Grossly-resected synovial sarcoma treated by the German and Italian Pediatric Soft Tissue Sarcoma Cooperative Groups: discussion on the role of adjuvant therapies

Differently from adult oncologists that considered synovial sarcoma (SS) a tumor with uncertain chemosensitiveness, since two decades pediatric oncologists in Europe assumed that chemotherapy played an important role in SS treatment, so most pediatric patients were included in rhabdomyosarcoma protocols, receiving adjuvant chemotherapy regardless of risk factors. The German and Italian groups reviewed the data of grossly resected SS patients in order to define a risk-adapted treatment program for the next European protocol. A total of 150 patients < 21 years with localized SS who underwent initial gross resection between 1975 and 2002 were the object of this study. All but four cases received adjuvant chemotherapy. Post-operative radiotherapy was administered to 50% Group I and to 92% Group II patients. Five-year event-free survival (EFS) and overall survival (OS) were 77% and 89%, respectively. Survival rates were influenced by tumor size (EFS 92% and 56% for size < or = and > 5 cm, respectively) and local invasiveness, not by surgical margins. No metastatic relapses occurred in Group I < or = 5 cm patients, while the outcome was poor for T2B patients (EFS 41%) due to a high rate of metastatic relapse. Our study was unable to assess the role of adjuvant treatments in grossly-resected SS, but identified a subset of low-risk patients (IRS Group I, size < or = 5 cm), for which the omission of adjuvant chemotherapy could be suggested, and a subset of high-risk patients (T2B), who need treatment intensification.     

Interstitial brachytherapy for childhood soft tissue sarcoma

BACKGROUND: To evaluate the efficacy of interstitial brachytherapy (BRT) in children undergoing combined modality treatment for soft tissue sarcomas (STS). PROCEDURE: From September 1984 to December 2003, 50 children (median age 13 years, range 1 to 18) with STS who received BRT as part of loco-regional treatment were included. There were 30 males and 20 females, the majority (68%) had primary lesions, synovial sarcoma (32%) was the most common histological type, and 26% had high-grade lesions. Treatment included wide local excision and BRT with or without external beam radiotherapy (EBRT). Thirty children (60%) received BRT alone. RESULTS: After a median follow-up of 51 months, the local control (LC), disease-free survival, and overall survival were 82%, 68%, and 71%, respectively. LC was superior in patients with tumor size 5 cm (96% vs. 67%, P = 0.04), symptom duration <2 months versus >2 months (100% vs. 73%, P = 0.05), and Grade I versus Grade II versus Grade III tumors (100% vs. 93% vs. 57%, P = 0.03). Children receiving a combination of BRT and EBRT had comparable LC to those receiving BRT alone (78% vs. 84%, P = 0.89). There was no significant difference in LC for patients receiving LDR versus HDR BRT (77% vs. 92%, P = 0.32, for BRT alone; and 67% vs. 100%, P = 0.17, for BRT + EBRT). CONCLUSION: Interstitial BRT with or without EBRT appears to result in satisfactory outcome in children with STS. Radical BRT alone, when used judiciously in select groups of children, results in excellent local control and functional outcome with reduced treatment-related morbidity.     

Treatment of soft tissue sarcomas in childhood and adolescence: results of the CWS-81 multicenter therapy study

344 previously untreated patients, under 19 years of age, with soft tissue sarcoma (STS) entered the first German STS Study, CWS-81. 218 of them with chemosensitive STS (Group A: rhabdomyosarcoma [RMS], synovial sarcoma, extraosseous Ewing's sarcoma, undifferentiated sarcoma and malignant peripheral neuroectodermal tumor) were evaluable for this analysis after a minimum potential follow-up of 6 years. A staging system based on the extent of disease, defined post-surgically, was used. The chemotherapy for stages I-III (VACA cycle) consisted of vincristine, dactinomycin, cyclophosphamide and doxorubicin. Patients with metastatic disease as well as stage III patients who failed to respond to VACA, were given ifosfamide instead of cyclophosphamide. The definitive local tumor control procedure for patients in stages II-III depended upon the tumor status at second-look surgery after 16 weeks of chemotherapy (no irradiation, 40Gy or 50Gy). The DFS rate after 5 years for group A was 57 +/- 4% and for patients with non-metastatic tumors (Stages I-III), 69 +/- 4%. There was no difference in prognosis between stages I and II (DFS rate 88 +/- 5% and 88 +/- 6% respectively). The DFS rate for stage III was 54 +/- 5% and for stage IV, 11 +/- 5%. Lack of local tumor control was the main cause of therapy failure: 10% of patients with localized disease never achieved CR, 18% relapsed locally. The most important prognostic factors were tumor size (p = .0002) and the degree of tumor regression after primary chemotherapy (p = .02).(ABSTRACT TRUNCATED AT 250 WORDS)     

