Sex Cord-Gonadal Stromal Tumors

性索性腺间质瘤
  • 文章类型: Journal Article
    同时广泛伴有性索及平滑肌样分化是低级别子宫间质肉瘤(low-grade endometrial stromal sarcoma,LGESS)的一种少见的形态,这种肿瘤常会掩盖LGESS的形态学特征,具有很大的迷惑性,在病理诊断中非常容易引起误诊,需要引起病理医师的高度关注。本文报道1例LGESS伴有广泛性索及平滑肌样分化的病例,肿瘤大部区域表现为性索样结构(约70%),部分为平滑肌瘤样形态(约25%),仅局灶见到短梭形细胞,间质富于螺旋小动脉血管的区域,需考虑子宫内膜间质肿瘤的可能。免疫组织化学及荧光原位杂交检测结果均支持LGESS。结合本病例和相关文献复习,本文探讨了LGESS伴有性索及平滑肌分化的诊断及主要鉴别诊断要点,以提高对该肿瘤的认识,为肿瘤治疗及判断预后提供依据。.
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  • 文章类型: Case Reports
    背景:硬化性间质瘤(SST)是一种罕见的卵巢肿瘤,通常表现为卵巢的单侧实体瘤,没有特定的临床或放射学表现。最终的治疗方法是手术切除。
    方法:我们介绍了4例女性硬化性卵巢间质瘤患者,其临床特征与卵巢恶性病变相似。有趣的是,我们的2例患者的抑制素B水平升高,所有患者均接受手术(卵巢切除术)治疗,无疾病复发.
    结论:肿瘤的宏观特征通常是非特异性的,常常提示可能的恶性肿瘤。因此诊断总是基于组织病理学报告。
    BACKGROUND: Sclerosing stromal tumors (SST) are rare ovarian neoplasms that often appear as solid unilateral tumors of the ovary with no specific clinical or radiological presentation. The definitive treatment is surgical removal.
    METHODS: Our article presents four cases of female patients with sclerosing stromal ovarian tumor with clinical characteristics mimicking malignant ovarian lesions. Interestingly, two of our cases had elevated levels of inhibin B. All patients were treated with surgery (oophorectomy) and had no disease recurrence.
    CONCLUSIONS: Tumors\' macroscopic features are usually non-specific and often suggestive of possible malignancy, therefore diagnosis is always based on histopathological report.
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  • 文章类型: Case Reports
    McCluggege于2015年在文献中首次报道了卵巢外性索基质的显微异位增生。之后,很少有类似的案例被描述。在这里,我们报告了第14例显微镜下异位性索基质增生,第3例位于盆腔腹膜。这些罕见病例的临床病史表明其良性性质。了解这种组织学模式对于鉴别诊断(例如恶性病理和转移性疾病)很重要。
    Microscopic heterotopic extraovarian sex cord-stromal proliferations were first reported in the literature in 2015 by McCluggege. Afterwards, few similar cases have been described. Herein, we report the fourteenth case of microscopic heterotopic sex cord-stromal proliferation and the third case sited in the pelvic peritoneum. The clinical history of these rare cases suggests their benign nature. Knowledge of this histological pattern is important for differential diagnoses such as malignant pathologies and metastatic diseases.
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  • 文章类型: Case Reports
    背景:具有环状小管的性索间质肿瘤是一种罕见的肿瘤,占所有卵巢恶性肿瘤的不到1%。然而,它们的特点是晚期复发,这可能是诊断和治疗后的30年。
    方法:一名16岁女性白种人患者在我科接受了IA期卵巢性索间质肿瘤伴环状小管治疗。她接受了左输卵管卵巢切除术和同侧盆腔淋巴结活检,没有辅助治疗。在失去随访16年后,她因闭经而被发现。通过放射学和血清抑制素B水平升高来诊断复发。病人接受了肿瘤切除,左段结肠切除术,和主动脉旁淋巴结清扫术,因为肿块大量粘附于左侧结肠系膜。组织学证实诊断无转移性淋巴结。无辅助治疗。该患者再次失去随访4年,并因闭经而重新表现。血清抑制素B水平高。建议第二次复发,患者接受了腹腔镜手术。我们做了左盆腔和主动脉旁淋巴结清扫术,手术后3个月患者怀孕。
    结论:具有环状小管的性索间质肿瘤是一种生长缓慢的卵巢肿瘤,具有很高的复发和转移潜力。手术是治疗的主要手段。由于这些肿瘤的稀有性,它们通常没有被怀疑,因此在初次手术前不完全分期;诊断是通过组织学检查做出的。这些患者的预后未知,他们需要长期随访。
    BACKGROUND: Sex cord-stromal tumors with annular tubules are a rare tumor accounting for less than 1% of all ovarian malignancies. However, they are characterized by very late recurrence, which can be as late as 30 years after diagnosis and treatment.
