Pattern of relapse in 290 patients with nonmetastatic Ewing's sarcoma family tumors treated at a single institution with adjuvant and neoadjuvant chemotherapy between 1972 and 1999.

AIMS: Evaluation of pattern of recurrences of 290 patients with an Ewing's sarcoma family tumor (ESFT), who relapsed after adjuvant or neoadjuvant chemotherapy. METHODS: Retrospective analysis at a median follow-up of 16.6 years (range: 5-32) from the primary therapy. RESULTS: There were 378 recurrences, treated by surgery, and/or chemotherapy, radiotherapy, or only palliative treatments. At the last control 18 patients were alive and free of disease 2.5 to 20 years (median 12.1 year) from the last treatment, 4 were alive with uncontrolled disease, 2 died of second line chemotherapy-related toxicity, and 266 died of the tumor 4 months to 20.5 years from the first relapse (median 3.2 years). The 5-year event free survival after the last relapse and overall survival were 5.1 and 7.9%, respectively, and resulted significantly correlated with the time of first relapse, the site of first metastases, the treatment performed after relapse (all patients presently free of disease had been treated by surgery alone or combined with a second line chemotherapy) and for patients treated with neoadjuvant chemotherapy and locally by surgery, with the histologic response to preoperative chemotherapy. CONCLUSIONS: We confirm that the post-relapse outcome of patients with ESFT who relapse after conventional treatment is very poor. Nonetheless specific subgroups of patients may be cured even after 2 or 3 relapses: patients who relapse 2 or more years after primary treatment, patients who relapse with only lung metastases, and patients whose recurrences can be surgically treated.     

Long-term results of conventional-dose salvage chemotherapy in patients with refractory and relapsed Hodgkin's disease (Croatian experience).

BACKGROUND: The aim of this study was to analyze outcome of patients with Hodgkin's disease (HD) in whom first-line chemotherapy with mustine/vincristine/procarbazine/prednisone (MOPP) had failed. PATIENTS AND METHODS: From January 1982 to December 1989 among 210 patients treated with MOPP and radiotherapy to initial bulky sites, 65 patients were primary refractory to or relapsed after initial treatment. RESULTS: Twenty-nine of 65 patients (44%) were primary refractory to initial chemotherapy, 20 relapsed within 12 months after complete remission (CR) and 16 relapsed after CR that lasted more than 12 months. Patients with primary refractory HD and early relapse (<12 months after CR) were treated with doxorubicin/bleomycin/vinblastine/darcarbazine. In patients with late relapse (>12 months after CR) MOPP was repeated. The median follow-up for all patients was 115 months. The overall response rate was 63%. Thirty-three patients (51%) achieved a second CR and eight patients (12%) partial response. Remission rate was greatest in patients with late relapse (CR >12 months) (75 versus 55% for early relapse versus 35% for primary refractory HD) (P <0.01). At 10 years, overall and failure-free survival rates were 21 and 16%, respectively. Patients who were in first remission longer than 12 months had a superior overall survival (37 versus 18% for early relapse) and failure-free survival (24 versus 10% for early relapse). No patient with primary refractory HD was alive beyond 52 months after initial treatment failure (P <0.01). Main prognostic factors were duration of the first remission and tumor bulk at relapse. CONCLUSIONS: Our results confirm previous observations that a significant proportion of patients with HD who experience induction treatment failure cannot be cured with conventional treatment and probably need more aggressive therapy.     

Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: recent experience at the istituto rizzoli.

