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      Long-Term Survival of Bifocal Paraganglioma: A Case Report

      case-report
      1 , , 2 , 2 , 1 , 2
      ,
      Cureus
      Cureus
      surgery, secreting tumor, head and neck cancer, radiation therapy, bifocal paraganglioma

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          Abstract

          Paragangliomas are sympathetic and parasympathetic para-ganglia neuroendocrine tumors of the autonomic nervous system.

          We analyzed a bifocal paraganglioma case of a 52-year-old patient in December 2013 with hearing loss and right ear pain, headaches, episodes of vomiting, and abdominal pain ten months before her medical consultation. The diagnosis of a right tympano-jugular glomus paraganglioma was based on cerebral magnetic resonance imaging and treated with radiotherapy. In 2016, the patient presented with worsening digestive symptoms; therefore, a second mesocolic localization was suspected by abdominal computed tomography and was histologically confirmed on the resection specimen of the mass. The surgery was the only treatment. After a follow-up of 11 years, the patient remained in good condition.

          Paraganliomas are rare tumors, their bifocal location in our patient represents an even rarer entity. Given the nonspecific symptomatology, the diagnosis of the retroperitoneal location simultaneously with that of the head and neck was difficult.

          Our objective is to emphasize the staging workup for paraganglioma, although it is mostly a benign tumor with slow growth.

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          Most cited references12

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          Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline.

          The aim was to formulate clinical practice guidelines for pheochromocytoma and paraganglioma (PPGL).
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            Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma.

            Pheochromocytoma or secreting paraganglioma may recur after initial surgery. Diagnostic methods have improved in recent decades. We determined whether features at presentation have changed over time and are associated with long-term outcome. In 192 patients with pheochromocytoma/paraganglioma seen between 1975 and 2003, we compared time from onset of hypertension to first operation, total metanephrine excretion, tumor size and site, the proportion of cases presenting as incidentalomas, and the probability of recurrence according to date of operation (divided into quartiles). The duration of hypertension and tumor size at first operation decreased significantly over time. Right-sided adrenal tumors were more frequent and larger, excreted greater amounts of metanephrines, and presented more frequently as incidentalomas than left-sided tumors. Age, familial disease, and tumor site and size were independent predictors of recurrence. The risk of recurrence was 3.4-fold higher in patients with familial disease than in those with sporadic tumors; it was, respectively, 3.1- and 11.2-fold higher in patients with right adrenal and extraadrenal tumors than in patients with left adrenal tumors. In conclusion, pheochromocytomas/paragangliomas can now be diagnosed earlier, with smaller tumors, and more frequently as incidentalomas. Familial, right adrenal, and extraadrenal tumors recur more frequently than left adrenal tumors.
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              National Cancer Data Base report on malignant paragangliomas of the head and neck.

              Malignant paragangliomas of the head and neck are rare, with previous reports limited to nine or fewer patients. The current review included 59 cases extracted from the National Cancer Data Base that were diagnosed between 1985-1996. The primary criterion for inclusion in the current study was verified metastatic spread from a paraganglioma of the head and neck. Patterns of presentation and treatment as well as clinically relevant associations were demonstrated in contingency tables. Relative survival was used for analysis of outcome. The average patient age at presentation was 44 years, and gender distribution was equivalent. Metastases were confined to regional lymph nodes in the majority of cases (68.6%), with carotid body tumors found to have an even higher rate of regional confinement (93.8%). Surgery was the most common treatment (76.3%). The use of adjuvant irradiation for regionally confined disease increased across time, from 27% (1985-1990) to 46% (1991-1996). The 5-year relative survival rate was 59.5% (76.8% for regionally confined carcinoma and 11.8% for distant metastasis). Among patients who were followed until death, those treated with adjuvant irradiation had a longer median survival (45 months) compared with those patients who were treated with surgery alone (12 months). Malignant paraganglioma represents metastatic spread of a tumor type that, when restricted to the site of origin, is considered benign. Metastases from malignant paragangliomas of the head and neck usually are regionally confined. The primary management of a recognized malignancy should be directed toward complete surgical removal of the primary tumor and regional lymph nodes. Postoperative irradiation may be beneficial in slowing the progression of residual disease. Copyright 2002 American Cancer Society. DOI 10.1002/cncr.10252
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                26 April 2024
                April 2024
                : 16
                : 4
                : e59048
                Affiliations
                [1 ] Radiation Therapy, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR
                [2 ] Radiation Oncology, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Mohammed First University, Oujda, MAR
                Author notes
                Article
                10.7759/cureus.59048
                11128073
                38800343
                df68230c-c16b-4306-bd93-441c89a6aeb2
                Copyright © 2024, Mouhib et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 25 April 2024
                Categories
                Radiation Oncology
                Radiology

                surgery,secreting tumor,head and neck cancer,radiation therapy,bifocal paraganglioma

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