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      Panhypopituitarism in a patient with a hypothalamic and pineal germ cell tumor

      research-article
      1 , , 1
      Endocrinology, Diabetes & Metabolism Case Reports
      Bioscientifica Ltd

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          Abstract

          Summary

          Central diabetes insipidus (DI) is characterized by decreased release of antidiuretic hormone, resulting in a variable degree of polyuria. The etiologies are variable, with the vast majority of cases either being idiopathic or resulting from primary or secondary tumors. Such tumors include craniopharyngioma, Langerhans cell histiocytosis, or a variety of inflammatory, vascular, or granulomatous diseases. It is exceptionally rare for pituitary adenomas to present with DI. We describe a young patient who first presented with symptoms consistent with DI. He was tested for DI and found to have panhypopituitarism due to a metastatic pineal germ cell tumor causing thickening of his pituitary stalk.

          Learning points
          • New onset central diabetes insipidus requires dedicated pituitary MRI for determination of etiology.

          • Germ cell tumors in the pineal and suprasellar regions most commonly cause central diabetes insipidus.

          • Determining the etiology of new-onset central diabetes insipidus can radically affect treatment.

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          Most cited references5

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          Intracranial germ cell tumours. A review with special reference to endocrine manifestations.

          Intracranial germ cell tumours (icGCTs) represent 3-15% of primary paediatric intracranial neoplasms with a considerable geographical variation in incidence. Ninety percent of patients diagnosed with icGCTs are under 20 years of age. Histologic characteristics and investigation of the tumour markers β-human chorionic gonadotropin (β-hCG) and alpha-fetoprotein (AFP) help define the different categories of icGCTs. The tumours are divided into two major groups called germinomas and non-germinomatous GCTs (NGGCTs). The clinical symptoms depend on the size and location of tumour in the brain, which is most commonly in the pineal or suprasellar region. Pineal GCTs often present with neurological symptoms because of their tendency to cause increased intracranial pressure. Suprasellar GCTs are often accompanied by endocrine abnormalities such as diabetes insipidus (DI), growth retardation and precocious or delayed puberty. A combination of clinical findings, endocrine and tumour marker evaluation, spinal fluid cytology, magnetic resonance imaging (MRI) and biopsy helps verifying the diagnosis of an icGCT. A summary of published data (n = 97) revealed that >90% of patients at diagnosis had at least one endocrine abnormality, DI being the most common (>80%). Classification of tumour is important for choice of treatment and for prognosis. A combination of chemotherapy and radiotherapy is often used, since most icGCTs have a great sensitivity to these treatment modalities. Endocrine symptoms are very frequently appearing in patients with icGCTs and they can present long before neuroimaging verification of tumour is possible. It is of the outmost importance to have the diagnosis of icGCTs in mind when children, adolescents and young adults are presenting with endocrine irregularities, because most icGCTs are very sensitive to radiotherapy and chemotherapy, and early onset of treatment is important in order to minimize morbidity and mortality.
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            Intracranial germinoma.

            Pineal region tumors make up less than 1% of all intracranial neoplasms, with the majority being of germ cell origin. We describe the diagnostic evaluation and treatment of a patient presenting with neurological deficits who was found to have a germinoma of the pineal gland.
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              Pituitary Dysfunction in Patients with Intracranial Germ Cell Tumors Treated with Radiotherapy.

              To evaluate the endocrine abnormalities in intracranial germ cell tumors (iGCTs) treated with radio-therapy (RT), and to discuss the effects of RT on pituitary functions.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                02 February 2022
                2022
                : 2022
                : 21-0021
                Affiliations
                [1 ]Department of Endocrinology , Lahey Hospital and Medical Center, Burlington, Massachusetts, USA
                Author notes
                Correspondence should be addressed to A Puchalski; Email: adam.puchalski@ 123456lahey.org
                Author information
                http://orcid.org/0000-0002-0808-6465
                Article
                EDM210021
                10.1530/EDM-21-0021
                8942320
                35266452
                e02ae406-17a5-4350-ba90-420a47847fcc
                © The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License..

                History
                : 17 December 2021
                : 02 February 2022
                Categories
                Case Reports

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