PURPOSE: The results achieved in 44 patients with nonmetastatic peripheral neuroectodermal tumor (PNET) of bone treated with neoadjuvant chemotherapy are reported. PATIENTS AND METHODS: A six-drug regimen of chemotherapy (vincristine, doxorubicin, dactinomycin, cyclophosphamide, ifosfamide, and etoposide) was administered to all patients. Local treatment consisted of surgery in 20 patients, surgery followed by radiotherapy in 13, and radiotherapy only in 11. RESULTS: At a mean follow-up of 4.5 years (range, 2 to 7 years), 23 patients (52%) remain event-free, 20 have relapsed (45%), and one has died of chemotherapy-related toxicity. The 5-year event-free survival and overall survival were 54.2% and 62.7%, respectively. To assess the prognostic significance of neural differentiation in the family of Ewing's sarcoma, these results have been compared with the outcomes of 138 concomitant patients with typical Ewing's sarcoma (TES) who were treated according to the same protocol. Of these, 103 (75%) remained continuously event-free, 34 (24%) relapsed, and one died of chemotherapy-related toxicity. It follows that PNET patients treated with this chemotherapy regimen have a significantly worse prognosis than typical ES patients (5-year event-free survival, 54.2% v 70.6%, P <.012; 5-year overall survival, 62.7% v 78.3%, P <.002). CONCLUSION: The authors conclude that studies into new adjuvant therapy for Ewing's sarcoma modulated according to risk of relapse should also consider neural differentiation as a risk factor.     

Prognostic factors in Ewing's tumor of bone: analysis of 975 patients from the European Intergroup Cooperative Ewing's Sarcoma Study Group.

PURPOSE: To further elaborate on prognostic factors for Ewing's sarcoma of bone and to document improvements in relapse-free survival (RFS) and trends in local therapy over the study period (1977 to 1993). PATIENTS AND METHODS: A retrospective analysis was performed on a combined Gesellschaft Für P?diatrische Onkologie und H?matologie/Cooperative Ewing Sarcoma Study and United Kingdom Children's Cancer Study Group/Medical Research Council data set of 975 patients registered with the respective trial offices before the current collaborative European Intergroup Cooperative Ewing's Sarcoma Study trial. Both groups independently undertook studies with similar chemotherapy during the period. RESULTS: The key adverse prognostic factor is metastases at diagnosis (5-year RFS, 22% of patients with metastases at diagnosis v 55% of patients without metastases at diagnosis; P: <.0001). For the group with metastases, there was a trend for better survival for those with lung involvement compared with those with bone metastases or a combination of lung and bone metastases (P: <.0001). In the group of patients with no metastases at diagnosis, multivariate analysis demonstrated that site (axial v other), age-group (< 15 v > or = 15 years), and period of diagnosis had significant influence on RFS (all P: <.005). RFS was superior in the period after 1985 compared with the period before 1985 for nonmetastatic patients (45% v 60%, respectively; P: <.0001) and for metastatic patients (16% v 30%, respectively; P: =.016). Patients who relapsed within 2 years of diagnosis had a less favorable prognosis than patients who relapsed later (5-year survival after relapse, 4% v 23%, respectively; P: <. 0001). There were other changes over the period; in particular, radiotherapy or amputation were more common in the period before 1986, whereas endoprosthetic surgery was widely used in the later period. CONCLUSION: Survival and RFS improved over the period. Prognostic factors are metastases at diagnosis, primary site, and age.     

Survival after recurrence of Ewing's sarcoma family of tumors.

PURPOSE: The overall survival (OS) of patients with relapsed Ewing's sarcoma family of tumors (ESFT) is poor, and the relative benefit of high-dose therapy (HDT) is controversial. PATIENTS AND METHODS: We retrospectively identified 55 consecutive ESFT patients with adequate medical records for review, who were treated at Children's Hospital and Regional Medical Center and who developed disease recurrence between January 1, 1985 and December 31, 2002. RESULTS: The median relapse-free interval (RFI) from diagnosis to first recurrence was 17 months (range, 5 to 90 months). Most recurrences were metastatic only (39 patients) or local and metastatic (10 patients). Twenty-seven patients (49%) achieved a partial or complete response to second-line treatment, with a median duration of response of 27 months (range, 5 to 119+ months). The 5-year OS rate for all relapsed patients was 23% (95% CI, 11% to 35%). By univariate analysis, improved OS was associated with response to second-line treatment versus no response (46% v 0%, respectively; P < .0001), RFI > or = 24 months versus less than 24 months (48% v 12%, respectively; P = .0001), and no metastases at initial diagnosis versus presence of metastases (31% v 12%, respectively; P = .05). Because all 13 patients who received HDT also had responsive relapse, we performed a multivariate analysis. Reduced risk of death was associated with response to second-line therapy (relative risk, 0.14; 95% CI, 0.05 to 0.40), RFI > or = 24 months (relative risk, 0.29; 95% CI, 0.13 to 0.66), and receiving HDT (relative risk, 0.26; 95% CI, 0.08 to 0.85). CONCLUSION: HDT as consolidation therapy for relapsed ESFT seems to be associated with improved OS, even after adjusting for RFI and response to second-line treatment.     

