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Pineal gland tumors are rare and account for less than 1% of all primary brain tumor diagnoses. They are more commonly seen in pediatric patients than in adults. We analyzed the available SEER data on pineal gland tumors that were diagnosed during the period 1973-2005. The cohort was subdivided into groups on the basis of tumor histology: germ cell tumors, pineal parenchymal tumors, gliomas, and other pineal tumors. Analyses of incidence, survival, factors influencing survival, and treatment modalities are provided. Among the 633 patients with pineal tumors, male sex was predominant, i.e., sex ratio was 3:1 for the whole group and 11.8:1 for those with germ cell tumors. The 5-year overall survival (OS) for the cohort was 65% +/- 2.1%. Those with germ cell tumors experienced the best survival (OS = 78.9% +/- 2.3%), followed by those with gliomas (OS = 61% +/- 9.3%), and those with pineal parenchymal tumors (OS = 47.2% +/- 4.2%). Non-germ cell tumors, absence of radiotherapy from treatment regimen, and diagnosis before 1993 were the only factors associated with a negative impact on survival. The extent of surgical tumor resection did not affect survival in any histologic subgroup. We conclude that, although pineal tumors are histologically diverse, they share some similarities due to their unique location. An aggressive surgical approach should be considered with caution in this region. Further studies on different pineal tumors subtypes are needed.

Background: Although screening for familial retinoblastoma has been shown to be beneficial we suspected that such screening programs may be less than optimal in developing countries (DC)., Methods: Retrospective cohort study comparing patients with familial retinoblastoma from five centers in DC (Argentina, Brazil, Turkey, Jordan, and Venezuela) versus a reference center in the USA., Results: Ninety-two (32 from the USA and 60 from DC) patients were included. Forty-one (44.6%) patients avoided enucleation, 42 (45.7%) had 1 eye removed, and 9 (9.8%) underwent bilateral enucleation. Eleven (11.9%) had major pathology risk factors at enucleation. There were no cases of metastatic disease at diagnosis. Detection via screening was significantly less common in DC than in the USA (23.3% vs. 71.8%, P < 0.0001). Patients in DC were diagnosed at a significantly later age and with more advanced intraocular disease that led to increased risk of bilateral enucleation. Patients detected by screening in DC were significantly younger at diagnosis, had less advanced intraocular disease, better ocular preservation rates and survival results than those whose retinoblastoma was not detected via early screening. Five-year pEFS was 0.92 for the patients treated in the USA and 0.81 for the patients in DC (P = 0.42). Seven events occurred (extraocular relapse four in patients from DC and second malignancies in three)., Conclusions: Patients with familial retinoblastoma are less likely to be diagnosed by screening in DC and had higher morbidity and mortality caused by recurrent extraocular retinoblastoma., ((c) 2009 Wiley-Liss, Inc.)

Purpose: To compare clinical features and outcomes of adults and children reported to have rhabdomyosarcoma., Patients and Methods: We analyzed data from 1,071 adults (age > 19 years) and 1,529 children (age < or = 19 years) reported in the public-access Surveillance, Epidemiology and End Results database as having rhabdomyosarcoma, diagnosed from 1973 to 2005. Survival estimates were determined using survival time with the end point being death from any cause., Results: Adults with rhabdomyosarcoma had significantly worse outcome than children (5-year overall survival rates, 27% +/- 1.4% and 61% +/- 1.4%, respectively; P < .0001). Tumors in adults were more likely to be at an unfavorable site (65% v 55%; P < .0001) and to have histologies that are unusual during childhood, particularly the pleomorphic subtype (19%) and not otherwise specified (43%). Regional and distant spread was not more frequent in adults. Adults had significantly worse outcome than children with similar tumors. The most significant difference was in localized disease; 5-year survival estimates were 82% +/- 2.0% for children and 47% +/- 2.9% for adults (P < .0001). Multivariate analysis showed that age, histologic subtype, primary site location, stage, and local control with surgery and/or radiation were significant predictors of survival. However, alveolar subtype and unfavorable primary site lost significance when analysis was restricted to adults., Conclusion: Adults reported to have rhabdomyosarcoma had worse survival than children with similar tumors. Predictors of poor outcome in children were valid in adults except for alveolar histology and unfavorable tumor site.