Paediatric and adolescent alveolar soft part sarcoma: A joint series from European cooperative groups

Background

Alveolar soft part sarcomas (ASPS) are generally chemo‐ and radio‐resistant mesenchymal tumours, with no standardized treatment guidelines. We describe the clinical behaviour of paediatric ASPS and compare these features to previously reported adult series.

Patients and Methods

The clinical data of 51 children and adolescents with ASPS, prospectively enrolled in or treated according to seven European Paediatric trials were analysed.

Results

Median age was 13 years [range: 2–21]. Primary sites included mostly limbs (63%). IRS post‐surgical staging was: IRS‐I (complete resection) 35%, II (microscopic residual disease) 20%, III (gross residual disease) 18% and IV (metastases) 27%. Only 3 of the 18 evaluable patients (17%) obtained a response to conventional chemotherapy. After a median follow‐up of 126 months (range: 9–240), 14/18 patients with IRS‐I tumour, 10/10 IRS‐II, 7/9 IRS‐III and 2/14 IRS‐IV were alive in remission. Sunitinib treatment achieved two very good partial responses in four patients. Ten‐year overall survival (OS) and event free survival (EFS) was 78.0 ± 7% and 62.8 ± 7% respectively. Stage IV, size >5 cm and T2 tumours had a poorer outcome, but only IRS staging was an independent prognostic factor.

Conclusions

ASPS is a very rare tumour frequently arising in adolescents and in the extremities, and chemo resistant. Local surgical control is critical. ASPS is a poorly chemo sensitive tumour. For IRS‐III/IV tumours, delayed radical local therapies including surgery are essential. Metastatic patients had a poor prognosis but targeted therapies showed promising results. Pediatr Blood Cancer 2013;60:1826–1832. © 2013 Wiley Periodicals, Inc.     

The value of postoperative radiotherapy in childhood nonrhabdomyosarcoma soft tissue sarcoma

OBJECTIVE: To determine the value of postoperative radiotherapy (RT) in the management of nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) of childhood. PROCEDURE: From 1964 to 2000, 62 children with a median age of 14 years were seen at the University of Iowa and underwent a wide local excision for non-metastatic NRSTS. Tumors were high grade in 36 (58%) and >5 cm in 24 (39%). Margins of resection were negative (Group I) in 37 (60%) and positive (Group II) in 25 (40%). Postoperative RT was delivered to 20 patients (32%); eight of 37 (22%) Group I and 12 of 25 (48%) Group II children received postoperative RT. Chemotherapy was employed in 19 patients (31%). Median follow-up was 9.6 years. RESULTS: The 5- and 10-year overall survival rates for Group I were 69 and 63% and for Group II were 66 and 60%. The 5- and 10-year local control rate was 66%. On multivariate analysis, size of tumor (P < 0.001) and postoperative RT (P = 0.017) were prognostic factors for local control. All 13 Group I children with low grade, 相似文献   

Treatment outcome and dose-response relationship in infants younger than 1 year treated for retinoblastoma with primary irradiation

Thirty-three infants (<1 year at diagnosis) were treated for retinoblastoma with primary irradiation at St. Jude Children's Research Hospital (SJCRH) between 1963 and June 1992. Staging of the 44 treated eyes was as follows: Reese-Ellsworth (R-E) Groups I (n = 20), Group II (n = 9), Group III (n = 6), Group IV (n = 2), Group V (n = 7). Irradiation was delivered using either a single anterior field (31 eyes) or lens-sparing techniques (13 eyes). Total doses ranged from 21–45 Gy (median = 36 Gy) in fractions of 150–180 cGy (n = 34) or >180 cGy (n = 10). One child died of metastatic disease at 42 months. Three patients have developed second malignant neoplasms; two have succumbed at 88 and 125 months post-RB diagnosis; the remaining patients are alive at 6–259 months postdiagnosis (median follow-up = 127 months). Local control with irradiation alone and supplemented cryotherapy given within 2 months (n = 2) was maintained in 29 eyes, with no statistical difference seen for total doses ≤36 Gy (21/28 eyes) vs. >36 Gy (8/16). Of 15 eyes that required salvage therapy, tumor control has been maintained in 13. Enucleation was required for four patients, two with recurrent retinoblastoma and one with a massive retinal hemorrhage, one with a phthisical eye. Cataract formation was documented in 23 eyes (87.5%) treated with anterior field. Three patient treated with lens-sparing techniques developed cataract. At last follow-up, 23 of 30 patients tested (77%) had visual acuity of 20/100 or better. This experience confirms early observations in that doses ≥36 Gy do not appear to improve local control with irradiation alone in infants (<365 days) with retinoblastoma. © 1996 Wiley-Liss, Inc.     

