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具有不寻常组织病理学表现的未成熟纵隔畸胎瘤:一例多谱系、体细胞型恶性转化病例报告并文献复习

Immature mediastinal teratoma with unusual histopathology: A case report of multi-lineage, somatic-type malignant transformation and a review of the literature.

作者信息

Mustafa Osama M, Mohammed Shamayel F, Aljubran Ali, Saleh Waleed N

机构信息

aCollege of Medicine, Alfaisal University bDepartment of Pathology and Laboratory Medicine cDepartment of Medical Oncology, Oncology Center dDivision of Thoracic Surgery, Department of Surgery, King Faisal Specialist Hospital and Research Center (KFSH&RC), Riyadh, Saudi Arabia.

出版信息

Medicine (Baltimore). 2016 Jun;95(26):e3378. doi: 10.1097/MD.0000000000003378.

DOI:10.1097/MD.0000000000003378
PMID:27367976
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4937890/
Abstract

Germ cell tumors (GCTs) represent a well-recognized group of heterogeneous neoplasms with diverse clinical, histopathological, diagnostic, and prognostic characteristics. We present a rare case of a locally aggressive, chemotherapy-resistant immature mediastinal teratoma with a peculiar histological finding of a multilineage somatic-type malignant degeneration. A 21-year-old male patient presented with a 3-week history of persistent, blood-tinged productive cough and shortness of breath. A contrast-enhanced computed tomography (CT) scan of the chest showed a heterogeneous mass occupying the right hemithorax and abutting on adjacent structures. CT-guided biopsy was consistent with immature teratoma. Combination chemotherapy with bleomycin, etoposide, and cisplatin was initiated, albeit without success; the mass showed interval progression in size, and surgical resection through clamshell incision was performed. Histological assessment of the resected mass confirmed the diagnosis of immature teratoma and revealed an extensive multilineage malignant differentiation into sarcomatous, carcinomatous, and melanomatous components. The patient underwent an uneventful recovery but presented 2 months later with extensive liver and bone melanomatous metastases. In this report, relevant findings from the literature are also highlighted. Despite being exceptionally rare, such tumors carry poor prognosis. Understanding the clinicopathological characteristics and biological behavior of such tumors may provide an insight into interventions tailored to improve the otherwise dismal disease outlook.

摘要

生殖细胞肿瘤(GCTs)是一组公认的异质性肿瘤,具有多样的临床、组织病理学、诊断和预后特征。我们报告了一例罕见的局部侵袭性、化疗耐药的未成熟纵隔畸胎瘤,其具有多谱系体细胞型恶性退变这一特殊的组织学表现。一名21岁男性患者,有持续3周的伴有血丝的咳痰和气短病史。胸部增强计算机断层扫描(CT)显示一个异质性肿块占据右半胸并毗邻相邻结构。CT引导下活检结果符合未成熟畸胎瘤。开始使用博来霉素、依托泊苷和顺铂进行联合化疗,但未成功;肿块大小出现进行性增大,遂通过蛤壳状切口进行手术切除。对切除肿块的组织学评估证实了未成熟畸胎瘤的诊断,并显示其广泛地多谱系恶性分化为肉瘤样、癌样和黑色素瘤样成分。患者术后恢复顺利,但2个月后出现广泛的肝脏和骨黑色素瘤转移。在本报告中,还突出了文献中的相关发现。尽管此类肿瘤极为罕见,但预后较差。了解此类肿瘤的临床病理特征和生物学行为可能有助于深入了解为改善原本不佳的疾病预后而量身定制的干预措施。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/1812090a6944/medi-95-e3378-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/cf40ec9cbfe9/medi-95-e3378-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/4417b4fd8971/medi-95-e3378-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/8b04d7bc09ae/medi-95-e3378-g003.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/d1007318121e/medi-95-e3378-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/1812090a6944/medi-95-e3378-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/cf40ec9cbfe9/medi-95-e3378-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/4417b4fd8971/medi-95-e3378-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/8b04d7bc09ae/medi-95-e3378-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/8bf8d5e6f4c0/medi-95-e3378-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/d1007318121e/medi-95-e3378-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0945/4937890/1812090a6944/medi-95-e3378-g006.jpg

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