The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas.

Introduction: We performed a retrospective study on clinical assessment, tumor location, radiological imaging, histopathological characteristics, and therapeutic management of 7 patients affected by choroid plexus carcinoma (CPC) or atypical choroid plexus papilloma (ACPP) who have been observed in the last 12 years.

Methods: Four patients fulfilled the criteria for classification as ACPP and three cases as CPC. The median age of the patients at the diagnosis was 42 months (range 3-190 months).

Salivary gland carcinomas in children and adolescents: the Italian TREP project experience.

Background: Salivary gland carcinomas are extremely rare in pediatric age. We report the clinical features of a series of children/adolescents with salivary gland carcinomas prospectively registered in the Italian TREP (Rare Tumors in Pediatric Age) project.

Procedures: Diagnostic/therapeutic guidelines were developed and shared among Italian pediatric oncology/surgical centers.

Constitutional 11q14-q22 chromosome deletion syndrome in a child with neuroblastoma MYCN single copy.

Constitutional 11q deletion is a chromosome imbalance possibly found in MCA/MR patients analyzed for chromosomal anomalies. Its role in determining the phenotype depends on extension and position of deleted region. Loss of heterozygosity of 11q (region 11q23) is also associated with neuroblastoma, the most frequent extra cranial cancer in children.

Phase I study of temozolomide combined with oral etoposide in children with malignant glial tumors.

The treatment of children with malignant glioma remains challenging. The aim of this multicenter phase I study is to establish the recommended dose (RD) of the combination therapy with temozolomide (TMZ) and oral etoposide (VP-16) in children with relapsed or refractory malignant glioma and brainstem glioma at diagnosis. A phase I trial was conducted to establish the maximum tolerated dose (MTD) of TMZ and oral VP-16.

The micro-RNA 199b-5p regulatory circuit involves Hes1, CD15, and epigenetic modifications in medulloblastoma.

Micro-RNA (miR) 199b-5p targets Hes1 in medulloblastoma, one of the downstream effectors of both the canonical Notch and noncanonical Sonic Hedgehog pathways. In medulloblastoma patients, expression of miR-199b-5p is significantly decreased in metastatic cases, thus suggesting a downregulation mechanism. We studied this mechanism, which is mediated mostly by Hes1 and epigenetic promoter modifications.

Epithelial thymic tumours in paediatric age: a report from the TREP project.

Background: Thymic epithelial tumours (thymoma and carcinoma) are exceptionally rare in children. We describe a national multicentre series with a view to illustrating their clinical behaviour and the results of treatment.

Methods: From January 2000 all patients under 18 years of age diagnosed with "rare paediatric tumours" were centrally registered by the Italian centres participating in the TREP project (Tumori Rari in Età Pediatrica [Rare Tumours in Paediatric Age]).

Phase I study of temozolomide combined with oral etoposide in children with recurrent or progressive medulloblastoma.

Background: The prognosis of recurrent or progressive medulloblastoma (MB) is still poor. This study was designed to investigate the potential therapeutic benefit of combination therapy with temozolomide (TMZ) and oral etoposide (VP-16) in children with progressive or relapsed MB. Given the oral administration of both drugs the regimen was administered outpatient.

Second-line chemotherapy with the association of liposomal daunorubicin, carboplatin and etoposide in children with recurrent malignant brain tumors.

Progressive or recurrent high-grade gliomas are characterized by a very poor prognosis, and the relevance of second-line chemotherapy is still unassessed. Although it has been reported that liposomal anthracyclines and carboplatin show some activity in these patients, their association has never been investigated. We have treated six children with recurrent high-grade glioma after surgery, radiotherapy and chemotherapy, and one child with progressive teratoid/rhabdoid tumor with the combination of liposomal daunorubicin and carboplatin plus etoposide.

Efficacy of sequential chemotherapy including methotrexate and doxorubicin in an infant with partially resected choroid plexus carcinoma.

This report refers to a 3-month-old male, with a residual choroid plexus carcinoma following partial resection, who was successfully treated with sequential chemotherapy without any postoperative radiation therapy. Along with carboplatin, we also used doxorubicin and methotrexate, hypothesizing that, given the patient's age, the blood-brain barrier should not hamper drug delivery to the tumor. According to this hypothesis, the treatment achieved complete remission of the disease, which lasts 27 months after the diagnosis.

