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.     

Challenges in the management of localized Ewing sarcoma in a developing country

Abstract

Survival in pediatric Ewing sarcoma (ES) lags in low- and middle-income countries (LMICs). This study analyzed factors contributing to a lower outcome in an LMIC center. A retrospective case review of children with localized ES treated from January 2011 till December 2017 was performed. Neoadjuvant chemotherapy with alternating cycles of vincristine, doxorubicin, cyclophosphamide; and ifosfamide, etoposide was administered 3-weekly for 48 weeks. Reassessment was planned for week 12, followed by local therapy (surgery/radiotherapy or both) tailed by adjuvant chemotherapy. Forty-eight patients with mean age 8 years (range: 0.7–14) were evaluated. Extremity and central axis tumors were seen in 25 (52%) and 23 (48%) patients. Three patients died of neutropenic sepsis and five abandoned therapy. Local therapy included primary surgery, radiotherapy and a combination of surgery and radiotherapy in 7 (16%), 20 (45%) and 17 (39%) patients. The 3-year event-free survival (EFS) and disease-free survival (DFS) for the cohort were 47.7?±?11% and 57.6?±?11.2%. Time to local therapy >16 weeks was associated with inferior DFS vs. local therapy administered within 16 weeks [46.6?±?12.4 vs. 63.9?±?19.4, p=.046]. Older age, axial site, large size and incomplete surgical resection did not predict relapse/progression. Patients who received wide local excision, as local therapy, had 100% DFS. Coordinated efforts to ensure timely therapy can improve outcome in pediatric ES. Abandonment and treatment-related mortality (TRM) are additional challenges that need to be tackled in LMICs.     

Testicular germ cell tumors in childhood: treatment results of 52 patients

We analysed the treatment results of 52 children with testicular germ cell tumors. Histopathological diagnoses were endodermal sinus tumor (63.4%), embryonal carcinoma (28.8%), teratocarcinoma (5.7%), and mixed tumors (2.1%). Radical inguinal orchiectomy was performed in 42 patients and retroperitoneal lymph node dissection in 10 (3/10 positive). Overall survival rates were: whole group: 71.2%; stage I: 89.7%; II: 68.5%; III: 31.2%; IV: 30% (p =.001). Five-year overall survival rates were 85.8% and 100% for stage I patients who received chemotherapy or not (p =.27); BEP regimen: 85.7%; classical VAC: 67.9%; vinblastine + bleomycin: 63.6%. Chemotherapy is not required in stage I. BEP regimen is effective in testicular germ cell tumors.     

LONG-TERM RESULTS OF HIGH-DOSE CHEMOTHERAPY AND AUTOLOGOUS STEM CELL RESCUE FOR HIGH-RISK NEUROBLASTOMA PATIENTS: A Report of the Spanish Working Party for BMT in Children (GETMON)

The authors retrospectively analyzed the long-term outcome of 67 patients over 1 year of age at diagnosis with high-risk neuroblastoma (stage 4 or stage 3 with N-myc amplification) who were treated with megatherapy and stem cell rescue from 1984 to 1998. Median age at transplant was 4 years (range 1.6–15 years). The source of cells was peripheral stem cells in 29 and bone marrow in 38 patients. In 12 patients, an in vitro purging of bone marrow harvest was performed. Most patients were conditioned with melphalan, BCNU, and VM-26. After transplant 19 patients received complementary treatment with IL-2 (16) or 13-cis-retinoic acid (3). Six patients (8%) died from transplant-related toxicity and 39 from disease progression. Three patients were alive with active disease at the time of analysis. Nineteen patients are alive and disease-free at a median follow-up of 104 months. Five-year event-free survival is 0.30. Survival of patients who received a purged graft was not significantly better than the rest. Post-transplant complementary treatment significantly improved overall and event-free survival (p =. 01 and p =. 04, respectively).     

