Primary central nervous system germ cell tumors in Central America and the Caribbean Region: an AHOPCA 20-year experience.

Background: Primary central nervous system germ cell tumors (GCT) are rare neoplasms in pediatrics. Treatment depends on the histological subtype and extent of the disease. Overall survival (OS) is above 90% for germinomas and 70%-80% for nongerminomatous GCT (NGGCT) in high-income countries (HIC) while data are usually lacking for patients in Low-Middle Income country (LMIC). Objective: This study aims to describe the experience of treating patients with CNS GCT in four of eight countries, members of the Asociación de Hemato-Oncología Pediátrica de Centro América (AHOPCA), and determine their 5-year OS. Design/methods: We conducted a retrospective chart review of patients treated for CNS GCT. Epidemiological and clinical characteristics, histology, treatment modalities, and outcomes were analyzed. Results: From 2001 to 2021, 48 patients were included: 22 from Guatemala, 18 from Nicaragua, three from the Dominican Republic, and five from El Salvador. Thirty-one (64.6%) were boys; the median age at diagnosis was 10.2 years (range: 1 to 17 years). Presenting symptoms were headaches (n = 24, 50%), visual disturbances (n = 17, 35.4%), vomiting (n = 12, 25%), nausea (n = 8, 16.7%), and diabetes insipidus (n = 7, 14.6%). Two patients with NGGCT presented with precocious puberty. Biopsy or tumor resection was performed in 38 cases (79.2%): 23 (88.4%) germinomas, 11 (78.6%) NGGCT, and four (50%) CNS GCT. Eight patients were diagnosed and treated based on CSF tumor marker elevation; four germinomas (BHCG 11.32-29.41 mUI/mL) and four NGGCT (BHCG 84.43-201.97 mUI/mL or positive AFP > 10 UI/mL). Tumor locations included suprasellar (n = 17, 35.4%), pineal (n = 13, 27.1%), thalamus/basal ganglia (n = 5, 10.4%), other (n = 12, 25%), and one bifocal. Four (8.3%) had metastatic disease, and six had positive CSF; staging data were incomplete in 25 patients (52%). Patients were treated with varied chemotherapy and radiotherapy modalities. Nine patients had incomplete data regarding treatment. Five-year OS was 65% (68% for germinoma, 50.6% for NGGCT, and 85.7% for unclassified GCT). Conclusions: Germinoma was the most common histology, and there was a male predominance. More than half of patients had incomplete staging data and treatment was variable across the region. OS is lower compared to HIC. Standardized treatment protocols will aid in adequate staging and treatment planning, prevent complications, and improve survival.

Rhabdomyosarcoma in low- and middle-income countries: A report from the Asociacion de Hemato-oncología Pediatrica de Centro América (AHOPCA).

This report describes the results of an observational study dedicated to rhabdomyosarcoma developed by the Asociación de Hemato-oncología Pediatrica de Centro América (AHOPCA) between 2001 and 2018. Overall, 337 previously untreated patients < 18 years old were included in the analysis; 58% had unresected disease, and 19% were metastatic at diagnosis. With a median follow-up of 6.6 years, five-year event-free and overall survival rates were 30% and 33%, respectively. Local progression/relapse was the main cause of treatment failure.

Ellipsoidal Process of the Femoral Head in Legg-Calvé-Perthes Disease: Effect of Prophylactic Hemiepiphysiodesis.

