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)

A case of abdominal Burkitt's lymphoma diagnosed through aspiration cytology is described. This 9-year-old boy presented with abdominal pain and distention for three months accompanied by fever and night sweat during the last month. An abdominal sonography and CT scan showed hepatosplenomegaly and an intrahepatic mass with celiac lymph node enlargement, ascites, and pleural fluid. A peripheral blood smear showed a few blast cells. Aspiration of the abdominal mass revealed very cellular aspirates consisting of diffusely scattered small monotonous round cells. The cells had little cytoplasm, along with round nuclei that showed clear-cut nuclear membrane, coarse chromatin pattern, and multiple small prominent nucleoli. Differential diagnoses considered were small round cell sarcomas such as malignant lymphoma, neuroblastoma, Ewing's sarcoma, and rhabdomyosarcoma. Of these, malignant lymphoma of the small noncleaved cell type was most consistent with the results of several studies including immunohistochemical staining, peripheral blood smear, and bone marrow biopsy. The cells were positive for leukocyte common antigen (LCA) and showed finely vacuolated basophilic cytoplasm in both the peripheral blood smear and bone marrow biopsy, characteristic of Burkitt's lymphoma cells.
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PMID:Abdominal Burkitt's lymphoma diagnosed by fine needle aspiration cytology--a case report. 227 68

Neuroblastoma is well recognized as a cause of soft tissue uptake of Tc-99m MDP. Two cases of neuroblastoma arising in the midline from the celiac axis are reported. Bone imaging performed on two separate days showed not only typical soft tissue uptake, but also the appearance of the radiopharmaceutical in the bowel. At surgery, a midline upper abdominal neuroblastoma was found in both patients without evidence of involvement of the liver, kidneys, bowel, gallbladder or mesentery. It became apparent with delayed images in the second patient that this activity was in the bowel and moving around the abdomen in a typical large bowel pattern. Bowel activity was not seen in other patients having bone scans at this time. Follow-up bone imaging on the first patient after resection of the tumor did not demonstrate diphosphonate activity in the bowel. These authors have never seen or read of this finding previously in this condition, and report it in these two patients.
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PMID:Diphosphonate intestinal activity seen on two bone images in neuroblastoma. 373 51

Neuroblastoma, a malignant tumor of neural crest origin, is the most common extracranial solid tumor in children. In 1971 Evans et al. introduced a clinical staging for neuroblastoma. Over sixty percent of patients present with neuroblastoma beyond stage I. Despite more aggressive therapy there has been only minimal improvement in survival. Since 1978, all patients with neuroblastoma have had CT scanning as part of their initial evaluation at our institution. Children with abdominal neuroblastoma beyond stage I form the basis of this report. Selected cases illustrating the permeative nature of neuroblastoma and the mechanism of direct abdominal spread by CT scanning are presented. The tumor originates in the retroperitoneum and spreads to the abdominal aorta where it gains access to the subperitoneal space via the celiac axis and superior mesenteric artery. These vessels course from the aorta to their ultimate destination within their peritoneal folds. These folds form the interconnecting space (subperitoneal space) between the retroperitoneum and the peritoneal organs. Such scanning is extremely sensitive in detecting neuroblastoma with early infiltration into adjacent tissues and contiguous spread through abdominal spaces. The clinical implications of the permeative nature of neuroblastoma and the mechanism of contiguous abdominal spread are discussed.
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PMID:Mechanism of direct spread of abdominal neuroblastoma: CT demonstration and clinical implications. 379 60

This multicenter study was designed to determine if CT can assess operability in stage III neuroblastoma. Nineteen children (11 boys, eight girls), aged 2-51 months, considered to have, by conventional clinical and radiographic examinations, localized neuroblastoma that crossed the midline were examined by CT. After intravenous and oral contrast media enhancements, CT was able to show the relation of the tumor to the adjacent vital vessels: aorta, celiac axis, and superior mesenteric artery. In all 15 patients, tumors that encased the aorta or its major branches were unresectable. However, three of four tumors crossing the midline but not coming into contact with the aorta or adherent only to one side of the vessel could be resected. Because surgery currently plays the major role in the treatment of neuroblastoma, the relation of the tumor to the aorta and great vessels is a more reliable and important factor in predicting the outcome of these children than the extension and location of the tumor with reference to the midline. CT after intravenous contrast enhancement can establish this relation and assess resectability.
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PMID:Computed tomography in stage III neuroblastoma. 387 39

