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[纵隔畸胎瘤伴躯体成分恶性转化。临床报告]

[Mediastinal teratoma with malignant transformation of the somatic component. Clinical report].

作者信息

Gerardo Rita, Morgado Carolina, Calvo Dolores, Pinto Eugénia, Bravio Ivan, Castelão Nelson, Martelo Fernando

机构信息

Unidade Local de Saúde do Baixo Alentejo, Serviço de Cirurgia Geral do Hospital de Santo António dos Capuchos, Centro Hospital Lisboa Central.

出版信息

Rev Port Cir Cardiotorac Vasc. 2009 Jul-Sep;16(3):143-7.

Abstract

Mediastinal germ cell tumours (M-GCT) are rare forms of neoplasms compared with other tumours of the same location. They are classified in seminomas, malignant non-seminomatous GCT and teratomas. The malignant transformation of the somatic component of the teratoma, with sarcomatous or carcinomatous degeneration, is even more uncommon. We report the clinical case of a 32 year old man who presented with severe chest pain on the right hemithorax. The image exams revealed the existence of a large heterogeneous lesion with a diameter of 7.7 cm, with areas of lipomatous density and a calcic image with the appearance of a tooth, in the right projection of the anterior mediastinum, in the vicinity of the large vessels, compatible with teratoma. The transthoracic biopsy (CT guided) showed morphologic aspects of sarcoma. The patient was operated on with the en bloc resection of the mediastinal mass, right lung, a segment of the pericardium and the thymus. The pathological studies showed a teratoma with malignant transformation of the mesenquimatous component, with muscular differentiation into leiomiosarcoma and rabdomiosarcoma. After surgery, the patient was treated with a scheme of doxorubicin and ifosfamide. The most prominent concepts related to this clinical entity, as well as its treatment, are debated in this article, based on the most recent publications dedicated to the subject.

摘要

与同一部位的其他肿瘤相比,纵隔生殖细胞肿瘤(M-GCT)是一种罕见的肿瘤形式。它们分为精原细胞瘤、恶性非精原性生殖细胞肿瘤和畸胎瘤。畸胎瘤的体细胞成分发生恶性转化,并伴有肉瘤样或癌样变性,这种情况更为罕见。我们报告了一名32岁男性的临床病例,该患者右半胸出现严重胸痛。影像学检查显示在前纵隔右侧投影、大血管附近存在一个直径7.7 cm的巨大异质性病变,有脂肪密度区域以及呈牙齿外观的钙化影像,符合畸胎瘤表现。经胸活检(CT引导)显示出肉瘤的形态学特征。患者接受了纵隔肿块、右肺、部分心包和胸腺的整块切除手术。病理研究显示为畸胎瘤,其中间叶成分发生恶性转化,肌肉分化为平滑肌肉瘤和横纹肌肉瘤。术后,患者接受了阿霉素和异环磷酰胺方案的治疗。本文基于最近关于该主题的出版物,对与这一临床实体及其治疗相关的最突出概念进行了讨论。

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