Synovial sarcoma in children and adolescents: thirty three years of experience with multimodal therapy

BACKGROUND: Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment. PROCEDURE: We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors. RESULTS: With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6% (95% Confidence Interval [CI] 62-89.2%) and 87.7% (95% CI 77.3-98.1%) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (< 5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS. CONCLUSIONS: Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers. Med Pediatr Oncol 2001;37:90-96.     

Orbital sarcoma with metastases at diagnosis: A report from the soft tissue sarcoma committee of the Children's Oncology Group

We reviewed clinicopathologic features and treatment outcomes in seven patients diagnosed with Stage 4/Group IV orbital sarcoma and treated on IRSG protocols I–III. Three patients had embryonal rhabdomyosarcoma (RMS), and two patients each had alveolar RMS or unclassified sarcoma. Median age at diagnosis was 1.8 years (range 0.2–6.9 years). All patients had bone marrow involvement, including six with normal complete blood count at diagnosis. Cerebrospinal fluid was normal in six patients. Three patients survived >5 years, including one with local recurrence. In conclusion, further study is needed to determine necessity of bone marrow and CSF examination in orbital sarcoma patients. Pediatr Blood Cancer 2010;54:1045–1047 © 2010 Wiley‐Liss, Inc.     

CDV与CiE两种方案联合术前化疗治疗晚期儿童神经母细胞瘤的近期疗效观察

目的观察CDV(环磷酰胺+柔红霉素+长春新碱)与CiE(顺铂+足叶乙甙)联合术前化疗治疗晚期神经母细胞瘤患儿的近期疗效及相关毒性。方法回顾分析27例IV期神经母细胞瘤患儿临床资料,年龄1岁2个月至8岁,原发部位腹部21例,后纵隔4例,盆腔2例,骨髓转移23例,多发性骨转移12例。均经CDV方案3疗程及CiE方案2疗程化疗。应用神经母细胞瘤治疗反应评价标准和美国国立癌症研究所通用不良反应分级标准对化疗近期疗效和相关毒副反应进行评价。结果27例患儿经术前化疗,总有效率为82%;辅助化疗后24例患儿接受手术治疗,完全切除原发肿瘤14例(58%),部分切除10例(42%)。化疗相关毒副反应主要是骨髓抑制,27例患儿粒细胞下降均为IV级;血红蛋白下降III级7例(26%),IV级20例(74%);血小板下降III级2例(7%),IV级25例(93%)。所有患儿均出现不同程度的感染,经积极的抗感染治疗可以控制。消化道、肝脏、肾脏损害多为Ⅰ、Ⅱ级,经治疗可恢复。2例(7%)出现Ⅰ级神经毒性病例。无心功能受损病例。结论CDV与CiE联合术前化疗方案治疗IV期神经母细胞瘤有转好的疗效,可为手术治疗创造条件。     

Use of Irradiation for Therapy of Retinoblastoma in Children More Than 1 Year Old: The St. Jude Children's Research Hospital Experience and Review of Literature

