1/37. 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 = 1keywords = acid (Clic here for more details about this article) |
2/37. 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 = 26038.226766279keywords = amplification (Clic here for more details about this article) |
3/37. Perianaesthetic risks and outcomes of abdominal surgery for metastatic carcinoid tumours.patients with metastatic carcinoid tumours often undergo surgical procedures to reduce the tumour burden and associated debilitating symptoms. These procedures and anaesthesia can precipitate a life-threatening carcinoid crisis. To assess perioperative outcomes, we studied retrospectively the medical records of adult patients from 1983 to 1996 who underwent abdominal surgery for metastatic carcinoid tumours. Preoperative risk factors, intraoperative complications and complications occurring in the 30 days after surgery were recorded. Perioperative complications or death occurred in 15 of 119 patients (12.6%, exact confidence interval 7.2-19.9). None of the 45 patients who received octreotide intraoperatively experienced intraoperative complications compared with eight of the 73 patients (11.0%) who did not receive octreotide (P=0.023). The presence of carcinoid heart disease and high urinary output of 5-hydroxyindoleacetic acid preoperatively were statistically significant risk factors for perioperative complications.- - - - - - - - - - ranking = 1keywords = acid (Clic here for more details about this article) |
4/37. An enormous abdominal mass associated with acute renal failure.We report a 67-year-old man with acute uric acid nephropathy, secondary to spontaneous tumor lysis syndrome, that presented itself as a huge intra-abdominal tumor that led to acute renal failure, hyperuricemia, and azotemia. Initial finding of hydronephrosis detected by ultrasonography led us to believe that the azotemia and decreasing amount of urine resulted from obstructive uropathy, a common complication of malignancy, caused by either a direct renal invasion or a urinary outflow tract compression because of a tumor mass effect. However, clinical observations and the response to therapeutic intervention confirmed the diagnosis of spontaneous tumor lysis syndrome, which is a rare cause of acute uric acid nephropathy.- - - - - - - - - - ranking = 2keywords = acid (Clic here for more details about this article) |
5/37. Hemodialysis for acute anuric uric acid nephropathy.A patient with acute oliguric uric acid nephropathy was treated with hemodialysis. Recovery in this disorder is based on treatment of both the uremic state and the intrarenal crystal obstruction. Hemodialysis with high uric acid clearance is much more efficient than other forms of therapy in this disorder.- - - - - - - - - - ranking = 6keywords = acid (Clic here for more details about this article) |
6/37. Abdominal and pelvic extra-adrenal paraganglioma: a review of literature and a report on 7 cases.INTRODUCTION: Extra-adrenal paraganglioma (pheochromocytoma) is a rare tumor. Herein we describe the clinical and pathological findings in patients with paragangliomas of the urinary bladder, seminal vesicle and retroperitoneum. methods: Between January 1994 and January 2001, extra-adrenal paragangliomas were diagnosed in 7 patients: 3 males and 4 females. The mean age of our patients was 32 /- 15.9 years. We reviewed the clinical data. Urinary metanephrines and vanillyl mandelic acid and blood catecholamine levels were estimated in 4 cases. CT scan and/or MRI were used in the imaging of all cases. 123I-MIBG was used in only 1 patient, who harbored multiple tumors. All the patients but one underwent surgical treatment. RESULTS: The definitive diagnosis was made by histopathological examination of the removed tumors and was confirmed in all cases by the immunohistochemical stains of chromogranin a and S100 protein. There was metastasis in the pelvic lymph nodes in 1 patient. Follow-up ranged from 3 to 82 months (mean = 37.9 /- 25.8). The catecholamine level was elevated in 3 patients under basal conditions and during endoscopic resection of the tumor in a fourth patient. In all cases, the catecholamine level was normalized after surgery. There was no recurrence or metastasis in any case following surgery. CONCLUSION: Pre-operative diagnosis of nonfunctioning bladder paraganglioma is difficult, but the tumors should be suspected in patients who have hypertension, hematuria or mass effects due to the tumor growth in the pelvis and/or retroperitoneum. Six of the seven cases reported here were found in the usual locations: 3 in the urinary bladder, 2 in the renal hilum and 1 in the organ of Zuckerkandl. One patient had multiple tumors, including a paraganglioma of the seminal vesicles. Resection is the treatment of choice, and in the case of urinary bladder paraganglioma should include total cystectomy. In patients with unresectable multiple tumors, medical therapy may be used to control hypertension.- - - - - - - - - - ranking = 1keywords = acid (Clic here for more details about this article) |
