关键词: Desmin Gonadal stromal tumor Inhibin S100 protein Smooth muscle actin Testicular neoplasm Testis-sparing surgery

Mesh : Male Humans Adult Middle Aged Sex Cord-Gonadal Stromal Tumors / surgery pathology Testicular Neoplasms / surgery pathology S100 Proteins Neoplasms, Germ Cell and Embryonal

来  源:   DOI:10.1186/s13256-024-04393-7   PDF(Pubmed)

Abstract:
BACKGROUND: Sex cord gonadal stromal tumors compose less than 10% of all testicular neoplasms and consist of a variety of histological subtypes. In 2016, the World Health Organization introduced a novel subtype, the myoid gonadal stromal tumor, that consists of spindle-shaped cells with immunohistologic features of muscle cells. Only few cases have been reported to date. Due to its rarity and owing to its only recent introduction, the current knowledge about myoid gonadal stromal tumor is limited, and particularly, appropriate clinical management is still ill-defined.
METHODS: A 47-year-old man of Caucasian descent presented with nonspecific scrotal discomfort. A roundish and well demarcated hypoechoic mass of 8.5 mm in diameter was detected in the cranial region of the left testis. Serum tumor marker levels were within normal ranges. Testis-sparing surgery revealed a 9-mm whitish, hard mass with sharp surgical margin. Histologically, the neoplasm consisted of microfibrillar tissue with spindle-shaped cells harboring elongated nuclei. Immunohistochemical work-up disclosed expression of desmin, small muscle actin, and S100 protein giving evidence for the myogenic nature of the neoplastic cells. There was no indication of malignancy, neither histologically nor clinically. Follow-up of 1 year was uneventful.
CONCLUSIONS: A literature survey revealed 22 previous cases of myoid gonadal stromal tumor. The median age was 37 years, the median size of the neoplasm was 20 mm, and there was no side-preponderance. Myoid gonadal stromal tumor is not much different from other subtypes of gonadal stromal tumors nor from testicular gem cell tumors regarding age and laterality; however, tumor size is smaller in myoid gonadal stromal tumors than in germ cell tumors. Although rarely performed so far, testis-sparing surgery probably constitutes an appropriate treatment of this neoplasm. Myoid gonadal stromal tumor represents an emerging novel entity of benign testicular new growths that caregivers of patients with testicular tumors should be aware of.
摘要:
背景:性索性腺间质肿瘤占所有睾丸肿瘤的不到10%,并且由多种组织学亚型组成。2016年,世界卫生组织引入了一种新的亚型,肌样性腺间质瘤,由具有肌肉细胞免疫组织学特征的梭形细胞组成。迄今为止,只有少数病例报告。由于它的稀有性和最近才推出的,目前关于肌样性腺间质瘤的知识是有限的,尤其是,适当的临床管理仍然不明确.
方法:一名47岁的高加索血统男子表现为非特异性阴囊不适。在左睾丸的颅骨区域检测到直径为8.5mm的圆形且边界清晰的低回声质量。血清肿瘤标志物水平在正常范围内。保留睾丸的手术显示出9毫米的白色,硬块有锋利的手术边缘。组织学上,肿瘤由微纤维组织和梭形细胞组成,这些细胞带有细长的核。免疫组织化学检查揭示了结蛋白的表达,小肌肉肌动蛋白,和S100蛋白为肿瘤细胞的生肌性质提供了证据。没有恶性肿瘤的迹象,无论是组织学还是临床。1年的随访是顺利的。
结论:文献调查显示22例肌样性腺间质瘤。中位年龄为37岁,肿瘤的中位大小为20毫米,也没有优势.肌样性腺间质瘤与性腺间质瘤的其他亚型和睾丸宝石细胞瘤在年龄和侧性方面没有太大区别;然而,肌样性腺间质瘤的肿瘤大小小于生殖细胞肿瘤。虽然到目前为止很少表演,保留睾丸的手术可能是这种肿瘤的适当治疗方法。肌样性腺间质瘤代表了睾丸良性新生长的新兴实体,睾丸肿瘤患者的护理人员应注意。
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