1/207. central nervous system relapse following bone marrow transplantation in stage IV neuroblastoma.neuroblastoma is the most common extracranial solid tumor in pediatrics. The disease-free survival rate for patients with stage IV neuroblastoma has improved over the past 10 years secondary to more aggressive induction chemotherapy regimens combined with autologous bone marrow transplantation. The usual sites of recurrence include the site of primary tumor, residual gross disease, bone, and bone narrow. The central nervous system, a rare site of relapse, is being involved with increasing frequency. The authors report two cases of patients with treated stage IV neuroblastoma who had relapses isolated to the CNS.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/207. Artificial abdominal hernia for the treatment of hepatomegaly in a neonate with stage 4S neuroblastoma.Surgical widening of the abdomen by a silastic pouch has been used very rarely in the management of critically ill infants with hepatomegaly due to neuroblastoma stage 4S. A female newborn baby was referred on the second day of life because of local compressive effects of a massive hepatomegaly, which lead to multiorgan failure. An artificial abdominal hernia was created on the third day of life using a silastic pouch. During the operation oxygenation and ventilation improved and urinary output returned. After chemotherapeutic reduction of hepatic metastases and primary tumor the pouch was successfully removed on day 57 without local complications. The child has survived for more than 1 year and is in complete remission. An artificial abdominal hernia should be considered more often in the critically ill neonate with stage 4S neuroblastoma and massive hepatomegaly.- - - - - - - - - - ranking = 0.00046525825303709keywords = lead (Clic here for more details about this article) |
3/207. Metastatic ovarian neuroblastoma: a case report.Ovarian metastasis of childhood tumors is rare despite their aggressive nature. The childhood tumor that spreads to the ovary most frequently is the neuroblastoma. The clinical features and frequent bilaterality of ovarian metastatic tumors are helpful diagnostic features in many cases, but when the ovarian tumor is the presenting manifestation of the disease, differentiation from various primary ovarian tumors may be difficult. In this paper, a rare case of metastatic ovarian neuroblastoma is reported.- - - - - - - - - - ranking = 1.0397788439377keywords = childhood (Clic here for more details about this article) |
4/207. Acute monoblastic leukemia in a child following chemotherapy for neuroblastoma.The long-term effects of childhood cancer and its therapy are serious problems that deserve attention. One of the most important late effects is the development of secondary malignancy. We encountered a girl with neuroblastoma who developed acute monoblastic leukemia as a secondary malignancy, 32 months after starting treatment for the primary tumor at the age of 4 years and 10 months. For the primary tumor, she had received cyclophosphamide, ifosphamide, etoposide, epirubicin, cisplatin, and vincristine during a period of 20 months; no radiotherapy was given. cytogenetic analysis of the leukemic cells showed no specific changes, but a rearrangement of the mixed lineage leukemia gene (chromosome 11q23 translocation) was subsequently found by reverse transcription polymerase chain reaction. The survival time after onset of the secondary malignancy was brief. The leukemogenic hazards of cancer treatment should be weighed against their therapeutic benefits.- - - - - - - - - - ranking = 0.51988942196885keywords = childhood (Clic here for more details about this article) |
5/207. 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 = 0.00046525825303709keywords = lead (Clic here for more details about this article) |
6/207. Full cytogenetic characterization of a new neuroblastoma cell line with a complex 17q translocation.Recent studies have shown that structural abnormalities of chromosome 17 resulting in gain of material are the most frequent genetic changes in neuroblastoma. We have established a new neuroblastoma cell line from a patient whose disease had evolved from stage 4s to 4, without evidence of deletion of the short arm of chromosome 1 and MYCN amplification, which are considered the most typical genetic indicators of aggressive disease. The cytogenetic study allowed a full characterization of the chromosome changes, and revealed a complex translocation of chromosome 17 leading to a derivative marker which may be described as follows: der(11)t(11;17)(p15;q12)t(11;17) (q22;q12). This resulted in a gain of part of the long arms of chromosome 17, which was recently associated with poor prognosis.- - - - - - - - - - ranking = 0.00046525825303709keywords = lead (Clic here for more details about this article) |
