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睾丸混合性生殖细胞肿瘤合并恶性转化为软骨肉瘤:一种非常罕见且侵袭性的疾病。

Testicular Mixed Germ Cell Tumor Combined with Malignant Transformation to Chondrosarcoma: A Very Rare and Aggressive Disease.

机构信息

Department of Internal Medicine, King Abdullah Medical City, Makkah, Saudi Arabia.

Department of Internal Medicine, Umm Alqura University, Makkah, Saudi Arabia.

出版信息

Am J Case Rep. 2020 Jun 26;21:e922933. doi: 10.12659/AJCR.922933.

Abstract

BACKGROUND Testicular mixed germ cell tumors (GCTs) represent a spectrum of malignancies that differ in terms of histopathology, clinical complications, and overall outcome. A variety of aggressive combinations containing different histological types have been described among such testicular tumors. However, a histopathology characterized by a combination of teratoma and choriocarcinoma, as seen in this case, in which the teratomatous component shows a secondary transformation to chondrosarcoma, is considered very rare. CASE REPORT The patient presented with progressive hemoptysis and dyspnea secondary to bilateral pulmonary cannon-ball lesions indicative of a metastatic process. His workup was remarkable for primary testicular cancer complicated by liver metastasis and very high levels of B-HCG at more than 175 000 mlU/ml. He deteriorated quickly with no improvement following the first cycle of Etoposide/Cisplatin (EP) chemotherapy regimen and died 15 days after starting cancer treatment. Such non-seminomatous GCTs with extrapulmonary visceral metastasis associated with very high tumor markers are deemed poor risk based on the International Germ Cell Cancer Collaborative Group (IGCCCG) criteria, with a reported 5-year overall survival rate reaching up to 73%. CONCLUSIONS This case is considered unique in terms of rapid clinical deterioration and lack of improvement following the standard EP chemotherapy regimen. This unusual dramatic presentation should draw attention to the possible association between the aggressiveness of the disease and its very rare histopathology.

摘要

背景

睾丸混合生殖细胞肿瘤(GCT)是一组在组织病理学、临床并发症和总体预后方面存在差异的恶性肿瘤。在这些睾丸肿瘤中,已经描述了多种包含不同组织学类型的侵袭性组合。然而,像本例中所见的以畸胎瘤和绒毛膜癌组合为特征的组织病理学,其中畸胎瘤成分发生继发性转化为软骨肉瘤,被认为非常罕见。

病例报告

患者因双侧肺炮弹状病变导致进行性咯血和呼吸困难而就诊,这些病变提示转移过程。他的检查结果显著提示原发性睾丸癌合并肝转移,且β-HCG 水平非常高,超过 175000 mlU/ml。他在接受依托泊苷/顺铂(EP)化疗方案的第一个周期后迅速恶化,且没有改善,在开始癌症治疗后 15 天死亡。根据国际生殖细胞癌协作组(IGCCCG)标准,此类伴有极高肿瘤标志物的非精原细胞瘤性 GCT 伴肺外内脏转移被认为是高危风险,报告的 5 年总生存率高达 73%。

结论

就标准 EP 化疗方案后迅速的临床恶化和无改善而言,本例被认为是独特的。这种不寻常的戏剧性表现应该引起人们对疾病侵袭性与其非常罕见的组织病理学之间可能存在的关联的关注。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c35c/7334836/c1770be28d70/amjcaserep-21-e922933-g001.jpg

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