For patients with diffuse pontine glioma, our institution offers local radiotherapy and supportive care only. The prognosis and do-not-resuscitate orders are discussed upfront with patients' parents. To investigate the effectiveness of this policy, we retrospectively reviewed records of patients with diffuse pontine glioma treated at the institution over a 49-month period. This study included 19 children (16 girls and 3 boys). The median age at diagnosis was 7 years. One patient remained alive at the end of the study, 2 were lost to follow-up, and 16 were confirmed dead. The median survival was 8.2 months. Do-not-resuscitate discussion was documented for 14 patients and successfully initiated for 11. None of those confirmed dead had been offered life support. Contrary to the common belief, our study showed that do not resuscitate should be addressed in Muslim patients with high-risk malignancies. We also found that diffuse pontine glioma warrants further study in developing countries.

Background: The impact of chemotherapy, focal therapies, radiation and co-existing ocular morbidities on histology of eyes with retinoblastoma enucleated following chemoreduction is not well known., Procedure: Twenty-five eyes (23 patients) with retinoblastoma enucleated after failing eye-salvage therapy were evaluated. Reasons for enucleation (tumor progression, subretinal or vitreous seeds) and co-morbid conditions (neovascular glaucoma, cataract, vitreous hemorrhage and retinal detachment) were documented. All specimens were reviewed for evidence of ciliary body, choroidal, optic nerve, and scleral invasion., Results: The median age at diagnosis was 14 months (range, 1-37 months). Twenty eyes were classified as Reese-Ellsworth Group IV-V at diagnosis. Twenty-four eyes had recurrent disease at enucleation; one eye was enucleated for neovascular glaucoma and vitreous hemorrhage. Co-existing ocular morbidities at enucleation included vitreous hemorrhage (n = 6), retinal detachment (n = 9), neovascular glaucoma (n = 9) and cataracts (n = 3). Histologic findings included choroidal invasion (n = 7), ciliary body invasion (n = 4), optic nerve invasion (n = 6) and scleral invasion (n = 3). The median time from diagnosis to enucleation was 11 months. Co-existing retinal detachment and vitreous hemorrhage significantly increased the likelihood of optic nerve invasion (P = 0.014 and P = 0.011, respectively). Prolonged time to enucleation was significantly associated with the likelihood of choroidal (P = 0.010) and ciliary body (P = 0.021) invasion as well as invasion of multiple sites., Conclusion: In eyes with retinoblastoma enucleated after chemoreduction, co-existing ocular morbidities and time to enucleation are predictive of extra-retinal extension.

Pediatric low-grade gliomas are the most common tumors of the central nervous system in children, accounting for almost 50% of all childhood brain tumors. They are a heterogeneous group of tumors with different histologic subtypes. Most treatment studies address low-grade gliomas as a single entity, depriving us of histology-specific treatment outcomes. This is mostly due to a lack of understanding of tumor biology at the molecular level. Pediatric low-grade gliomas are not benign, and most incompletely resected tumors will progress and negatively affect quality of life. The advancements made in understanding sporadic pilocytic astrocytoma and neurofibromatosis 1-associated pilocytic astrocytoma in particular have paved the way for potential targeted therapy and biological stratification. Such progress in pilocytic astrocytoma needs to be consolidated and expanded to other histologic varieties of pediatric low-grade gliomas.

Despite the important role of nurses in a pediatric oncology team, the marginalization of nursing is common, especially in developing countries. At the King Hussein Cancer Center (KHCC) in Jordan, the position of the clinical nurse coordinator (CNC) was created in the pediatric neuro-oncology and ocular oncology services to empower the role of nursing. Our advanced nursing practice is based on the domains of the Strong Model of Advanced Practice, which are direct patient care, support of systems, education, research and publications, and professional leadership. There is strong involvement of the CNC in clinical care, patient education, follow-up, and interdisciplinary collaboration, which has resulted in improved survival, reduced morbidity, and increased compliance of patients and also their successful reintegration into society. The coordination of twinning initiatives and telemedicine activities has helped ensure the quality of treatment and supported research initiatives in the services. Such a positive impact has led to a significant increase in the recruitment of CNCs at KHCC. We propose that the role of nursing be strengthened in developing countries to improve the level of care provided to patients and their families.