Outcome after relapse in an unselected cohort of children and adolescents with Ewing sarcoma

BACKGROUND: Survival after relapse in patients with Ewing sarcoma is very poor and this retrospective study attempts to identify of prognostic factors predicting survival after relapse. PROCEDURE: A total of 191 patients with localised Ewing sarcoma were registered in the ET-2 trial of the United Kingdom Children's Cancer Study Group (UKCCSG). All patients received standardised primary treatment with chemotherapy and surgery and or radiotherapy as local modality treatment. Sixty-four patients who relapsed are included in this report. Treatment at relapse was variable and included chemotherapy, surgery, radiotherapy and high dose therapy (HDT) or megatherapy with peripheral stem cell transplantation (PBSCT) or autologous bone marrow transplantation (ABMT) in various combinations. A subgroup of patients had only non-specific symptomatic treatment at relapse. Both univariate and multivariate methods were used to investigate variables affecting survival after relapse. RESULTS: The overall actuarial median survival from relapse for all patients was 14 months (95% CI 11-16 months). Univariate analysis showed that males had a longer survival (median, 16 months vs. 11 months); patients who relapsed while on treatment did worse (median, 3 months vs. 16 months) and patients who had a longer disease-free interval (DFI) prior to relapse had a better outcome (DFI <1 year, median survival = 3 months; DFI 1-2 years, survival = 8 months; DFI > 2 years, median survival = 24 months, P < 0.001). Multivariate analysis confirmed that duration of first remission was the only factor associated with longer survival after relapse. CONCLUSIONS: These data suggest that although aggressive therapy may delay disease progression after relapse for some children, the course of the disease after relapse is usually fatal. International co-operative studies are needed to evaluate new strategies.     

放疗及二次根治手术对儿童横纹肌肉瘤的疗效影响分析

目的探讨儿童横纹肌肉瘤(RMS)的治疗手段与疗效、预后的关系。方法回顾性分析中山大学孙逸仙纪念医院儿科2013年1月—2017年12月收治的24例儿童RMS的临床资料,总结儿童RMS的治疗策略、疗效,分析放疗及二次根治手术对儿童RMS预后的影响。结果初治儿童RMS共24例,男18例,女6例,发病中位年龄3.6岁(11个月~11岁),其中原发于颌面部14例,颈部1例,腹盆腔2例,肢体4例,生殖系统2例,肝脏1例;病理类型包括胚胎型22例,腺泡型2例;低危组3例,中危组18例,高危组3例。所有患儿均按横纹肌肉瘤全国协作组重庆方案(RMS-CQ-2009)进行化疗,并依据实际病情及疗效反应选择性采取根治手术及放疗。截止2020年6月1日共9例患儿死亡,3年总体生存率(OS)为65.7%。化疗+放疗组的3年OS(80.0%)高于化疗+二次根治术组(50.0%)(χ²=3.896,P=0.048)。对于颌面部RMS,行二次根治术的患儿3年0S(25.0%)低于未行二次根治术的患儿(80.0%)(χ²=6.521,P=0.011);行放疗的患儿3年OS(80.0%)高于未行放疗的患儿(25.0%)(χ²=4.168,P=0.041)。对于非颌面部RMS,行二次根治术和未行二次根治术的患儿3年OS分别是68.6%和66.7%(χ²=0.035,P=0.852),行放疗和未行放疗的患儿3年OS分别为53.3%和80.0%(χ²=0.076,P=0.783)。结论放疗有助于提高儿童RMS生存率,二次根治术并不能显著改善颌面部RMS的预后。     

Advanced Nasopharyngeal Carcinoma in the Young: The Northern Israel Oncology Center Experience, 1973-1991

Between 1973 and 1991, 10 patients with locally advanced [stages III and IV] nasopharyngeal carcinoma were treated at the Northern Israel Oncology Center. All patients were treated with wide-field irradiation to the primary tumor, including the base of skull, neck, and supraclavicular region. After 1984, 6 patients also received cisplatin/5FU-based chemotherapy prior to radiotherapy and 1 patient received it after radiotherapy. All the patients who received chemotherapy are alive with no evidence of disease, for a mean disease-free survival of 96 months (range, 77 to 108 months), and no serious therapy-related late side-effects have been noted, except in one patient. We conclude that adjuvant chemotherapy may be effective in improving outcome, but only randomized prospective studies can evaluate its exact role.     

Medulloblastoma—A retrospective analysis

A retrospective review of 45 patients was undertaken at the All India Institute of Medical Sciences to assess the outcome and prognostic factors for these patients who received post operative radiotherapy with or without chemotherapy for medulloblastoma. The median age at diagnosis was 11 years, with 34 males and 11 female patients. Thirty four tumours were confined to midline structures, and 11 were localised to one cerebellar hemisphere or involved midllne and lateral structures. Complete macroscopic removal was achieved in 24 patients and subtotal removal in 21 patients. Forty one patients underwent craniospinal irradiation and 27 patients received adjuvant chemotherapy. Median overall and disease free survival was 57 and 31 months respectively and 3 year overall, survival was 76%. The addition of adjuvant chemotherapy was a significant factor for disease free survival (p = 0.01) whereas extent of surgery (total vs subtotal, p = 0.01) was a significant factor for overall survival only. Eleven patients developed recurrent disease, with ten relapsing first in the posterior fossa.     

