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一名曾患隆突性皮肤纤维肉瘤的年轻男性的纵隔肿物:转移性疾病还是不同的实体——病例报告

Mediastinal mass in a young man with a previous history of dermatofibrosarcoma protuberans: metastatic disease or different entity-a case report.

作者信息

Reyntiens Pieter, Driessen Ann, Rasschaert Marika, Snoeckx Annemiek

机构信息

Antwerp University Hospital and University of Antwerp, Edegem, Belgium.

出版信息

Mediastinum. 2021 Dec 25;5:35. doi: 10.21037/med-21-18. eCollection 2021.

DOI:10.21037/med-21-18
PMID:35118340
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8794385/
Abstract

A 34-year-old man presented to the clinician with a persisting cutaneous swelling in the lower back. Diagnosis of dermatofibrosarcoma protuberans (DFSP) was made based on histopathologic examination of the resected mass and molecular testing. Further diagnostic workup with computed tomography (CT) showed a mass in the prevascular mediastinum, composed of a soft tissue component, areas of fat density and some small foci of calcification. Location of the mass and typical imaging findings of fat component and calcification were suggestive for a teratoma. Robotic assisted surgery with complete resection of the mediastinal mass was performed. Histopathological examination showed that there was no relationship with the primary tumor and confirmed the diagnosis of a teratoma. Teratomas are to be classified as mature, immature and teratomas with malignant transformation. In this patient, histopathology shows a teratoma with somatic-type malignancy. The patient was treated with adjuvant radiotherapy. Upon progressive disease with lung metastasis, pleural metastasis and lymph node metastasis, palliative chemotherapy was started. Knowledge of classic metastatic patterns is essential in imaging oncologic patient. Since DFSP rarely metastasizes, a metastatic origin of the mediastinal mass would not be likely. In a patient with typical imaging findings of a mature teratoma, associated findings such as adenopathy and pulmonary nodules should alert the radiologist to the possibility of a teratoma with somatic-type malignancy.

摘要

一名34岁男性因下背部持续皮肤肿胀就诊于临床医生。根据切除肿块的组织病理学检查和分子检测,诊断为隆突性皮肤纤维肉瘤(DFSP)。进一步的计算机断层扫描(CT)检查显示前纵隔有一肿块,由软组织成分、脂肪密度区域和一些小钙化灶组成。肿块的位置以及脂肪成分和钙化的典型影像学表现提示为畸胎瘤。进行了机器人辅助手术,完整切除了纵隔肿块。组织病理学检查显示与原发肿瘤无关,并确诊为畸胎瘤。畸胎瘤可分为成熟型、未成熟型和恶性转化型畸胎瘤。在该患者中,组织病理学显示为具有体细胞型恶性肿瘤的畸胎瘤。患者接受了辅助放疗。在出现肺转移、胸膜转移和淋巴结转移的疾病进展后,开始了姑息化疗。了解经典的转移模式对于肿瘤患者的影像学检查至关重要。由于DFSP很少发生转移,纵隔肿块不太可能起源于转移瘤。在具有成熟畸胎瘤典型影像学表现的患者中,如淋巴结肿大和肺结节等相关表现应提醒放射科医生注意存在体细胞型恶性肿瘤的畸胎瘤的可能性。

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本文引用的文献

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Robot-assisted thoracoscopic surgery for mediastinal masses: a single-institution experience.机器人辅助胸腔镜手术治疗纵隔肿物:单机构经验
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Mediastinal Mature Teratoma With Malignant Carcinomatous Transformation (Somatic-Type Malignancy) With Metastatic Course.伴有恶性癌转化(体细胞型恶性肿瘤)及转移过程的纵隔成熟畸胎瘤。
Int J Surg Pathol. 2015 Dec;23(8):682-4. doi: 10.1177/1066896915591583. Epub 2015 Jun 24.
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Current treatment options for dermatofibrosarcoma protuberans.隆突性皮肤纤维肉瘤的当前治疗选择。
Expert Rev Anticancer Ther. 2015;15(8):901-9. doi: 10.1586/14737140.2015.1052799. Epub 2015 May 31.
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Virchows Arch. 2013 Nov;463(5):731-5. doi: 10.1007/s00428-013-1476-3. Epub 2013 Aug 25.
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AIRP best cases in radiologic-pathologic correlation: mediastinal mature cystic teratoma.放射学-病理学相关性最佳病例:纵隔成熟囊性畸胎瘤。
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