Shunt-related abdominal metastases in an infant with medulloblastoma: long-term remission by systemic chemotherapy and surgery.

This is the first reported case of long remission of abdominal metastases spread through a ventriculo-peritoneal shunt in an infant diagnosed, four years ago, at age 1 year and 10 months, to have cerebral medulloblastoma. Two years later, while in second complete remission of his cerebral tumor, he showed abdominal metastases, successfully treated by platinum based chemotherapy and surgery. One year later, a second abdominal relapse and hepatic metastases were treated by doxorubicin administration and surgery.

Radiation late effects in children treated for orbital rhabdomyosarcoma.

Background And Purpose: The experience resulting from large cooperative studies shows that correct radiation therapy at doses adequate to the tumor bulk are crucial for local control of rhabdomyosarcoma. The aim of the present study was to document the correlation between modalities and doses of radiotherapy and radiation side effects.

Patients And Methods: Between 1980 and 1997, 19 patients affected by primary orbital rhabdomyosarcoma have been followed at the University Federico II of Naples.

Treatment of EBV-induced lymphoproliferative disorder with epipodophyllotoxin VP16-213.

A case of haemophagocytic lymphohistiocytosis consistent with X-linked lymphoproliferative disorder is described. Remission was observed after administration of VP-213, a cytotoxic drug generally used to treat histiocytosis. The child is currently in good clinical health.

Glucose 6-phosphate dehydrogenase deficiency and red cell membrane defects: additive or synergistic interaction in producing chronic haemolytic anaemia.

We have investigated two unrelated patients with congenital haemolytic anaemia in both of whom we found a combination of hereditary spherocytosis (HS) and glucose-6-phosphate dehydrogenase (G6PD) deficiency. Segregation of the two defects was documented in both families, who had different molecular abnormalities for both HS and G6PD deficiency. In one family the propositus had a reduced level of spectrin and G6PD Seattle (282Asp-->His).

A novel chromosomal abnormality detected in a case of acute myelomonocytic leukemia.

A not yet recorded translocation, t(3:17)(q21;q23), detected in a case of acute myelomonocytic leukemia, is reported. In spite of an aggressive cytotoxic chemotherapy, the disease showed a rapid fatal course, confirming the dismal prognostic significance of structural, 'primary' chromosomal abnormalities in acute leukemias.

Familial erythrophagocytic lymphohistiocytosis: adverse prognostic significance of delayed diagnosis.

Familial erythrophagocytic lymphohistiocytosis (FEL) is a rare disorder of the monocyte-macrophage system, for which an autosomal recessive mode of inheritance has been postulated. It is characterized by a dismal prognosis and is peculiar of early infancy. Three new cases of infants affected by FEL are reported.

Multidisciplinary treatment of primary orbital rhabdomyosarcoma. A single-institution experience.

Orbital rhabdomyosarcoma accounts for one-fourth of the primary tumors in the head and neck region. Modern treatment modalities have led to a 2-year survival rate of about 90% in these patients. However, new therapeutic trials are designed to reduce complications and salvage more than 90% of orbital cases.

Primary cerebral neuroblastomas.

Two children with primary cerebral neuroblastomas are reported and the other cases from the literature are reviewed. Cerebral neuroblastomas are a distinct pathological entity, which differs from other neuroectodermal tumours, although the clinical features are aspecific. The review of the CT findings shows that neuroblastomas appear as large solid tumours in half of the cases and as cystic lesions in the other half; massive contrast enhancement, calcification and cystic hypodensity are typical CT features.

Non-Hodgkin's lymphoma in childhood presenting as thyroid enlargement.

The authors report a case of a 10-year-old girl with early involvement of the thyroid gland by non-Hodgkin's lymphoma, an uncommon site of presentation of childhood lymphomas. In pediatrics, thyroid enlargement is more often caused by lymphocytic thyroiditis. The good response to therapy, in spite of the advanced stage of the disease, is noted.