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原发性睾丸外精原细胞瘤:一种罕见病理类型的诊断

The primitive extratesticular seminoma: diagnosis of a rare pathology.

作者信息

Saba Luca

机构信息

.

出版信息

Acta Biomed. 2017 Apr 28;88(1):82-85. doi: 10.23750/abm.v88i1.5601.

DOI:10.23750/abm.v88i1.5601
PMID:28467339
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6166189/
Abstract

UNLABELLED

Background: The Primitive Extratesticular Seminoma is a very rare condition and represents 3% of germ cell tumors; it is an indeterminate origin disease, whose diagnosis is often complicated by a nonspecific and highly variable clinical finding.

CASE PRESENTATION

A caucasian 55 years old male, non-smoker, arrived to our centre with cough, severe respiratory distress and dysphagia, in a context of the superior vena cava syndrome. A Computed Tomography was performed, which shows the presence of a mediastinal anterior mass with aorto-pulmonary window and left paracardiac invasion. A biopsy of the mediastinal mass was performed with mediastinoscopy; the hystological diagnosis was seminoma. After, first of all the 18FDG PET-CT is performed, which shows the presence of an intense hypermetabolism (SUV max=20.3 and metabolic volume 867 cc) at the level of bulky mediastinal mass, with paratracheal, aorto-pulmonary window and left paracardiac invasion. The mass presents also a sternal manubrium invasion. There were no other detectable tissue metabolic alterations with the 18FDG PET-CT and, in particular, the testicles examination was negative. A bilateral testicular ultrasound (US) was executed, which confirms the absence of testicular germ tumor. Clinical laboratory tests show a significant increase of beta-HCG (123 IU/L); AFP is negative. A final diagnosis of primitive extratesticular seminoma was carried.

CONCLUSIONS

The Primitive Extratesticolar Seminoma is a rare patology, and, for its massive size at the onset diagnosis, curable in early stage often only with radiochemotherapy. The Diagnostic Imaging and Nuclear Medicine, as Testicular Ultrasound, the CT with contrast medium and the 18FDG PET-CT total body examination, are fundamental to the staging and localisation. MRI sometimes is useful for testicular evaluation. The biopsy is needed for tissue characterization.

摘要

未标注

背景:原发性睾丸外精原细胞瘤是一种非常罕见的疾病,占生殖细胞肿瘤的3%;它是一种起源不明的疾病,其诊断常常因非特异性且高度可变的临床表现而复杂化。

病例报告

一名55岁的白种男性,不吸烟,因上腔静脉综合征出现咳嗽、严重呼吸窘迫和吞咽困难,前来我们中心就诊。进行了计算机断层扫描,显示纵隔前部有一个肿块,侵犯了主动脉肺窗和左心旁区域。通过纵隔镜对纵隔肿块进行了活检;组织学诊断为精原细胞瘤。之后,首先进行了18氟脱氧葡萄糖正电子发射断层扫描计算机断层扫描(18FDG PET-CT),显示在巨大纵隔肿块处存在强烈的高代谢(最大标准摄取值=20.3,代谢体积867立方厘米),伴有气管旁、主动脉肺窗和左心旁侵犯。肿块还侵犯了胸骨柄。18FDG PET-CT未发现其他可检测到的组织代谢改变,特别是睾丸检查为阴性。进行了双侧睾丸超声检查,证实没有睾丸生殖细胞瘤。临床实验室检查显示β-人绒毛膜促性腺激素(β-HCG)显著升高(123国际单位/升);甲胎蛋白(AFP)为阴性。最终诊断为原发性睾丸外精原细胞瘤。

结论

原发性睾丸外精原细胞瘤是一种罕见的疾病,由于其在诊断时体积巨大,早期通常仅通过放化疗可治愈。诊断性影像学和核医学检查,如睾丸超声、增强CT和18FDG PET-CT全身检查,对于分期和定位至关重要。磁共振成像(MRI)有时对睾丸评估有用。需要进行活检以明确组织特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2373/6166189/9edd29d8dbb6/ACTA-88-82-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2373/6166189/7bc430feafdd/ACTA-88-82-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2373/6166189/bd3013e42acc/ACTA-88-82-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2373/6166189/9edd29d8dbb6/ACTA-88-82-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2373/6166189/7bc430feafdd/ACTA-88-82-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2373/6166189/bd3013e42acc/ACTA-88-82-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2373/6166189/9edd29d8dbb6/ACTA-88-82-g003.jpg

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Nuclear Protein of the Testis Midline Carcinoma Masquerading as a Primary Mediastinal Seminoma.伪装成原发性纵隔精原细胞瘤的睾丸中线癌核蛋白
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