Frequency of pathogenic germline variants in pediatric medulloblastoma survivors.

Background: Medulloblastoma is the most common malignant brain tumor in children. Most cases are sporadic, but well characterized germline alterations in , , , , , and predispose to medulloblastoma. However, knowledge about pathogenic/likely pathogenic (P/LP) variants that predispose to medulloblastoma vary based on genes evaluated, patient demographics, and pathogenicity definitions.

Increase of breast-feeding in the past decade in Greece, but still low uptake: cross-sectional studies in 2007 and 2017.

Objective: To estimate breast-feeding prevalence in Greece in 2007 and 2017, compare breast-feeding indicators and maternity hospital practices between these years, and investigate breast-feeding determinants.

Design: Two national cross-sectional studies (2007 and 2017) using systematic cluster sampling of babies with the same sampling design, data collection and analysis methodology.

Setting: Telephone interview with babies' mothers or fathers.

Socioeconomic factors play a more important role in childhood vaccination coverage than parental perceptions: a cross-sectional study in Greece.

To identify predictive factors of complete and age-appropriate vaccination status in Greece, we conducted a cross-sectional study, using stratified cluster sampling, among children attending the first year of the Greek Grammar school (about 6 years of age) and their parents/guardians. Almost 88% (N=3878) of pupils in the selected clusters (school classrooms) provided their vaccination booklet and their parents/guardians completed a questionnaire regarding beliefs and attitudes towards immunization. Belonging to a minority group, having other siblings and perceiving long distance to immunization site as a barrier were independent predictors of both incomplete and delayed vaccination status in the final logistic regression model.

Survival among children with medulloblastoma in Greece: gains from transition to chemotherapy and socio-economic differentials.

The objective of this study was to quantify improvements in survival due to chemotherapy among children with medulloblastoma treated during the last three decades at a university unit in Greece, compare these gains with figures derived from a specialized unit in the United States and explore the role of extrinsic factors affecting survival. The records of all children with medulloblastoma (n=50) treated at the University Childhood Oncology Unit in Athens, Greece during the period 1973-2003 were reviewed. The role on survival of socio-demographic factors was studied by modeling the data through Cox's proportional-hazards regression, controlling for the mode of treatment (chemotherapy, yes vs.

Retrospective family study of childhood medulloblastoma.

Medulloblastoma is the most common malignant central nervous system tumor of childhood and can occur sporadically or in association with inherited cancer susceptibility syndromes such as the nevoid basal cell carcinoma syndrome (NBCCS). To determine whether an association existed between the risk of developing medulloblastoma and undiagnosed syndromes, we retrospectively reviewed clinical data on 33 patients with medulloblastoma from a single institution and compared them with their unaffected relatives (n = 46). Six patients had tumors showing desmoplastic histology.

Mutations in SUFU predispose to medulloblastoma.

The sonic hedgehog (SHH) signaling pathway directs the embryonic development of diverse organisms and is disrupted in a variety of malignancies. Pathway activation is triggered by binding of hedgehog proteins to the multipass Patched-1 (PTCH) receptor, which in the absence of hedgehog suppresses the activity of the seven-pass membrane protein Smoothened (SMOH). De-repression of SMOH culminates in the activation of one or more of the GLI transcription factors that regulate the transcription of downstream targets.

Prognostic factors and secondary malignancies in childhood medulloblastoma.

Purpose: Little is known of the outcome of long-term survivors of childhood medulloblastoma, one of the most common pediatric malignancies. To determine the potential for secondary malignancies, a retrospective outcome evaluation in 88 consecutive cases of childhood medulloblastoma was performed.

Patients And Methods: The records of all patients with childhood medulloblastoma diagnosed at Children's National Medical Center in Washington, DC from 1969 through 1997 were reviewed.

Growth hormone replacement therapy in children with medulloblastoma: use and effect on tumor control.

Purpose: Progress has been made in the treatment of medulloblastoma, the most common childhood malignant brain tumor: However, many long-term survivors will have posttherapy growth hormone insufficiency with resultant linear growth retardation. Growth hormone replacement therapy (GHRT) may significantly improve growth, but there is often reluctance to initiate GHRT because of concerns of an increased likelihood of tumor relapse.

Patients And Methods: This study retrospectively reviewed the use of GHRT for survivors of medulloblastoma in 11 neuro-oncology centers in North America who received initial treatment for disease between 1980 and 1993 to determine its impact on disease control.

Intracranial calcifications in childhood medulloblastoma: relation to nevoid basal cell carcinoma syndrome.

Background And Purpose: Medulloblastoma is one of the most common posterior fossa tumors to occur in children. Our purpose was to document the frequency, location, and time of occurrence of intracranial calcifications in cranial CT studies of children with medulloblastoma.

Methods: We retrospectively reviewed cranial CT studies of 56 patients diagnosed with medulloblastoma from 1983 through 1997 for the presence of intracranial calcifications.

Deletion analysis of the adenomatous polyposis coli and PTCH gene loci in patients with sporadic and nevoid basal cell carcinoma syndrome-associated medulloblastoma.

Background: Medulloblastomas can occur sporadically or may be associated with hereditary tumor syndromes including familial adenomatous polyposis (FAP) and nevoid basal cell carcinoma syndrome (NBCCS).

Methods: The authors performed a retrospective analysis for allelic deletion of the adenomatous polyposis coli (APC) and PTCH gene loci using paraffin embedded medulloblastoma specimens from patients who were admitted to Children's National Medical Center in Washington, DC, between 1982 and 1997. Thirty-five cases from which tumor and normal tissue could be procured were analyzed.