A chemotherapy only therapeutic approach to pediatric Hodgkin lymphoma: AHOPCA LH 1999.

BACKGROUND: AHOPCA is a collaborative group that designs uniform treatment regimens (protocols) for children diagnosed with cancer in Central America. Based on a preliminary report from one of the AHOPCA centers, AHOPCA adopted a treatment regimen to maintain a good event-free survival (EFS) as well as eliminate radiation therapy from the treatment of children with Hodgkin lymphoma. PROCEDURE: Newly diagnosed patients with histologically proven Hodgkin lymphoma were staged according to the Ann Arbor classification and divided into favorable (stage I, stage IIA, and IIIA) and unfavorable (stage IIB, IIIB, and IV) groups. Subjects classified as group 1 (favorable) were treated with six 28-day cycles of chemotherapy (COPP/COPP ± ABV). Subjects classified as group 2 (unfavorable) were treated with eight 28-day cycles of COPP/ABV chemotherapy. RESULTS: Of 269 patients registered, 216 were eligible for evaluation. The mean age at diagnosis was 7.5 years with a male to female ratio of 3.7-1. The predominant histology was nodular sclerosis (44%) but with a relatively high proportion of mixed cellularity (35.2%) The EFS at 5 and 10 years was 71% and 68%, respectively. There was a 14% rate of abandonment of therapy. CONCLUSION: This treatment regimen for children with Hodgkin lymphoma, when applied as a multi-institutional regimen, had poorer outcome than our previously reported preliminary data and was inferior to the EFS reported in high-income countries. The major contributor adversely affecting EFS in this report is abandonment of therapy. Given these results, AHOPCA initiated a concerted effort to decrease abandonment of therapy.

Barriers to effective treatment of pediatric solid tumors in middle-income countries: can we make sense of the spectrum of nonbiologic factors that influence outcomes?

BACKGROUND: The delivery of effective treatment for pediatric solid tumors poses a particular challenge to centers in middle-income countries (MICs) that already are vigorously addressing pediatric cancer. The objective of this study was to improve the current understanding of barriers to effective treatment of pediatric solid tumors in MICs. METHODS: An ecologic model centered on pediatric sarcoma and expanded to country as the environment was used as a benchmark for studying the delivery of solid tumor care in MICs. Data on resources were gathered from 7 centers that were members of the Central American Association of Pediatric Hematologists and Oncologists (AHOPCA) using an infrastructure assessment tool. Pediatric sarcoma outcomes data were available, were retrieved from hospital-based cancer registries for 6 of the 7 centers, and were analyzed by country. Patients who were diagnosed from January 1, 2000 to December 31, 2009 with osteosarcoma, Ewing sarcoma, rhabdomyosarcoma, and other soft tissue sarcomas were included in the analysis. To explore correlations between resources and outcomes, a pilot performance index was created. RESULTS: The analyses identified specific deficits in human resources, communication, quality, and infrastructure. The treatment abandonment rate, the proportion of metastatic disease at diagnosis, the relapse rate, and the 4-year abandonment-sensitive overall survival (AOS) rate varied considerably by country, ranging from 1% to 38%, from 15% to 54%, from 24% to 52%, and from 21% to 51%, respectively. The treatment abandonment rate correlated inversely with health economic expenditure per capita (r = -0.86; P = .03) and life expectancy at birth (r = -0.93; P = .007). The 4-year AOS rate correlated inversely with the mortality rate among children aged <5 years (r = -0.80; P = 0.05) and correlated directly with the pilot performance index (r = 0.98; P = 0.005). CONCLUSIONS: Initiatives to improve the effectiveness of treatment for pediatric solid tumors in MICs are warranted, particularly for pediatric sarcomas. Building capacity and infrastructure, improving supportive care and communication, and fostering comprehensive, multidisciplinary teams are identified as keystones in Central America. A measure that meaningfully describes performance in delivering pediatric cancer care is feasible and needed to advance comparative, prospective analysis of pediatric cancer care and to define resource clusters internationally.

Pediatric sarcoma in Central America: outcomes, challenges, and plans for improvement.

