Treatment failure in intracranial primary germinomas

Object A radiation dose of 40–50 Gy is able to produce a cure rate of more than 90% in intracranial pure germinoma. However, many attempts have been made to reduce the dose and volume of radiation without compromising the disease control rate because of the toxicity of irradiation. This retrospective study is intended to provide the physician with an appropriate therapeutic strategy. Materials and methods We reviewed a series of 10 recurrent germinomas among 117 germinomas diagnosed histologically or clinically between 1979 and 2002. These patients involved underwent three different treatment modalities; radiation alone (N = 71), chemotherapy alone (N = 9), and combined therapy (N = 37). The 10-year overall and relapse-free survival rates were 97 and 93% in the radiation alone group, 89 and 67% in the chemotherapy alone group, and 92 and 92% in the combined therapy group, respectively. As expected, both radiation therapy and combined therapy were effective in controlling the disease. Tumor recurrence was closely related to the volume of radiation but not to the dose of radiation. If the tumor bed and craniospinal axis were fully covered, the radiation dose might be reduced. Chemotherapy alone showed earlier recurrence and a higher tumor recurrence rate. In the case of combined therapy, chemotherapy was useful in reducing the radiation dose but revealed some toxicity (death of two patients). Conclusions The investigation of a possible further dose reduction seems worthwhile. Radiation therapy alone with a dose of less than 40 Gy should be compared with ongoing chemotherapeutic protocols combined with low-dose irradiation.     

Radiation therapy for histologically confirmed primary central nervous system germinoma

: To evaluate survival and patterns of recurrence in patients with primary central nervous system germinoma treated with radiation therapy.

: Data regarding 48 patients with histologically confirmed, primary central nervous system germinoma were reviewed. All had been operated on at the Mayo Clinic between the years 1935 and 1993. Thirty-two patients (67%) were treated since 1973. The study group included 39 males and 9 females, with a median age at diagnosis of 17 years (range, 6–42 years). Twelwe patients (25%) were treated with craniospinal axis irradiation, 11 (23%) received whole-brain irradiation without spinal axis irradiation, and 24 (50%) underwent partial-brain irradiation. Treatment volumes were unknown in one patient. The median dose to the primary tumor was 44.00 Gy (range, 7.44–59.40 Gy). The median follow-up was 5.5 years (range, 4 months to 37 years).

: Actuarial 5-year and 10-year survival for the entire study group of patients was 80%. There was a trend toward improved survival in patients treated after 1973 (introduction of computed tomography) with 5-year and 10-year survival of 91% vs. 63% in prior years (p = 0.07). For the group of 31 patients treated since 1973 with known treatment volumes, the spinal axis failure rate at 5 years was 49% for patients treated with partial brain fields (11 patients) vs. 0% for those having undergone whole brain (10 patients) or craniospinal axis (10 patients) irradiation (p = 0.007). The rate of brain failure was also significantly higher in patients receiving less than whole-brain irradiation; at 5 years, 45% of the patients treated with partial-brain fields had intracranial recurrence of disease compared to 6% of patients treated with craniospinal axis or whole-brain irradiation (p = 0.01). Among the 32 modern era patients, the rate of brain failure was higher in patients who received doses less than 40 Gy (median dose, 48.55 Gy; range, 30.60-59.40 Gy) to the primary tumor (5-year brain failure rate 52% vs. 11%, p = 0.002).

: The long-term survival of patients with histologically proven CNS germinoma treated with radiation is excellent. Whole-brain or craniospinal axis irradiation appears to result in fewer spine and brain failures than does partial-brain irradiation. Furthermore, the administration of doses greater than 40 Gy to the primary tumor is associated with better local control.     

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.     

Numbers and Letters: Exploring an Autistic Savant’s Unpractised Ability

This paper describes an individual with autism and high-level calendar calculation ability who could perform a set of unpractised letter/number association tasks. The savant’s performance was compared with that of two control participants, one a departmental secretary and the other a professor of mathematics. The facility with which the savant could master the rules governing the relationships between the series of items suggests that he possessed a flexibility of mental processing transcending his ability of calendar calculation. Furthermore, he could recalibrate previous knowledge to solve new hitherto unpractised tasks. When presented with novel problems, the savant, unlike the mathematician, made no initial errors at all on any of the presented tasks, thereby indicating his fast and spontaneous recognition of new rules and of new relationships between items. It is concluded that a cognitive style of ‘weak central coherence’ as adopted by autistic savants may protect single representations from being retained in the form of stable enduring wholes, and that such a segmentation strategy may allow for the transformation, reorganization and reconstruction of the relationship between single items of information.     

Lymphocytic Hypophysitis Masking a Suprasellar Germinoma in a 12-year-old Girl<197>A Case Report

Case history, light and electron microscopic findings of a case of a lymphocytic hypophysitis in coincidence with a suprasellar germinoma in a 12-year-old girl are reported. The girl presented with a long time case history of diabetes insipidus and subsequent panhypopituitarism. Two years after the diagnosis of diabetes insipidus magnetic resonance imaging (MRI) showed a tumorous enlargement of the sellar content and pituitary stalk. A transnasal exploration was initially performed and revealed a lymphocytic hypophysitis. Light microscopy showed a dense infiltration of mature lymphocytes and plasma cells in the interstitium of the anterior pituitary gland. The stalk area could not be exposed to exclude a germinoma. One year later the lesion relapsed despite dexamethason therapy and a second operation by another neurosurgeon had to be performed. Light microscopy showed lymphocytic infiltrates, fibrosis and necrosis. The diagnosis was a lymphocytic hypophysitis again. Though transcranially exposed only pituitary tissue was removed. No infundibular mass became visible at surgery as shown by MRI. The girl developed five months later multiple cerebral lesions, which revealed to be a germinoma. Lymphocytic hypophysitis in children is very rare and a coincedence with a germinoma has not been described from histopathological aspect until now. The origin of the pituitary infiltration is discussed.     

