Nasopharyngeal carcinoma in Turkish children: review of 33 cases.

A retrospective and prospective analysis is reported of epidemiological, clinical, and therapeutic aspects of 33 children with nasopharyngeal carcinoma who were treated in a single institution over a period of 10 years. Twenty-three male and 10 female children ranging from 9 to 17 years were referred to our center. Histopathology was WHO type 3 carcinoma in 21, WHO type 2 in 8, WHO type 1 in 1, and unclassified in 3 patients. Disease extent was T2a (n = 15), T2b (n = 2), T3 (n = 11), and T4 (n = 5); N1 (n = 5), N2 (n = 12), and N3a (n = 16). Five patients had base of skull invasion. Four patients had M1 disease on admission. Four patients were treated with irradiation only. Three patients received neoadjuvant, 4 patients received adjuvant, and 22 patients received neoadjuvant + adjuvant chemotherapy in addition to radiotherapy. Patients received 50-72 Gy to the primary tumor and involved nodes and 45-50 Gy to uninvolved regions. Chemotherapy consisted of combinations of cisplatin, fluorouracil or Adriamycin, vincristine, and cyclophosphamide. Twenty-nine patients (88%) attained locoregional control. Overall, 10 patients died with progressive disease or infectious complications, and 2 patients are still receiving therapy. Three patients are still living with multiple metastases and stable disease. Eight patients were lost to follow-up. Twelve patients are alive without relapse 3 and 63 months from diagnosis. Seven patients had 6 relapses at distant and 1 relapse at local site. The median time for first relapse was 8 months. Overall, the 5-year survival rate was 63% and disease-free survival rate was 53%. Although the locoregional control rate is high, long-term survival rates will be the real test of the impact of chemotherapy. Further studies are needed to confirm the optimal combination of effective chemotherapeutic agents and radiotherapy.     

Intensive chemotherapy in children with stage IV neuroblastoma

A retrospective analysis of effectiveness of sequential chemotherapy with cyclophosphamide, doxorubicin, cisplatin and etoposide in children with stage IV neuroblastoma was undertaken. Study group included 17 children of mores than one year old with median age of 3 years (range 18 months to 7 years). Fourteen were males and three females. Sites of primary tumor were abdomen in 12 patients, pelvis in 3, paravertebral in 1 and unknown in 1. Metastatic sites included bone marrow (88%), bone (82%), orbit (29.4%) and lymph node (11.7%). One patient had brain parenchymal disease and another had cerebrospinal fluid positivity for malignant cells. Fifteen of the 17 patients had major response with chemotherapy (complete response in two and partial response in 13). Ten of the 15 patients completed four courses of chemotherapy and five patients progressed while on chemotherapy and died. Only two of the ten patients, who had four courses chemotherapy are alive after 2 years. Hence the 2-year survival in this series is 11.7%. There was no toxic death in this study.     

Combined radiation and chemotherapy for advanced undifferentiated nasopharyngeal carcinoma in children

Five children, 11–16 years of age at diagnosis, with advanced (stage IV) undifferentiated nasopharyngeal carcinoma, are reviewed. All had radiotherapy and chemotherapy, four at first treatment, are disease free in 8 months, 6, 10, and 13 years from diagnosis. One patient who had radiotherapy only as primary treatment and chemotherapy for metastases, died 15 months from diagnosis. One patient who received 60 Gy and chemotherapy according to the BACON protocol had severe early toxicity and severe late sequelae of treatment. Three patients who received chemotherapy according to a modified UICC-2 protocol for nasopharyngeal carcinoma and 50 to 54 Gy to the primary site had only mild early toxicity and mild late effects of treatment. With lower radiation doses, adjusted to the effect of preradiation chemotherapy, complete tumor control was achieved and acute and long-term morbidity reduced. Med. Pediatr. Oncol. 28:366–369, 1997. © 1997 Wiley-Liss, Inc.     