Early stage infradiaphragmatic Hodgkin's disease: results of radiotherapy and review of the literature.

PURPOSE: To assess the impact of modality therapy on long-term outcome for infradiaphragmatic Hodgkin's disease (IDHD). METHODS AND MATERIALS: During the period 1965-1997, 847 patients with early stage Hodgkin's disease (HD) were evaluated and treated at our institution, 20 of them had IDHD (2.4%). Patients characteristics: stage I, nine patients (five pathological stage (PS), and four clinical stage (CS)) and stage II: 11 patients (six PS and five CS). Two modalities of treatment were used: combined modality (CMT), consisting of chemotherapy followed by extended field radiotherapy or radiotherapy alone (XRT). All patients with CS or PS II, except in one case, were treated with CMT. Overall, 12 patients were treated with CMT and the remaining eight patients were treated with XRT. RESULTS: The relapse rate after initial treatment was 30%. Ten-year disease free survival (DFS) and 10-year cause-specific survival were 60% and 92%, respectively. There was a non-significant trend to a better DFS for the CMT group of patients (76% vs. 35% for the whole series and 100% vs. 24% for stage I patients). The four relapsed patients in the XRT group were inguino-femoral PS I. In four out of the six patients who relapsed (66%) the failure was located solely in the supradiaphragmatic area, outside of the radiation fields. CONCLUSIONS: In our experience, inguino-femoral stage I patients have a high relapse rate after XRT; consequently, CMT consisting of chemotherapy plus involved field radiotherapy should be recommended for early stage HD confined below diaphragm.     

Local therapy and other factors influencing site of relapse in patients with localised Ewing's sarcoma. United Kingdom Children's Cancer Study Group (UKCCSG)

Relapse patterns have been documented in 191 children with localised Ewing's sarcoma treated with the United Kingdom Children's Cancer Group (UKCCSG) Ewing's Tumour regimen ET2. All received chemotherapy comprising ifosfamide, vincristine, doxorubicin and actinomycin D. Local treatment modality was excision and or radiotherapy depending on tumour site and response to primary chemotherapy. Although not strictly comparable, due to the clinical indications used for each modality, local relapse rates were very low and were similar, irrespective of the type of local treatment modality: radiotherapy (3/56), surgery (7/114) or a combination (0/20). Combined relapse (local + distant) rates were similarly low irrespective of the type of local therapy: radiotherapy (4/56), surgery (4/114) or a combination (0/20). Overall survival was lower in females (P = < 0.04), older children (P = < 0.002) and those with primaries at sites other than long bones (P = < 0.02). It is concluded that with effective intensive chemotherapy combined with either radiotherapy or surgery, local control in this study was excellent at sites other than the pelvis. Preventing distant relapse, predominantly to lung and bone, remains the major challenge.     

Surgery for lung metastases in Ewing's sarcoma of bone.

The addition of chemotherapy and radiotherapy to local treatment for Ewing's sarcoma of bone (ES) significantly bettered its prognosis. Nonetheless, although with no signs of local recurrence some patients still relapse with lung metastases. These patients are generally treated with non-surgical procedures. The few reports in literature on surgical resection of metachronous pulmonary metastases do not allow any conclusions. This study aims to report the results achieved in 24 Ewing's sarcoma patients who relapsed only with lung metastases, and no local recurrence, treated with surgery at the authors' institution.     

Does the incidence and outcome of brain metastases in locally advanced non-small cell lung cancer justify prophylactic cranial irradiation or early detection?

