Non-Hodgkin's lymphoma in children

In a study of non-Hodgkin's lymphoma in children, 104 children were treated and followed at Memorial. Sloan-Kettering Cancer Center from 1964 through June 1974. Forty-three patients, previously treated and untreated, received a nonspecific group of various chemotherapeutic agents and attained an 11% disease-free survival rate. A second group of 18 previously untreated patients, who received a chemotherapeutic regimen consisting of cyclophosphamide alone, achieved a 33% disease-free survival rate. The last group, 43 previously untreated patients (77% of whom had far advanced disease and 86% of whom had diffuse histological types) who received a new and intensive multiple-drug regimen (the LSA₂-L₂ protocol) consisting of induction, consolidation, and maintenance phases, has maintained an 81% disease-free survival rate after a median observation time of 21+ months. Although nervous system involvement and recurrence or metastases at any time are poor prognostic factors, initial marrow involvement and the amount of bulky disease are no longer considered negative prognosticators when intensive treatment is initiated immediately after diagnosis, is continued for 2 - 3 years, and includes radiation therapy to sites of bulky disease and CNS prophylaxis. The LSA₂-L₂ treatment is effective in accomplishing the dual aims of not only increasing the numbers of disease-free patients but also prolonging their survival.     

Evaluation of the LSA2L2 protocol for treatment of childhood non-Hodgkin's lymphoma. A report from the Polish Children's Leukemia/Lymphoma Study Group

From 1979 to 1982, 97 previously untreated children with non-Hodgkin's lymphoma were treated with the LSA2L2 protocol proposed by Wollner. Staging was done according to the criteria proposed by Wollner and re-staged according to Murphy's criteria. Each patient, regardless of clinical stage and histologic group, was given the same chemotherapy. A total of 28 nonrandomized patients received either cranial irradiation or intermediate-dose intravenous methotrexate as CNS prophylaxis. The complete remission rate was 72.6%. The 3-year actuarial estimate of survival was 73% and the disease-free survival rate was 62% for all responders, and was influenced by stage and main clinical features present at the time of initial presentation. The overall survival rate at 3 years is 52%. Of 26 children who failed to achieve complete remission, 21 had presented with disseminated disease. Also, 20/67 patients who entered remission have suffered relapses: four in the bone marrow, seven in the CNS, and nine with local relapses. Only one of 28 children who received CNS prophylaxis developed CNS disease as the site of first relapse, whereas six of those who received only intrathecal chemotherapy did so. This study confirms the improved outlook in comparison with a historical group for children with non-Hodgkin's lymphoma by the use of an intensive multiple-drug regimen and CNS prophylaxis.     

Outcome for young children newly diagnosed with ependymoma, treated with intensive induction chemotherapy followed by myeloablative chemotherapy and autologous stem cell rescue

BACKGROUND: The purpose of this study is to investigate the efficacy of an intensive chemotherapy induction regimen followed by myeloablative chemotherapy and autologous hematopoietic stem cell rescue (AHSCR) in children with newly diagnosed ependymoma. PATIENTS AND METHODS: Twenty-nine children less than 10 years of age at diagnosis of ependymoma were enrolled on the "Head Start" studies. Twenty-four patients with localized disease received an induction regimen including five cycles of chemotherapy (cisplatin, vincristine, etoposide cyclophosphamide, and high dose methotrexate for patients with metastatic disease). Following induction, individuals without evidence of disease proceeded to marrow-ablative chemotherapy (thiotepa, carboplatin, and etoposide) with AHSCR. RESULTS: The estimated 5-year event free survival (EFS) and overall survival (OS) from diagnosis were 12% (+/-6%) and 38% (+/-10%), respectively. The toxic mortality amongst this group of 29 patients was 10.3%. Younger age (less than 18 months at diagnosis) was the only statistically significant prognostic factor. The estimated 5-year OS rate for the five patients with metastatic disease at presentation was 80% (+/-18%). Overall, radiation-free survival at 5 years from diagnosis was 8% (+/-5%). CONCLUSIONS: The use of an intensive induction chemotherapy regimen including myeloablative chemotherapy followed by AHSCR in newly diagnosed young children with ependymoma is not superior to other previously reported chemotherapeutic strategies.     