Fifteen children (>1 year old at diagnosis) were treated for retinoblastoma (RB) with primary irradiation at St. Jude Children's Research Hospital between January 1963 and January 1992. Staging of the 19 treated eyes was as follows: Reese-Ellsworth (R-E) Groups I and II, n = 7; Group III, n = 3; Group IV, n = 1; Group V, n = 7; information on one globe is incomplete. Total dosage ranged from 25 to 45 Gy (median = 40 Gy) in fractions of 170–225 cGy delivered 5x/week (n = 13) or 225–265 cGy delivered 4x/week (n = 4 eyes) or x5/week (n = 2 eyes). Treatment techniques included anterior field (n = 11) or lens-sparing technique (n = 8). One patient has died of progressive central nervous system (CNS) disease at 13 months; one patient succumbed to second malignant neoplasm within the irradiated field at 194 months; the remaining 13 patients remain alive from 27 to 301 months (median = 178 months). Local control with irradiation alone has been maintained in 12 eyes. Four eyes with local recurrence were salvaged using cryotherapy or reirradiation. Enucleation was required for three eyes for progressive disease at 4, 7, and 7 months postirradiation. Cataract formation was documented in nine eyes treated with anterior fields and in one patient treated with lens-sparing technique. At last follow-up, 7 patients had visual acuity of 20/100 or better. Radiation dose of 40 Gy appears to be adequate for local control of early stage RB (R-E Groups I-III and VB whose tumors are R-E Groups I-III) in patients >1 year old. The results of this limited series which has lengthy follow-up is compared with the results of previously published data in an effort to define the benefits and disadvantages of the different treatment techniques which have been reported in the treatment of RB in children >1 year old. © 1995 Wi1ey-Liss, Inc.     

Cerebrospinal fluid C-reactive protein measurement--a bedside test in the rapid diagnosis of bacterial meningitis

C-reactive protein (C-RP) determinations were performed by the Latex agglutination method on the cerebrospinal fluid (CSF) samples of 212 patients with clinical features suggestive of meningitis. Patients were grouped as follows Group I: bacterial meningitis and partially treated bacterial meningitis (n = 22). Group II: viral encephalitis (n = 11). Group III: tuberculous meningitis (n = 18). Group IV: (i) febrile convulsions (n = 87); (ii) epileptic seizures (n = 70); (iii) intracranial haemorrhage (n = 4). C-RP was a better indicator of bacterial meningitis (sensitivity 91 per cent) than the Gram's stain (sensitivity 46 per cent). C-RP was positive in 91 per cent of patients in Group I, none in Groups II and III and 0.6 per cent in Group IV. C-RP determination in CSF proved to be a useful indicator of bacterial meningitis and served to distinguish it from viral encephalitis, tuberculous meningitis, febrile convulsions and other central nervous system disorders.     

Alveolar soft part sarcoma in children and adolescents: Clinical features and outcome of 11 patients

The clinical features and response to therapy of pediatric alveolar soft part sarcoma, a rare soft tissue sarcoma of uncertain histogenesis, have not been previously described in detail in the literature. We retrospectively reviewed the clinical characteristics of all patients with alveolar soft part sarcoma who were seen at our institution over a 32-year period. We found 11 patients with the diagnosis of alveolar soft part sarcoma. Their ages ranged from 2.8–16 years (median 9.8). Staging was determined using the Intergroup Rhabdomyosarcoma Study clinical grouping system and the UICC TNM system. Accordingly, there were six patients with grossly resected tumors (clinical groups I and II) and five with unresected or metastatic disease (clinical groups III and IV). Children with resected disease were more likely to have smaller noninvasive tumors. The main feature predictive of survival was tumor resectability, since chemotherapy in various combinations failed to produce significant tumor responses. Nine patients are disease-free with a median follow-up of 11.9 years. Surgical resection remains the mainstay of therapy for pediatric alveolar soft part sarcoma. Since active chemotherapy agents have not been identified, patients with unresected or metastatic disease may benefit from experimental agents. The survival rate of this cohort is superior to that seen in adults. © 1996 Wiley-Liss, Inc.     

Positive skin test and age

OBJECTIVE: To evaluate positive responses to skin tests for immediate hypersensitivity to allergens in children with asthma and rhinitis at different ages. METHOD: We observed positive skin test reactivity in prick tests using fifteen allergens of same origin (total dust and Dermatophagoides sp.; Dermatophagoides pteronyssinus; Dermatophagoides farinae; Blomia tropicalis; Penicillium sp; Alternaria alternata; Cladosporium herbarium; Aspergillus fumigatus; Bermuda grass; forage grass; dog and cat epithelia; feathers; Blatella germanica and wool). We placed 713 selected patients into different age groups - Group I: 6 to 11 months; Group II: 1 to 3 years and 11 months; Group III: 4 to 8 years and 11 months; and Group IV: 9 to 15 years. We used the chi-square test for statistical analysis. RESULTS: The total significant differences between these groups were: I to II = 5; II to III = 5; II to IV = 5; III to IV = 6; I to III = 10; and I to IV = 10. CONCLUSION: Skin test reactivity is acquired progressively with age, and can be observed as early as at 12 months. Reactivity is significantly more positive from the age of 4 on.     