14例肝母细胞瘤患儿的临床疗效观察

目的：通过对儿童肝母细胞瘤患儿的临床治疗结果的回顾总结,对ICE化疗方案的有效性和安全性进行评估。方法：自2000年6月至2008年6月,14例初发患儿入选,男7例,女7例,中位年龄：1.33岁（范围0.25～8.25岁）。临床分期：Ⅰ期6例,Ⅱ期1例,Ⅲ期5例,Ⅳ期2例。诊断时血甲胎蛋白（AFP）水平显著升高13例,1例AFP正常。采用多科室协作模式进行治疗,其中一期手术8例,3例进行了二期手术。化疗方案采用ICE方案,14例患儿共接受了73个疗程化疗,其中术前化疗25个疗程。结果：14例患儿治疗后有效12例（85.7%）,其中完全缓解10例（71.4%）,部分缓解2例,2例无效。随访至2008年7月31日,疾病处于长期完全缓解者9例（64.3%）,中位随访时间为35个月（范围：16～96个月）。5年总生存率（OS）为：（70.71±12.37）%,5年无事件生存率（EFS）为：（64.29±12.81）%。1例患儿复发,2例失访。结论：ICE化疗方案联合手术治疗能有效并且安全地治疗儿童肝母细胞瘤,Ⅳ期患儿的治疗有待于进一步研究。［中国当代儿科杂志,2009,11（8）：659-662］     

NASOPHARYNGEAL CARCINOMA IN TURKISH CHILDREN: Review of 33 Cases

A retrospective and prospective analysis is reported of epidemiological, clinical, and therapeutic aspects of 33 children with nasopharyngeal carcinoma who were treated in a single institution over a period of 10 years. Twenty-three male and 10 female children ranging from 9 to 17 years were referred to our center. Histopathology was WHO type 3 carcinoma in 21, WHO type 2 in 8, WHO type 1 in 1, and unclassified in 3 patients. Disease extent was T2a (n = 15), T2b (n = 2), T3 (n = 11), and T4 (n = 5); N1 (n = 5), N2 (n = 12), and N3a (n = 16). Five patients had base of skull invasion. Four patients had M1 disease on admission. Four patients were treated with irradiation only. Three patients received neoadjuvant, 4 patients received adjuvant, and 22 patients received neoadjuvant + adjuvant chemotherapy in addition to radiotherapy. Patients received 50-72 Gy to the primary tumor and involved nodes and 45-50 Gy to uninvolved regions. Chemotherapy consisted of combinations of cisplatin, fluorouracil or Adriamycin, vincristine, and cyclophosphamide. Twenty-nine patients (88%) attained locoregional control. Overall, 10 patients died with progressive disease or infectious complications, and 2 patients are still receiving therapy. Three patients are still living with multiple metastases and stable disease. Eight patients were lost to follow-up. Twelve patients are alive without relapse 3 and 63 months from diagnosis. Seven patients had 6 relapses at distant and 1 relapse at local site. The median time for first relapse was 8 months. Overall, the 5-year survival rate was 63% and disease-free survival rate was 53%. Although the locoregional control rate is high, long-term survival rates will be the real test of the impact of chemotherapy. Further studies are needed to confirm the optimal combination of effective chemotherapeutic agents and radiotherapy.     

Nasopharyngeal carcinoma in children and young adults—Beyond 5‐year survival

Nasopharyngeal carcinoma (NPC) is a rare and locally aggressive form of childhood cancer. Treatment of NPC includes chemotherapy and radiotherapy. With current treatment protocols, survival rates for patients with nonmetastatic disease is over 80%. Data regarding very late events including long‐term treatment‐related morbidities and second malignancies are scarce. We present our data on 42 patients with NPC treated in Israel between 1989 and 2014, and followed until 2019. During follow up, five patients had disease recurrence, and four children developed secondary malignancy. Median time to diagnosis of secondary malignancy was 105 months. Eighty‐eight percent of patients have long‐term treatment‐related morbidities.     

Nasopharyngeal cancer of childhood and adolescence: a single institution experience

Nasopharyngeal cancer is rare in childhood and results with radiotherapy are far from encouraging. A total of 52 patients with stage I to IVB nasopharygeal cancer and age <18, received radiotherapy to 60-66 Gy in 2-Gy fractions to the nasopharynx and cervical nodes, while 22 of these patients also received chemotherapy with cisplatin and 5 FU. Three-year disease-free survival with concurrent chemotherapy was 82% compared to 40% for patients who had radiotherapy alone (p = .001; HR 0.33; 95% CI 0.25-0.74). The 3-year overall survival in the patients who received radiotherapy was 72% and that in the patients who received concurrent chemotherapy was 77% (p = .38). A statistically significant improvement in disease-free survival was observed with concurrent chemoradiation in nonmetastatic nasopharyngeal cancer in young patients.     

