1/110. Neuroblastoma in two siblings supports the role of 1p36 deletion in tumor development.Familial neuroblastoma occurs rarely. We studied a family with three children; one of them has a disseminated (stage 4) and another has a localized (stage 2) neuroblastoma. We observed subtelomeric locus D1Z2 (1p36) deletion in both tumors by using double-color fluorescence in situ hybridization. The MYNC gene was found in single copy in both tumors. loss of heterozygosity (LOH) and restriction fragment length polymorphism analyses were performed by using dna from frozen tumor cells and from microdissected tumor areas excised from paraffin-embedded sections. We detected somatic LOH at locus D1S468 (1p36) in a tumor-cell population with a trisomy 1 of the stage-2 patient. Neuroblastoma cells of the stage-4 patient were diploid and showed allelic loss at the following loci: D1S172, D1S80, D1S94, D1S243, D1S468, D1S214, D1S241, and D1S164. Haplotype study showed that the siblings inherited the same paternal 1p36-->pter chromosome region by homologous recombination and that, in the two tumors, arm 1p of different chromosomes of maternal origin was damaged. Our results suggest that the siblings inherited the predisposition to neuroblastoma associated with paternal 1p36 region and that tumors developed as a consequence of somatic loss of the maternal 1p36 allele.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
2/110. Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement.Neuroblastic tumors comprise neuroblastoma (NB), ganglioneuroblastoma, and ganglioneuroma (GN). NBs have shown remarkable differentiation capabilities, which include spontaneous regression. In the last 3 decades, the prognosis for metastatic NB has improved significantly. The ability to distinguish prognostic subtypes based on clinical and biologic features allows for understanding of this disease process and development of management procedures. NBs are often asymptomatic and remain undetected until a large abdominal mass or metastasis is found. GN, the most differentiated variant, is a benign neoplasm with significant growth potential and ability to cause clinical complications. GN of the mandible has seldom been reported in the literature in English. We present a case of NB metastatic to the mandible exhibiting ganglioneuromatous differentiation. We discuss the current understanding of the biology, grading, classification, and prognostic implications of NB.- - - - - - - - - - ranking = 2keywords = neuroblastoma (Clic here for more details about this article) |
3/110. Injuries of large vessels in high stage neuroblastoma surgery. A case report.Complete resection of the primary lesion in stage III neuroblastoma improves survival Neuroblastoma has a tendency towards surrounding and infiltrating the large vessels, leading to injuries during tumor resection. We operated on a stage III neuroblastoma, which resulted in the right and left common iliac artery and vein damage. The right common iliac artery and, veins were repaired by end to end anastomosis. There was a long gap between the two ends of the left common iliac artery and it was repaired using a mesenteric vein (marginal vein of the colon) graft. Digital subtraction angiography performed 6 months after the operation did not reveal any stenosis or aneurysmatic changes in the anastomoses. We conclude that short segments of large vessels may be sacrificed during the resection of neuroblastomas invading the vessel wall, and the resulting defects may be repaired by end to end anastomosis, or even by substituting mesenteric vein grafts, for the purpose of total or near total removal- - - - - - - - - - ranking = 2.3333333333333keywords = neuroblastoma (Clic here for more details about this article) |
4/110. Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma.Neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.- - - - - - - - - - ranking = 2keywords = neuroblastoma (Clic here for more details about this article) |
5/110. Cystic neuroblastoma with hepatic metastasis: report of one case.Neuroblastoma is the most common infant malignancy, but cystic neuroblastoma is a rare presentation of this disease. There were only thirty-one reported cases in literature. Here we report another case. A 4-month-old female baby presented with a huge abdominal mass which was histologically proved to be a cystic neuroblastoma. Her urinary vanillylmandelic acid (VMA) was elevated. The image studies, including abdominal sonography, computed tomography, and magnetic resonance image, revealed a huge main mass with calcification and septa in the abdominal cavity, and multiple metastatic nodules with septation in the liver. A three-layer fluid-fluid level was found in the main mass and also in the metastatic nodules. No previous report showed a three-layer fluid-fluid level in the cystic component of neuroblastoma like the case presented here. It may be evidence of easy hemorrhagic character of this tumor.- - - - - - - - - - ranking = 2.3333333333333keywords = neuroblastoma (Clic here for more details about this article) |
