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      Mature teratoma of the anterior mediastinum revealed by supravalvular pulmonary stenosis: a case report

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          Abstract

          The mature teratoma of the mediastinum is a benign tumour made up of several adult-type tissue components which result from the abnormal development of two or three embryonic layers (ectoderm, endoderm or mesoderm). We report the case of a 31-year-old patient, admitted for management of a mediastinal tumour, revealed by a pulmonary supravalvular stenosis, symptomatic of exertional dyspnea and mid-thoracic pain. The clinical examination objectified a respiratory rate at 18 c/min and an oxygen saturation at 96% in ambient air. The chest X-ray showed a mediastinal enlargement with a left paracardiac opacity, while the echocardiac showed a supravalvular pulmonary stenosis, hence the realization of a thoracic computed tomography (CT), which objectified a voluminous anterior mediastinal cystic formation and superior lateralized on the left, with an effect of compression on the trunk of the pulmonary artery. The median sternotomy allowed the complete exeresis of this tumor and the postoperative course was simple. The mature teratoma of the anterior mediastinum should be suspected in view of these clinical and radiological signs in a young patient. Surgery with a complete resection remains the treatment of choice.

          Most cited references13

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          Primary germ cell tumors in the mediastinum: a 50-year experience at a single Japanese institution.

          Primary germ cell tumors (GCT) of the mediastinum share similar clinical and biologic characteristics, which are different from their testicular counterpart. The purpose of the current study was to review the authors' institutional experience of mediastinal GCT, emphasizing the clinical spectrum, time trends of treatment, and recent advances in therapeutic modalities for malignant GCT. Between 1951 and 2000, 129 patients (70 males and 59 females) underwent surgical treatment for GCT, which accounted for 16.0% of the mediastinal tumors during the same period. There were 95 patients with mature teratomas, 13 patients with seminomas, and 21 patients with nonseminomatous germ cell tumors (NSGCT) with median ages of 26.4 years, 27.6 years, and 28.5 years, respectively. Adult patients with mature teratomas were less symptomatic (33.3%) than pediatric patients (52.4%). All patients with mature teratoma were cured by resection alone. Eight of the 13 patients (61.5%) with seminoma were symptomatic and 10 of 13 patients (83.3%) survived after surgery and radiation with/without chemotherapy. Nineteen of 21 patients (90.5%) with NSGCT had dyspnea, chest pain, and superior vena cava syndrome. Before 1985, patients received radical resection and/or chemoradiotherapy. However, all patients died due to disease progression, with a median survival period of 7.6 months. After 1986, six of eight patients received cisplatin-based chemotherapy, including three who received additional high-dose chemotherapy with a supporting peripheral blood stem cell transplantation until tumor markers normalized. Five patients who underwent salvage resection are currently disease free with a median survival period of 58.3 months. The institutional experience indicates the benign nature of mediastinal mature teratomas and the excellent prognosis for patients with seminomas after resection. An improved survival advantage was ensured with cisplatin-based preoperative chemotherapy in patients with NSGCT. Copyright 2003 American Cancer Society
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            Germ cell tumors of the mediastinum. A 30-year experience.

            The records of 98 consecutive patients (58 males and 40 females; median age, 27 years; age range, 2-64 years) who presented with a primary germ cell tumor (GCT) of the mediastinum between January 1960 and December 1990 were reviewed. There were 45 mature teratomas, 8 immature teratomas, 16 pure seminomas, and 24 malignant nonseminomatous GCT (MNSGCT). All patients with mature teratomas were cured by radical resection alone, except one patient who died intraoperatively. Among the eight patients with immature teratomas, five were treated before the advent of cisplatin treatment (two children younger than 15 years were cured by surgery alone and three adults died within 7 months after operation). Three patients underwent surgery followed by cisplatin-based chemotherapy (two are still alive and one died of an associated rhabdomyosarcoma). Thirteen of 16 patients with seminomas (81%) were cured by surgery either alone (5 patients) or with adjuvant radiation therapy (8 patients). Among the 24 MNSGCT, 10 were treated before 1980 without cisplatin and all but 1 died of disease progression. Fourteen patients were treated by initial high-dose cisplatin combination chemotherapy and 8 (57%) achieved complete remission (2 died of systemic mastocytosis development). Results indicate the benignity of mature teratomas of the mediastinum, the age-dependent clinical course of immature teratomas, and the excellent prognosis of seminomas. The improved survival advantage resulting from cisplatin-based chemotherapy in MNSGCT is impaired by the propensity to nongerminal solid tumor development and hematologic malignancies.
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              Mediastinal mature teratoma: imaging features.

              The purpose of this study was to characterize the cross-sectional imaging features of mediastinal mature teratomas. Sixty-six cases of mediastinal mature teratoma were retrospectively reviewed, noting clinical, radiologic, surgical, and pathologic findings. The patient population consisted of 38 females and 28 males who were 1 week to 67 years old (mean age, 23 years 10 months). Forty-six patients presented with symptoms, predominantly chest pain, dyspnea, and cough. Twenty patients were asymptomatic. Fifty-four tumors were found in the anterior mediastinum, two in the posterior mediastinum, one in the middle mediastinum, and nine in multiple compartments. CT studies (n = 66) showed masses of heterogeneous attenuation with varying combinations of soft tissue, fluid, fat, and calcium. Soft-tissue attenuation was observed in 66 tumors (100%), fluid in 58 tumors (88%), fat in 50 tumors (76%), and calcification in 35 tumors (53%). The most frequent combination of attenuations was soft tissue, fluid, fat, and calcium, which was noted in 26 masses (39%). The combination of soft tissue, fluid, and fat was seen in 16 tumors (24%); and the combination of soft tissue and fluid was seen in 10 tumors (15%). Fat-fluid levels were seen in seven masses (11%). Mediastinal mature teratoma typically manifests on CT as a heterogeneous anterior mediastinal mass containing soft-tissue, fluid, fat, or calcium attenuation, or any combination of the four. Fluid-containing cystic areas, fat, and calcification occur frequently. Cystic lesions without fat or calcium were seen in 15% of tumors. Fat-fluid levels, considered highly specific for the diagnosis of mediastinal mature teratoma, are uncommon. CT is the imaging technique of choice in the evaluation of these lesions.
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                Author and article information

                Contributors
                Journal
                Pan Afr Med J
                Pan Afr Med J
                PAMJ
                The Pan African Medical Journal
                The African Field Epidemiology Network
                1937-8688
                28 October 2022
                2022
                : 43
                : 109
                Affiliations
                [1 ]Faculty of Medicine, Cardiovascular Surgery Department, Monastir, Tunisia,
                [2 ]Faculty of Medicine, Pharmacy Department, Monastir, Tunisia
                Author notes
                [& ] Corresponding author: Hafedh Daly, Faculty of Medicine, Cardiovascular Surgery Department, Monastir, Tunisia. daly.hafedh@ 123456yahoo.fr
                Article
                PAMJ-43-109
                10.11604/pamj.2022.43.109.32333
                9834795
                36699971
                fdbf10a8-62aa-4003-afb6-f4c79d25575f
                Copyright: Hafedh Daly et al.

                The Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License ( https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 07 November 2021
                : 01 October 2022
                Categories
                Case Report

                Medicine
                teratoma,mature,mediastinum,supravalvular pulmonary stenosis,case report
                Medicine
                teratoma, mature, mediastinum, supravalvular pulmonary stenosis, case report

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