Maria Eleonora Basso

Treatment with oral etoposide for childhood recurrent ependymomas

In this study the authors retrospectively evaluated the feasibility and effectiveness of prolonged oral etoposide therapy in children with recurrent ependymoma. Twelve ependymoma patients with documented recurrent or persistent disease were treated between May 1998 and October 2003. All patients were treated monthly with oral VP-16 administered at a dose of 50 mg/m2/d for 21 days, with a 7-day interval between cycles, for a planned minimum number of six cycles. Response (complete plus partial) after two cycles occurred in 5 of the 12 patients (41.6%). Response plus stable disease occurred in 10 of the 12 (83.3%), with a median duration of response or stable disease of 7 months (range 4-30). The median survival was 7 months; the 2-year progression-free survival was 16.7%. These results emphasize that oral etoposide is an attractive option for childhood recurrent ependymomas in terms of administration, tolerability, and neuroradiologic response.

Schinzel-Giedion syndrome with sacrococcygeal teratoma

A case of Schinzel-Giedion syndrome, a rare malformation syndrome, is described. In addition to the classic features of the syndrome, the patient had a malignant sacrococcygeal teratoma and agenesis of the corpus callosum. So far, this patient is the fifth case with a sacrococcygeal tumor and the eighth with anomalies of the corpus callosum. According to this occurrence of uncommon tumors, risk of malignancy could be a component of Schinzel-Giedion syndrome.

End-of-life care in pediatric neuro-oncology

The management of children with cancer during the end‐of‐life (EOL) period is often difficult and requires skilled medical professionals. Patients with tumors of the central nervous system (CNS) with relapse or disease progression might have additional needs because of the presence of unique issues, such as neurological impairment and altered consciousness. Very few reports specifically concerning the EOL period in pediatric neuro‐oncology are available.

A complex karyotype including a t(2;11) in a paediatric ependymoma: case report and review of the literature

Ependymomas are glial tumours representing approximately 5–10% of all intracranial tumours and are the third most common primary brain tumour in childhood. Only a few karyotypic studies on paediatric ependymomas have been published and no specific chromosomal aberration has been specifically related to this type of cancer. We performed cytogenetic analysis of an ependymoma in an 11-year-old boy. Our patient showed a complex karyotype, characterized by a near-tetraploidy and a sole structural unbalanced aberration: der(2)t(2;11)(q11.2;q13.1), which has not been described before. We here discuss such cytogenetic findings, comparing our data with those reported in the literature.