Controversies in the management of paratesticular rhabdomyosarcoma: is staging retroperitoneal lymph node dissection necessary for adolescents with resected paratesticular rhabdomyosarcoma?

PURPOSE: Use of retroperitoneal lymph node dissection (RPLND) in paratesticular rhabdomyosarcoma (PTRMS) is controversial and has changed over the past 2 decades. The Intergroup Rhabdomyosarcoma Study Group (IRSG) required ipsilateral RPLND (IRPLND) for all patients with PTRMS treated on IRS-III (1984-91), but changed to clinical evaluation of RPLNs using computerized tomography (CT) in IRS-IV (1991 through 1997). In IRS-IV, only those patients with identified lymph node involvement on CT required surgical evaluation of the RPLNs. Nodal radiation therapy was administered only to patients with RPLNs recognized as positive; such patients received more intensive chemotherapy as well. Thus, they compared the incidence of recognized RPLN involvement using these 2 different approaches. They then analyzed patient outcome to determine whether this change in management affected outcome. METHODS: Eligible patients with group I or II PTRMS who were treated on IRS III (n = 100) or IRS IV (n = 134) were analyzed. Failure-free survival (FFS) and survival (S) rates were estimated using the Kaplan-Meier method and compared using the log-rank test. RESULTS: There was a significant change in the distribution of patients with group I versus II tumors from IRS-III to IRS-IV (group I, 68% in IRS-III versus 82% in IRS-IV). This was the result of decreased node recognition when CT was used to stage RPLNs in IRS-IV and was most notable for adolescents (>10 years of age). Overall, 3-year FFS was 92% for patients treated on IRS-III and 86% for those treated on IRS-IV (P =.10), whereas survival estimates were 96% and 92%, respectively (P =.30). Adolescents were at higher risk of RPLN relapse than were children (<10 years of age) and their FFS and survival were worse, regardless of IRS protocol. Furthermore, adolescents with recognized group II tumors experienced better 3-year FFS than those with group I tumors on IRS-IV (100% versus 68%, P =.06), most likely as a result of receiving radiotherapy and intensified chemotherapy. CONCLUSIONS: Use of only CT scan evaluation of RPLN in IRS-IV led to a decrease in identification of RPLN involvement in boys who present with localized PTRMS, and a higher rate of regional relapse as compared with IRS-III. Adolescents had much higher likelihood of RPLN disease, and they fared significantly worse than did younger children on both studies. Furthermore, adolescent boys with group I tumors experienced worse FFS than those with Group II tumors on IRS-IV, probably because some patients with group II tumors were not identified by CT imaging and thus received less effective therapy. These data suggest that adolescents should have ipsilateral RPLN dissection as part of their routine staging, and those with positive lymph nodes require intensified chemotherapy as well as nodal irradiation.     

Delayed resection in the management of non-orbital rhabdomyosarcoma of the head and neck in childhood

This retrospective study was undertaken to evaluate the effect of delayed resection on outcome of head and neck rms in a single institution which has experience in cranial base surgery. Since 1988, patients with primary non-orbital rms of the head and neck following treatment at the Children's Hospital of Pittsburgh, were evaluated by the Department of Otolaryngology, Eye and Ear Hospital at the University of Pittsburgh Medical Center either at the time of presentation or when response to chemotherapy and/or radiation therapy was thought to have been optimized for the possibility of definitive surgery. Medical records of patients who did or did not have delayed surgery were reviewed and compared with respect to demographics, tumor stage, response to therapy, survival, and cosmetic results. Of 16 children diagnosed with non-orbital head and neck rms from 1988–1994 and treated with chemotherapy according to IRS II–IV, 3 had group I or II disease following extensive surgery at diagnosis. Thirteen had group III or IV disease. Of these, 6 patients had delayed resection and 7 did not. Delayed resection was undertaken 3–12 months (median, 4 months) from diagnosis in 4 children who had a partial response (PR) and 2 children who had stable disease (SD) with chemotherapy and/or radiation. Delayed resection converted all children to complete responses (CR), including one child with clinical SD and one with PR who were found to have no viable tumor at surgery. The overall percentages of CRs for patients with group II or IV disease (documented any time post-diagnosis) were at least as good for patients who had undergone delayed surgery as for those who had not (100% vs. 71%, p = .465). Median survivals for patients with advanced disease were 3 ½ years and 2 years, respectively (p = .2801). Cosmetic and functional problems attributable to surgery were not severe but included facial asymmetry (n = 4), trismus (n = 1), cranial nerve deficits (n = 1), and abnormal dentition (n = 1). In locally extensive head and neck rms, cranial base surgery should be considered after initial cytoreductive therapy, since it may contribute to achievement of CR and to survival with acceptable morbidity. Med. Pediatr. Oncol. 28:294–298. © 1997 Wiley-Liss, Inc.     

