[Non-conventional therapies in childhood cancer: guidelines for distinguishing non-harmful from harmful therapies: a report of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology]. / Unkonventionelle Therapien bei Krebserkrankungen im Kindesalter: Richtlinien zur Abgrenzung unbedenklicher von schädlichen Behandlungsmethoden: Ein Bericht der SIOP-Arbeitsgruppe für Psychosoziale Fragen in der Pädiatrischen Onkologie.

This is the 11th official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, instituted in 1991. There is a tendency for some physicians to make blanket statements against the use of non-proven, nonconventional therapies, even when these therapies are not harmful. There is an equal and opposite tendency on the part of many parents to do all that they possibly can for their children, including using any non-conventional therapy they feel might do some good. The health care team must open a healthy dialogue with parents that will lead to a clear distinction between those complementary therapies that are harmful and those that are not, indeed, might even be helpful psychologically if not therapeutically.

P73 expression in neuroblastoma: a role in the biology of advanced tumors?

p73, a recently identified gene showing high homology to p53 and mapping to 1p36.33, was presented as a candidate gene for neuroblastoma. In this study the authors evaluate the levels and allelic nature of p73 expression in primary neuroblastomas using reverse transcription-polymerase chain reaction-restriction fragment length polymorphism strategies based on intragenic polymorphisms. From 32 neuroblastoma patients, 11 were heterozygous for the p73 polymorphisms analyzed. p73 expression was found to be low in the correspondent tumors and while all 6 stages 1 and 2 tumors presented biallelic expression, 4 out of the 5 stage 4 tumors showed only one active p73 allele. Analysis of blood samples from 8 healthy donors and 4 neuroblastoma patients revealed much higher levels of p73 expression, and exclusively of biallelic nature. These results are supportive of a role for p73 in the biology of neuroblastoma, particularly in some advanced tumors. Nevertheless, the G81A/C91T polymorphism, previously implicated in regulating the expression of p73, did not show any significant association with neuroblastoma development.

Guidelines for assistance to siblings of children with cancer: report of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology.

This is the seventh official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, instituted in 1991. This document develops an additional topic discussed and approved by the SIOP Committee, "Assistance to siblings of children with cancer. " It is addressed to the pediatric oncology community and outlines general principles for helping siblings throughout phases of treatment: at diagnosis, during treatment, in the event of relapse, during bone marrow transplantation, after completion of therapy, during palliative care and the terminal phase.

Spinal cord vascular injuries following surgery of advanced thoracic neuroblastoma: an unusual catastrophic complication.

BACKGROUND: Spinal cord injury is a possible complication associated with removal of thoracic dumbbell neuroblastomas. Our experience with two children whose postsurgical course was complicated by midthoracic spinal cord ischemia is reported there. Permanent paraplegia resulted in both. PROCEDURE AND RESULTS: Preoperative awareness of the origin and distribution of the Adamkiewicz artery (arteria radiculomedullaris magna, ARMM) and of the possible collateral pathways for spinal cord blood supply may be helpful in the planning of operations that involve dissection in the midthoracic posterior mediastinum. Otherwise, a flaccid paraplegia may result. CONCLUSIONS: The syndrome is presumed to be triggered by a spasm, an embolism, or a iatrogenic interruption of the ARMM.

Guidelines for assistance to terminally ill children with cancer: a report of the SIOP Working Committee on psychosocial issues in pediatric oncology.

This, the sixth official document of the SIOP Working Committee on psychosocial issues in pediatric oncology, develops another important and especially difficult topic: assistance for terminally ill children with cancer. This is provided for the pediatric oncology community as a useful set of guidelines. It should be always possible for a declining child to die without unnecessary physical pain, fear, or anxiety. It is essential that he or she receive adequate medical, spiritual, and psychological support, and that the child at no point feels abandoned. Palliative care, in the terminal phase of cancer, should be tailored to the different needs and desires of the child and the family, with the goal of providing the best possible quality of life for the days that remain.

Guidelines for a therapeutic alliance between families and staff: a report of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology.

This, the fifth official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, develops another important topic: the Therapeutic Alliance between families and staff. This is addressed to the Pediatric Oncology Community as Guidelines that could be followed. Every parent, medical staff member, and psychosocial professional involved in the care of the child should be responsible for cooperating in the child's best interest. Everyone must work together toward the common goal of curing the cancer and minimizing its medical and psychosocial side-effects.

Loss of heterozygosity at chromosome 9p21 in primary neuroblastomas: evidence for two deleted regions.