Comprehensive treatment of advanced neuroblastoma involving autologous bone marrow transplant

Encouraging results are reported with high-dose chemotherapy and total body irradiation followed by autologous bone marrow transplantation in the treatment of advanced neuroblastoma. However, relapse remains a significant problem. We used high-dose chemotherapy, surgery, intraoperative radiation and an autologous bone marrow transplant treated in vitro to remove tumor cells followed by 13-cis-retinoic acid to treat 36 children with advanced neuroblastoma. This comprehensive treatment appears to improve the survival rate of patients with advanced neuroblastoma, including those with N-myc amplification and bony involvement. The disease-free survival rate was 66% (95% confidence interval, 49–84%) at 3 years. All patients who received 13-cis-retinoic acid developed cheilitis, but no bone marrow depression occurred in these patients. Five patients developed hemolytic uremic syndrome (HUS) post-transplant. This may have been related to the procedure used for total body irradiation. Patients who had their kidneys shielded during this procedure did not develop this syndrome. Patients who received local irradiation at the primary site showed no evidence of relapse in this region, indicating that such therapy may help to prevent a relapse. These data suggest a high rate of 3 year disease-free survival with this treatment strategy. The nonrandomized nature of the study and use of multiple modalities precludes analysis of the specific contribution of each.     

Primary chemotherapy followed by radiotherapy for localized non-Hodgkin's lymphomas: a preliminary report

Twenty-eight evaluable patients with clinical stage I-II of non-Hodgkin's lymphoma were treated with primary chemotherapy followed by involved field radiotherapy. The frequency of the follicular versus diffuse histological pattern was 14.3 versus 85.7%, and 24/28 (86%) of patients were in stage II. No lymphoma examined was identified as T-cell in origin. A complete response was obtained in 21 (78%) of 27 patients with measurable disorder. [Gastrectomy rendered one patient disease-free survival, and one partially responded showing a complete response after radiotherapy.] Sixteen (76%) of the 21 employed for complete response. After 36 months, elapsed disease-free survival was rated at 100% together with actuarial survival of all patients at 81%. Chemotherapy contributed to reduce the radiation dosage to 3000 rads and to control the areas infiltrated with preexisting tumors. These findings, although the median follow-up time in our study remains short (28 months), provide a strong rationale for further clinical trials of primary chemotherapy, followed by regional radiotherapy for localized stages of non-Hodgkin's lymphomas.     

Sparing strategy does not compromise prognosis in pediatric localized synovial sarcoma: experience of the International Society of Pediatric Oncology, Malignant Mesenchymal Tumors (SIOP-MMT) Working Group

Background

The aim of this analysis was to identify if the modified indications of radiotherapy (RT) or radical surgery compromised survival in pediatric synovial sarcoma (SS).

Procedure

Children with non‐metastatic SS, prospectively enrolled in three trials, were analyzed. After primary surgery or biopsy, they received chemotherapy. RT was planned after chemotherapy in patients who had not achieved a complete response (CR). The considered outcome was 5‐year overall survival (OS) and event‐free survival (EFS).

Results

Eighty‐eight patients were identified. Primary tumors were mainly located in limbs (66%). The first‐line therapy for 65 patients was primary resection. Of the 49 patients who had gross tumor resection, 43 received adjuvant chemotherapy, and 8 had RT. All of the 39 patients with macroscopic residual disease received chemotherapy, then only surgery (n = 12) ± RT (n = 22). The 5‐year EFS and OS rates were 68% and 85%, respectively. The TNM stage was a prognostic factor for relapse, whereas primary site of the tumor and TNM stage were prognostic factors for death.

Conclusions

Only 32% of survivors received RT. OS was similar to published data. Omission of RT may be considered in younger children, to limit the potential sequelae in patients with tumors less than 5 cm in size initially submitted to marginal resection. This strategy may also be considered in initially unresected cases, when the tumor is resected at delayed surgery with microscopically free margins, and in patients in complete remission after primary chemotherapy. Pediatr Blood Cancer 2011; 57: 1130–1136. © 2011 Wiley Periodicals, Inc.     