Interstitial irradiation of cerebral gliomas in childhood by permanently implanted 125-iodine--preliminary results

19 children with a deeply located cerebral glioma were treated with continuous interstitial irradiation (stereotactically implanted 125-iodine). The accumulated dose at the tumor surface ranged in the low grade glioma group (Group A) from 55 to 100 Gy and in the high grade glioma group (group B) from 50 to 65 Gy. Patients in group B additionally received a fractionated external beam irradiation (15-20 Gy boost dose). Tumor shrinkage as seen on CT-scans 6 months postoperatively could be achieved in 100% of patients with a low grade glioma. The response rate of 6 children with a high grade glioma was 83%. With a mean follow up of 57.0 months (group A) and 56.2 months (group B) respectively the estimated 4.5 year survival probability was 92% for low grade gliomas and 83% for grade III/grade IV lesions. Permanent interstitial irradiation offers the possibility of local tumor control with low risk of severe side effects. The survival rates are promising and comparable with results of other study groups. For gliomas grade I and grade II a dose reduction may be feasible.     

Role of ethamsylate in preventing periventricular-intraventricular hemorrhage in premature infants below 34 weeks of gestation

OBJECTIVE: To determine the role of ethamsylate in prevention of PVH-IVH in premature infants <34 weeks gestational age. DESIGN: Prospective, randomized, controlled study. METHODS: Infants less than 34 weeks gestational age were included in the trial. Neonates with congenital malformations, family history of bleeding disorders and with Apgar scores <5 at 5 minutes were excluded. Subjects were randomized into two groups--Group A infants received intravenous ethamsylate (12.5 mg/kg) six hourly for four days and Group B infants served as a control group. Regular cranial ultrasounds to detect the presence of PVH-IVH were done between days 3-5, 10-14 and 28-30 of post natal age, and before hospital discharge in all infants and weekly in infants detected to have PVH-IVH on earlier scans. Various antenatal and postnatal factors known to affect the incidence of PVH-IVH were recorded. RESULTS: A total of 192 infants underwent the trial, 93 in Group A and 99 in Group B. Antenatal corticosteroids (1 or 2 doses) were administered to 32 ( 34.4%) and 36 (36.3%) women in Group A and Group B, respectively. None of the mothers received phenobarbitone, vitamin K or indomethacin antenatally and none of the infants received phenobarbitone, vitamin E or indomethacin postnatally during the study period. PVH-IVH was seen in 26 infants in Group A, of which Grade I IVH occurred in 9, Grade II in 14, Grade III in 2 and Grade IV in one infant. Twenty-nine infants had PVH-IVH in Group B of which 11 had Grade I, 15 Grade II and 3 Grade III. None of the differences were statistically significant. CONCLUSION: Postnatal administration of ethamsylate did not decrease the incidence of PVH-IVH in the study infants.     

