In the December 2009 issue of Journal of Neuro-Oncology, volume 95, we read with great interest the article by Cerase et al. [1]. The authors reported the neuroradiological follow-up of a 56-year-old male patient with papillary tumor of the pineal region (PTPR). The title and conclusion of the article, we felt, did not accurately reflect their observation. Moreover, it was not clear to us why the authors opted for a simple clinical and radiological observation for such a long period of time. Nevertheless, their article, quite admirably, attempts to address a contemporary need for more knowledge on the natural history and the best ways of managing this relatively uncommon clinicopathological entity, formally codified in the 2007 edition of the World Health Organization Classification of Tumours of the Central Nervous System [2]. Since the first description of PTPR as a distinct pathological entity with specific morphology and immunohistochemistry [3], there has been growing evidence of different clinical and immunophenotypic profiles [1, 4–9]. Herein, we would like to present our experience with a shorter natural history of PTPR, hoping it will add more information to this important discussion. One year ago, a 29-year-old male patient came to our attention for a 1-month history of progressively worsening diplopia on lateral gaze and headaches. Brain computed tomography (CT) and magnetic resonance imaging (MRI) scan revealed the presence of a heterogenous lesion at the pineal region lesion, protruding in the Sylvian aqueduct and resulting in active three-ventricular hydrocephalus (Fig. 1a). Ventriculo-peritoneal shunt (VPS) was placed in November 2008, which resulted in immediate and complete resolution of both the symptoms and the hydrocephalus. In December 2008 (Fig. 1b), through occipital craniotomy (supracerebellar approach), subtotal removal of the tumor was performed. Histopathological examination concluded an ependymoma. Because follow-up brain MRI in March 2009 (Fig. 1c) revealed a radiological progression trend, a second histological review was recommended. Microscopic re-evaluation revealed a neoplasm with papillary architecture demonstrating cells with large eosinophilic cytoplasm and round-to-oval nuclei; some true rosettes were noted; mitoses were absent; immunostaining was positive for synaptophysin and cytokeratin, and focally positive for EMA; Ki-67 proliferative index was 3–4%, meeting the diagnostic criteria for PTPR. In August 2009, clinical deterioration (decreased upward gaze, nystagmus, and broad-based gait) was compatible with clear radiological progression of the tumor extending to the mesencephalon (Fig. 1d). Thus, in an 8-month period, this tumor presented an aggressive growth pattern. Because the patient refused a second surgery, cranial conformational radiotherapy was recommended. While waiting for the radiotherapy, he received two cycles of platinum-based chemotherapy. Currently, the patient is completing the radiotherapy with satisfactory clinical and radiological improvement. As of today, it remains difficult to predict the biological behavior, natural course, and appropriate management of PTPR. In our opinion, the different growth and recurrence rates are probably due to intrinsic characteristics of these tumors demonstrating a large spectrum of differentiation, sharing similar immunohistochemical features with G. Kaloshi (&) A. Rroji A. Lame L. Leka E. Haxhihyseni G. Vreto M. Petrela Department of Neurosurgery, University Hospital Center ‘‘Mother Theresa’’, 327 Rr e Dibres, Tirana, Albania e-mail: g_kaloshi@yahoo.com