鞍区生殖细胞瘤的MRI特征分析

目的：分析鞍区生殖细胞瘤的磁共振成像(MRI)表现特征及临床特点,以提高在临床工作中对本病的认识。方法回顾性分析12例鞍区生殖细胞瘤患者的临床和MRI资料,均行MRI的T1WI、T2WI、DWI、增强序列扫描。结果鞍区生殖细胞瘤呈较均质T1WI等或稍低信号、T2WI等或稍高信号, DWI为高信号,增强呈较均匀强化;鞍区生殖细胞瘤均有垂体柄增粗达15 mm以上和垂体后叶正常短T1高信号消失;根据肿瘤发生的部位及侵犯范围不同分为3类：垂体柄增粗病灶、V形或Y形病灶、不规则形肿块,所占比例分别为2/12(16.7%)、6/12(50.0%)、4/12(33.3%);1例鞍区生殖细胞瘤沿穹窿播散;12例患者主要临床症状为中枢性尿崩症。结论 MRI软组织分辨率高,并能多方位成像,可以清晰显示肿瘤形态、信号特点、累及范围及播散灶,再结合患者的性别、年龄及临床表现,有助于颅内生殖细胞瘤诊断。     

颅内生殖细胞瘤致垂体柄增粗误诊为淋巴细胞性垂体炎临床报告并文献复习

目的探讨颅内生殖细胞瘤致垂体柄增粗伴中枢性尿崩症的临床特点及诊治措施,以减少误诊误治。方法回顾性分析我院收治的1例误诊为淋巴细胞性垂体炎的颅内生殖细胞瘤致垂体柄增粗的临床资料,并复习相关文献。结果本例因多饮、多尿,伴胡须、腋毛稀疏3年,发热1周入院,曾经相关检查误诊为淋巴细胞性垂体炎导致中枢性尿崩症,给予口服糖皮质激素治疗3个月无效,后行经蝶垂体柄占位活组织病理检查确诊为生殖细胞瘤。行2次化学治疗、1次放射治疗后,瘤体明显缩小。随后给予激素替代治疗,效果良好。1周前出现上呼吸道感染,停用激素替代,进而出现垂体危象,予激素等治疗后患者病情好转出院。现病情稳定。结论颅内生殖细胞瘤致垂体柄增粗诊断除需考虑年龄、临床表现及实验室、影像学检查资料外,确诊需依据病理检查结果,以做到早诊断、早治疗,改善患者预后。     

Early response to chemotherapy as an indicator for the management of germinoma-like tumors of the pineal and/or suprasellar regions

Recent advances in diagnostic imaging and experience with germinomas may allow for the differentiation of central nervous system germinomas from other tumors based on clinical information, without histological verification. We retrospectively analyzed clinically diagnosed germinoma-like tumors of the pineal and/or suprasellar regions. This was done to evaluate the efficacy of our strategy of defining germinoma-compatible tumors based on good responses to initial chemotherapy. The responses to chemotherapy and survival of 34 consecutive patients with germinoma-like tumors who underwent initial treatment from July 2001 to October 2010 were analyzed. The minimum apparent diffusion coefficient (minADC) value and proton magnetic resonance spectroscopy (MRS) were evaluated in recent patients. Twelve patients with histologically verified germinomas and 18 with germinoma-compatible tumors showed early logarithmic decreases in tumor volume in response to initial chemotherapy, typical low minADC values and typical MRS characteristics, including increased choline/creatine ratios, decreased N-acetylasparate/creatine ratios, and large lipid peaks. These patients had good progression-free survival. The other four patients, with histologically verified non-germinomas, showed no response to chemotherapy, and one patient with a pineoblastoma showed a similar minADC value and MRS characteristics to those of patients with germinomas. The response to initial chemotherapy can be used to distinguish germinoma-compatible tumors from non-germinoma in patients with germinoma-like tumors of the pineal and/or suprasellar regions. The evaluation of minADC and proton MRS are useful for distinguishing germinomas from other tumors. However, a subset of non-germinomas may show similar characteristics to germinomas. The benefit of bypassing unnecessary surgical intervention can be achieved, at least in Asian populations with a high incidence of germinomas.     

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.     

原发性颅内生殖细胞瘤

患者1年前出现右手抖,右侧肢体肌力下降,2个月前出现头痛及癫痫样发作一次,行CT检查发现颅内占位,行左额颞叶占位性病变次全切除术,术后病理诊断为:左额颞叶生殖细胞瘤。经过多学科讨论认为原发性颅内生殖细胞瘤为少见病,放疗为其最主要治疗手段,具有较高的治愈率,应继续关注联合放化疗在该病治疗中的应用进展,这可能有助于我们在保持疗效的情况下减低放疗的远期毒副作用。对于该例患者,我们给予了根治性放疗,先行全中枢放疗,随后缩野至瘤床局部推量,完成后再次缩野至肿瘤实质区放疗,总放疗剂量为46.8Gy。并在放疗后予PEB方案辅助性化疗2程。治疗结束后1周复查MR疗效评价为CR。