6/110. Intolerance to an extensively hydrolysed formula mistaken for postoperative diarrhoea in a child with neuroblastoma.BACKGROUND: Persistent diarrhoea has recently been reported as a common problem following surgical resection for advanced abdominal neuroblastoma. CASE STUDY: A 10-month-old child, who had previously had no nutritional problems whilst undergoing chemotherapy treatment for a stage IV neuroblastoma, developed severe diarrhoea following tumour resection. He required nutritional support and was commenced on a continuous overnight nasogastric feed of a non-milk protein hydrolysate feed (MCT Pepdite 0-2, S.H.S. International Ltd, UK) His diarrhoea persisted and it was assumed that this was a postoperative problem which in time would regress. Two months later the child was admitted with a rectal prolapse, and a colonoscopy and biopsy suggested allergic protocolitis. The diarrhoea resolved rapidly on stopping the feed. CONCLUSION: Although he was never re-challenged with the non-milk protein hydrolysate, it appears that he had a rare intolerance to an extensively hydrolysed protein formula which was masked by the assumption that his diarrhoea was a postoperative problem.- - - - - - - - - - ranking = 2keywords = neuroblastoma (Clic here for more details about this article) |
7/110. Rearrangement in the coding region of the MYCN gene in a subset of amplicons in a case of neuroblastoma with MYCN amplification.The MYCN gene is often amplified but rarely rearranged in neuroblastoma. We report, for the first time, a rearrangement within the MYCN coding region in a metastatic neuroblastoma in a 3-year-old boy with MYCN amplification in his primary tumor. The rearrangement occurred 46 nucleotides downstream from the ATG codon in exon 2 of MYCN. The amplification level of the rearranged copies of the MYCN gene was lower than that of the unrearranged copies of MYCN. These results indicate that the rearrangement occurred after initial MYCN gene amplification. Monochromosomal somatic cell hybrid mapping of the novel region fused to exon 2 of MYCN localized it to chromosome 2, suggesting that this rearrangement resulted from an interstitial deletion, presumably within the MYCN amplicon itself.- - - - - - - - - - ranking = 2keywords = neuroblastoma (Clic here for more details about this article) |
8/110. Can the growth of a neuroblastoma be influenced by a child's nutritional state? Observations in a patient treated for kwashiorkor and later given a restricted diet.A 17-month-old Thai female with neuroblastoma presented with an abdominal mass and the classical findings of kwashiorkor. Concomitant with effective repair of the child's protein deficit, the mass enlarged dramatically and metastases were noted. This is the first known report of such an occurrence.- - - - - - - - - - ranking = 1.6666666666667keywords = neuroblastoma (Clic here for more details about this article) |
9/110. Neuroblastoma with concomitant giardiasis: report of a case with diagnosis by fine needle aspiration cytology.BACKGROUND: diagnosis of two pathologies, including a neoplasm and infectious condition, by fine needle aspiration (FNA) cytology in the same patient is rare. CASE: A 2-year-old, male child presented with fever, abdominal pain and abdominal mass. Imaging findings were strongly in favor of a neuroblastoma. FNA smears from the mass revealed fecal material containing numerous trophozoites of giardia lamblia. FNA was repeated in view of the imaging findings. Repeat smears showed a small round cell tumor with rosettes and background filamentous/fibrillar material consistent with a neuroblastoma. Chemotherapy reduced the mass considerably. Histopathology of the resected residual mass revealed a ganglioneuroma in addition to remnants of neuroblastoma. The patient was free of disease two years after the initiation of chemotherapy. CONCLUSION: When FNA cytology shows an infectious pathology in the clinical and imaging setting of a tumor, FNA should be repeated so that an important component of the diagnosis is not missed.- - - - - - - - - - ranking = 1keywords = neuroblastoma (Clic here for more details about this article) |
10/110. Successful treatment of pseudomonas aeruginosa respiratory tract infection with a sugar solution--a case report on a lectin based therapeutic principle.BACKGROUND: Airway infections with pseudomonas aeruginosa often represent a life-threatening event in immuno-compromised patients or patients with cystic fibrosis. The adhesion of this bacterium to surfaces such as the airway epithelium is mediated by two lectins, sugar binding proteins. In addition to their adhesive properties, these lectins have been shown to stop human ciliary beating thus compromising the mucociliary clearance as an important non-specific defence mechanism of the airways. Inhibition of these lectins by their specific sugars galactose and fucose, respectively, could therefore be of benefit in the elimination therapy of P. aeruginosa. CASE REPORT: An infant suffering from P. aeruginosa airway infection after chemotherapy for neuroblastoma, which could not successfully be treated by antibiotics, was subjected to a series of additional galactose/fucose inhalations, which eliminated the germ as evidenced by microbiological testing. This is the first report suggesting the effectiveness of a lectin-based therapeutic principle in P. aeruginosa airway infection. CONCLUSION: The competitive inhibition of P. aeruginosa lectins by the lectin specific sugars galactose and fucose may overcome particular mechanisms of bacterial resistance in patients with P. aeruginosa airway infection. This underlying biochemical mechanism and the outcome of our patient suggest a clinical benefit of this novel therapeutic approach for immunocompromised patients or patients with cystic fibrosis suffering from infection with P. aeruginosa.- - - - - - - - - - ranking = 0.33333333333333keywords = neuroblastoma (Clic here for more details about this article) |
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