7/37. Large-dose intravenous methotrexate-induced cutaneous toxicity: can oral magnesium oxide reduce pain?IMPLICATIONS: Chemotherapy for cancer is associated with pain including cutaneous vasculitis. Magnesium, an N-methyl-d-aspartic acid-receptor antagonist, was used successfully to treat an adolescent with pain caused by cutaneous vasculitis secondary to methotrexate therapy.- - - - - - - - - - ranking = 1keywords = acid (Clic here for more details about this article) |
8/37. Intranodal palisaded myofibroblastoma with overexpression of cyclin d1.Intranodal palisaded myofibroblastoma (IPM) is a rare benign spindle-cell tumor of lymph nodes with myofibroblastic/smooth muscle differentiation. We present another case of IPM that confirms the myofibroblastic differentiation of the tumor cells and identifies the so-called amianthoid fibers as collagen deposits by immunohistochemical and ultrastructural techniques. Because IPM shares histomorphologic characteristics with an inflammatory myofibroblastic tumor that has been associated with a virus-induced alteration of cell cycle regulation, the diagnostic approach was extended in this case. We were able to demonstrate cyclin d1 overexpression but could detect neither amplification of the CCND1 gene nor allelic loss at chromosomes 9p22-21 and 13q (surrounding the genes p16 and Rb, respectively). Furthermore, no evidence of human herpesvirus-8 or Epstein-Barr virus infection could be found by polymerase chain reaction or immunostaining. Nevertheless, our results point to the cell cycle regulatory genes as a factor in the pathogenesis of IPM.- - - - - - - - - - ranking = 3719.746680897keywords = amplification (Clic here for more details about this article) |
9/37. pneumocystis carinii pneumonia: a late presentation following treatment for stage IV neuroblastoma.This report describes a child who develops pneumocystis carinii pneumonia 7 months after high-dose chemotherapy for stage IV neuroblastoma. In addition to chemotherapy the child had also been treated with abdominal radiotherapy and 13-cis-retinoic acid. Standard practice has been to treat patients with prophylactic co-trimoxazole for 3 months after high-dose therapy, but this report highlights the intensity and complexity of current treatment for stage IV neuroblastoma and the need to be aware of prolonged lymphopenia after such treatment.- - - - - - - - - - ranking = 1keywords = acid (Clic here for more details about this article) |
10/37. Evolution of unbalanced gain of distal chromosome 2p in neuroblastoma.neuroblastoma, one of the most common tumors of childhood, presents at diagnosis with a vast number of recurrent chromosomal imbalances that include hyperdiploidy for whole chromosomes, partial loss of 1p, 3p, 4p, 11q, 14q, partial gain of 1q, 7q, 17q and amplification of MYCN. These abnormalities are nonrandomly distributed in neuroblastoma as loss of 3p and 11q rarely occur in MYCN amplified neuroblastomas. Here, we report on a patient who had a non-MYCN amplified 3p-/11q- neuroblastoma at diagnosis who subsequently developed a high level of MYCN amplification in bone marrow metastases 41 months after induction of complete remission. The tumor at diagnosis had low level unbalanced gain of distal 2p. In order to assess the frequency of low level gain of distal 2p in neuroblastoma, we examined the comparative genomic hybridization results from 60 neuroblastomas. Among non-MYCN amplified neuroblastomas, 8/45 (18%) had low level gain of distal 2p. Low level gain for a segment of 2p (i.e. a region larger than the 2p23-->p24 undergoing amplification) was also detected in five of the 15 tumors that had high level MYCN amplification. The possibility that low level gain of distal 2p is a risk factor for high level MYCN amplification is discussed.- - - - - - - - - - ranking = 18598.733404485keywords = amplification (Clic here for more details about this article) |
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