BACKGROUND: Children with cancer in middle-income countries have inferior outcomes compared with similar children in high-income countries. The magnitude and drivers of this survival gap are not well understood. In the current report, the authors sought to describe patterns of clinical presentation, magnitude of treatment abandonment, and survival in children with sarcoma in Central America. METHODS: A retrospective review was conducted of hospital-based registries from national pediatric oncology referral centers. Patients with newly diagnosed osteosarcoma, Ewing sarcoma, rhabdomyosarcoma (RMS), and soft tissue sarcoma (STS) between January 1, 2000 and December 31, 2009 were included. Survival analyses were performed first using standard definitions of overall survival (OS) and event-free survival (EFS) and then with abandonment included as an event (abandonment-sensitive OS and abandonment-sensitive EFS). RESULTS: In total, 785 new cases of pediatric sarcoma were reported (264 diagnoses of osteosarcoma, 175 diagnoses of Ewing sarcoma, 240 diagnoses of RMS, and 106 diagnoses of STS). The rate of metastatic disease at presentation was high (osteosarcoma, 38%; Ewing sarcoma, 39%; RMS, 29%; and STS, 21%). The treatment abandonment rate also was high, particularly among patients with extremity bone sarcomas (osteosarcoma, 30%; Ewing sarcoma, 15%; RMS, 25%; and STS, 15%). Of 559 patients who experienced a first event, 59% had either recurrent or progressive disease. The 4-year OS rate (±standard error) was 40% ± 3%, and the EFS rate was 30% ± 2%; however, these rates decreased further to 31% ± 2% and 24% ± 2%, respectively, when abandonment was taken into account. CONCLUSIONS: The current results indicated that high rates of metastases and treatment abandonment and difficulty with upfront treatment effectiveness are important contributors to the poor survival of children with pediatric sarcomas in Central America. Initiatives for early diagnosis, psychosocial support, quality improvement, and multidisciplinary care are warranted to improve outcomes.

Colon carcinoma in children and adolescents: prognostic factors and outcome-a review of 11 cases.

BACKGROUND: Carcinoma of the colon and rectum is rare in the pediatric age group, and usually presents with an advanced stage disease bearing a poor prognosis. Colorectal carcinoma should be considered in children with signs of intestinal obstruction, alteration in bowel habits, gastrointestinal bleeding and chronic abdominal pain. We performed a retrospective study to evaluate the clinical characteristics, and prognosis of these patients. METHODS: Between 1974 and 2007, 11 patients were identified and treated for colorectal carcinoma at the Oncology Unit. The medical records were studied to analyze the age, sex, clinical presentation, diagnostic procedures, extent of disease (Dukes staging), treatment, histological types, and outcome. RESULTS: There were seven boys and four girls ranging from 7 to 17 of age. Predisposing diseases and syndromes were encountered in three children, (1 with Turner's syndrome and two with adenomatous familial polyposis). Abdominal pain, acute intestinal obstruction, rectal bleeding and weight loss were the commonest symptoms. Surgical procedures were done in 11 patients (incomplete resection with segmental resection in 4 patients, complete resection in the other 4, and biopsy alone in 3 patients).The predominant histological type was mucinous carcinoma. Seven patients received adjuvant chemotherapy, all of whom did not survive. Two patients died shortly after initial surgery, and two patients are alive, and well. CONCLUSIONS: Colorectal carcinoma in children is very uncommon and could be easily misdiagnosed, resulting in advanced stage disease at diagnosis. Because radical surgery which is the mainstay of treatment is possible only in patients with early stage disease, a high level of awareness and early diagnosis are critical.

Diagnosis of anterior mediastinal mass lesions using the Chamberlain procedure in children.

The mediastinum is the most common site for primary intra-thoracic pathology in childhood. This paper describes the versatility of the Chamberlain operation in establishing tissue diagnosis and guiding definitive treatment in children with mediastinal mass lesions. During 1999-2006, 28 consecutive patients were referred to a National Oncology Center with anterior mediastinal pathology. Eleven underwent the Chamberlain procedure. Demographic data, diagnostic sensitivity, operating time and morbidity were recorded. All patients had preoperative chest X-ray and computed tomography scans. The Chamberlain operation was deployed in only those children without other ways of accurately establishing their diagnosis. Others with mediastinal pathology had a diagnosis established by lymph node biopsy, thoracentesis or other method(s). Eleven patients (nine male, two female; age range 2-13 years) underwent the Chamberlain procedure. In these children, there was no pre-existent diagnosis and this was the primary procedure employed. Diagnostic accuracy was 100%. Three patients had pleural disruption and chest tubes were placed at the time of surgery. No patient required a thoracotomy. Average operating time was 1.3 h. Five patients were diagnosed with Hodgkin's lymphoma, four had non-Hodgkin's lymphoma and two children thymic hyperplasia. The Chamberlain operation provides excellent access to the antero-superior mediastinum for biopsy of obscure mediastinal mass lesions in childhood. Complications from this procedure are very rare.