Purpose: Legg-Calvé-Perthes disease (LCPD) often causes the residual deformity, typically appearing as an ellipsoidal shape. In some cases, this ovalization is related to the asymmetric growth of the femoral head physis, which presents a growth-inhibiting necrotic area in the anterosuperior quadrant. The objective of the present study is to evaluate the effectiveness of selective hemiepiphysiodesis of the healthy physis in the posteroinferior quadrant as a means of disrupting femoral head ovalization in cases of LCPD with the previous onset of ovalization. Methods: We performed a prospective study of 39 LCPD hips operated on consecutively by selective hemiepiphysiodesis of the posteroinferior portion of the head during the reossification phase.Surgical indication was based on a progressive increase in the ellipsoidal index (EI), the presence of a double epiphyseal nucleus of reossification, physeal narrowing, and physeal angulation. Hemiepiphysiodesis was performed at the mean patient age of 8.8 years (SD 1.5) and a mean of 4.7 years (SD 1.4) following disease onset. Results: Preoperative EI was 1.80 (SD 0.2), which was reduced to 1.72 (SD 0.2) postoperatively (P > 0.05). Physeal angulation increased from 50° preoperatively (SD 9.3) to 54.29° (SD 9.7) at the end of growth (P > 0.05). The final result according to the Stulberg classification revealed 20 class-II cases, 16 class III, and 3 class IV, and SDS was 25.97 (SD 9.95), range: 9.36-51.67. Conclusion: As revealed by the EI, the ellipsoidal process may be stopped by selective hemiepiphysiodesis in the posteroinferior quadrant of the femoral head. Level of Evidence: II.

Ellipsoidal process of the femoral head in Legg-Calvé-Perthes disease. Part I.

Legg-Calvé-Perthes disease (LCPD) often produces a residual deformity, typically consistent with coxa magna, coxa plana, and ellipsoidal shape. Depending on the degree of asphericity and flatness, this morphology was classified by Stulberg in stages III and IV. Thus far, few studies have investigated physeal injury as an etiological cause or evaluated its progressive profile throughout Waldenström's reossification stage and the remodelling stage. In this study, we analysed the ellipsoidal process of the femoral head. This was a retrospective control case study involving 83 unoperated hips with LCPD and Stulberg stages III and IV outcome. The data were compared with those obtained for 49 healthy contralateral hips (control). The Ellipsoidal Index, the presence of a double epiphyseal reossification nucleus, physeal narrowing, intraphyseal angle, epiphyseal height, diameter of the head, and Reimer's Index were determined. Measurements were performed at four-time points: the year the reossification stage was initiated, the final growth stage, and two equally spaced time points in between. The Ellipsoidal Index gradually increased throughout the course of the disease from 1.6 in the initial reossification stage to 2.0 at the end of growth. In the control cases, this value was consistently 1.4. More ellipsoidal deformity was observed in Stulberg stage IV versus Stulberg stage III patients (P < 0.05). Moreover, there was a direct link between a high Ellipsoidal Index and the appearance of a double reossification nucleus, a physeal narrowing in the area underlying the anterosuperior nucleus, intraphyseal angle. Reimer's Index showed a gradual extrusion from baseline to the end of growth (26.1 versus 31.8, respectively; P < 0.05). The ellipsoidal process of the femoral head occurs gradually throughout the reossification and remodelling stages. This was linked to the appearance of a double epiphyseal nucleus, gradual extrusion, an angulated physis appearance, an asymmetrical narrowing of the physis and a high Ellipsoidal Index, which may be indicative of poor prognosis. Levels of Evidence for Primary Research Question: Level III, case-control study.

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.

External manual reduction of paediatric idiopathic ileocolic intussusception with US assistance: a new, standardised, effective and safe manoeuvre.

BACKGROUND: The ultimate goal in the management of paediatric ileocolic intussusception is to achieve non-operative reduction and therefore patient cure. The standard non-surgical treatment consists of enema (air or liquid media). OBJECTIVE: The purpose of this study is to present external manual reduction for paediatric ileocolic intussusception. We present a new manoeuvre that is standardised, simple, safe and effective for the radiologist in the non-surgical management of this pathology. MATERIALS AND METHODS: External manual reduction is performed under sedation. Execution of the manoeuvre is detailed and illustrated. The procedure was carried out 15 times in 13 paediatric patients with idiopathic ileocolic intussusception. RESULTS: Complete reduction exclusively by external manual reduction was accomplished on 12 occasions (80%). In the remaining three procedures, partial reduction to the cecum was obtained. Subsequent enema achieved complete reduction in two. Overall non-surgical reduction rate was 93%. CONCLUSION: External manual reduction is a radiation-free, safe and effective procedure. In case of incomplete reduction, it facilitates enema performance. External manual reduction incorporates and additional initial step in the non-surgical reduction of intussusception and should be considered a first-line procedure.