    METHODS: A 16-year-old female Caucasian patient was treated in our department for a stage IA ovarian sex cord-stromal tumors with annular tubules. She underwent a left salpingo-oophorectomy and ipsilateral pelvic node biopsy with no adjuvant treatment. She was seen for amenorrhea after being lost to follow up for 16 years. The diagnosis of recurrence was made by radiology and the elevation of serum inhibin B level. The patient underwent resection of the tumor, left segmental colectomy, and paraaortic lymphadenectomy because the mass was massively adherent to the left mesocolon. Histology confirmed the diagnosis with no metastatic lymph nodes. No adjuvant therapy was indicated. The patient was lost to follow-up again for 4 years and re-presented for amenorrhea. Serum inhibin B level was high. A second recurrence was suggested, and the patient underwent a laparoscopic surgery. We performed left pelvic and paraaortic lymphadenectomy, and 3 months after surgery the patient was pregnant.
    CONCLUSIONS: Sex cord-stromal tumors with annular tubules is a slow-growing ovarian tumor with a high potential for recurrence and metastasis. Surgery is the mainstay of treatment. Due to the rarity of these tumors, they are often unsuspected and thus incompletely staged before primary surgery; the diagnosis is made by histological examination. The prognosis of these patients is unknown, and they require long-term follow-up.
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  • 文章类型: Case Reports
    硬化性间质瘤是一种良性性索间质瘤,这通常发生在第二个和第三个十年的年龄。由于罕见发生并且可以模仿恶性病变,因此对该实体的术中诊断提出了巨大挑战。一名15岁女性出现右卵巢肿块。血清标志物在正常范围内。放射学评估显示,大小为16×14×9cm的大量非均相增强的实性囊性腹盆腔肿块,两个卵巢均未分别可见,少数钙化区域伴有轻度腹水。进行了开放性膀胱切除术。囊肿壁的一部分被送往术中冷冻切片。据报道为硬化性间质瘤,术后标本也证实了这一点。钙化和骨化区域也被确定为其他发现。我们报告了这个病例,因为不常见,突出其他组织学特征,并做了文献综述,尤其是集中于术中诊断。
    UNASSIGNED: Sclerosing stromal tumor is a benign sex cord-stromal tumor, that commonly occurs in the second and third decades of age. Intraoperative diagnosis of this entity poses a great challenge because of the rare occurrence and can mimic malignant lesions. A 15-year-old female presented with a right ovarian mass. Serum markers were within normal limits. The radiological evaluation showed a large heterogeneously enhancing solid cystic abdominopelvic mass of size 16 × 14 × 9 cm with non-visualization of both ovaries separately and a few areas of calcification with mild ascites. An open cystectomy was performed. A part of the cyst wall was sent for an intraoperative frozen section. It was reported as sclerosing stromal tumor, and the post-operative specimen also confirmed the same. Areas of calcification and ossification were also identified as additional findings. We reported this case because of the uncommon occurrence, highlighting additional histological features, and also did a literature review, especially focussing on the intra-operative diagnosis.
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  • 文章类型: Review
    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。
    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.
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  • 文章类型: Case Reports
    一名怀孕12周零3天的妇女因患有严重的高血压而被转诊至急诊科,尽管积极的医疗管理,仍然不受控制。肾素和醛固酮水平明显升高,超过了妊娠早期的典型水平,与右卵巢囊肿的发现一起指出了肾外肾癌的可能诊断。并决定在妊娠16周时进行右侧卵巢切除术.组织学显示出与类固醇细胞肿瘤最一致的染色模式,导致诊断为继发于卵巢类固醇细胞肿瘤的难治性高血压。卵巢切除术后血液生化迅速恢复正常,和我们的病人的高血压缓慢解决,允许大幅减少降压药物的需求和成功的妊娠结局。
    A woman 12 weeks and 3 days pregnant was referred to the emergency department with significant hypertension which, despite aggressive medical management, remained uncontrolled. Markedly elevated levels of renin and aldosterone beyond what is typical in early pregnancy were present, which together with the finding of a right ovarian cyst pointed to the possible diagnosis of an extrarenal reninoma.and the decision was made to perform a right-sided oophorectomy at 16 weeks gestation. Histology demonstrated a staining pattern most consistent with a steroid cell tumour leading to the diagnosis of refractory hypertension secondary to an ovarian steroid cell tumour. Post oophorectomy blood biochemistry rapidly returned to normal, and our patient\'s hypertension slowly resolved allowing for a large reduction in antihypertensive agent requirements and a successful pregnancy outcome.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    卵巢黏液瘤是一种罕见的卵巢良性肿瘤,类似发生在软组织的黏液瘤,为第5版WHO女性生殖系统肿瘤分类中新增的肿瘤类型。本文报道1例卵巢黏液瘤,局部合并硬化性间质瘤,复习相关文献,总结其临床病理特征,以提高病理医师对该病的认识。.
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  • 文章类型: Journal Article
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