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

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

The current role of surgery was evaluated in seven consecutive patients with high-risk neuroblastoma (six stage 4 patients and one stage 3, abdominal origin, and all over 12 months of age at diagnosis) treated with new modalities utilizing bone marrow transplantation (BMT). In six of these seven patients, a grossly complete excision of the primary tumor was achieved, and four have survived for 133, 69, 39, and 28 months with no further evidence of disease. The remaining patient with celiac neuroblastoma only underwent a biopsy during a second-look laparotomy after chemoradiotherapy, and thereafter developed local and distant recurrences and ultimately succumbed to the tumor. The timing of surgical intervention varied, either before or after chemotherapy, and did not appear to affect the ultimate survival. Although surgical excision of the primary tumor remains a very high priority in the overall treatment scheme, the most important factor remains the eradication of the tumor by well-planned courses of intensive chemotherapy (e.g., A1 Protocol of the Study Group of Japan). Thus, after having induced complete remission, for consolidation, it is necessary to kill all remaining tumor cells by giving supralethal doses of chemotherapy including total body irradiation (TBI) assisted by BMT.
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PMID:The strategy to treat disseminated neuroblastoma utilizing bone marrow transplantation: what is the surgeon's role? 789 87

Ganglioneuroma is a rare neoplasm arising from the sympathoadrenal neuroendocrine system and has anatomic distribution paralleling the sympathetic chain ganglia and the adrenal medulla. In some cases, ganglioneuroma is the end stage maturation of less-differentiated neoplasms such as neuroblastoma or ganglioneuroblastoma, but based on age at diagnosis (over 10 years of age) and anatomic location, many of these tumors appear to arise de novo. It must be included in the differential diagnosis of posterior mediastinal and retroperitoneal mass. We report a case of retroperitoneal ganglioneuroma involving the celiac axis and superior mesenteric arteries in a 40-year-old female.
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PMID:Retroperitoneal ganglioneuroma encasing the celiac and superior mesenteric arteries. 1557 21

Down-regulated in renal cell carcinoma 1 (DRR1) is mapped at 3p21.1, and is a candidate tumor suppressor gene. However, its biological roles have yet to be elucidated. Here, we developed polyclonal antibodies against DRR1 protein, and examined its expression during embryogenesis and carcinogenesis. The DRR1 protein was preferentially expressed in axonal projections of the central and peripheral nervous system of mice during embryonic days 10.5-16.5. Consistent with this expression pattern, the protein was detected in the neurites of primary cultured cortical neurons of rats at embryonic day 18.5. Survival of these cells was significantly inhibited by RNAi-induced downregulation of DRR1 expression. DRR1 was poorly expressed in established cancer cell lines, including neuroblastoma cells, whereas strong expression was observed in normal cells. A neuroblastoma model, MYCN transgenic mice, revealed that DRR1 protein was expressed in the celiac ganglion 2 weeks after birth when neuroblast hyperplasia was also observed; however, there was no longer any expression of DRR1 protein in tumors originating from the ganglion 8 weeks after birth. Together, our data indicate that DRR1 protein is expressed in normal cells, particularly in the nervous system during embryogenesis, is involved in neuronal cell survival, and is downregulated during neuroblastoma carcinogenesis.
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PMID:DRR1 is expressed in the developing nervous system and downregulated during neuroblastoma carcinogenesis. 2029 74

The basic helix-loop-helix transcription factor NeuroD1 has been implicated in the neurogenesis and early differentiation of pancreatic endocrine cells. However, its function in relation to cancer has been poorly examined. In this study, we found that NeuroD1 is involved in the tumorigenesis of neuroblastoma. NeuroD1 was strongly expressed in a hyperplastic region comprising neuroblasts in the celiac sympathetic ganglion of 2-week-old MYCN transgenic (Tg) mice and was consistently expressed in the subsequently generated neuroblastoma tissue. NeuroD1 knockdown by short hairpin RNA (shRNA) resulted in motility inhibition of the human neuroblastoma cell lines, and this effect was reversed by shRNA-resistant NeuroD1. The motility inhibition by NeuroD1 knockdown was associated with induction of Slit2 expression, and knockdown of Slit2 could restore cell motility. Consistent with this finding, shRNA-resistant NeuroD1 suppressed Slit2 expression. NeuroD1 directly bound to the first and second E-box of the Slit2 promoter region. Moreover, we found that the growth of tumor spheres, established from neuroblastoma cell lines in MYCN Tg mice, was suppressed by NeuroD1 suppression. The functions identified for NeuroD1 in cell motility and tumor sphere growth may suggest a link between NeuroD1 and the tumorigenesis of neuroblastoma. Indeed, tumor formation of tumor sphere-derived cells was significantly suppressed by NeuroD1 knockdown. These data are relevant to the clinical features of human neuroblastoma: high NeuroD1 expression was closely associated with poor prognosis. Our findings establish the critical role of the neuronal differentiation factor NeuroD1 in neuroblastoma as well as its functional relationship with the neuronal repellent factor Slit2.
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PMID:The neuronal differentiation factor NeuroD1 downregulates the neuronal repellent factor Slit2 expression and promotes cell motility and tumor formation of neuroblastoma. 2134 47


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