Prognostic factors and secondary malignancies in childhood medulloblastoma

PURPOSE: Little is known of the outcome of long-term survivors of childhood medulloblastoma, one of the most common pediatric malignancies. To determine the potential for secondary malignancies, a retrospective outcome evaluation in 88 consecutive cases of childhood medulloblastoma was performed. PATIENTS AND METHODS: The records of all patients with childhood medulloblastoma diagnosed at Children's National Medical Center in Washington, DC from 1969 through 1997 were reviewed. RESULTS: The median follow-up time was 92 months (range 6-257 months). Overall survival was 59% at 5 years and 52% at 10 years. Univariate analysis showed that age at diagnosis, extent of surgical resection, presence of metastatic disease (M stage), ventriculoperitoneal shunt placement within 30 days from diagnosis, posterior fossa radiation therapy dose, and adjuvant chemotherapy significantly affected survival. Although based on small numbers, the risk of second neoplasms was significantly increased in this cohort. Multiple basal cell carcinomas developed in the areas of radiation therapy in two patients; these patients also had nevoid basal cell carcinoma syndrome (NBCCS) diagnosed. One other patient died of glioblastoma multiforme 8 years after treatment of medulloblastoma. A meningioma developed in another patient 10 years after radiation therapy. CONCLUSION: As survival of medulloblastoma patients improves, increased surveillance regarding secondary malignancies is required, especially because radiation-induced tumors may occur many years after treatment. These two cases of NBCCS also illustrate the importance of considering the concomitant diagnosis of NBCCS in young patients with medulloblastoma. In those patients, alternative therapy should be considered to minimize radiation therapy-related sequelae.     

Results of Treatment of 18 Children With Hodgkin Disease With MOPP Chemotherapy as the Only Treatment Modality

Eighteen children with Hodgkin disease (16 previously untreated; two relapsed) were treated with MOPP chemotherapy (nitrogen mustard, vincristine, prednisone, procarbazine) only. Ten had clinical stage I and II disease, four had stage III, and four had stage IV. In ten patients, the clinical stage was confirmed by staging laparotomy. Six courses of MOPP were given to eight stage I and II patients and two stage IV patients. Between 7 and 12 courses were given to two stage I and II, and six stage III and IV patients. Dose reduction of 75-50% was required in 13% and delay of treatment in 22% of the first six courses of MOPP. Hematologic toxicity, minor and major viral infections, and nausea and vomiting were the major complications. Complete remission (CR) was obtained in 17 patients. Of these 17, there has been one death in CR, and one relapse. Sixteen patients have discontinued treatment and have been observed off treatment for 8 months to 7.5 years. The actuarial disease-free survival with a median follow-up of 28 months is 80% and overall survival is 92%.     

Total body irradiation and melphalan as a conditioning regimen for children with hematological malignancies undergoing transplantation with stem cells from HLA‐identical related donors

Watanabe N, Takahashi Y, Matsumoto K, Horikoshi Y, Hama A, Muramatsu H, Yoshida N, Yagasaki H, Kudo K, Horibe K, Kato K, Kojima S. Total body irradiation and melphalan as a conditioning regimen for children with hematological malignancies undergoing transplantation with stem cells from HLA‐identical related donors.
Pediatr Transplantation 2011: 15: 642–649. © 2011 John Wiley & Sons A/S. Abstract: Although some studies have reported that TBI and MEL offer an effective conditioning regimen for autologous SCT in acute leukemia, little has been reported regarding outcomes of allogeneic SCT. We retrospectively evaluated outcomes for 50 pediatric patients who underwent allo‐SCT conditioned with intravenous MEL (180–210 mg/m²) and fractionated TBI (12–13.2 Gy) from HLA‐identical related donors. Nineteen patients were in CR1, 18 were in CR2, and 13 showed advanced‐stage disease (≥CR3). Patients had received allo‐SCT from HLA‐identical siblings (n = 45) or phenotypically HLA‐identical family donors (n = 5). Median duration of follow‐up for all disease‐free patients was 61 months (range, 8.8–177 months). At the time of analysis, 12 patients had died. Eleven of those died of relapse, and one died of TRM. DFS rates for all patients, patients with AML (n = 12), and patients with lymphoid malignancy (n = 38) were 61.4% and 82.1%, respectively. DFS rates for CR1, CR2, and ≥CR3 cases were 89.2%, 88.1%, and 23.1%, respectively (p < 0.05). MEL/TBI for pediatric patients with hematological malignancies was associated with lower relapse rates and no increase in toxicity, resulting in better survival.     

