Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

N-myc oncogene is amplified in several human neuroblastoma cell lines and fresh neoplastic tissues; amplification would be highly correlated with advanced stages of neoplasia and associated with poor outcome of the disease. According to these data, N-myc amplification should be a new prognostic marker in addition to classical clinical and laboratory findings commonly monitored in neuroblastoma; some of them are levels of urinary Vanillylmandelic Acid (VMA) and Homovanillic Acid (HVA), serum Lactic Dehydrogenase (LDH) and serum Ferritin (sFe). We studied 4 patients in stage III and IV; N-myc amplification was detected in 2 cases in stage IV in primary tumor cells and in one case this finding was associated with high levels of serum Ferritin and LDH, while no correlation between other laboratory parameters and N-myc amplification has been found. We propose that N-myc amplification has to be evaluated with other prognostic factors to obtain useful information about the disease and the survival of the patient.
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PMID:[Amplification of the N-myc cellular oncogene in neuroblastoma. Relation to other tumor markers]. 379 Feb 77

Fourteen children with histopathologically confirmed neuroblastoma underwent 38 studies using 99mTc-methylene-diphosphonate (MDP) and galliumcitrate Ga67 whole-body scintigraphy during various stages of the disease. Ten patients (71%) showed 99mTc-MDP accumulation in the primary tumoral site, whereas 11 patients (78.6%) showed 67Ga concentration. In 12 patients (86%), at least one of these two radiopharmaceuticals concentrated in the primary tumor. Nine patients had osseous or extraosseous metastases. All of these metastases (100%) were positive on 99mTc-MDP scintigraphy. No 67Ga-citrate uptake was demonstrable in osseous metastases; only one extraosseous lung metastasis concentrated this radiopharmaceutical. 67Ga-citrate was superior to 99mTc-MDP with regard to accurately demonstrating the extent of primary tumors. Only 99mTc-MDP indicated the relationship of the tumor to the kidneys and neighbouring osseous structures, providing early screening of kidney compression and possible damage caused by the tumor. From these results, we found these two methods to be complementary for the diagnosis and follow-up of neuroblastoma; their combined use resulted in high diagnostic accuracy and a considerable gain of information. We therefore recommend sequential 99mTc-MDP and 67Ga-citrate scans for the diagnosis and evaluation of the primary tumor; periodic 99mTc-MDP whole-body scans should be used in the follow-up of treatment, and for discovering disease exacerbations and metastases.
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PMID:The complementary role of sequential 99mTc-MDP and 67Ga-citrate scanning in the diagnosis and follow-up of neuroblastoma. 386 71

The proportion of malignancies in children differs from that in adults: Leukemias and malignant lymphomas predominate with a total of 50%, followed by tumors of the nervous system, of the kidneys, and of connective and supportive tissue. Most of these diseases respond well to cytostatic therapy. Therefore chemotherapy occupies a major role in the curative concepts for nearly all childhood malignancies. Its objective is the destruction of micrometastases as well as the reduction of primary tumor mass in inoperable cases, and it often helps to limit the extent of radical surgery. Radiotherapy, too, can be reduced under the influence of cytostatic therapy. In nearly all childhood cancers, prognosis has improved substantially over the past 10 to 15 years. Today, our aim is not the mere limited survival, but a definitive cure. Modern strategies have raised the cure rates of Hodgkin's disease to 90%, of Wilms' tumor, acute lymphoblastic leukemia and non- Hodgkin lymphomas to 70-75%, of soft tissue sarcomas and osteosarcomas to about 50%, and of acute myelogenous leukemia, neuroblastoma and medulloblastoma to 30-35%. Centralized management of childhood cancers in specially staffed hospitals is mandatory on account of their relative low frequency, the risks of chemotherapy, and the high staff workload.
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PMID:What's new in pediatric oncology? Epidemiology, treatment principles and prognosis in childhood malignancies. 388 97

Neuroblastoma in adults is uncommon. Previous reports have suggested that adult patients with neuroblastoma have a better prognosis than children with these tumors. We have examined the clinical features of eight adults with neuroblastoma and related these data to tumor histopathology and immunohistochemistry using an antibody to neuron-specific enolase. The results show that when children and adults with neuroblastoma are compared by stage, adults do not have a better prognosis. Adults tend to have a different anatomic distribution of primary tumor sites, with more frequent extra-abdominal sites than are seen in children. Neuroblastomas arising in adults are similar to those seen in children by containing neuron-specific enolase, an enzyme associated with cells of neuroectodermal origin. These findings show that adult neuroblastomas are similar to their childhood counterparts in clinical behavior and pathologic features.
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PMID:Neuroblastoma in adults. Pathologic findings and clinical outcome. 388 99

