of the Central nervous system. Lyon: IARC Press. 2007:50-2.

Inoue T, Kumabe T, Kanamori M, Sonoda Y, Watanabe M, Tominaga T. Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases. Neurosurg Rev. 2010;34(2):197-208.

Artigas J, Cervos-Navarro J, Iglesias JR, Ebhardt G. Gliomatosis cerebri: Clinical and histological findings. Clin Neuropathol. 1985;4(4):135-48.

Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, et al. The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol. 2002;61(3):215-25.

Jennings MT, Frenchman M, Shehab T, Johnson MD, Creasy J, LaPorte K, et al. Gliomatosis cerebri presenting as intractable epilepsy during early childhood. J Child Neurol. 1995;10(1):37-45.

Cozad SC, Townsend P, Morantz RA, Jenny AB, Kepes JJ, Smalley SR. Gliomatosis cerebri: results with radiation therapy. Cancer. 1996;78:1789-93.

Horst E, Micke O, Romppainen ML, Pyhtinen J, Paulus W, Schäfer U, et al. Radiation therapy approach in gliomatosis cerebri: case reports and literature review. Acta Oncol. 2000;39:747-51.

Kim DG, Yang HJ, Park IA, Chi JG, Jung HW, Han DH, et al. Gliomatosis cerebri: clinical features, treatment, and prognosis. Acta Neurochir (Wien). 1998;140(8):755-62.

Malamud N, Wise BL, Jones OW. Gliomatosis cerebri. J Neurosurg. 1952;9:409-17.

Levin N, Gomori JM, Siegal T. Chemotherapy as initial treatment in gliomatosis cerebri: results with temozolomide. Neurology. 2004;27:63(2):354-6.

Perkins GH, Schomer DF, Fuller GN, Allen PK, Maor MH. Gliomatosis cerebri: improved outcome with radiotherapy. Int J Radiat Oncol Biol Phys. 2003;15:56(4):1137-46.

Chen S, Tanaka S, Giannini C, Morris J, Yan ES, Buckner J, et al. Gliomatosis cerebri: clinical characteristics, management, and outcomes. J Neurooncol. 2013;112(2):267-75.

Park S, Suh YL, Nam DH, Kim ST. Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. Clin Neuropathol. 2009;28(2):73-82.

Kannuki S, Hirose T, Horiguchi H, Kageji T, Nagahiro S. Gliomatosis cerebri with secondary glioblastoma formation: report of two cases. Brain Tumor Pathol. 1998;15(2):111-6.

Duffau H, Taillandier L. New concepts in the management of diffuse low-grade glioma: Proposal of a multistage and individualized therapeutic approach. Neuro Oncol. 2015;17(3):332-42.

Duffau, Hugues. Diffuse Low-Grade Gliomas in Adults. Natural History, Interaction with the Brain, and New Individualized Therapeutic Strategies. Springer, London, 2013.

Senatus PB, McClelland S, Tanji K, Khandji A, Huang J, Feldstein N. The transformation of pediatric gliomatosis cerebri to cerebellar glioblastoma multiforme presenting as supra- and infratentorial acute disseminated encephalomyelitis. Case report. J Neurosurg. 2005;102(1):72-7.

Vates GE, Chang S, Lamborn KR, Prados M, Berger MS. Gliomatosis cerebri: a review of 22 cases. Neurosurgery. 2003;53:261-71.