Secondary neoplasms after radiotherapy for a childhood solid tumor

This study was conducted to determine the outcome of patients who develop a second neoplasm after radiotherapy (RT) for a childhood solid tumor. From 1956 to 1998, 429 children with a malignant solid tumor were treated at a single radiation oncology facility. The medical records and radiotherapy charts were reviewed to determine if the patient developed a secondary neoplasm after treatment for malignancy. Twenty-three (5.4%) patients developed a second neoplasm. There were 12 males and 11 females with a median age at RT of 6.6 years (range, 2 months to 20 years). There were 14 malignant neoplasms in 13 (3.0%) and 14 benign neoplasms in 11 patients (2.6%). The types of initial solid tumors treated with RT were Ewing sarcoma in 6, Wilms tumor in 6, medulloblastoma in 5, neuroblastoma in 3, and other in 3. Median RT dose was 45 Gy (range, 12.3 to 60 Gy) using 4 MV in 9, 1.25 MV in 8, 250 KV in 4, and 6 MV photons in 1 patient. One child was treated using 15-MeV electrons. Fourteen had chemotherapy. Median follow-up was 23.2 years (range, 5.3 to 44.4 years). For the 14 malignant neoplasms, the median time interval from initial tumor to second malignancy was 10.1 years. The 14 second malignant neoplasms (SMN) were osteosarcoma in 3, breast carcinoma in 2, melanoma in 2, malignant fibrous his- tiocytoma in 1, dermatofibrosarcoma in 1, leiomyosarcoma in 1, mucoepidermoid carcinoma in 1, colon cancer in 1, chronic myelogenous leukemia in 1, and basal cell carcinoma in 1. Ten of the 14 SMN (71%) were at the edge or inside the RT field. The 5- and 10-year overall survival rate after diagnosis of an SMN was 69.2%; it was 70% for children with a SMN at the edge or inside the RT field and 66.7% for those outside of the RT field. The 14 benign neoplasms appeared at a median time of 16.9 years and included cervical intraepithelial neoplasia in 3, osteochondroma in 3, thyroid adenoma in 1, duodenal adenoma in 1, lipoma in 1, cherry angioma in 1, uterine leiomyoma in 1, ovarian cystadenofibroma in 1, and giant cell tumor in 1. Only 5 (36%) of the 14 benign tumors occurred in the RT field, with osteochondroma being the most common. Of 189 deaths occurring in 429 patients, only 3 (1.6%) were secondary to radiation-induced malignancy. Not all SMN in children receiving RT occur in the irradiated field. More than two-thirds of children with a radiation-induced malignancy are alive 10 years after the diagnosis of a SMN.     

NASOPHARYNGEAL CANCER OF CHILDHOOD AND ADOLESCENCE: A Single Institution Experience

Nasopharyngeal cancer is rare in childhood and results with radiotherapy are far from encouraging. A total of 52 patients with stage I to IVB nasopharygeal cancer and age <18, received radiotherapy to 60–66 Gy in 2-Gy fractions to the nasopharynx and cervical nodes, while 22 of these patients also received chemotherapy with cisplatin and 5 FU. Three-year disease-free survival with concurrent chemotherapy was 82% compared to 40% for patients who had radiotherapy alone (p =. 001; HR 0.33; 95% CI 0.25–0.74). The 3-year overall survival in the patients who received radiotherapy was 72% and that in the patients who received concurrent chemotherapy was 77% (p =. 38). A statistically significant improvement in disease-free survival was observed with concurrent chemoradiation in nonmetastatic nasopharyngeal cancer in young patients.     

