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睾丸原发性单纯血管肉瘤:一种罕见的恶性肿瘤。病例报告及文献复习。

Primary pure angiosarcoma of the testis: a vanishingly rare malignancy. Case report and literature review.

机构信息

Kingston Hospital NHS Foundation Trust, Galsworthy Road, Surrey, KT2 7QB, UK.

Department of Urology, Kent and Canterbury Hospital, Ethelbert Road, Canterbury, CT1 3NG, UK.

出版信息

BMC Urol. 2020 Oct 31;20(1):175. doi: 10.1186/s12894-020-00747-7.

DOI:10.1186/s12894-020-00747-7
PMID:33129286
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7602293/
Abstract

BACKGROUND

Primary pure angiosarcoma of the testis is an exceptionally rare testicular malignancy, which is poorly understood. We present the fifth and youngest case in the current medical literature. Additionally, all cases of angiosarcoma of the testicle, both occurring with associated germ cell tumour and without, were compared in an extended tabular format.

CASE PRESENTATION

A 56-year old man presented with unilateral scrotal pain, swelling and erythema. Ultrasonography revealed two testicular lesions with a high suspicion of malignancy but serum tumour markers were negative. A radical orchidectomy was performed with clear surgical margins. Diagnosis of primary pure angiosarcoma of the testis was confirmed on subsequent histopathology.

CONCLUSIONS

Primary pure angiosarcoma is a rare testicular neoplasm. We present the fifth case in the literature. Clinical and radiological features are non-specific. The diagnosis is purely histological, with the pathologist choosing immunohistochemistry based on abnormal morphology. Local invasiveness is variable but metastatic sites are typical for extra-gonadal angiosarcomas. Primary pure testicular angiosarcoma diagnosis confers a relatively better prognosis compared to angiosarcoma arising in the context of a testicular germ cell tumour. While extra-gonadal angiosarcomas are associated with high rates of local recurrence following resection, in all cases of testicular angiosarcoma there were no local recurrences following radical orchidectomy. Surgical resection remains the most effective treatment for both subtypes of testicular angiosarcoma.

摘要

背景

原发性单纯睾丸血管肉瘤是一种极为罕见的睾丸恶性肿瘤,目前人们对此了解甚少。我们报告了目前医学文献中的第五例也是年龄最小的病例。此外,还以扩展表格的形式比较了所有发生伴或不伴生殖细胞肿瘤的睾丸血管肉瘤病例。

病例介绍

一名 56 岁男性因单侧阴囊疼痛、肿胀和红斑就诊。超声检查显示两个睾丸病变,高度怀疑恶性肿瘤,但血清肿瘤标志物为阴性。随后进行了根治性睾丸切除术,切缘无肿瘤累及。随后的组织病理学检查证实为原发性单纯睾丸血管肉瘤。

结论

原发性单纯睾丸血管肉瘤是一种罕见的睾丸肿瘤。我们报告了文献中的第五例病例。临床表现和影像学特征无特异性。诊断完全基于组织病理学,病理学家根据异常形态选择免疫组织化学。局部侵袭性各不相同,但转移部位与睾丸外的血管肉瘤典型部位一致。与睾丸生殖细胞肿瘤相关的血管肉瘤相比,原发性单纯睾丸血管肉瘤的诊断预后相对较好。虽然睾丸外血管肉瘤在切除后局部复发率较高,但所有睾丸血管肉瘤患者在根治性睾丸切除术后均无局部复发。手术切除仍然是两种类型睾丸血管肉瘤最有效的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2986/7602293/d99f8e70d8f1/12894_2020_747_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2986/7602293/d99f8e70d8f1/12894_2020_747_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2986/7602293/d99f8e70d8f1/12894_2020_747_Fig1_HTML.jpg

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