OBJECTIVE: The radical treatment of locally advanced non-small cell lung cancer (LA-NSCLC) currently involves combined modality therapy (CMT) with the use of chemotherapy in addition to radiation therapy and/or surgery. Chemotherapy has been shown to improve survival, but does not alter brain relapse. We reviewed the outcomes of Stage IIIA and IIIB LA-NSCLC patients treated with CMT at our institution. We assessed the incidence of brain metastases and the management and outcome of these patients. METHODS: Using our radiation-planning database (RSTS), we identified 230 consecutive patients from the years 1999 and 2000 who received radical radiation therapy to the lung. Extracting data from the chart, we identified 83 patients who were treated radically with chemotherapy, radiation and possibly surgery. These patients form the basis of this study. RESULTS: At 2 years, the actuarial rates for any brain failure, first failure in the brain and sole failure in the brain were 34.2%, 24.6% and 11.0%, respectively. Age was the only factor among sex, histology, stage, weight loss and the timing of chemotherapy and radiation that predicted for an increased risk of first failure in the brain. Patients less than age 60 had a risk of 25.6% versus 11.4% for those greater than 60 (p = 0.022). Among the patients who failed first in the brain, those who had aggressive management of their brain metastases with surgical resection in addition to whole brain radiotherapy had a median survival of 26.3 months compared with 3.3 months for those treated with palliative whole brain radiotherapy alone. CONCLUSION: Brain metastases are common in patients with LA-NSCLC treated with CMT. These patients may benefit from either prophylactic cranial irradiation or early detection and aggressive treatment of brain metastases.     

Treatment and outcome of recurrent osteosarcoma: experience at Rizzoli in 235 patients initially treated with neoadjuvant chemotherapy

The pattern of relapse, treatment and final outcome of 235 patients with osteosarcoma of the extremity who relapsed after neoadjuvant treatments performed between 1986 and 1998 at a single institution is reported. The 235 relapses were treated by surgery, surgery plus second line chemotherapy, and only second line chemotherapy or radiotherapy. The 5-year post-relapse-event-free-survival (PREFS) was 27.6% and the post-relapse-overall-survival (PROS) 28.7%. All 69 patients who are presently alive and free of disease were treated by surgery, alone or combined with chemotherapy. None of patients treated only by chemotherapy or radiotherapy survived. We conclude that it is possible to obtain prolonged survival and cure in about 1/4 of relapsing osteosarcoma patients with aggressive treatments. The complete removal of the recurrence is essential for outcome, while the role of the association of second-line chemotherapy remains to be defined.     

Radiotherapy in Ewing tumors of the vertebrae: treatment results and local relapse analysis of the CESS 81/86 and EICESS 92 trials

PURPOSE: Treatment results in patients with Ewing tumors of the vertebrae enrolled in the Cooperative Ewing's Sarcoma Study (CESS) 81, 86, and the European Intergroup Cooperative Ewing's Sarcoma Study (EICESS) 92 trials were analyzed with special emphasis on radiation-associated factors. PATIENTS AND METHODS: A retrospective analysis was performed on 116 patients with primary tumors of the cervical, thoracic, or lumbar vertebrae treated between 1981 and 1999. Furthermore, a relapse analysis was done on those patients who underwent radiotherapy and subsequently had a local recurrence. RESULTS: A total of 64.6% of the patients received definitive radiotherapy; 27.5% of patients had surgery and radiotherapy. Only 4 patients (3.4%) underwent definitive surgery. Twenty-seven patients presented with metastases at diagnosis. 22.4% of the total group developed a local relapse. Among the subgroup with definitive radiotherapy, local recurrence was seen in 17 of 75 patients (22.6%). Event-free survival and survival at 5 years were 47% and 58%, respectively. Of the 14 evaluable patients with a local relapse after radiotherapy, 13 were in-field. No correlation between radiation dose and local control could be found. CONCLUSION: Surgery with wide resection margins is rarely possible. The results after definitive radiotherapy in vertebral tumors are comparable to those of other tumor sites when definitive radiotherapy is given. Nearly all local relapses after radiotherapy are in-field.     

Relapse after high-dose therapy in relapsed Ewing's tumor patients: effects of maintenance chemotherapy in two selected patients?