Intraoperative electron beam treatment for pediatric malignancies: The Ohio State University experience

PURPOSE: To report the result of intraoperative electron beam radiation therapy (IOERT) in patients with extensive pediatric tumors. METHOD: From October 1989 through June 2000, 13 children were treated with chemotherapy, maximal surgery, and 10-15 Gy IOERT at a total of 18 sites. IOERT was used for palliative purposes in 5 children with metastatic disease and in 3 others who were previously treated with external beam radiation (EBRT). The remaining five patients received definitive IOERT. Postoperative EBRT of 35.4-45 Gy was given in 5 patients. RESULTS: After a median follow-up of 42 months (range = 18-63 months), 4 patients were alive and without evidence of disease. Overall and 3 year actuarial survival rates were 31% (4/13) and 26%, respectively. Local control was achieved at 13/18 sites (72%). Poor prognostic factors included metastatic disease, recurrent disease, and the absence of adjuvant EBRT. Two children with Wilms tumors had 100% local control, disease-free survival, and overall survival without the addition of EBRT. CONCLUSION: A boost dose of IOERT allows for reduction in the dose of EBRT, thereby limiting growth-related morbidity without compromising local control or disease-free survival. Except for Wilms tumors, which achieved 100% local control and disease-free survival, adjuvant EBRT is necessary for successful local control and survival in children with soft tissue sarcomas. Based on this study and others, intraoperative irradiation should be considered for inclusion in prospective, multi-institutional trials designed to treat localized malignancies in young children.     

Local control of parameningeal rhabdomyosarcoma: outcomes in non-complete responders to chemoradiation

PURPOSE: To determine whether there is a role for surgery in Group III and IV patients with residual parameningeal rhabdomyosarcoma (PM-RMS) after radiotherapy (RT). MATERIALS AND METHODS: From 1965 to 2000, 29 patients with PM-RMS (Group III 27, Group IV 2) were diagnosed and treated with RT at the University of Iowa. All patients received chemotherapy consisting of vincristine, dactinomycin, and cyclophosphamide (VAC) in 17 (59%), VAC + doxorubicin in six (21%), VC + doxorubicin in three (10%), and other in three. RT was given to the primary site. Median dose was 50.4 Gy (range, 41.4-65 Gy). Two had hyperfractionated RT (59.4 Gy in 54 fractions). Median follow-up time for surviving patients was 17.9 years (range, 1.5-31.5 years). RESULTS: The 2- and 5-year overall survival rates were 78.9% and 45.7% while the 2- and 5-year freedom from local progression rates were 56.8% and 42.1%. For the 11 patients who did not achieve a complete response to chemoradiotherapy at the primary site, eight underwent surgical resection 1.5-7 months after RT. The 2- and 5-year survival rates for the eight who had a surgical salvage were 100% and 60%. None of the other three survived. Six of 18 patients (33%) relapsed at the primary site after a complete response to chemoradiation and all died. CONCLUSION: Surgical salvage after an incomplete response to chemoradiation in PM-RMS is feasible and can be curative in some cases.     

Renal cell carcinoma in children

Data from four pediatric hospitals concerning 20 children treated for renal cell carcinoma (RCC) from 1964-1978 were reviewed. Median age of the patients (pts) was 11.8 years (range, 14 months-19 years). Twelve were male and eight female; 17 were white and three black. Most patients presented with pain and hematuria with or without a palpable mass. An intrarenal tumor was detected at IV urography (17 pts), arteriography (2 pts), or at surgery (1 pt). Treatment consisted of nephrectomy in 15 pts, renal biopsy (4 pts), or no surgery (1 pt), followed by chemotherapy (5 pts), radiation therapy (1 pt), or both (7 pts). Ten pts died of distant metastases at a median of one year (range, 0.2 to two years) after diagnosis. The other 10 pts (50%) survive free of relapse at a median of 4 years (range, two to ten years) from diagnosis. Proportions surviving free of recurrent disease two or more years by National Wilms' Tumor Study (NWTS) Group were 5/5 in Group 1, 3/7 in Group 11, 1/3 in Group III, and 1/5 in Group IV; by age at diagnosis, 6/6 in those under 11 years old and 4/14 in those 11 or older; and by type of surgery, 10/15 who had nephrectomy and 0/5 with limited or no surgery. The data indicate that radiation and chemotherapy had only minor if any influences on relapse-free survival. We conclude that (1) RCC in children is similar to its counterpart in adults; (2) RCC has a worse prognosis than Wilms' tumor except for the earliest stage; (3) nephrectomy alone is adequate treatment for Group I RCC, and (4) young age (< 11 years old) may be prognostically favorable.     