7/207. neuroblastoma associated with seizures and arrested development.Two unrelated cases of childhood peripheral neuroblastoma associated with infantile seizures and developmental problems (but without opsoclonus-myoclonus) are presented. The considerable body of evidence supporting the view that the opsoclonus-myoclonus syndrome associated with neuroblastoma has an immunologic basis is reviewed. patients with neuroblastoma and opsoclonus-myoclonus syndrome commonly have subsequent developmental problems and, rarely, may have seizures. The authors postulate that the seizures and developmental problems in their two patients may result from an immunologic mechanism similar to that suggested for the opsoclonus-myoclonus syndrome of neuroblastoma. The only laboratory evidence to support an immunologic mechanism in these two patients was the presence of raised cerebrospinal fluid immunoglobulins in Patient 2. Specific antineuronal antibody tests in Patient 2 were negative. It is therefore possible that the association reported in these two unrelated cases is coincidental. However, reasonably extensive investigations did not uncover an alternative etiology for the presence of the seizures and developmental delay.- - - - - - - - - - ranking = 0.51988942196885keywords = childhood (Clic here for more details about this article) |
8/207. incidence of occult cancer in children presenting with musculoskeletal symptoms: a 10-year survey in a pediatric rheumatology unit.OBJECTIVES: To assess the frequency and types of cancer found in children presenting to our Unit with musculoskeletal symptoms over a 10-year period. methods: The medical records of patients with musculoskeletal symptoms and a final diagnosis of cancer were reviewed. In each case age, gender, presenting symptoms, laboratory data, diagnostic procedures, provisional and final diagnoses, and time between clinical onset and correct diagnosis were reviewed. RESULTS: An underlying neoplasia was found in 10 of 1,254 patients (<1%) complaining of musculoskeletal symptoms. The types of malignancies found included acute lymphocytic leukemia (ALL) (6 cases), lymphoma (2 cases), neuroblastoma (1 case), and Ewing's sarcoma (1 case). The mean time between disease onset and final diagnosis was 3.2 months. The most common presenting feature was monoarthritis, involving the larger joints such as the elbows, knees or ankles. Juvenile idiopathic arthritis (JIA) was the most frequent provisional diagnosis. In the preliminary hematologic evaluation, eight patients had an increased erythrocyte sedimentation rate (ESR) or c-reactive protein (CRP) value. White blood cell (WBC) count was normal in almost all children, with a normal differential count. Lactic dehydrogenase (LDH) was raised in all children. Bone marrow aspirates and lymph node or bone biopsies were necessary to reach the final diagnosis. CONCLUSIONS: A malignancy should always be excluded in children with musculoskeletal symptoms, especially when the clinical pattern is not characteristic of a specific rheumatic disease. Routine laboratory tests may be misleading. The simultaneous presence of high LDH or alpha-hydroxybutyric dehydrogenase (alpha-HBDH) levels and raised ESR or CRP, even with normal blood cell counts, should lead to additional investigations. RELEVANCE: All patients presenting with arthritis or other musculoskeletal symptoms should have a thorough clinical examination. Disproportionate pain levels and an atypical pattern of "arthritis," especially in the presence of systemic manifestations, suggest a possible underlying malignancy.- - - - - - - - - - ranking = 0.00093051650607417keywords = lead (Clic here for more details about this article) |
9/207. The homeobox gene MEIS1 is amplified in IMR-32 and highly expressed in other neuroblastoma cell lines.neuroblastoma is a childhood tumour of the sympathetic nervous system that demonstrates striking clinical heterogeneity. In order to determine which genes are abnormally expressed in neuroblastoma, we screened regions of amplification from the short arm of chromosome 2 in the neuroblastoma cell line IMR-32 and found that the homeobox gene, myeloid ecotropic integration site 1 (MEIS1), is highly amplified. MEIS1 normally maps to chromosome band 2p14. High expression of MEIS1 without amplification was also found in other neuroblastoma cell lines, with and without MYCN amplification, and in medulloblastoma and crythroleukaemia cell lines. MEIS1 is highly expressed in cerebellum and ubiquitously expressed in normal immunohaematopoietic tissues and is thought to be important in cell proliferation and differentiation. While several lines of evidence point towards a role for homeobox genes in the development of other malignancies, this is the first report showing the amplification of a homeobox gene in neuroblastoma.- - - - - - - - - - ranking = 0.71988942196885keywords = childhood, nervous system (Clic here for more details about this article) |
10/207. Simultaneous occurrence of advanced neuroblastoma and acute myeloid leukemia.The authors report the case of a 4-year-old boy with a diagnosis of stage IV neuroblastoma (NB), who had been treated with 6 cycles of cyclophosphamide, doxorubicin, cisplatin, and etoposide for 12 months. The patient reached partial remission and presented a diagnosis of acute myelomonocytic leukemia (M4 AML), confirmed by immunophenotyping. After 2 months of therapy for leukemia, the child died with both malignancies in activity. A necropsy histologically confirmed the simultaneity of the two diseases. The authors review the possibilities of this association. The review leads to the conclusion that AML can occur as a secondary malignancy after the onset of the neuroblastoma, or be suggested by a misdiagnosis. The simultaneous occurrence of both as described here is not, however, found in the literature, to the best of the authors' knowledge.- - - - - - - - - - ranking = 0.00046525825303709keywords = lead (Clic here for more details about this article) |
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