BACKGROUND: The prognosis for patients with high-risk Ewing's tumor, i.e. primarily multifocal or early relapsed disease, is extremely poor with conventional relapse therapy. High-dose radio/chemotherapy (HDC) with subsequent stem cell transplantation seems to improve outcome in this patient cohort. In spite of this intensified therapy however, relapse remains the most frequent cause of death. In the majority of patients the time to second relapse after HDC is shorter than the time to first relapse after conventional therapy (3.4 vs. 18 months). Event-free survival in Ewing's tumor patients who suffer a second relapse after first-line therapy (EICESS 92) is 2% after 18 months. CASE REPORT: The present report describes the clinical course of two girls who relapsed after HDC and subsequently received low-dose oral trofosfamide and etoposide. The patient with very late multifocal relapse after first-line treatment and a second localized relapse 30 months after HDC remains disease-free for 5 years after the last relapse. However, the other patient with 2 early relapses after first-line treatment and HDC, respectively, did not benefit from this regime. CONCLUSION: We propose that low-dose maintenance therapy may be beneficial in the subgroup of Ewing's tumor patients with late relapse after HDC.     

Carcinoma of the nasopharynx. An analysis of 91 cases and a comparison of differing treatment approaches

Ninety-one patients with malignant epithelial tumors of the nasopharynx seen in our department from 1970 to 1982 were evaluated. The 5- and 10-year actuarial survival rates were 62.0% and 42.0%, respectively. Patients seen from 1970 to 1979 were treated by radiotherapy to the primary site and upper neck, the lower neck being irradiated only in instances of massive disease. Those treated from 1980 to 1982 received elective irradiation of the whole neck, as well as adjuvant chemotherapy (consisting of cyclophosphamide, methotrexate, and either 5-fluorouracil or bleomycin) for 6 to 12 months after completion of radiotherapy. Comparison of 3-year actuarial survival (61.4% versus 83.3%) and disease-free survival (49.7 versus 77.0%) rates show significantly improved results (P less than 0.05) for those receiving combined therapy. In addition, significantly fewer (P less than 0.05) distant metastases appeared in the combined therapy group at 18 months. A retrospective analysis comparing those patients who received full-neck irradiation and adjuvant chemotherapy with those who received full-neck irradiation alone showed a significantly improved survival (P less than 0.02) and disease-free survival (P less than 0.05) for those patients with undifferentiated carcinomas, including lymphoepitheliomas, who received adjuvant chemotherapy.     

食管癌根治术后复发的治疗策略

  目的  探讨食管癌根治性切除术后复发患者的放射治疗策略和预后因素。  方法  回顾性分析病理确诊为食管癌且经R0切除后出现复发转移的66例患者,肿瘤中位复发时间10.6个月;50例患者局部区域复发,16例患者复发合并远处转移;全组患者中,单纯放疗10例,单纯化疗23例,放化联合治疗33例;联合放化疗组:先行化疗者22例,先行放疗者11例。放疗采用6MV-X线,中位放疗剂量60 Gy。  结果  全组患者1、2、3年生存率和中位生存时间分别为61.9%、25.9%、16.5%和14.3个月（95% CI=2.4~16.2个月）。三种治疗模式中单纯化疗、单纯放疗和联合放化疗的患者中位生存时间分别为11.4、25.5和14.3个月（P= 0.037）。复发合并远处转移的联合放化疗患者,先行化疗较先行放疗生存有获益（P=0.032）。单因素分析显示患者初治时的肿瘤分段、术式、复发是否合并转移、复发后治疗方式以及复发后是否放疗与患者预后相关（P < 0.05）。多因素分析显示患者初始治疗时的肿瘤分段为影响患者预后的独立因素（P < 0.01）。  结论  术前食管癌分段或许能提示复发的预后;对于有远处转移的患者,先行化疗后再放疗,对延长生存更有益。      

A comparison of patients with relapsed and chemo-refractory gestational trophoblastic neoplasia

