Mariasavina Severino1, Alessandro Consales2, Matteo Doglio3, Domenico Tortora4, Giovanni Morana4, Salvina Barra5, Paolo Nozza6, Maria Luisa Garrè3. 1. Neuroradiology Unit, Istituto Giannina Gaslini, Genoa, Italy. Electronic address: msavina@libero.it. 2. Neurosurgery Unit, Istituto Giannina Gaslini, Genoa, Italy. 3. Neuroncology Unit, Istituto Giannina Gaslini, Genoa, Italy. 4. Neuroradiology Unit, Istituto Giannina Gaslini, Genoa, Italy. 5. Department of Radiation Oncology, IRCCS San Martino IST, Genoa, Italy. 6. Pathology Unit, Istituto Giannina Gaslini, Genoa, Italy.
Abstract
BACKGROUND: Primary intradural extramedullary ependymomas are very rare tumors and have never been described in children. CASE DESCRIPTION: We report on an 11-year-old girl presenting with a 1-month history of neck pain, left arm weakness, paresthesia in the fingers of the left hand and gait disturbances. Magnetic resonance imaging on admission revealed an intradural extramedullary cystic lesion at the cervical level with craniospinal leptomeningeal nodules causing mild hydrocephalus. The multicystic lesion was surgically removed and neuropathologic examination revealed a World Health Organization grade II ependymoma. The patient underwent adjuvant radiotherapy with progressive reduction of the metastatic nodules. At her 2-year follow-up, the patient was symptom free with no evidence of recurrence on magnetic resonance imaging. CONCLUSIONS: Although a rare entity, intradural extramedullary ependymomas should be included in the differential diagnosis of intradural extramedullary lesions in children. Surgical treatment seems to play a pivotal role in the prognosis of these rare tumors, with a possible role for adjunctive radiotherapy in the case of recurrence, anaplastic transformation, and metastasis.
BACKGROUND: Primary intradural extramedullary ependymomas are very rare tumors and have never been described in children. CASE DESCRIPTION: We report on an 11-year-old girl presenting with a 1-month history of neck pain, left arm weakness, paresthesia in the fingers of the left hand and gait disturbances. Magnetic resonance imaging on admission revealed an intradural extramedullary cystic lesion at the cervical level with craniospinal leptomeningeal nodules causing mild hydrocephalus. The multicystic lesion was surgically removed and neuropathologic examination revealed a World Health Organization grade II ependymoma. The patient underwent adjuvant radiotherapy with progressive reduction of the metastatic nodules. At her 2-year follow-up, the patient was symptom free with no evidence of recurrence on magnetic resonance imaging. CONCLUSIONS: Although a rare entity, intradural extramedullary ependymomas should be included in the differential diagnosis of intradural extramedullary lesions in children. Surgical treatment seems to play a pivotal role in the prognosis of these rare tumors, with a possible role for adjunctive radiotherapy in the case of recurrence, anaplastic transformation, and metastasis.
Authors: David R Ghasemi; Martin Sill; Konstantin Okonechnikov; Andrey Korshunov; Stephen Yip; Peter W Schutz; David Scheie; Anders Kruse; Patrick N Harter; Marina Kastelan; Marlies Wagner; Christian Hartmann; Julia Benzel; Kendra K Maass; Mustafa Khasraw; Ronald Sträter; Christian Thomas; Werner Paulus; Christian P Kratz; Hendrik Witt; Daisuke Kawauchi; Christel Herold-Mende; Felix Sahm; Sebastian Brandner; Marcel Kool; David T W Jones; Andreas von Deimling; Stefan M Pfister; David E Reuss; Kristian W Pajtler Journal: Acta Neuropathol Date: 2019-08-14 Impact factor: 17.088
Authors: Daniel Diaz-Aguilar; Sergei Terterov; Alexander M Tucker; Shaina Sedighim; Rudi Scharnweber; Stephanie Wang; Catherine Merna; Shayan Rahman Journal: Surg Neurol Int Date: 2018-04-26