Poor-risk non-lymphoblastic lymphoma of childhood: results of an intensive pilot study

Children with "poor-risk" nonlymphoblastic lymphoma, especially those with marrow or nervous system (CNS) involvement at presentation, have fared poorly even on aggressive chemotherapy regimens. We report here the results of a pilot study of 30 children treated with a highly intensive chemotherapy regimen. This regimen includes an intensive Induction Phase consisting of three cycles of CHOP therapy (cyclophosphamide, doxorubicin, vincristine, and corticosteroids) as well as intensive intrathecal therapy with each cycle. This is followed by a CNS Consolidation Phase consisting of a single cycle of CHOP therapy with five intrathecal doses of "triple" chemotherapy (methotrexate, cytosine arabinoside, and hydrocortisone). Thereafter, a Maintenance Phase consists of alternating cycles of 1) cytosine arabinoside and 6-thioguanine, 2) oral methotrexate and VP-16, and 3) CHOP, for a duration that varied from 36 to 72 wk. Neither debulking surgery nor radiation therapy were recommended. There were 20 patients with Stage III disease (St. Jude's Staging System) and an additional ten patients with bone marrow and/or CNS involvement. The latter group included six patients with B-cell leukemia, three of whom also had CNS disease at presentation. Two additional patients had CNS disease without marrow involvement. Twenty-nine of 30 patients achieved a complete response. Six patients died with recurrent or progressive disease. Twenty-three patients are alive without any adverse events between 21 and 65 mo after diagnosis, with the median time of survival not yet reached (at least 32 mo). All seven adverse events occurred within 7 mo of diagnosis. Event-free survival for all patients is 77%, for Stage III patients is 80%, and for patients with marrow and/or CNS involvement is 70%. This pilot study offers encouragement for improvement in the prognosis of children with "poor-risk" nonlymphoblastic lymphoma and merits evaluation in a Phase III randomized trial in the multicenter cooperative group setting.     

Long-term survival in an adolescent with widely metastatic renal cell carcinoma with rhabdoid features

Renal cell carcinoma is rarely seen in children and adolescents. Patients with widespread disease at diagnosis have a particularly poor survival rate. Currently, all known chemotherapy has been ineffective in improving the median survival in patients with advanced disease. A 13-year-old black boy with stage IV renal cell carcinoma with rhabdoid features is a long-term disease-free survivor after aggressive multiagent chemotherapy. After the initial evaluation and histologic diagnosis of renal cell carcinoma, the patient received three courses of an aggressive chemotherapy regimen consisting of vincristine, doxorubicin, cyclophosphamide with mesna uroprotection, granulocyte colony-stimulating factor and erythropoietin (Epogen). After an almost complete response, a radical nephrectomy was performed and results demonstrated a solitary small nodule with viable tumor. After surgery, he received floxuridine infusion for 14 days by circadian schedule at 28-day intervals for a total of 1 year. The patient is well and free of disease 5 years after initial presentation. The dramatic response to treatment and long-term disease-free survival of this patient suggest this chemotherapeutic approach warrants additional investigation.     

Treatment of advanced stage diffuse,small non-cleaved cell lymphoma in childhood: Further experience with total therapy B

Prior to the development of intensive treatments for patients with advanced stage B-cell non-Hodgkin's lymphoma (B-NHL), the prognosis for such patients was dismal. A minority of patients attained long-term, disease-free survival. Since 1981, we have treated 28 children with advanced stage B-NHL with an intensive chemotherapeutic protocol, Total Therapy B. This regimen employs cycles of fractionated high-dose cyclophosphamide, doxorubicin, and vincristine alternating with sequential infusions of high-dose methotrexate and escalating doses of cytarabine, in addition to intensive intrathecal therapy. The planned duration of therapy is approximately 6 months. Two patients had B-cell acute lymphoblastic leukemia and 26 had stage III B-NHL; none had CNS involvement. The median age was 7 years. All 28 patients achieved complete remission (CR). Both patients with B-ALL and 21 of 26 with stage III B-NHL remain in CR, with a median follow-up of 51 months. Treatment failures included 3 patients with recurrent or progressive disease, 1 toxic death in CR, and 1 patient who developed a secondary mediastinal T-cell lymphoblastic lymphoma 4 1/2 years after the diagnosis of B-NHL. The 2- and 5-year event-free survival rates were 85.7 ± 6.6% (SE) and 79.6 ± 8.5%, respectively. Total Therapy B is a highly effective therapy for children with advanced stage B-NHL without CNS involvement. © 1994 Wiley-Liss, Inc.     