The majority of women requiring chemotherapy for gestational trophoblastic disease (GTN) are cured with their initial chemotherapy treatment. However, a small percentage either become refractory to treatment, or relapse after the completion of treatment. This study investigates the characteristics and outcome of these patients. Patients were identified from the Charing Cross Hospital GTD database. The outcome of these patients with relapsed disease was compared to those with refractory disease. Between 1980 and 2004, 1708 patients were treated with chemotherapy for GTN. Sixty (3.5%) patents relapsed following completion of initial therapy. The overall 5-year survival for patients with relapsed GTN was 93% (95% CI 86-100%). The overall survival for patients with low-risk and high-risk disease at presentation, who subsequently relapsed was 100% (n=35), and 84% (n=25) (95% CI: 66-96%: P<0.05), respectively. Eleven patients were identified who failed to enter remission and had refractory disease. These patients had a worse outcome compared to patients with relapsed disease (5-year survival 43% (95% CI:12-73% P<0.01)). The outcome of patients with relapsed GTN is good. However, patients with primary chemo-refractory disease do poorly and novel therapies are required for this group of patients.     

Integrated treatment in locally advanced carcinoma of the oropharynx

BACKGROUND AND OBJECTIVES: Oropharyngeal carcinoma tends to be aggressive and deeply infiltrative of nearby sites, with an high incidence of lymph node metastases. The last treatment decision generally depends on the stage of the lesion and the patient's general status. Oropharyngeal tumor is generally treated by integrated treatments. METHODS: We retrospectively studied 115 patients with locally advanced oropharyngeal tumors treated in our institution with combined therapies compare the results in two different groups of patients (surgery plus radiotherapy and chemotherapy plus radiotherapy). RESULTS: The 3-year overall survival rate in patients who underwent surgery plus radiotherapy was 82% and in those who underwent chemotherapy plus radiotherapy was 49%. CONCLUSION: The results suggest that surgery followed by radiotherapy seems to be the best treatment in the case of locally advanced oropharyngeal tumor.     

TNM因素对非小细胞肺癌治疗效果的影响

目的 探讨肺部原发肿瘤的大小、淋巴结不同区域的转移及有无远处转移对非小细胞肺癌(NSCLC)综合治疗效果的影响.方法 回顾性分析手术治疗的987例NSCLC患者的临床资料,将其中以手术+化、放疗的574例患者与单纯手术治疗的413例患者进行对比分析.结果 全组患者的1、3、5、10年生存率分别为87.7%、57.5%、54.6%和54.5%.其中综合治疗组的1年生存率高于单纯手术组(P<0.01).在T4患者中,手术+放疗组的5年生存率高于单纯手术组(P<0.05).在N0患者中,手术+化疗组、手术+放疗组与单纯手术组比较,1年生存率差异有统计学意义(均P<0.05);在N1患者中,手术+化疗组、手术+放化疗组与单纯手术组比较,1年生存率差异有统计学意义(P<0.01);在N2患者中,手术+化疗组与单纯手术组比较,1年和3年生存率差异均有统计学意义(均P<0.05).结论 对于NSCLC患者,以手术为主的综合治疗疗效优于单纯手术.对T4患者应加强术后局部放疗,对NO和N1患者应辅以适度化、放疗,对N2患者则应强调辅以足够的化疗.     

Clinical stage IEA-IIEA orbital lymphomas: outcomes in the era of modern staging and treatment.

