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      Gangliocytic Paraganglioma of Dorsolumbar Spine: A Rare Tumor at Rare Site

      case-report

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          Abstract

          Spinal paragangliomas are rare benign tumors. The gangliocytic paragangliomas (GP) of spine are even rarer. The GPs are almost exclusively seen in duodenum. In spine, the usual site of affection is cauda equina region. The involvement of other spinal levels is far less often and includes thoracic and cervical region. We report this case of GP involving the conus medullaris region and reaching up to the upper part of cauda equina. The prognosis of spinal GP is excellent after total excision, as it is classified under WHO Grade I tumor. The patient made a full recovery after tumor removal. Clinical, radiological, and pathological characteristics of this rare tumor are discussed here along with.

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          Most cited references10

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          Literature survey on epidemiology and pathology of gangliocytic paraganglioma

          Background Although gangliocytic paraganglioma (GP) has generally been regarded as a neuroendocrine tumor, its origin remains unclear. We therefore aimed to investigate the details of this disease by carefully analyzing and extracting common features of the disease as presented in selected publications. Methods We searched for English and Japanese cases of GP using the PubMed and IgakuChuoZasshi databases on August 2010. We then extracted and sampled raw data from the selected publications and performed appropriate statistical analyses. Additionally, we evaluated the expression of hormone receptors based on our previously reported case. Results 192 patients with GP were retrieved from the databases. Patient ages ranged from 15 y to 84 y (mean: 52.3 y). The gender ratio was 114:76 (male to female, 2 not reported). Maximum diameter of the tumors ranged from 5.5 mm to 100 mm (mean: 25.0 mm). The duodenum (90.1%, 173/192) was found to be the most common site of the disease. In 173 patients with duodenal GP, gastrointestinal bleeding (45.1%, 78/173) was found to be the most common symptom of the disease, followed by abdominal pain (42.8%, 74/173), and anemia (14.5%, 25/173). Rate of lymph node metastasis was 6.9% (12/173). Our statistical analysis indicated that significant differences were found for gender between GP within the submucosal layer and exceeding the submucosal layer. Furthermore, our immunohistochemical evaluation showed that both epithelioid and pancreatic islet cells showed positive reactivity for progesterone receptors. Conclusions Our literature survey revealed that there were many more cases of GP exceeding the submucosal layer than were expected. Meanwhile, our statistical analyses and immunohistochemical evaluation supported the following two hypotheses. First, vertical growth of GP might be affected by progesterone exposure. Second, the origin of GP might be pancreatic islet cells. However, it is strongly suspected that our data have been affected by publication bias and to confirm these hypotheses, further investigation is required.
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            Gastrointestinal ganglioneuromas; brief review with report of a duodenal ganglioneuroma.

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              Gangliocytic paragangliomas of the duodenum. A report of two cases with light and electron microscopic examination.

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                Author and article information

                Journal
                Asian J Neurosurg
                Asian J Neurosurg
                AJNS
                Asian Journal of Neurosurgery
                Wolters Kluwer - Medknow (India )
                1793-5482
                2248-9614
                Jul-Sep 2019
                : 14
                : 3
                : 907-910
                Affiliations
                [1 ] Department of Pathology, Dr. Ulhas Patil Medical College and Hospital, Jalgaon, Maharashtra, India
                [2 ] Department of Pathology, Mamata Medical College, Khammam, Telangana, India
                [3 ] Department of Pathology, Apollo Institute of Medical Sciences and Research, Chittoor, Andhra Pradesh, India
                Author notes
                Address for correspondence: Dr. Vaishali Baburao Nagose, Department of Pathology, Dr. Ulhas Patil Medical College and Hospital, Jalgaon Khurd, Jalgaon - 425 309, Maharashtra, India. E-mail: vaishali.nagose@ 123456gmail.com
                Article
                AJNS-14-907
                10.4103/ajns.AJNS_80_19
                6702988
                e4671fdd-2a71-46a2-99b9-760ae0146b88
                Copyright: © 2019 Asian Journal of Neurosurgery

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                Categories
                Case Report

                Surgery
                cauda equina,gangliocytic paragangliomas,spinal paragangliomas
                Surgery
                cauda equina, gangliocytic paragangliomas, spinal paragangliomas

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