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.     

Experience and outcomes of nephroblastoma in Johannesburg, 1998 - 2003.

INTRODUCTION: Outcomes for children with cancer in the developing world are compromised by the difficulties for patients in accessing health services and by competition for resources between oncological services and the myriad other health problems of emerging nations. The purpose of this study is to document and analyse our experience and the outcomes of children with nephroblastoma over recent years. METHODS: This is a retrospective review of all patients who underwent combined oncological and surgical treatment for nephroblastoma in the Paediatric Oncology Unit between 1998 and 2003. RESULTS: Sixty-three patients were treated for Wilms' tumour; the mean age was 3 years 8 months (range 4 months to 11 years). The majority of children presented with an abdominal swelling or mass. Preoperative chemotherapy was given in forty-six cases (73 %). The tumour stage distribution was 11/63 stage I (17 %), 11/63 stage II (17 %), 21/63 stage III (33 %), 16/63 stage IV (25 %) and 4/63 stage V (6 %). Postoperative chemotherapy and radiotherapy was given according to the SIOP protocol. During the study period, thirteen patients (21 %) died (7 cancer-specific, 2 postoperative, 4 sepsis related), thirteen (21 %) were lost to follow-up and thirty-seven (59 %) are free of disease with a mean follow-up period of 3.67 years. Children with stage I and stage II had a disease-free survival at 4 years of 89 %. However, those with stage III, IV and V disease had 4-year survival of 66.75 % (p = 0.07). Overall, four-year post-nephrectomy survival was 76 %. CONCLUSION: Outcomes for children with cancer have improved dramatically over recent years; however, in the developing world, the scarcity of hospital resources and the overwhelming burden of non-cancer diseases can mean that oncological treatment is extremely challenging. In our society, children tend to present with nephroblastoma at an advanced stage; however, treatment by dedicated, multidisciplinary teams can achieve good results.     

Rhabdomyosarcoma Treatment and Outcome at a Multidisciplinary Pediatric Cancer Center in Lebanon

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Outcome of patients treated on standard protocols, in a multidisciplinary cancer center setting outside of clinical trials, is not well reported. We reviewed characteristics and outcome of 23 pediatric patients treated at a single, multidisciplinary cancer center in Lebanon, between April 2002 and December 2010. Median follow-up was 41 months. The most commonly affected primary site was the head and neck (48%, n = 11). Nineteen tumors (82.6%) were of embryonal histology. Tumor size was ≥5 cm in eight (34.8%) patients. Sixteen patients (69.6%) had localized disease, and one (4.4%) had metastatic disease. Fifteen (65.2%) had Group III tumors. All patients received chemotherapy, for a duration ranging 21–51 weeks. Upfront surgical resection was performed in 10 patients (43.5%). Eighteen patients (78.3%) received radiation therapy. The 5-year overall and disease-free survival rates were 83% and 64%, respectively. Relapse correlated with absence of surgery. Treatment of childhood RMS in a multidisciplinary cancer center in Lebanon results in similar survival to that in developed countries when similar protocols are applied. There was a higher incidence of local relapse, but those were salvageable with further therapy and surgical local control.     

MultiCenter outcome of pediatric oncology patients requiring intensive care

A retrospective cohort study was conducted to evaluate the intensive care outcome of pediatric cancer patients between 1996 and 1998. The study comprised 20 pediatric intensive care units (PICUs) and 802 patients with cancer requiring PICU care. Patients with a history of cancer were identified from PICUs participating in the Pediatric Intensive Care Unit Evaluations program. Their demographics, resource requirements, and outcomes were compared with those of noncancer patients. Cancer patients comprised 3.3% (802/24,431) of PICU admissions. Overall PICU survival was not different between cancer and noncancer patients (95% vs. 96%, p =.2). Cancer patients were older (99 +/- 3 vs. 72 +/- 1 months, p <.001), had similar gender distributions, and had similar lengths of stay (3.3 +/- 0.2 vs. 3.9 +/- 0.1 days, p =.98). The majority (72%) were admitted to the PICU for postoperative care; PICU survival in these patients was 100, 100, and 71% for those not receiving mechanical ventilation or vasoactive agent infusion, those receiving either mechanical ventilation or vasoactive agent infusion, and those receiving both, respectively. PICU survival in nonoperative patients was 87% overall; survival for those requiring ventilation, vasoactive infusions, or both was 93, 89 and 46%. Overall hospital survival was 99% in operative cancer patients and 81% in nonoperative patients (p =.004, operative vs. nonoperative patients). Pediatric cancer patients receiving intensive care do well overall. Outcomes have substantially improved and, in general, the diagnosis of cancer should not limit the provision of intensive care. Additionally, resource use in terms of lengths of stay in the PICU is not different between cancer and noncancer patients.     