TESTICULAR GERM CELL TUMORS IN PREPUBERTAL CHILDREN

Pediatric testicular germ cell tumors are rare. Fifteen children, all less than 5 years of age, were evaluated and treated during February 1987 to July 1996. The median age was 18 months (range, 4-60 months). All were staged according to the Pediatric Oncology Group/Children's Cancer Study Group staging system. Seven patients had stage III disease. Histologically, 9 patients had pure endodermal sinus tumor, 1 had endodermal sinus tumor with embryonal carcinoma, 1 had embryonal carcinoma alone, 2 had immature teratoma, and 2 had mature teratoma. Six children were kept on surveillance. All others received chemotherapy with cisplatin, bleomycin, and vinblastine. The 10-year actuarial overall survival rate was 86.7%.     

Influence of different treatment modalities on the curability of childhood rhabdomyosarcoma of the head or neck

We reviewed the clinical courses of 43 patients with localized and regional rhabdomyosarcoma primary in the head or neck treated according to three sequential plans of combined-modality therapy to identify independent contributions of surgery, chemotherapy and radiotherapy to disease control, survival and development of central nervous system (CNS) involvement. In the majority of patients complete surgical resection was not feasible by vitrue of tumor location or extent of disease. Combinations of vincristine, cyclophosphamide, and dactinomycin with or without adriamycin were used simultaneously or sequentially with irradiation to induce tumor regression. Radiation doses ranged from 35 to 55 Gy. In the sequential treatment group, six of 22 patients with measurable disease had complete responses to chemotherapy. Ten of 11 partial responders and 2 of 5 non-responders to chemotherapy attained complete responses with addition of radiotherapy (82% overall CR). Simultaneous chemotherapy and irradiation induced complete responses in 4 of 9 patients. Local control of minimal residual disease was uniformly achieved with radiation dose of < 50 Gy, but similar doses were effective in only 11 of 26 patients with gross residual disease. Twenty-three percent of patients developed CNS involvement as a consequence of uncontrolled or recurrent primary tumors. Although local tumor control was achieved in 79% of patients, it could not be maintained in more than 63%. Twenty-four patients (56%) have been surviving disease-free for 2.5 to 15.5 years (median 7.5 years). We conclude that a 6-week course of chemotherapy preceding radiotherapy does not result in loss of short term disease control of unresectable primary tumor. Radiation doses < 50 Gy appear adequate for eradication of minimal residual disease but inadequate for gross disease.     

Excellent local control and survival after intraoperative and external beam radiotherapy for pediatric solid tumors: long-term follow-up of the Mayo Clinic experience

Use of external beam radiotherapy (EBRT) for pediatric solid malignancies is generally limited by the tolerance of normal tissue in developing organs. Intraoperative electron radiotherapy (IOERT) allows a more focal delivery of radiation dose because vital organs can be displaced and avoided during treatment. From February 1983 to July 2003, 20 children underwent IOERT for treatment of locally advanced or recurrent malignancies of the extremity or abdominopelvic area. All patients underwent EBRT and received IOERT doses of 7.5 to 25 Gy with 6-MeV to 15-MeV electrons. At a median follow-up of 11.6 years (range, 2.1 to 25.5 y), 13 patients (65%) were alive and without evidence of disease. Patients who underwent gross total resection had better local control (88% vs. 67%) and survival (71% vs. 33%) than patients for whom the resection was not achieved. Among 7 patients, 11 grade 3 toxicity events were reported. No grade >3 toxicities or second malignancies were observed during follow-up. Use of IOERT in combination with surgery and EBRT in management of pediatric solid malignancies provides excellent local control with reasonable toxicity. IOERT should be considered as an integral part of a multimodality regimen for pediatric solid malignancies, especially for patients with abdominopelvic malignancies.     