The genes responsible for the development of neuroblastoma following in vivo deletion or mutation are largely unknown. We have performed loss of heterozygosity studies on a series of 24 Portuguese primary neuroblastomas using 6 polymorphic markers located at chromosome 9p21 spanning the p16/MTS1/CDKN2, p15/MTS2/CDKN2B, and the interferon alpha and beta genes. Loss of heterozygosity was observed in 4 of the 24 tumors (17%), a somewhat lower percentage than a previous study that identified patients by a mass screening program. A correlation was also observed between 9p21 LOH and 1p36 LOH in our group of tumors. Two distinct regions of 9p21 deletion were observed: one located in the region adjacent to the markers D9S162 and D9S1747 and a second located centromerically of the p16 gene near the D9S171 marker. The latter region is exclusive of the p16 gene. This result suggests the presence of at least one other tumor suppressor gene at 9p21, apart from the p16 and p15 genes, which may be of importance to the development of neuroblastoma.

Epithelial salivary gland tumors of children and adolescents in southern Portugal. A clinicopathologic study of twenty-four cases.

During a 30-year period 24 epithelial salivary gland tumors were diagnosed in children and adolescents less than 18 years of age. The cases were retrieved from a series of 759 consecutive cases of salivary gland tumors (3.2%) from the area corresponding to southern Portugal during the same period of time. The mean age of the patients was 13.4 years, and one case was congenital. There was a slight female predominance (male/female ratio 1:1.7). The parotid gland was affected in most cases (70.8%). Seventeen neoplasms were benign, and the remaining seven were malignant. As in the adult group, pleomorphic adenoma was the most frequent benign tumor (66.6%), with similar histologic findings and clinical course. Mucoepidermoid carcinoma was the prevalent malignant tumor (20.8%), had a high grade of differentiation, and had a favorable outcome. The histologic pattern of the congenital neoplasm was similar to that of adult epithelial-myoepithelial carcinoma.

Ewing's sarcoma in children: twenty-five years of experience at the Instituto Portugês de Oncologia de Francisco Gentil (I.P.O.F.G.).

Fifty children with Ewing's sarcoma were consecutively treated from 1962 to 1987 and retrospectively analyzed at the I.P.O.F.G. of Lisbon. At first diagnosis, 10 cases had distant metastases. The remaining 40 patients had clinically localized disease, and different protocols were followed over the years. The best results were obtained with chemotherapy and radiotherapy with or without surgery; and for these children the two-year survival rate was 42.8% vs. 8.3% for the group of patients submitted to local treatment alone. Besides the treatment modality, other factors influenced the prognosis, such as inflammatory signs, sex, tumor volume, and tumor site as well as evidence of distant metastases.

[Application of aspiration cytology in the diagnosis of tumor lesions in children]. / Aplicações da citologia aspirativa no diagnostico das lesões tumorais das crianças.

Fine needle aspiration cytology (FNAC) became increasingly popular for evaluating of both superficial and deep seated lesions, but its use in children has been limited. We report our recent experience with FNAC in the pediatric group. The study population included 34 males and 12 females and the age ranged between 11 months to 16 years. The aspirated sites were: testis (n = 14), lymph node (n = 12), bone (n = 9), pelvis (n = 2), retroperitoneum (n = 1), soft tissue (n = 6), lung (n = 1) and orbit (n = 1). The aims of FNAC were to diagnose a primary lesion (25 cases) and to document relapse of neoplastic disease (21 cases). Of the 46 aspirates, 28 were diagnosed as malignant, 4 as benign, 2 were inconclusive; in the remaining case the material was considered inadequate. Eleven out of 14 testicular cases were normal. FNAC diagnosis was confirmed either by histology (25 cases) or by the clinical evolution (18 cases). We conclude that FNAC can be considered a major diagnostic tool for the characterization of the tumoral lesions in childhood, both for diagnostic purposes and for evaluation of the progression of neoplasia. It may also provide useful information for guiding the therapeutical strategy.

Submandibular endodermal sinus (yolk sac) tumor in a female infant. A case report.

A submandibular endodermal sinus (yolk sac) tumor (EST-YST) is reported. The patient was a 1.5-year-old girl exhibiting a rapidly growing tumor in the submandibular region. The lesion showed the typical microscopic features including Schiller-Duval bodies and colloid bodies. Alpha-fetoprotein immunoreactivity was expressed by most tumor cells. There was no clinical or radiological evidence of the presence of this tumor elsewhere in the body.