Background: The prognosis for children with M1 medulloblastoma (positive CSF cytology) has not been well-defined., Methods: We retrospectively reviewed the records of 285 newly diagnosed medulloblastoma patients treated between 1984 and 2006. Older children received post-operative craniospinal and tumor bed irradiation; radiotherapy for younger children depended on treatment era and physician/family preference., Results: 55 patients were <3 years old and 230 patients were >or= 3 years old at diagnosis. We detected significant (P < 0.0001) associations between M1 disease and EFS for the entire cohort and for both younger and older patients. Among younger children, M1 patients had lower EFS than M0 (P = 0.0044)., Conclusions: Children <3 years old with M1 medulloblastoma fared poorly in our small series. Survival for older children with M1 disease treated with higher-dose CSI was better than that of M2/M3 patients, but still less than optimal; our findings do not support reduction in therapy for either cohort.

Central nervous system tumors are the most common pediatric solid tumors and a leading cause of cancer-related mortality and morbidity in this age group. Survival rates have improved significantly over the last decades for most of the tumor types, as a consequence of improvements in neuroimaging, neurosurgery and neuroanesthesia, radiation oncology, and medical oncology. The complexity of the management of these patients requires a multidisciplinary approach and has led to the emergence of a new subspecialty of pediatric neuro-oncologists who are dedicated to the management and follow-up of this population. This review highlights the most critical advances in the diagnostic and treatment modalities of pediatric brain tumors. A specific review of the most common tumor types discusses treatment options, controversies, and ongoing developments, with an emphasis on cooperative trials.

Retinoblastoma is curable when diagnosed early and treated appropriately; however, the prognosis is dismal when the basic elements of diagnosis and treatment are lacking. In developing countries, poor education, lower socioeconomic conditions, and inefficient health care systems result in delayed diagnosis and suboptimal care. Furthermore, the complexity of multidisciplinary care required is seldom possible. Whereas ocular salvage is a priority in the Western world, death from retinoblastoma is still a major problem in developing countries. To bring the 2 ends of this spectrum together and provide a forum for discussion, the "One World, One Vision" symposium was organized, at which clinicians and researchers from various cultural, geographic, and socioeconomic backgrounds converged to discuss their experiences. Strategies for early diagnosis in developing countries were discussed. Elements of the development of retinoblastoma centers in developing countries were discussed, and examples of successful programs were highlighted. An important component in this process is twinning between centers in developing countries and mentor institutions in high-income countries. Global initiatives by nongovernmental organizations such as the International Network for Cancer Treatment and Research, Orbis International, and the International Agency for Prevention of Blindness were presented. Treatment of retinoblastoma in developing countries remains a challenge; however, it is possible to coordinate efforts at multiple levels, including public administrations and nonprofit organizations, to improve the diagnosis and treatment of retinoblastoma and to improve the outcome for these children.

Background: This study evaluated the outcome of retinoblastoma patients, when employing a telemedicine-based twinning program in Jordan., Procedure: This cohort study included patients at the King Hussein Cancer Centre (KHCC; Amman, Jordan) who received consultations for retinoblastoma from March 2003 to September 2006. A collaborative program was established with the International Outreach Program at St. Jude Children's Research Hospital in Memphis, Tennessee. Cases were discussed using an Internet consultation service where fundus images, clinical history, and proposed treatment were reviewed. Selected cases were further discussed via videoconferencing and electronic mail., Results: Thirty-three children with retinoblastoma (20 bilateral) were treated at KHCC. The median age at diagnosis was 7 months for patients with bilateral retinoblastoma and 35 months for patients with unilateral retinoblastoma. Of the 20 patients with bilateral disease, 12 were newly diagnosed and 8 had received prior treatment. Our success in the bilateral cases was most evident in the previously untreated group, in which only six eyes (25%) were enucleated and four eyes (17%) were irradiated. Of the 13 patients with unilateral retinoblastoma, 12 underwent enucleation, and 6 required radiation. Neither group experienced mortality., Conclusions: Twinning has positively impacted survival and ocular salvage in Jordan. By partnering a team of professionals with mentors willing to provide close supervision, the highly specialized management of retinoblastoma can be successfully implemented in a developing country.

