Intratumoral hemorrhage because of primary spinal mixed germ cell tumor presenting with atypical radiological features in an adult

Background context

Germ cell tumors are known to arise in the central nervous system, usually in the intracranial regions. However, primary spinal mixed germ cell tumors are extremely rare.

Purpose

This is the first reported case of intratumoral hemorrhage because of a primary spinal mixed germ cell tumor consisting of germinoma and immature teratoma in the conus medullaris of an adult patient that presented with rapid changes on magnetic resonance image (MRI). We report this rare case and discuss the clinical manifestations of an intramedullary spinal mixed germ cell tumor in adult.

Study design

A case report.

Methods

A 42-year-old woman experienced buttock numbness, and a spinal cord tumor was observed on the conus medullaris on MRI. The patient was scheduled for an operation in 1 month, but she developed sudden-onset neurologic deterioration. Rapid progression of the tumor was observed on follow-up MRI. The tumor was removed by emergency surgery and was identified as a primary mixed germinoma and immature teratoma.

Results

The patient received adjuvant chemotherapy and radiotherapy after gross total resection. The neurologic deficit of the patient was relieved, and recurrence of the tumor was not observed 26 months after the surgery.

Conclusions

We present this rare case and emphasize the necessity of precise diagnosis and early treatment of primary spinal germ cell tumor. Close observation on MRI is required after surgery, and adjuvant chemotherapy and radiotherapy should be considered according to the pathologic features.     

Re-evaluation of the significance of cerebrospinal fluid human chorionic gonadotropin in detecting intracranial ectopic germinomas

This study was conducted to establish a reference value for cerebrospinal fluid (CSF) human chorionic gonadotropin (hCG) levels. We also evaluated the sensitivity of CSF hCG as a biomarker to detect intracranial ectopic germinomas that arise in rare sites other than the pineal and suprasellar regions. CSF hCG was measured in 201 male patients who had various types of neurological disease (not tumours of germ cell origin or other malignant tumours). A reference value of 1.009 U/L was established, and the CSF hCG level among different age groups was not significantly different. CSF and serum hCG were measured before and after radiotherapy in 14 consecutive patients with intracranial ectopic germinomas. The CSF hCG levels were all above 1.009 U/L before radiotherapy. In male patients, a CSF hCG value above 1.009 U/L suggests abnormal intrathecal hCG secretion. These results demonstrate that the CSF hCG assay is a sensitive method for diagnosing intracranial ectopic germinoma.     

原发性纵隔恶性生殖细胞肿瘤临床和预后分析

目的探讨原发性纵隔恶性生殖细胞肿瘤(PMGCT)的临床病理特点、治疗方法和预后因素。方法回顾性分析29例PMGCT患者的临床资料。结果29例患者均为男性,平均发病年龄26.1岁,肿瘤均来源于前纵隔,平均最大径16.0 cm。其中原发性纵隔精原细胞瘤(PMSGCT)5例(17.2%),原发性纵隔非精原细胞瘤(PMNSGCT)24例(82.8%)。PMGCT最常见症状是憋气、咳嗽与胸痛,其治疗采用化疗、手术、放疗相结合的综合治疗模式。PMNSGCT组中化生存期为19.0个月, 1年和2年生存率分别为65.3%和28.1%。PMSGCT组均长期生存,预后优于PMNSGCT组(P= 0.008)。多因素分析结果显示,病变局限于纵隔、以顺铂为基础的联合化疗是PMNSGCT患者预后的独立影响因素。结论PMGCT以PMNSGCT为主,主要治疗手段是以顺铂为基础的联合化疗。PMNSGCT预后明显差于PMSGCT,并与病变范围、化疗与否相关。     

原发纵隔恶性生殖细胞瘤32例临床分析

目的:探讨纵隔恶性生殖细胞瘤(malignant germ cell tumors,MGCT)的临床特点、治疗和预后。方法:32例纵隔MGCT患者,精原细胞瘤18例,非精原细胞瘤14例。所有患者均采用手术和(或)放疗和(或)化疗等多学科综合治疗的方法。结果:非精原细胞瘤患者中位生存期(OS)32.4个月,中位无进展生存期(PFS)18个月,5年无复发生存率和总生存率均为28.6%。精原细胞瘤患者5年无复发生存率和总生存率分别为83.3%和85.6%,中位OS和PFS均未到达。精原细胞瘤患者OS和PFS均明显好于非精原细胞瘤患者,P值分别为0.001 4和0.000 7。结论:纵隔精原细胞瘤采用多学科综合治疗方法能取得较好的治疗效果,本研究的结果与文献报道相符。纵隔非精原细胞瘤的治疗效果有待进一步提高。非精原细胞瘤是影响纵隔恶性生殖细胞瘤预后的重要因素。     

74例颅内生殖细胞瘤放疗疗效分析

目的 分析颅内生殖细胞瘤的放疗疗效.方法 搜集2007年11月前接近18年内收治的颅内生殖细胞瘤病例74例,其中男35例,女39例,中位年龄15(5～45)岁.放疗前病理诊断9例,余65例20 Gy放疗后MRI显示病灶均明显缩小(>50%)或消失为临床诊断.应用6 MV X线全脑全脊髓放疗加局部补量照射、全脑放疗加局部补量照射、全脑室放疗加局部补量或肿瘤区局部照射,原发病灶区38.5～50.0 Gy,全脑或全脑室18～25 Gy,全脊髓21～25 Gy,分割剂量1.6～2.0Gy/次,5次/周.结果 中位随访时间80(12～168)个月,总随访率为97%,10年随访例数和随访率分别为14例和19%.1、5、10年总生存率和无复发生存率分别为99%和97%、96%和90%、93%和83%.共9例患者治疗失败,6例为照射野内复发,3例为照射野外转移.照射野内复发者中肿瘤剂量<40 Gy者3例.38例患者放疗后有不同程度的垂体前叶功能低下,部分需强的松等激素替代治疗.结论 放疗是颅内生殖细胞瘤的主要治疗手段,其照射剂量、范围要根据病灶数目、脑脊液检查等结果 来决定.     