Childhood non-Hodgkin's lymphoma and "leukemia-lymphoma syndrome": long-term results with the modified LSA2-L2 protocol

From June 1976 to December 1984, 48 previously untreated children with non-Hodgkin's lymphoma (NHL) were treated according to the LSA2-L2 protocol, modified by inclusion of cranial irradiation for patients in stage III and stage IV disease. According to the staging system proposed by Wollner, 4 patients were in stage I, 8 in stage II, 11 in stage III, 8 in stage IVA (less than or equal to 25% blasts in the bone marrow), 15 in stage IVB (greater than 25% blasts in the bone marrow), and 2 in stage IV central nervous system disease. The complete remission rate was 95.8%. The relapse-free survival (RFS) rate of 46 complete responders was 76% after a median observation time of 47+ months. Only 1 of 35 high-risk responder patients developed CNS relapse after prophylactic treatment. Clinical stages were related to the RFS: 100% in stage I-II vs. 69% in stage III-IV. All 8 patients in stage IV were alive without evidence of disease with a median observation time of 59+ months. Fifteen patients in stage IVB who had leukemia-lymphoma syndrome attained 59% RFS with a median observation time of 39+ months. After a median observation time of 38+ months, 29 of 37 patients are off therapy. The results emphasize the value of both the histologic and immunologic features and the stage of disease in predicting the outcome of NHL in children. The LSA2-L2 regimen appears to be a very effective protocol for children with lymphoblastic lymphoma, although it may be less efficacious for patients with large bone marrow involvement.     

The role of local radiation in the treatment of non-hodgkin lymphoma in children

Eighty-one patients between 1 and 15 years of age with non-Hodgkin lymphoma were seen at Memorial Sloan-Kettering Cancer Center (MSKCC) in an 8-year period ending December 1973. There was no statistically significant difference among the survival distributions for site or histologic type. The patients also were divided into three groups according to the chemotherapeutic regimen employed in their care, and further subdivided as to whether they received “curative” radiotherapeutic attempts. Children treated with multiple agent chemotherapeutic regimen (LSA-2) did significantly better than children of the LSA-1 or nonprotocol (NP) group. Children with Stage I and Stage II disease did significantly better than children with Stage III and Stage IV disease. Radiation therapy as employed in this study prevented recurrence of local disease. All patients in whom the disease recurred died within a year from the appearance of recurrence. However, no statistically significant association between radiation and survival could be shown in this series.     

Non-hodgkin's lymphoma in children: Results of treatment with the modified LSA2-L2 protocol

From June 1976 to May 1980, 25 previously untreated children with non-Hodgkin's lymphoma (NHL) were studied and treated with a protocol modified from the one (LSA₂L₂) proposed by Wollner [8]. Stage III and IV had, in addition, prophylactic treatment of the central nervous system (CNS) with cranial irradiation (2,400 rad plus intrathecal metho-trexate). The complete remission rate is 96%. Of these patients, 76% are disease free surviving after a median observation time of nearly two years. The disease-free actuarial survival is 100% for 6 children with Stage I-II disease and 68% for 19 Stage III-IV children after median observation times respectively of 25 + and 19 + months. None of the 19 high-risk patients developed CNS disease after prophylactic treatment. Mediastinal involvement and leukemic conversion at diagnosis were not unfavorable prognostic factors, but primary skeletal or subcutaneous disease and Burkitt-type histology were ominous features in patients treated by this regimen. It is concluded that good results are obtained when an aggressive multimodal and multiple drug regimen like the LSA₂-L₂ is coupled with CNS prophylaxis and is used assiduously to maintain complete remission during the first 12 months of treatment.     

Treatment of refractory and relapsed Hodgkin's disease: intensive chemotherapy and autologous bone marrow or peripheral blood stem cell support

Thirty-three patients with recurrent or refractory Hodgkin's disease were treated with high-dose cyclophosphamide, BCNU, and etoposide and supported with either autologous bone marrow or peripheral blood stem cells or both. Peripheral blood stem cells were comparable to bone marrow in supporting the recovery of hematopoiesis. Twenty-five patients (76%) were in complete remission following this therapy of whom 13 have subsequently relapsed. Twelve remain alive and disease free from 10 to 47 months. The Kaplan-Meier estimate of disease-free survival at 28 months for the entire 33 patients is 32% (95% confidence interval, 13-50%). Poor outcome in six patients was associated with bone marrow involvement by Hodgkin's disease at the time of peripheral blood stem cell collection. These six patients' survival, disease-free survival, the duration of complete remission were all significantly worse than for the 27 patients who were supported with bone marrow (n = 23), peripheral blood stem cells (n = 2), or both (n = 2), and whose marrows were free of disease at the time of stem cell collection. These data demonstrate that intensive therapy with autologous transplantation can produce extended disease-free survival for some patients with advanced Hodgkin's disease and that peripheral blood stem cell support can effectively be used for hematopoietic reconstitution. However, our observations also suggest that with this preparative regimen, bone marrow involvement at the time of peripheral blood stem cell collection is predictive for a poor outcome and alternate approaches to treatment should be considered for this subset of patients.     