Neuroblastoma originates in the adrenal medulla or anywhere in the body that sympathetic tissue normally is present. It may present with a variety of symptoms due to primary tumor, metastatic disease, or unusual signs and symptoms such as opsoclonus-myoclonus or severe diarrhea. Despite the fact that this neoplasm responds to a variety of therapeutic modalities, it remains one of the most frustrating and difficult childhood tumors to treat and cure.
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PMID:Neuroblastoma. 388 4

A 14-year-old boy was presented with a mass in the epigastrium. The tumor developed 13 years after removal of a neuroblastoma of the left adrenal gland. Therapy of the primary tumor had included irradiation and chemotherapy. Diagnosis of the gastric tumor was made by ultrasound and endoscopy. A total gastrectomy was performed showing a malignant undifferentiated sarcoma. Primary malignant tumors of the stomach such as gastric teratoma of the newborn [4], leiomyosarcoma [6], malignant lymphoma and carcinoma [1] are very rare in childhood. As cancer is treated more aggressively with radiation and cytostatics, more therapy-induced-tumors, including gastric sarcoma, must be expected in children.
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PMID:Malignant gastric sarcoma--diagnosis by ultrasound and endoscopy. 394 5

Meta-iodine-benzylguanidine (MIBG) scanning provides, for the first time, specific radiological means for diagnosis, treatment follow-up and post-treatment care of patients with neuroblastoma. Of 10 such patients studied by MIBG scanning, 7 had histologically confirmed neuroblastoma. In 6 of them there was markedly increased activity in the primary tumor, in 3 metastases were demonstrated. In at least one patient the intensity of increased activity suggested the possibility of selective therapeutic administration.
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PMID:[Diagnosis and therapy control of neuroblastoma using meta-iodo- benzylguanidine]. 396 3

To assess the resectability of retroperitoneal neuroblastoma and determine the timing of a "second-look" resection of primary tumor on the basis of aortographic findings, 32 abdominal aortographies performed on 26 patients with retroperitoneal neuroblastoma (22 adrenal, 4 paraspinal) were examined retrospectively. Angiographic findings of both displacement of aorta and narrowing of aorta indicate the difficulty of complete removal of the neuroblastoma. These findings were particularly useful in determining the resectability of retroperitoneal neuroblastoma. On the other hand, the absence of angiographic findings of both displacement of main vessels (celiac axis, origin of superior mesenteric artery, or renal arteries) and stretching of main vessels indicate the feasibility of complete removal of retroperitoneal neuroblastoma. On the basis of angiographic findings, the decision to resect the retroperitoneal neuroblastoma could be made in 6 patients, who underwent the "second-look" operation.
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PMID:Evaluation of aortography in assessing the resectability of retroperitoneal neuroblastoma in children. 405

This report documents, in seven infants younger than 2 years of age, a previously unrecognized association of a renal embryonal neoplasm (malignant rhabdoid tumor in six patients and a Wilms' tumor in one) with an embryonal primary tumor originating in the central nervous system. The neuroepithelial tumors included three cerebellar medulloblastomas, one pineoblastoma, one primitive neuroepithelial tumor (probably cerebral neuroblastoma), one malignant subependymal giant cell astrocytoma, and one cerebellar medulloepithelioma with divergent glial and neuronal differentiation. There is no evidence that this association is based on the selective neoplastic transformation of embryonal cells of similar histogenetic or cytogenetic origin. The relationship between these dissimilar, embryologically unrelated tumors remains enigmatic.
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PMID:The association of embryonal tumors originating in the kidney and in the brain. A report of seven cases. 609 60

Forty-two infants and children with retroperitoneal solid tumors underwent second operative procedures. Twenty-three children had stage III (4) or stage IV (19) neuroblastoma and 19 had Wilms' tumor. This report suggests that second-look procedures are quite beneficial in selected cases. In children with Wilms' tumor, second procedures are most useful in those patients inadequately staged with flank operations, in cases of bilateral tumors, and in children with initially unresectable tumors following cytoreduction. Patients with significant tumor spill at a previous procedure may also benefit from early reoperation. Late recurrence of tumor, especially in children with unfavorable histology and/or hematogenous liver metastases, carries an ominous prognosis. Ten of the 19 patients with Wilms' tumor are long-term survivors (52.6%). Patients with initially unresectable (stage III) neuroblastoma occurring in the pelvis respond well to second-look resection. Children with primary tumors surrounding the celiac axis eventually died despite subsequent resection. Although the efficacy of primary tumor resection in cases of metastatic neuroblastoma remains controversial, stage IV patients with tumor resection had a longer duration of survival. Our only long-term survivors had their primary tumors resected. Second-look or delayed abdominal operations in metastatic cases may be useful in assessing the effectiveness of treatment and for sampling of retroperitoneal lymph nodes as a prognostic indicator.
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PMID:The role of second-look procedures in the management of retroperitoneal tumors in children. 609 86


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