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      Primary hypothalamic lymphoma with clinical findings mimicking pituitary apoplexy: a case report

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          Abstract

          Background

          Primary central nervous system lymphoma (PCNSL) is a rare but well-known extra-nodal lymphoma, which usually presents with non-Hodgkin B-cell lymphomas. PCNSL is generally located around the ventricle and is often detected as multiple lesions. It is rarely seen in the area of the hypothalamus.

          Case presentation

          We report the case of a 48-year-old Caucasian woman with progressive short-term memory deterioration, headache, mental confusion, diabetes insipidus (DI) and hypopituitarism. Early findings were suggestive of a pituitary apoplexy. The results of tests performed during the initial admission at the tertiary health center revealed hypernatremia, hypopituitarism and DI. Intravenous hydrocortisone treatment was initiated for the secondary adrenal insufficiency, and 75 mcg/day of levothyroxine was started for the secondary hypothyroidism on the fourth day following hydrocortisone treatment. A daily dose of 120 mg desmopressin melt tablet was started twice a day for polyuria/polydipsia after the patient’s volume status was balanced. A brain magnetic resonance imaging scan revealed a mass lesion in the hypothalamic area, which was surrounded by marked edema. Anti-edema treatment was initially started considering the suggestion by our neurosurgery team. The patient’s clinical and laboratory findings improved after the initiation of the anti-edema therapy. Afterwards, a biopsy was performed, which diagnosed a malignant diffuse large B-cell lymphoma. Subsequently, intravenous high-dose methotrexate-based therapy was started; however, after the second cycle of chemotherapy, the patient died due to sepsis.

          Conclusion

          In this report, we present a case of hypopituitarism that developed due to the mass effect of hypothalamic lymphoma with clinical findings of pituitary apoplexy. Intracranial masses may cause obvious endocrinological findings related to hypopituitarism, while vague findings may also be observed due to partial failure. Therefore, it is important to perform a comprehensive endocrinological examination at the time of diagnosis in patients with intracranial masses.

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          Most cited references24

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          Chemoimmunotherapy with methotrexate, cytarabine, thiotepa, and rituximab (MATRix regimen) in patients with primary CNS lymphoma: results of the first randomisation of the International Extranodal Lymphoma Study Group-32 (IELSG32) phase 2 trial

          Standard treatment for patients with primary CNS lymphoma remains to be defined. Active therapies are often associated with increased risk of haematological or neurological toxicity. In this trial, we addressed the tolerability and efficacy of adding rituximab with or without thiotepa to methotrexate-cytarabine combination therapy (the MATRix regimen), followed by a second randomisation comparing consolidation with whole-brain radiotherapy or autologous stem cell transplantation in patients with primary CNS lymphoma. We report the results of the first randomisation in this Article.
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            Primary CNS Lymphoma.

            Primary CNS lymphoma (PCNSL) is a rare form of extranodal non-Hodgkin lymphoma that is typically confined to the brain, eyes, and cerebrospinal fluid without evidence of systemic spread. The prognosis of patients with PCNSL has improved during the last decades with the introduction of high-dose methotrexate. However, despite recent progress, results after treatment are durable in half of patients, and therapy can be associated with late neurotoxicity. PCNSL is an uncommon tumor, and only four randomized trials and one phase III trial have been completed so far, all in the first-line setting. To our knowledge, no randomized trial has been conducted for recurrent/refractory disease, leaving many questions unanswered about optimal first-line and salvage treatments. This review will give an overview of the presentation, evaluation, and treatment of immunocompetent patients with PCNSL.
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              Differentiation between glioblastomas, solitary brain metastases, and primary cerebral lymphomas using diffusion tensor and dynamic susceptibility contrast-enhanced MR imaging.

              Glioblastomas, brain metastases, and PCLs may have similar enhancement patterns on MR imaging, making the differential diagnosis difficult or even impossible. The purpose of this study was to determine whether a combination of DTI and DSC can assist in the differentiation of glioblastomas, solitary brain metastases, and PCLs. Twenty-six glioblastomas, 25 brain metastases, and 16 PCLs were retrospectively identified. DTI metrics, including FA, ADC, CL, CP, CS, and rCBV were measured from the enhancing, immediate peritumoral and distant peritumoral regions. A 2-level decision tree was designed, and a multivariate logistic regression analysis was used at each level to determine the best model for classification. From the enhancing region, significantly elevated FA, CL, and CP and decreased CS values were observed in glioblastomas compared with brain metastases and PCLs (P < .001), whereas ADC, rCBV, and rCBV(max) values of glioblastomas were significantly higher than those of PCLs (P < .01). The best model to distinguish glioblastomas from nonglioblastomas consisted of ADC, CS (or FA) from the enhancing region, and rCBV from the immediate peritumoral region, resulting in AUC = 0.938. The best predictor to differentiate PCLs from brain metastases comprised ADC from the enhancing region and CP from the immediate peritumoral region with AUC = 0.909. The combination of DTI metrics and rCBV measurement can help in the differentiation of glioblastomas from brain metastases and PCLs.
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                Author and article information

                Contributors
                mehmetsozen07@gmail.com
                bsr2004_86@hotmail.com
                alevselek@gmail.com
                zeynepcanturk@hotmail.com
                barslan@kocaeli.edu.tr
                gezeremre@gmail.com
                Journal
                J Med Case Rep
                J Med Case Rep
                Journal of Medical Case Reports
                BioMed Central (London )
                1752-1947
                25 May 2021
                25 May 2021
                2021
                : 15
                : 293
                Affiliations
                [1 ]GRID grid.411105.0, ISNI 0000 0001 0691 9040, Department of Endocrinology and Metabolism, , Kocaeli University College of Medicine, ; Kocaeli, 41000 Turkey
                [2 ]GRID grid.411105.0, ISNI 0000 0001 0691 9040, Department of Pathology, , Kocaeli University College of Medicine, ; Kocaeli, Turkey
                Author information
                http://orcid.org/0000-0002-8428-1115
                Article
                2866
                10.1186/s13256-021-02866-7
                8145818
                34030739
                f79c10f4-4147-49b2-841b-ff7756ee83df
                © The Author(s) 2021

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 19 January 2021
                : 15 April 2021
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2021

                Medicine
                hypothalamic lymphoma,hypopituitarism,diabetes insipidus,empty sella
                Medicine
                hypothalamic lymphoma, hypopituitarism, diabetes insipidus, empty sella

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