Results of a randomized study of early stage Hodgkin's disease using ABVD,EBVD, or MBVD

From January 1986 to December 1989, 157 previously untreated patients, with Hodgkin's disease stage I or II without bulky disease, were enrolled in a clinical comparative study. The objectives of the study were to compare the efficacy and safety of using epirubicine or mitoxantrone instead of adriamycin in the combination chemotherapy regimen ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine). The complete response rate was better in the patients treated with the ABVD or EBVD regimens compared to the MBVD arm. Also, differences in overall survival and relapse-free survival were better in the patients who received ABVD or EBVD compared to the MBVD regimen. Hematological, gastrointestinal and cardiac toxicity were similar in the three groups. Dose intensity, delays and complications were also similar in the three groups. The mitoxantrone-containing regimen was found to have less efficacy in comparison to the other regimens tested in the present study in patients with favorable stage I or II Hodgkin's disease. © 1995 Wi1ey-Liss Inc.     

Treatment of non-Hodgkin's lymphoma in Mexican children. The effectiveness of chemotherapy during malnutrition

The histological diagnosis of non-Hodgkin's lymphoma (Burkitt's lymphoma excluded) in 147 children was reviewed. The most common site of presentation was in the abdomen (32.6%). The most frequent site of metastatic disease at diagnosis was the bone marrow (27.2%). The most common histology was diffuse undifferentiated non-Burkitt type (37.4%). According to the Murphy staging system, 40.1% were stage III and 27.2% were stage IV. In a nonrandomized prospective study, 121 patients were submitted to a treatment regimen (protocol 8001) and compared with 26 historical controls treated with the COP regimen, consisting of cyclophosphamide, vincristine, and prednisone. Of those patients treated with protocol 8001, nine had intestinal perforation at the site of primary disease. All patients in this group were malnourished at the time of perforation. The overall rate of initial complete remission in those patients treated with protocol 8001 was 90.7%. The duration of remission was from 16 to 108 months, with a median of 39 months. The actuarial rate of disease-free survival was 69% at 2 years and 63% at 6 years, compared with 36% at 6 years of the control group (COP) (p less than 0.01). None of the patients have relapsed after 4 years.     

Current status of treatment for pediatric rhabdomyosarcoma in the USA and Japan

This article reviews the current status of treatment for children with rhabdomyosarcoma, according to the four risk groups. Low‐risk subgroup A: the Children's Oncology Group in the USA recently performed a clinical trial consisting of a chemotherapy regimen with a shortened treatment period and a reduced drug dosage. Patients in this group received only four cycles of vincristine and actinomycin D (VA) after four cycles of vincristine, actinomycin D, and cyclophosphamide (VAC) with cyclophosphamide (CPM) 1.2 g/m² and their outcome was no worse than that obtained with previous regimens. Low‐risk subgroup B: although marked improvement in survival was seen with an intensive VAC regimen with CPM 2.2 g/m²/cycle (Intergroup Rhabdomyosarcoma Study [IRS]‐V, 1997–2004), the total dose of CPM in this regimen caused serious and fatal hepatic veno‐occlusive disease during treatment and probably cannot avoid infertility or possible secondary cancer as a late effect. Thereafter, a reduced‐dose regimen consisting of four cycles of VAC with CPM 1.2 g/m² followed by 12 cycles of VA was investigated in the next study, but the outcome appeared to be worse than in IRS‐V. Intermediate‐risk group: no significant difference was found between VAC/vincristine, topotecan and cyclophispahamide (VTC) and intensive VAC in IRS‐V. The results of a subsequent regimen of VAC with CPM 1.2 g/m² alternating with vincristine and irinotecan are awaited. High‐risk group: overall survival is approximately 30% and has not improved over the last 25 years. Although 18 month failure‐free survival (FFS) was improved with an intensive combination therapy regimen, 36 month FFS dropped to 32% and thus better novel approaches or additive treatments are needed.     

