CLINICAL STUDY Temozolomide after radiotherapy in recurrent ‘‘low grade’’ diffuse brainstem glioma in adults Germa ´n Reyes-Botero • Florence Laigle-Donadey • Karima Mokhtari • Nadine Martin-Duverneuil • Jean-Yves Delattre Received: 21 May 2014 / Accepted: 9 August 2014 / Published online: 20 August 2014 Ó Springer Science+Business Media New York 2014 Abstract Diffuse brainstem glioma is a rare disease in adults. Radiotherapy (RT) is usually considered to be the standard treatment. However, the role of chemotherapy in treating relapses after RT is unclear, and this study aimed to assess the use of temozolomide (TMZ) in this situation. We conducted a retrospective analysis of patients from our database with ‘‘low grade’’ adult diffuse infiltrating brain- stem glioma who received TMZ at relapse after failing RT. The patients were diagnosed by histology or MRI criteria compatible with a low-grade glioma. The tumors were localized in the pons, medulla oblongata or midbrain, excluding supratentorial or infratentorial tumors that had infiltrated the brainstem secondarily. The patients’ clinical and radiological responses were assessed, and their pro- gression free survival (PFS) and overall survival (OS) time were estimated. Fifteen adult patients (median age 34 years) fulfilled the inclusion criteria. Histological ana- lysis was available in 5 cases and showed grade II oligo- dendroglioma (2 cases), grade II oligoastrocytoma (2 cases), and grade II astrocytoma (1 case). Ten patients were selected by MRI criteria only. All patients received RT as initial treatment and had a median PFS of 34.2 months (95 % CI 24.1–44.2). The median KPS at the time of relapse was 80. TMZ was administered orally at 150–200 mg/m 2 for 5 days, every 28 days. Clinical improvement after TMZ was observed in 9 cases (60 %), whereas radiological assessment detected responses in 6/15 cases, including 4 partial and 2 minor responses. The estimated median PFS after TMZ was 9.5 months (95 % CI 7.9–11), and the median OS was 14.4 months (95 % CI 10.5–18.2). Grade 3 thrombopenia was observed in 26 % of cases. TMZ could be useful after RT failure in adult patients with recurrent diffuse ‘‘low grade’’ brainstem glioma. Keywords Adult brainstem glioma Á Diffuse intrinsic pontine glioma Á Temozolomide Introduction Brainstem gliomas are primary tumors of the central ner- vous system originating in the pons, midbrain or medulla oblongata. [1] In contrast to diffuse intrinsic pontine gli- oma (DIPG) in children, which occur frequently and have a dismal outcome, these tumors are rare in adults and have a better prognosis. [2, 3] Because the risks of biopsy often prevent definite histological confirmation, MRI criteria are often used to evaluate brainstem tumors and to decide on the appropriate treatment strategy. [3, 4] In adults, contrast enhancement on MRI has been associated with high-grade tumors and short survival. [3] On the other hand, an MRI clinical-radiological entity characterized by a diffuse intrinsic non-enhancing brainstem infiltration sometimes G. Reyes-Botero Á F. Laigle-Donadey (&) Á J.-Y. Delattre Service de Neurologie 2, AP-HP, Groupe Hospitalier Pitie ´- Salpe ˆtrie `re, 47-83 boulevard de l’Ho ˆpital, 75013 Paris, France e-mail: florence.laigle-donadey@psl.aphp.fr K. Mokhtari Laboratoire de Neuropathologie Raymond Escourolle AP-HP, Groupe Hospitalier Pitie ´-Salpe ˆtrie `re, Paris, France N. Martin-Duverneuil Service de Neuroradiologie, AP-HP, Groupe Hospitalier Pitie ´- Salpe ˆtrie `re, Paris, France J.-Y. Delattre Inserm U 1127, CNRS UMR 7225, Sorbonne Universite ´s, UPMC Univ Paris 06 UMR S 1127, Centre de Recherche de L’Institut du Cerveau et de la Moelle e ´pinie `re (CRICM) UMR- S975, Universite ´ Pierre et Marie Curie-Paris 6, Paris, France 123 J Neurooncol (2014) 120:581–586 DOI 10.1007/s11060-014-1589-9