Successful twinning initiatives have been reported in childhood leukemia. Pediatric neuro-oncology requires a complex multidisciplinary approach and the feasibility of similar twinning programs is unknown. Twinning between King Hussein Cancer Center in Amman and the Hospital for Sick Children in Toronto started with e-mail communications, and subsequently included monthly videoconferences and exchanges between institutions. The outcome of 35 newly diagnosed medulloblastoma patients (22 high-risk and 13 average-risk) treated during this period is reported. The 3-year overall survival for average risk and high-risk patients was 100 and 81%, respectively. This experience suggests that twinning may facilitate the implementation of multidisciplinary neuro-oncology programs in low-income countries. Videoconferencing allows interactive exchanges with a significant learning impact., ((c) 2007 Wiley-Liss, Inc.)

Following from the publication of the International Retinoblastoma Staging System, an open internet discussion group was created at the www.cure4kids.org resource. The results of a survey distributed among participants are discussed. Although most patients with retinoblastoma were treated under prospective protocols, there was a wide variation in the definition of risk criteria and in the criteria for giving adjuvant chemotherapy following enucleation. Definition of high-risk histological features and the criteria for use of adjuvant therapy will be standardized in future studies. Internet meetings are a valuable mechanism for enabling participation from under-resourced countries in the development of cooperative studies., ((c) 2007 Wiley-Liss, Inc.)

Background: Telemedicine is widely used in industrialized countries for educational purposes. Twinning experiences using telemedicine between institutions in industrialized and developing countries (DC) have been limited. Pediatric neuro-oncology is a complex multidisciplinary discipline that is underserved in most of DC and provides a model to test the feasibility of such tool for twinning purposes., Methods: A computer, an EMLO visual presenter HV-7600SX document camera, and a TANDBERG 6000 model videoconference unit were used to present data. For connectivity, we used a six-channel ISDN telephone line. Each channel is 64 megabytes/sec., Results: Between December 2004 and May 2006, 20 sessions of videoconference were held between King Hussein Cancer Center and the Hospital for Sick Children to discuss 72 cases of 64 patients with various brain tumors (5 patients were discussed twice and 1 patient four times). In 23 patients (36%), major changes from original plan were recommended on different aspects of the care. In 21 patients (91%), those recommendations were followed, with potentially significant positive impact on patients' care., Conclusions: Videoconferencing is a feasible and practical twinning tool in pediatric neuro-oncology with a potentially major impact on patient care., ((c) 2006 Wiley-Liss, Inc.)

Desmoplastic infantile ganglioglioma is a rare superficial supratentorial tumor that occurs within the first two years of life. Despite the worrisome radiological and histological appearance, the tumors are often curable following gross total resection. Tumors with similar characteristics are exceedingly rare in the noninfantile population. We present a six-year-old boy with seizures, weakness, and unsteady gait. Radiographic imaging confirmed a very large, solid and cystic mass in the right temporal-parietal region. Pathological examination demonstrated a tumor with severe desmoplasia identical to those reported as "desmoplastic infantile ganglioglioma." This case adds to the limited data available for desmoplastic gangliogliomas in the noninfantile population. It is not clear, yet likely, that the noninfantile form of this neoplasm is biologically similar to the infantile form. It is also unclear whether the desmoplastic noninfantile ganglioglioma has characteristics similar to classical ganglioglioma. This rare case highlights the remarkable versatility of glioneuronal tumors in children.

Background: Carboplatin-based regimens have demonstrated activity in unresectable low-grade glioma (LGG) in children. Despite an interesting toxicity profile, the use of these regimens has been limited by the development of carboplatin hypersensitivity reaction (HSR) in up to 30% of patients. Desensitization has been the recommended approach for HSR. However, no guidelines have existed to aid physicians when carboplatin desensitization techniques fail., Methods: A pilot study of monotherapy with weekly vinblastine for LGG in 9 children who developed carboplatin HSR on a carboplatin and vincristine regimen was performed., Results: Vinblastine toxicity was moderate and readily manageable. None of the 9 patients had disease progression on therapy. Magnetic resonance imaging evaluation of tumor size from diagnosis to the end of vinblastine treatment showed 1 complete response (CR), 1 partial response (PR), 5 objective effects (OE), and 2 stable diseases (SD)., Conclusions: This experience suggested that weekly vinblastine has a good efficacy to toxicity ratio in the treatment of LGG and can be a valuable option for children who develop severe HSR., (Copyright 2005 American Cancer Society.)