Nasopharyngeal carcinoma in childhood and adolescence: a single institution's experience with treatment modalities during the last 15 years

Pediatric nasopharyngeal carcinoma (NPC) represents a locally advanced undifferentiated tumor with widely varying epidemiological features and with a high cure rate when combined modality treatment is provided. Both local and systemic treatment is necessary, and additional treatment with biologic modifiers seems promising. In this study, clinical experience and therapeutic results of 10 children with newly diagnosed NPC, treated in a single pediatric hematology/oncology institution in Athens over a period of 15 years, are analyzed. Results from Greece on NPC in young patients are reported for the first time. Ten patients (6 male, 4 female) 7-14 years old (median = 12.5) with a nasopharyngeal tumor were retrospectively evaluated. Disease extent was staged according to the TNM system. EBV-DNA, EBERs, and LMP-1 from paraffin-embedded tissues were studied in 8 patients. All patients received both local and systemic treatment. All cases were classified as type WHO-3. The presence of EBV-DNA and expression of EBER 1-2 mRNAs was demonstrated in the 8 tumors examined, while LMP-1 protein was expressed in 4/8 of the studied cases. Disease stage was III in 4 and IV in 6 patients. Time from the onset of symptoms to diagnosis ranged from 4 to 24 weeks (mean 8 weeks). All patients received preradiation chemotherapy and radiotherapy, and 5/10 received postradiation chemotherapy due to either resistant or advanced disease. In 9/10 patients, complete locoregional control was achieved. In addition to chemotherapy and radiotherapy the latest patient of this series was treated with recombinant IFN-beta (10(5) IU/Kg i.v. 3 times a week) for 6 months and at 18 months remains in continuous complete remission. One patient was lost to follow-up 3 years after cessation of treatment while remaining in complete remission. Of the remaining 9 patients, 7 are alive for a median follow-up of 54 months (range 18-186); 5/7 are free of disease, and 2/7 are with disease but stable. The median time for first relapse was 17 months. The data confirm the good results of combined chemo-radiotherapy treatment for high-risk NPC in young patients. The documented EBV latency underlying this tumor, which possibly critically mediates its pathogenesis, justifies the use of biological modifiers with antiviral and immunoregulatory activity, like the IFNs, which may offer better therapeutic results in the future.     

大剂量化疗造血干细胞移植治疗IV期神经母细胞瘤的长期疗效研究

目的：目前IV期神经母细胞瘤患儿无论采用何种方法治疗均疗效差,长期生存率低,需要探索新的治疗途径。该文采用大剂量化疗、自体外周血造血干细胞移植及13-顺式维甲酸治疗等方法,试图提高IV期神经母细胞瘤的长期疗效。方法：选择IV期神经母细胞瘤患儿28例,年龄2.1~11.5岁,平均3.3±1.9岁,发病时间1~7个月,平均3.1±0.7个月。原发部位:肾上腺23例,胸部3例,胸腹联合1例,骶骨1例。强烈化疗6疗程,期间进行外周血造血干细胞采集、手术切除,然后进行自体外周血造血干细胞移植,术后行局部放疗及13-顺式维甲酸治疗,定期随访。结果：28例患儿诱导化疗结束时13例取得完全缓解,11例取得部分缓解,4例化疗中病情进展。完全缓解及部分缓解的24例患儿完成治疗进入本研究。随访3.5±0.7年,两组4年无病生存率29.2%。完全缓解组中位无复发生存时间4.1±0.7年;部分缓解组中位无复发生存时间2.8±0.5年,两组中位无复发生存时间差异有显著性(t=3.9,P<0.01)。结论：大剂量化疗、自体外周血造血干细胞移植及13-顺式维甲酸治疗IV期神经母细胞瘤可取得较好疗效,4年无病生存率29.2%,移植前达到完全缓解时可取得更好疗效