BACKGROUND AND PURPOSE: The present study examines outcomes in patients with primary orbital lymphomas who underwent complete staging. MATERIALS AND METHODS: From 1978 to 1997, 21 adult patients at the M.D. Anderson Cancer Center had stage IEA-IIEA orbital non-Hodgkin's lymphomas based on staging that included computed tomography scans. Sixteen (76%) patients had working formulation low-grade lymphomas, and five (24%) had aggressive lymphomas. Fourteen of 16 (88%) patients with low-grade lymphomas were treated with radiotherapy alone, and four of five (80%) patients with aggressive lymphomas were treated using combination chemotherapy with or without radiotherapy. Total radiotherapy doses ranged from 30.0 to 40.0 Gy using daily 1.5-2.0 Gy fractions. RESULTS: The median follow-up was 84 months. For the low-grade lymphomas, the 5-year local control, progression-free survival, and overall survival rates were 100, 100, and 92%, respectively. For the seven low-grade lymphomas treated with radiotherapy alone to 30.0 Gy in 20 fractions, the 5-year local control, progression-free, and overall survival rates were 100, 100, and 75%, respectively. The 5-year incidence of complications, which were typically mild, in eyes irradiated to 30 Gy in 20 fractions versus higher biologically effective doses were 25 and 38%, respectively (P = 0.62). Of the five patients with aggressive lymphomas, none of the four who underwent chemotherapy with or without radiotherapy relapsed (all four remain alive), whereas the one treated with radiotherapy alone for stage IEA disease experienced a distant relapse. CONCLUSIONS: In patients with low-grade lymphomas, a good therapeutic ratio was obtained with low-dose radiotherapy alone. In patients with aggressive lymphomas, chemotherapy with or without radiotherapy resulted in excellent local control, progression-free survival, and overall survival; however, the statistical power was limited.     

嗅神经母细胞瘤综合治疗疗效及失败模式分析

目的 分析嗅神经母细胞瘤单中心的治疗疗效及失败模式。方法 回顾性分析本院1979—2014年间初诊112例无远处转移嗅神经母细胞瘤病例,改良Kadish分期A期1例、B期23例、C期60例、D期28例。手术+术后放疗±化疗者51例,放疗±化疗者46例,术前放疗+手术±化疗者11例,手术±化疗者3例,单纯化疗者1例。Kaplan-Meier法计算生存率。结果 全组5年样本数44例, 5年OS和DFS分别为66%和55%,术前放疗+手术±化疗者分别为91%和82%,手术+术后放疗±化疗者分别为80%和66%,放疗±化疗者分别为46%和37%。3例单纯手术均出现复发,1例姑息化疗者生存时间为6个月。全组共47例治疗失败,失败率为42%,以远处转移为首次失败方式者占总失败的53%、局部区域复发占36%、同时存在局部区域复发及远处转移者为11%。结论 嗅神经母细胞瘤治疗仍推荐放疗+手术综合治疗方案。综合治疗模式下嗅神经母细胞瘤局部区域控制率及疗效尚可,主要失败方式为远处转移。     

The clinical characteristics and treatment outcome of 57 children and adolescents with primary central nervous system germ cell tumors

Primary central nervous system germ cell tumors （CNS-GCTs） in children and adolescents have unique clinical features and methods of treatment compared with those in adults. There is little information about Chinese children and adolescents with CNS-GCTs. Therefore, in this study we retrospectively analyzed the clinical features and treatment outcome of Chinese children and adolescents with primary CNS-GCTs. Between January 2002 and December 2012, 57 untreated patients from a single institution were enrolled. They were diagnosed with CNS-GCTs after pathologic or clinical assessment. Of the 57 patients, 41 were males and 16 were females, with a median age of 12.8 years （range, 2.7 to 18.0 years） at diagnosis; 43 （75.4%） had non-germinomatous germ cell tumors （NGGCTs） and 14 （24.6%） had germinomas; 44 （77.2%） had localized disease and 13 （22.8%） had extensive lesions. Fifty-three patients completed the prescribed treatment, of which 18 underwent monotherapy of surgery, radiotherapy, or chemotherapy, and 35 underwent multimodality therapies that included radiotherapy combined with chemotherapy or surgery combined with chemotherapy and/or radiotherapy. PEB （cisplatin, etoposide, and bleomycin） protocol was the major chemotherapy regimen. The median follow-up time was 32.3 months （range, 1.2 to 139 months）. Fourteen patients died of relapse or disease progression. The 3-year event-free survival （EFS） and overall survival rates for all patients were 72.2% and 73.8%, respectively. The 3-year EFS was 92.9% for germinomas and 64.8% for NGGCTs （P = 0.064）. The 3-year EFS rates for patients with NGGCTs who underwent monotherapy and multimodality therapies were 50.6% and 73.5%, respectively （P = 0.042）. Our results indicate that multimodality therapies including chemotherapy plus radiotherapy were better treatment option for children and adolescents with CNS-GCTs.