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Metastatic mediastinal mature teratoma with malignant transformation in a young man with an adenocarcinoma in a Klinefelter's syndrome: Case report and review of the literature.
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- Author(s): Le Fèvre, C.1; Vigneron, C.1; Schuster, H.2; Walter, A.3; Marcellin, L.4; Massard, G.5; Lutz, P.6; Noël, G.1,7
- Source:
Cancer Radiothérapie. May2018, Vol. 22 Issue 3, p255-263. 9p.- Subject Terms:
- Source:
- Additional Information
- Abstract: Malignant transformation of mediastinal mature teratoma is extremely rare and worsens the prognosis of the disease. Transformation can appear synchronously to or several years after the initial diagnosis. Clinical and radiological signs can orientate the clinician but the definitive diagnosis is obtained thanks to histology. An 11 year-old boy presented with a mediastinal mature teratoma and bone and pulmonary metastases. He received six cycles of chemotherapy combining etoposide, ifosfamide, cisplatin, followed by resection of a 16 × 14 × 9 cm mediastinal mass. Karyotype analysis revealed the presence of an additional sex chromosome X (47 XXY) pathognomonic of Klinefelter's syndrome. Ten years later, sciatalgia revealed malignant transformation of a pre-existing sacral bone metastasis into gastrointestinal adenocarcinoma. The patient received four cycles of chemotherapy combining oxaliplatin, 5-fluorouracil and cetuximab. This treatment was followed by a complete resection of the sacral metastasis and completed with adjuvant irradiation of 54 Gy in 30 daily fractions. Twelve months after the diagnosis of relapse, the patient remained alive without disease. To our knowledge, this is the first case of adenocarcinoma developed in bone metastases of a mediastinal mature teratoma in a boy with a Klinefelter's syndrome. We propose a review of the literature and an analysis of 20 others published cases of mediastinal teratoma with malignant transformation into adenocarcinoma. [ABSTRACT FROM AUTHOR]
- Abstract: Résumé La transformation maligne d’un tératome mature du médiastin est rare et aggrave le pronostic de la maladie. Une transformation maligne peut apparaître de manière synchrone ou jusqu’à plusieurs années après le diagnostic initial. Le diagnostic est clinique et radiologique, mais le diagnostic de certitude est histologique. Nous rapportons le cas d’un jeune garçon âgé de 11 ans qui a été atteint d’un tératome mature du médiastin métastatique osseux et pulmonaire d’emblée. Il a été traité par six cycles d’étoposide, ifosfamide, cisplatine, suivis de la résection chirurgicale de la tumeur médiastinale, qui mesurait 16 × 14 × 9 cm. À l’examen du caryotype du patient, il existait un chromosome X surnuméraire (47 XXY) et le diagnostic de syndrome de Kinefelter a été posé. Dix ans plus tard, une sciatalgie a révélé l’existence d’une transformation maligne en adénocarcinome de type intestinal au sein d’une métastase osseuse sacrée pré-existente. Le patient a reçu quatre cycles d’oxaliplatine, 5-fluoro-uracile et cétuximab. Il a bénéficié ensuite d’une résection complète de la métastase sacrée et d’une irradiation postopératoire de 54 Gy en 30 fractions. Le patient était en vie à 12 mois du diagnostic de transformation maligne du tératome. À notre connaissance, il s’agit du premier cas de tératome mature médiastinal métastatique avec transformation maligne en adénocarcinome associé au syndrome de Klinefelter rapporté dans la littérature. Nous proposons une revue de la littérature et une analyse des 20 cas de tératome mature du médiastin avec transformation maligne en adénocarcinome retrouvés dans la littérature. [ABSTRACT FROM AUTHOR]
- Abstract: Copyright of Cancer Radiothérapie is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
- Abstract:
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