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      Management of dual intracranial germinoma by radiotherapy alone

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          Abstract

          Synchronous intracranial germ cell tumor in the pineal and suprasellar region is rare. They represent only 5–10% of all intracranial germinomas. They are also known by the entity “double midline atypical teratoma” and are common in the second decade of life. We report a case of an 11-year-old girl having dual midline intracranial lesions with obstructive hydrocephalus treated by ventriculo-peritoneal shunt. Diagnosis of germinoma was made on the basis of imaging and elevated beta-human chorionic gonadotropin in cerebrospinal fluid. Radiotherapy was instituted, which resulted in the total disappearance of both the lesions. Clinical expression, diagnosis and management strategies are discussed.

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          Intracranial germ cell tumors.

          Intracranial germ cell tumors are a heterogeneous group of lesions which occur in children and adults. Within the classification of intracranial germ cell tumors, there are a variety of different tumor types which carry different prognoses. The diagnosis of an intracranial germ cell tumor usually requires histological information, but a subgroup of tumors will secrete specific tumor markers, including alpha-fetoprotein and beta-human chorionic gonadotropin, which may obviate the need for surgical intervention. The management of intracranial germ cell tumors in both children and adults remains unsettled. Germinomas have a good prognosis, as over 90% of patients can be effectively treated with radiation therapy. The dose and volume of radiation therapy needed for disease control is not well established, and controversy exists concerning the need for whole brain or craniospinal radiation therapy for localized tumors. Germinomas are also chemosensitive and recent reports suggest that the dose and volume of radiation therapy required for disease control can be lessened with the addition of adjuvant chemotherapy. The outcome for patients with nongerminomatous germ cell tumors is less favorable. Radiation therapy alone will result in disease control in 40%-60% of patients. The addition of chemotherapy to radiation therapy may improve the rate of survival.
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            Current concepts and future strategies in the management of intracranial germinoma.

            Radiation therapy is the backbone in the management of intracranial germinoma. In localized disease chemotherapy followed by whole brain irradiation is the present standard providing cure rates in excess of 90%. Craniospinal irradiation alone in metastatic disease provides equally excellent outcome. Chemotherapy is able to convert macroscopic to microscopic disease permitting a dose reduction to the tumor site and possibly the ventricular system and is investigated in prospective trials. Chemotherapy alone cannot replace radiotherapy as sole treatment. Whole-ventricular radiotherapy followed by a boost to tumor site without chemotherapy might be feasible. New treatment technologies such as intensity-modulated radiotherapy or proton therapy permit a dose reduction to non-target brain. Data on functional outcome are conflicting and based on small heterogeneous series only mandating prospective investigations.
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              Intracranial multiple midline germinomas: is histological verification crucial for therapy?

              In this report we present two patients with intracranial multiple midline tumours in the suprasellar region and pineal gland. We postulate that in a patient with multiple midline tumours and normal values of the tumour markers human chorionic gonadotropin and alpha-fetoprotein in serum and cerebrospinal fluid, the only possible diagnosis is a germinoma. In such a situation no histological confirmation is required to start low-dose radiotherapy.
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                Author and article information

                Journal
                J Pediatr Neurosci
                J Pediatr Neurosci
                JPN
                Journal of Pediatric Neurosciences
                Medknow Publications & Media Pvt Ltd (India )
                1817-1745
                1998-3948
                Jan-Mar 2015
                : 10
                : 1
                : 38-40
                Affiliations
                [1]Department of Neurosurgery, Sri Aurobindo Institute of Medical Sciences and P.G. Institute, Indore, Madhya Pradesh, India
                Author notes
                Address for correspondence: Dr. Rakesh Gupta, 101, Sukun Villa 3/2, New Palasia, Indore - 452 001, Madhya Pradesh, India. E-mail: drrakeshgupta@ 123456yahoo.com
                Article
                JPN-10-38
                10.4103/1817-1745.154330
                4395943
                dc1e5474-e809-46fe-9e27-b4a6d5171e5b
                Copyright: © Journal of Pediatric Neurosciences

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Case Report

                Neurosciences
                alpha feto protein,beta-human chorionic gonadotropin,germinomas,radiotherapy,synchronous lesions

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