Mixed germ cell tumors with abundant sarcomatous component in the temporal lobe after radiochemotherapy of neurohypophyseal germinoma: a case report

We report a case of intracranial germ cell tumor that showed pathological changes from neurohypophyseal germinoma to mixed germ cell tumors consisting exclusively of undifferentiated sarcomatous component after radiochemotherapy. Three surgical specimens and autopsied brain from the patient were histologically examined. An initial specimen from the neurohypophyseal tumor was diagnosed as germinoma with a two-cell pattern. Five years later, after repeated radiochemotherapy, the second specimen resected from the right temporal lobe showed mixed germ cell tumors consisting of the three components of germinoma, choriocarcinoma, and immature teratoma. Six months later after intensive radiotherapy, the right temporal tumor recurred and was surgically removed. The histological diagnosis was mixed germ cell tumors with abundant immature teratoma component. The patient died of uncontrollable tumor growth with repeated intratumoral hemorrhages. The autopsied brain showed sarcoma with angionecrosis. This pathological alteration indicated an increase in the sarcomatous component after undergoing various treatments. We discuss the histological changes of intracranial germ cell tumor modified by treatment.     

74例颅内生殖细胞瘤放疗疗效分析

目的 分析颅内生殖细胞瘤的放疗疗效.方法 搜集2007年11月前接近18年内收治的颅内生殖细胞瘤病例74例,其中男35例,女39例,中位年龄15(5～45)岁.放疗前病理诊断9例,余65例20 Gy放疗后MRI显示病灶均明显缩小(>50%)或消失为临床诊断.应用6 MV X线全脑全脊髓放疗加局部补量照射、全脑放疗加局部补量照射、全脑室放疗加局部补量或肿瘤区局部照射,原发病灶区38.5～50.0 Gy,全脑或全脑室18～25 Gy,全脊髓21～25 Gy,分割剂量1.6～2.0Gy/次,5次/周.结果 中位随访时间80(12～168)个月,总随访率为97%,10年随访例数和随访率分别为14例和19%.1、5、10年总生存率和无复发生存率分别为99%和97%、96%和90%、93%和83%.共9例患者治疗失败,6例为照射野内复发,3例为照射野外转移.照射野内复发者中肿瘤剂量<40 Gy者3例.38例患者放疗后有不同程度的垂体前叶功能低下,部分需强的松等激素替代治疗.结论 放疗是颅内生殖细胞瘤的主要治疗手段,其照射剂量、范围要根据病灶数目、脑脊液检查等结果 来决定.     

74例颅内生殖细胞瘤放疗疗效分析

目的 分析颅内生殖细胞瘤的放疗疗效.方法 搜集2007年11月前接近18年内收治的颅内生殖细胞瘤病例74例,其中男35例,女39例,中位年龄15(5～45)岁.放疗前病理诊断9例,余65例20 Gy放疗后MRI显示病灶均明显缩小(>50%)或消失为临床诊断.应用6 MV X线全脑全脊髓放疗加局部补量照射、全脑放疗加局部补量照射、全脑室放疗加局部补量或肿瘤区局部照射,原发病灶区38.5～50.0 Gy,全脑或全脑室18～25 Gy,全脊髓21～25 Gy,分割剂量1.6～2.0Gy/次,5次/周.结果 中位随访时间80(12～168)个月,总随访率为97%,10年随访例数和随访率分别为14例和19%.1、5、10年总生存率和无复发生存率分别为99%和97%、96%和90%、93%和83%.共9例患者治疗失败,6例为照射野内复发,3例为照射野外转移.照射野内复发者中肿瘤剂量<40 Gy者3例.38例患者放疗后有不同程度的垂体前叶功能低下,部分需强的松等激素替代治疗.结论 放疗是颅内生殖细胞瘤的主要治疗手段,其照射剂量、范围要根据病灶数目、脑脊液检查等结果 来决定.     

Pineal region tumors: Analysis of treatment results

This article represents a review of 32 patients with pineal region tumors seen and treated at the University of Michigan Medical Center from January 1950 to December 1980. All patients presented with manifestations of increased intracranial pressure: limitation of the upward gaze (Parinaud's syndrome), hydrocephalus and a mass in the posterior aspect of the third ventricle. The tumor was demonstrated by pneumoencephalography, ventric ulography, angiography or CT scans. Ventricular decompression was performed in all patients. Eighteen of the 32 patients (56%) had a histological diagnosis of germinoma, one patient had a diagnosis of hamartoma; no histological diagnosis was obtained in the remainder of the patients. Twenty-seven patients received post-operative irradiation. Irradiation dosage ranged between 30 and 55 Gy. The overall 10 year survival for evaluable patients was $\text{16}\text{24}$ (67%). The low incidence of spinal cord metastasis in these patients does not justify routine use of spinal irradiation.