APE chemotherapy for children with relapsed Hodgkin disease: a Pediatric Oncology Group trial

BACKGROUND: MOPP (mechlorethamine, vincristine, procarbazine, prednisone) and ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) are effective therapies for Hodgkin disease (HD) that may cause long-term toxicities in children. APE (cytosine arabinoside, cisplatin, etoposide) is a non-cross-resistant regimen with limited toxicities. We evaluated this regimen for patients with recurrent or refractory disease. METHODS: Patients with recurrent Hodgkin disease who were 相似文献   

Clinical efficacy of combined therapy in children with stage 4 neuroblastoma北大核心CSCD

目的 观察4期神经母细胞瘤联合治疗的早期疗效,探讨提高生存率及改善生活质量的治疗方案。 方法 对2016年1月至2021年6月在香港大学深圳医院确诊的14例4期神经母细胞瘤患儿的临床资料、治疗及随访情况进行回顾性分析。 结果 14例患儿中位发病年龄为3岁7.5个月。骨髓检查阳性9例,N-Myc基因扩增4例,神经元特异性烯醇化酶增高13例,尿香草扁桃酸增高7例。病理结果：分化型6例,未分化型1例,混合型1例,分化差型6例。N7方案化疗10例（含2例N7方案+三氧化二砷化疗患儿）,Rapid COJEC方案化疗4例。手术13例;自体造血干细胞移植14例;放疗10例。Ch14.18/CHO免疫治疗8例,其中1例因在治疗过程中出现过敏性休克而停止免疫治疗;其余7例完成治疗,期间未观察到严重不良反应。完成免疫治疗患儿中1例复发后进行3次Lu177 Dotatate治疗,目前仍在化疗中。14例患儿中位随访时间为45个月。2年内复发4例,2年内总生存率100%;3年内复发4例,3年内无病存活7例。 结论 儿童4期神经母细胞瘤建议选择多学科联合方案治疗,使4期神经母细胞瘤患儿获得更好的生存及预后。     

Treatment results of advanced neuroblastoma with the first Japanese study group protocol. Study Group of Japan for Treatment of Advanced Neuroblastoma.

PURPOSE: To elucidate the efficacy of intensive induction and consolidation chemotherapy regimens (Study Group of Japan for Advanced Neuroblastoma [JANB] 85) for patients with advanced neuroblastoma aged 1 year or older. PATIENT AND METHODS: One hundred fifty-seven patients with newly diagnosed advanced neuroblastoma were entered into this study between January 1985 and December 1990. Eligible patients were 12 months old or older with stage III or IV disease. The patients first received six cyclic courses of intensive induction chemotherapy (designated regimen A1) consisting of cyclophosphamide (1,200 mg/m2), vincristine (1.5 mg/m2), tetrahydro-pyranyl Adriamycin (pirarubicin; 40 mg/m2), and cisplatin (90 mg/m2). The patients were further treated with three different consolidation protocols: 3-[(4-amino-2-methyl-5-pyrimidinyl)methyl]-1-(2-chloroethyl)-1-nitrosour ea, dacarbazine, and bone marrow transplantation. RESULTS: Overall survival rates for patients with stage III disease without reference to the consolidation protocols were 80.8%, 76.9%, and 66.3% at 2, 5, and 10 years, respectively. The overall survival rates for patients with stage IV disease were 58.8%, 34.4%, and 28.9% at 2, 5, and 10 years, respectively. There were no statistically significant differences between the three consolidation treatment groups. Patients who did not achieve complete remission (CR) with induction chemotherapy and surgery all died, suggesting that CR is essential for the cure of advanced neuroblastoma. The overall 5-year survival rate of the 24 patients with N-myc amplified stage III and IV disease was 33.3%, and the longest survival time of a relapse-free patient was 103 months. CONCLUSION: The intensive induction chemotherapy regimen used in this study may be of significant value in increasing the CR rate and survival for patients with N-myc amplified and nonamplified advanced neuroblastoma.     