Programa de Gestión de Calidad en Salud Ministerio de Salud - Gobierno de la Ciudad de Buenos Aires - Análisis organizacional / Program Quality Management in Health Ministry of Health-government of the city of Buenos Aires-Organizational analysis

Se realizó un análisis organizacional del Programa de Gestión de Calidad del Ministerio de Salud del Gobierno de la Ciudad de Buenos Aires en diciembre de 2006. Las dimensiones del análisis seleccionadas fueron la historia, los actores, las actividades, el contexto socio político, las expectativas sociales, la conducción, elliderazgo, el poder, los roles, los controles nstitucionales y la relación con el medio. Se utilizaron fuentes de información primaria obrantes en la sede del programa lo que permitió una visión tanto sincrónica como diacrónica del mismo. Finalmente se presentan debilidades, fortalezas, amenazas, oportunidades de mejora, y logros generados por cambios culturales promovidas por el programa.

Programa de Gestión de Calidad en Salud Ministerio de Salud - Gobierno de la Ciudad de Buenos Aires - Análisis organizacional / Program Quality Management in Health Ministry of Health-government of the city of Buenos Aires-Organizational analysis

Se realizó un análisis organizacional del Programa de Gestión de Calidad del Ministerio de Salud del Gobierno de la Ciudad de Buenos Aires en diciembre de 2006. Las dimensiones del análisis seleccionadas fueron la historia, los actores, las actividades, el contexto socio político, las expectativas sociales, la conducción, elliderazgo, el poder, los roles, los controles nstitucionales y la relación con el medio. Se utilizaron fuentes de información primaria obrantes en la sede del programa lo que permitió una visión tanto sincrónica como diacrónica del mismo. Finalmente se presentan debilidades, fortalezas, amenazas, oportunidades de mejora, y logros generados por cambios culturales promovidas por el programa.(AU)

Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature.

A case of pancreatoblastoma (PB) in a 2 month-old male infant with incomplete Beckwith-Wiedemann syndrome is presented. Clinical examination disclosed left hemihypertrophy, macroglossia, bilateral exophthalmos, and enlargement of the left testis. Imaging with ultrasound and computed tomography scan showed a well-defined, heterogeneous, and grossly cystic mass arising from the head of the pancreas. Serum alpha-fetoprotein (AFP) level was elevated. The tumor was completely resected, and the histological analysis showed PB. The patient's recovery was uneventful, and AFP returned to normal values after surgery. The child has been disease-free for 5 years, and his serum AFP remained within normal values. Six other examples of this association, PB, and Beckwith-Wiedemann syndrome are recorded in the literature. The risk of developing tumor in this syndrome (complete and incomplete form) increases when hemihypertrophy is present, and the need for routine screening examination is warranted. Beckwith-Wiedemann syndrome was suggested to be a favorable biological marker for survival in children who have intraabdominal tumors.

Acute torsion of the spleen: diagnosis and management.

We present a 2-year-old girl with a 24-hour history of abdominal pain, fever, and vomiting. The diagnosis of acute splenic torsion was made by means of color and power Doppler ultrasound. Management of this rare surgical emergency is discussed.

Serous cystadenoma of the pancreas in a child.

Pancreatic tumors are very rare in children. Until now, approximately 150 cases have been reported in the English-language medical literature. Only 4 of them represented serous cystadenoma. Although the tumor is not potentially malignant, all patients required partial pancreatic resection. The authors present a 4-year-old girl with a short history of abdominal pain and vomiting. Sonography and computed tomography showed a pancreatic mass. The mass was proved to be a serous cystadenoma, and resection of the tumor was performed. Clinical discussion with review of the literature is presented.

Heterotopic brain tissue in the upper lip of a neonate mimicking bilateral cleft lip features.

Heterotopic brain tissue is an extremely rare developmental anomaly frequently diagnosed in the newborn period. This entity has been described in various sites of the head and neck, most commonly in the nasal area. Computed tomography and magnetic resonance imaging are necessary for exclude associated cranial communications. Heterotopic brain tissue is an extremely rare developmental anomaly frequently diagnosed in the newborn period. This entity has been described in various sites of the head and neck, most commonly in the nasal area. Computed tomography and magnetic resonance imaging are necessary for exclude associated cranial communications.