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腹膜后畸胎瘤伴体部恶性转化:铂类化疗后继发于睾丸生殖细胞肿瘤转移的乳头状肾细胞癌。

Retroperitoneal teratoma with somatic malignant transformation: a papillary renal cell carcinoma in a testicular germ cell tumour metastasis following platinum-based chemotherapy.

机构信息

Department of Urology, University Hospital Zürich, Zürich, Switzerland.

出版信息

BMC Urol. 2013 Feb 12;13:9. doi: 10.1186/1471-2490-13-9.

Abstract

BACKGROUND

Malignant transformation describes the phenomenon in which a somatic component of a germ cell teratoma undergoes malignant differentiation. A variety of different types of sarcoma and carcinoma, all non-germ cell, have been described as a result of malignant transformation.

CASE PRESENTATION

A 33-year-old man presented with a left testicular mass and elevated tumour markers. Staging investigations revealed retroperitoneal lymphadenopathy with obstruction of the left ureter and distant metastases. Histopathology from the left radical orchiectomy showed a mixed germ cell tumour (Stage III, poor prognosis). The ureter was stented and four cycles of cisplatin, etoposide and bleomycin chemotherapy administered. After initial remission, the patient recurred four years later with a large retroperitoneal mass involving the renal vessels and the left ureter. Left retroperitoneal lymph node dissection with en-bloc resection of the left kidney was performed.Histopathology revealed a germ cell tumour metastasis consisting mainly of mature teratoma. Additionally, within the teratoma a papillary renal cell carcinoma was found. The diagnosis was supported by immunohistochemistry showing positivity for AMACR, CD10 and focal expression of RCC and CK7. There was no radiological or histo-pathological evidence of a primary renal cell cancer.

CONCLUSIONS

To the best of our knowledge, malignant transformation into a papillary renal cell carcinoma has not been reported in a testicular germ cell tumour metastasis following platinum-based chemotherapy. This histological diagnosis might have implications for potential future therapies. In the case of disease recurrence, renal cell cancer as origin of the recurrent tumour has to be excluded because renal cell carcinoma metastases would not respond well to the classical germ cell tumour chemotherapy regimens.

摘要

背景

恶性转化描述了生殖细胞瘤的体细胞成分发生恶性分化的现象。多种不同类型的肉瘤和癌,均非生殖细胞来源,已被描述为恶性转化的结果。

病例介绍

一名 33 岁男性因左侧睾丸肿块和肿瘤标志物升高就诊。分期检查显示腹膜后淋巴结肿大伴左侧输尿管梗阻和远处转移。左侧根治性睾丸切除术的组织病理学检查显示混合生殖细胞瘤(III 期,预后不良)。输尿管放置支架,并给予顺铂、依托泊苷和博来霉素化疗 4 个周期。初始缓解后,患者 4 年后复发,出现累及肾血管和左侧输尿管的巨大腹膜后肿块。行左侧腹膜后淋巴结清扫术和左肾整块切除术。组织病理学检查显示生殖细胞瘤转移,主要由成熟畸胎瘤组成。此外,在畸胎瘤中还发现了乳头状肾细胞癌。免疫组织化学显示 AMACR、CD10 阳性和 RCC 和 CK7 局灶性表达,支持该诊断。没有影像学或组织病理学证据表明存在原发性肾细胞癌。

结论

据我们所知,在接受铂类化疗后的睾丸生殖细胞瘤转移中,尚未有报道恶性转化为乳头状肾细胞癌。这种组织学诊断可能对未来的潜在治疗方案有影响。在疾病复发的情况下,必须排除肾细胞癌作为复发性肿瘤的起源,因为肾细胞癌转移对经典生殖细胞瘤化疗方案反应不佳。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/77ed/3577457/719d6c193c0e/1471-2490-13-9-1.jpg

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