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.     

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.     

Chemotherapy with cyclophosphamide,vincristine, cytosine arabinoside,and prednisone (COAP) in childhood acute lymphoblastic leukemia (ALL)

Three groups of children with acute lymphoblastic leukemia (ALL) were treated with intermittent cyclophosphamide, vincristine, cytosine arabinoside, and prednisone (COAP). Group A (no prior relapse) and Group B (prior single-agent relapse) received COAP after 12 months on another chemotherapy regimen. Children in Group C (prior relapse on multiagent regimens) received COAP following A-COAP (asparaginase plus COAP) reinduction. Median disease-free survival after beginning COAP was not reached for Group A, but was only 7 months for Groups B and C. As of November 1976, there were 8 of 15 Group A patients, 1 of 12 Group B patients, and 1 of 28 Group C patients who had remained disease-free from 38 to 60 (median 54.5) months and were off chemotherapy. COAP has activity in childhood ALL. However, effectiveness in markedly diminished in patients with prior bone marrow relapse.     

Isolated extramedullary relapse in acute myeloid leukemia: A retrospective analysis

BACKGROUND: Little is known about the characteristics and outcome of children with acute myeloid leukemia (AML) experiencing an isolated extramedullary relapse (IEMR). PROCEDURE: The tumor registry of The Children's Hospital of Philadelphia identified 215 patients with AML diagnosed between 1970 and 2000, of which 16 (7.4%) experienced IEMR. Patient- and disease-related features and outcome of patients with IEMR and other patients with AML were compared. RESULTS: IEMR occurred a median of 4.5 months (1.5-74 months) from diagnosis. Male to female ratio was 4.3:1 in patients with IEMR and 1.1:1 in the other patients with AML (P = 0.048). Median age at diagnosis and median presenting WBC were not significantly different in patients with and without IEMR. Patients with IEMR were more likely to have extramedullary disease (EMD) at diagnosis (31 vs. 4.5%) (P =.002) and FAB M4 or M5 morphology (P =.0001). Leukemic cells in 7 of 13 patients (54%) had t(11q23), inversion 16 or t(8;21) with IEMR compared to 21 of 93 other patients (23%) (P = 0.166). Six of 16 (37.5%) patients survive a median of 4.5 years (range 1.5-15 years) after IEMR and there are 13 survivors (23%) of 57 patients after marrow or combined relapse (P = 0.56). One survivor of IEMR received local irradiation and continued on maintenance therapy while the other five received chemotherapy, irradiation, and allogeneic marrow transplant in second or third remission. CONCLUSIONS: Patients with isolated EMR are typically young males with monoblastic or myeloblastic leukemia who present with EMD at diagnosis. Marrow transplant following chemotherapy and local radiotherapy offer the potential for long-term survival.     

Chemotherapy with cyclophosphamide, vincristine, cytosine arabinoside, and prednisone (COAP) in childhood acute lymphoblastic leukemia (ALL).

Three groups of children with acute lymphoblastic leukemia (ALL) were treated with intermittent cyclophosphamide, vincristine, cytosine arabinoside, and prednisone (COAP). Group A (no prior relapse) and Group B (prior single-agent relapse) received COAP after 12 months on another chemotherapy regimen. Children in Group C (prior relapse on multiagent regimens) received COAP following A-COAP (asparaginase plus COAP) reinduction. Median disease-free survival after beginning COAP was not reached for Group A, but was only 7 months for Groups B and C. As of November 1976, there were 8 of 15 Group A patients, 1 of 12 Group B patients, and 1 of 28 Group C patients who had remained disease-free from 38 to 60 (median 54.5) months and were off chemotherapy. COAP has activity in childhood ALL. However, effectiveness is markedly diminished in patients with prior bone marrow relapse.