Pineal germ cell tumors: Two cases with review of histopathologies and biomarkers

Pineal germ cell tumors (GCTs) are primarily seen in pediatric and Asian populations. These tumors are divided into germinomatous and non-germinomatous GCTs (NGGCTs). GCTs are thought to arise by misplacement of totipotent stem cells en route to gonads during embryogenesis. Intracranial GCTs display an affinity to develop along the pineal-suprasellar axis and have variable manifestations dependent upon the location of the tumor. Management and outcomes are driven by histopathologies. In this study, we highlight two cases of pineal GCTs and present a review of the literature with an emphasis on histopathologies and biomarkers.     

小儿睾丸恶性生殖细胞瘤的回顾性分析

目的：探索小儿睾丸恶性生殖细胞瘤合理的治疗方案。方法：I期无高危因素18例行睾丸高位切除术后密切随访（A组）;I期有高危因素和Ⅱ期31例分为13例行睾丸切除加腹膜后淋巴结清扫加不规则化疗（B组）,10例行睾丸切除加腹膜后淋巴结清扫加规则化疗（C组）,8例行睾丸切除加规则化疗（D组）。结果：A组、B组间生存率差异有显著性意义（P＜0．05）,B组与C组、D组间生存率差异有显著性意义（P＜0．05）,C组、D组间生存率差异无显著性意义（P＞0．05）。结论：年龄小、分期早预后好,I期有高危因素及Ⅱ期者应行与化疗的综合治疗,有效联合化疗可代替常规腹膜后淋巴结清扫。     

颅内生殖细胞肿瘤研究进展

颅内生殖细胞肿瘤(Intracranial germcell tumors GCTs)是一种少见的发生于中线位置的颅内胚胎性肿瘤,主要发生于儿童和青少年。其组织学形态与起源与性腺和其他性腺外的颅内生殖细胞肿瘤相似。2007年WHO将颅内生殖细胞肿瘤分为8个亚型。不同组织学亚型在治疗及预后方面差别很大。随着MRI及显微外科等的发展,对颅内生殖细胞肿瘤的认识有了很大提高。本文论述了颅内生殖细胞肿瘤在组织病理学特点、组织起源、遗传学改变、临床特点及神经影像特点等方面的研究进展。     

颅内生殖细胞瘤的影像学诊断

目的探寻颅内生殖细胞瘤的CT和MRI特点。方法回顾性分析18例经病理或放疗证实的颅内生殖细胞瘤患者的CT和MRI表现。结果18例颅内生殖细胞瘤患者中,9例位于松果体区,3例位于基底节区及丘脑,6例位于鞍区。松果体区肿瘤MRI呈长T1、等或长T2信号,边缘光滑,出血少见,CT呈均匀等密度或稍高密度,松果体钙化常被肿瘤包埋,增强呈均匀显著强化。鞍区肿瘤垂体柄增粗,垂体后叶正常短T1信号消失,肿瘤T1WI呈等信号,T2WI上信号可以表现为等或高信号,CT上肿瘤实体部分呈高密度,增强扫描有明显强化。基底节及丘脑肿瘤,瘤体较大,信号及密度不均匀,边缘欠清晰,出血多见,增强呈不均匀强化。结论颅内生殖细胞瘤影像学表现具有一定的特征性,结合临床表现可进一步提高诊断正确率。     

松果体区混合性生殖细胞肿瘤临床与病理分析

目的 总结松果体区混合性生殖细胞肿瘤的临床特点、病理组织学构成,并探讨其相关的治疗策略.方法 对经手术切除,且病理检查详尽的14例松果体区混合性生殖细胞肿瘤进行回顾性分析.结果 14例均为男性,平均年龄17.3岁.其中13例包含生殖细胞瘤,其他成分即可能是单一的成熟畸胎瘤,也可能是多种高度恶性的生殖细胞肿瘤.血清肿瘤标志物升高基本反映了肿瘤中包含有相应的肿瘤成分.结论 松果体区混合性生殖细胞肿瘤临床表现无明显特殊.肿瘤大多包含有生殖细胞瘤成分,术前全面准确的定性诊断困难.先手术切除肿瘤以获取完整的病理诊断,而后采取针对性综合治疗似乎更合理.当血清肿瘤标志物正常,且影像学上肿瘤密度或信号混杂时,“试验性”放疗应慎重.     

松果体区肿瘤的治疗策略选择

目的探讨关于松果体区肿瘤治疗方式的选择。 方法回顾性分析解放军第九六〇医院神经外科自2004年9月至2016年12月收治的21例松果体区肿瘤患者的临床资料,根据不同的组织学类型对患者采用不同的治疗方式,并随访观察预后情况。 结果本组患者中显微手术9例,穿刺活检4例,脑室镜下活检4例,脑室-腹腔分流术后行实验性放射治疗4例。死亡2例,19例随访6个月~15年,生活自理、轻残者8例,中残者4例,重残者1例,失访者6例。 结论松果体区肿瘤应根据肿瘤的组织学类型、患者年龄、是否合并脑积水而决定治疗方式：生殖细胞瘤者尽快选择全脑、全脊髓放射治疗,儿童生殖细胞瘤者建议先行化学治疗后再行低剂量的放射治疗,合并脑积水的患者应解除脑积水症状后再行其他治疗。     

Imaging of pediatric pelvic neoplasms

Pediatric pelvic neoplasms, although relatively uncommon, are often difficult to evaluate and characterize with imaging, due to their overlapping appearances. This article discusses key clinical features and imaging findings that help limit the differential diagnosis of pediatric pelvic neoplasms. Testicular, ovarian, uterine, and bladder masses are included in addition to sacrococcygeal teratoma.     

Sellar abnormalities in female first-degree relatives

Non-pituitary lesions account for a minority of sellar region abnormalities. We report the unusual occurrence of non-pituitary sellar/suprasellar lesions in a mother and her two daughters. Each of these cases was diagnosed and managed differently, illustrating the relative importance of radiographic imaging, tumor markers and histopathologic examination in the diagnosis and treatment of intracranial disease. The mother had histologically confirmed Rathke's cleft cyst (RCC) with typical radiographic and histologic appearance. One daughter was treated presumptively for germinoma based on characteristic radiographic studies and slightly elevated tumor marker. The other daughter's lesion exhibited radiographic characteristics concerning for pituitary macroadenoma but with slightly elevated germ cell tumor marker, raising the suspicion for germinoma. Biopsy of the intrasellar mass revealed only proteinaceous material and normal anterior pituitary, consistent with cyst content without evidence of neoplasm. Without a clear unifying diagnosis it is difficult to posit an underlying pathology or genetic mechanisms in this unusual set of cases. At least two of the patients had benign cysts. The diagnosis of the third patient is unclear as there was no tissue biopsy. However, it is highly improbable that three female first-degree relatives would develop such lesions in the same brain region simply by chance.     

原发延髓生殖细胞瘤二例报告及文献复习

目的 报告2例原发延髓生殖细胞瘤病例并文献复习.方法 通过手术治疗明确诊断,并进行随访.结果 2例病例均手术治疗,生殖细胞瘤诊断成立.1例术后放化疗,肿瘤完全消失.另1例未行放化疗,术后肿瘤复发,病人术后8个月死亡.结论 原发延髓生殖细胞瘤罕见,手术辅以放疗及化疗病人能取得良好预后.