CDV与CiE两种方案联合术前化疗治疗晚期儿童神经母细胞瘤的近期疗效观察

目的:观察CDV(环磷酰胺+柔红霉素+长春新碱)与CiE(顺铂+足叶乙甙)联合术前化疗治疗晚期神经母细胞瘤患儿的近期疗效及相关毒性。方法:回顾分析27例IV期神经母细胞瘤患儿临床资料,年龄1岁2个月至8岁,原发部位腹部21例,后纵隔4例,盆腔2例,骨髓转移23例,多发性骨转移12例。均经CDV方案3疗程及CiE方案2疗程化疗。应用神经母细胞瘤治疗反应评价标准和美国国立癌症研究所通用不良反应分级标准对化疗近期疗效和相关毒副反应进行评价。结果:27例患儿经术前化疗,总有效率为82%;辅助化疗后24例患儿接受手术治疗,完全切除原发肿瘤14例（58%）,部分切除10例（42%）。化疗相关毒副反应主要是骨髓抑制,27例患儿粒细胞下降均为IV级;血红蛋白下降III级7例(26%),IV级20例(74%);血小板下降III级2例(7%),IV级25例(93%)。所有患儿均出现不同程度的感染,经积极的抗感染治疗可以控制。消化道、肝脏、肾脏损害多为Ⅰ、Ⅱ级,经治疗可恢复。2例（7%）出现Ⅰ级神经毒性病例。无心功能受损病例。结论: CDV与CiE联合术前化疗方案治疗IV期神经母细胞瘤有转好的疗效,可为手术治疗创造条件。［中国当代儿科杂志,2009,11（11）：885-887］     

Treatment results for patients with localized,completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984–1997: A report from the Children's Oncology Group

Purpose

To assess local control, event‐free survival (EFS), and overall survival (OS) rates in 71 patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma (ALV RMS) and their relation to radiation therapy (RT) on IRSG Protocols III and IV, 1984–1997.

Methods

Chart review and standard statistical procedures.

Patients and Tumors

Patients were 1–18 years at diagnosis (median, 6 years). Primary tumor sites were extremity/trunk (N = 54), head/neck (N = 9), genitourinary tract (N = 7), and perineum (N = 1). Thirty patients received VA ± C with RT; 41 received VA ± C alone. RT was assigned, not randomized.

Results

Fifty‐four patients had Stage 1 (favorable site, any size) or Stage 2 (unfavorable site, ≤5 cm) tumors. Eight‐year EFS was 90%, with 100% local control for 17 patients given RT. Eight‐year EFS was 88%, with 92% local control for 37 patients without RT; P = 0.52 for EFS comparisons, 0.3 for local control comparisons. In 17 Stage 3 patients (unfavorable site, tumors >5 cm, N0), 8‐year EFS was 84% with 100% local control in 13 patients given RT; 8‐year EFS was only 25% and local control 50% in 4 patients without RT. Local recurrence was the most common site of first failure in non‐irradiated patients.

Conclusion

Patients with Stage 1–2 ALV RMS had slightly but statistically insignificantly improved local control, EFS, and OS rates when local RT was given. The need for local RT in Stage 1–2 patients deserves evaluation in a randomized study. Local control, EFS, and OS rates were significantly improved in Stage 3 patients receiving local RT. Pediatr Blood Cancer. 2010;55:612–616. © 2010 Wiley‐Liss, Inc.     

Outcome of patients with undifferentiated embryonal sarcoma of the liver treated according to European soft tissue sarcoma protocols

Background

To assess the outcomes of pediatric patients with undifferentiated embryonal sarcoma of the liver (UESL) and treatment including at least surgery and systemic chemotherapy.

Methods

This study included patients aged up to 21 years with a pathological diagnosis of UESL prospectively enrolled from 1995 to 2016 in three European trials focusing on the effects of surgical margins, preoperative chemotherapy, use of radiotherapy (RT), and chemotherapy.

Results

Out of 65 patients with a median age at diagnosis of 8.7 years (0.6–20.8), 15 had T2 tumors, and one had lymph node spread, 14 were Intergroup Rhabdomyosarcoma Study (IRS) I, nine IRS II, 38 IRS III, and four IRS IV. Twenty-eight upfront surgeries resulted in five operative spillages and 11 infiltrated surgical margins, whereas 37 delayed surgeries resulted in no spillages (p = .0119) and three infiltrated margins (p = .0238). All patients received chemotherapy, including anthracyclines in 47. RT was administered in 15 patients. With a median follow-up of 78.6 months, 5-year overall and event-free survivals (EFS) were 90.1% (95% confidence interval [CI]: 79.2–95.5) and 89.1% (95% CI: 78.4–94.6), respectively. Two out four local relapses had previous infiltrated margins and two out of three patients with metastatic relapses received reduced doses of alkylating agents. Infiltrated margins (p = .1607), T2 stage (p = .3870), use of RT (p = .8731), and anthracycline-based chemotherapy (p = .1181) were not correlated with EFS.

Conclusions

Multimodal therapy improved the outcome of UESL. Neoadjuvant chemotherapy for pediatric patients increases the probability of complete surgical resection. The role of anthracyclines and RT for localized disease remains unclear.