Disseminated nonlymphoblastic lymphoma of childhood: A childrens cancer group study,CCG-552

(1) Purpose: To assess the efficacy of a chemotherapy-only regimen in pediatric patients with disseminated nonlymphoblastic lymphoma and acute B-cell leukemia (B-ALL). (2) Patients and Methods: Sixty-eight eligible patients with previously untreated disseminated non-lymphoblastic lymphoma were enrolled on a Childrens Cancer Group study. Therapy included cycles of chemotherapy, systemic and intrathecal (IT), ever 3 weeks for a total maximal duration of 57 weeks. Fifty-five patients had small non-cleaved cell lymphoma (SNCCL) and 13 had diffuse large cell lymphoma (DLCL). Forty-seven were stage III, six were stage IV, and 15 had B-ALL; 13 had central nervous system (CNS) involvement. (3) Results: Four year event-free survival (EFS) was 53% (SE ± 12%). Stage III SNCCL patients with LDH < 500 IU/L achieved an improved EFS compared to other SNCCL patients (86% vs. 42% 4 year EFS, P = .072). The primary site of failure for advanced stage SNCCL patients was the CNS. All Ki-1-positive DLCL patients relapsed. Patterns of failure, time to relapse, and outcome following relapse differed between SNCCL and DLCL patients. (4) Conclusions: Advanced stage SNCCL requires better CNS-directed chemotherapy to reduce the CNS failure rate; however, the achievement of durable disease-free survival in four of 11 patients with CNS disease without use of cranial irradiation suggests merit for further evaluation of chemotherapy-only strategies. DLCL patients do not need intensive CNS-directed chemotherapy. © 1994 Wiley-Liss, Inc.     

Cyclophosphamide-based, seven-drug hybrid and low-dose involved field radiation for the treatment of childhood and adolescent Hodgkin disease

PURPOSE: The outlook for children and adolescents with Hodgkin disease (HD) is excellent with combined modality therapy. However, the long-term toxicities of multiagent therapy and radiation therapy remain of concern for these patients with curable disease. In an attempt to reduce long-term toxicities while preserving excellent cure rates, we developed a combined-modality protocol using a modified seven-drug hybrid and low-dose (2,000 cGy) involved field radiation therapy (RT). The hybrid used cumulative doses of alkylating agents and anthracyclines that were lower than those used in previous four-drug regimens and substituted a less leukemogenic agent, cyclophosphamide, for nitrogen mustard. PATIENTS AND METHODS: From 1991 through 1994 a cyclophosphamide, vincristine, procarbazine, and prednisone/adriamycin, bleomycin, and vinblastine hybrid was used to treat 29 patients with HD. Median age was 12 years (range 6-16 yrs). Patients who were postpubertal with early stage disease as determined by surgical staging were excluded. Treatment consisted of four cycles of therapy for stages I and IIA, six cycles for stages IIB and III, and eight cycles for stage IV. Twenty-two patients also received low-dose RT to areas of bulky disease. RESULTS: Twenty-eight patients (97%) had a complete response to chemotherapy. Five patients experienced relapse; two died from disease 27 and 29 months after initial diagnosis; three received additional therapy and are alive with no evidence of disease. Follow-up for all other patients is a median of 56 months (range 24-78 mos) from cessation of therapy and all have remained disease-free. At 5 years follow-up, actuarial disease-free survival is 82%, and the overall survival is 93%. There have been no clinically significant cardiac or pulmonary toxicities and no secondary malignancies. CONCLUSIONS: This therapy has resulted in 5-year overall survival and disease-free survival rates similar to regimens using higher doses of alkylating agents, anthracyclines, and radiation. Longer follow-up will be necessary to fully evaluate disease-free survival, organ damage, and quality of life.     

ACUTE LYMPHOBLASTIC LEUKEMIA IN SWEDISH CHILDREN 1973–1978

ABSTRACT. Gustafsson, G., Kreuger, A. and Dohlwitz, A. (Departments of Paediatrics, University Hospital, Uppsala, and County Hospital, Nykoping, Sweden). Acute lymphoblastic leukemia in Swedish children 1973–1978. Acta Paediatr Scand, 70:609,.–Three hundred and sixty-seven children with acute lymphoblastic leukemia have been diagnosed in Sweden 1973–1978, 345 of whom were treated according to the national uniform regimens of the Swedish Child Leukemia Group (SCLG). The patients were classified into an SR (standard risk) and an IR (increased risk) group. Remission was obtained in 354 patients (96%). With 12–84 months observation time the total survival was 54% and the diseasefree survival 44 %. A more intensive cytostatic regimen in the induction period increased considerably the diseasefree survival for the SR and to some extent also for the IR patients. Relapses were significantly more common in the IR group in spite of a more intensive cytostatic regimen. The most decisive IR criteria were B-LPK and age at diagnosis. Prognosis was significantly worse for boys in all groups. After 3 years in CCR treatment was discontinued in 95 out of 246 children (38%) of whom 19 later relapsed (20%)     

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.