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 lactobezoar is described in an eleven weeks old infant with a history of prolonged vomiting and constipation. The feeding history revealed no abnormalities, but unusually high environmental temperatures plus increased sweating may have been responsible for the formation of the gastric milk coagulum which presented as a firm epigastric tumour persisting for several weeks. An abdominal neuroblastoma was suspected but the correct diagnosis was established by a barium meal. Conservative therapy with parenteral fluids and gentle gastric lavage resulted in prompt disintegration of the lactobezoar. The 9 previously reported cases in the literature are briefly discussed.
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PMID:Lactobezoar in an infant: an unusual cause of upper abdominal tumour persisting for several weeks. Case report and review of the literature. 57 98

A 5-day continuous infusion of vincristine (VCR; total dose 4 mg/m2) has been given as part of a high-dose chemoradiotherapy regimen with bone marrow transplantation. Evidence of neurotoxicity, such as weakness, paraesthesia and intestinal hypomotility, was evaluated prospectively in nine patients. Five patients had advanced neuroblastoma and four, relapsed sarcomas, and all had responded to initial conventional-dose therapy. VCR was combined with high-dose melphalan (180 mg/m2) and fractionated total-body irradiation. Plasma concentrations of VCR were measured by radioimmunoassay during and up to 24 h after the infusion. Serum and urine electrolytes and liver function tests were measured during VCR treatment and at regular intervals thereafter. VCR concentration at 1 h ranged from 1.8 to 10.9 (median 6.6) ng/ml, and a steady state was achieved by 13-30 h (median 16 h). Levels above 1 ng/ml were maintained throughout the 5-day period with a mean steady-state concentration of 1.7 ng/ml (range 1.3-2.15). After cessation of the infusion, serum concentrations fell to below 0.25 ng/ml within 24 h. Abdominal pain occurred in one patient, but neither constipation nor ileus was seen. In two patients severe muscle pain occurred in the lower limbs towards the end of the infusion. Significant electrolyte problems did not occur and, in particular, there was no evidence of inappropriate ADH secretion. Transient increases in liver enzymes were common but bilirubin was not elevated during the period of monitoring. This regimen allows a two-fold escalation in the dose of VCR to be administered, producing sustained high serum drug levels without major toxicity.
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PMID:Continuous vincristine infusion as part of a high dose chemoradiotherapy regimen: drug kinetics and toxicity. 304 35

After a 3-year history of severe constipation, a 16-year-old girl required surgery to be relieved of impacted stools. Histologic examination showed ganglionitis in the myenteric plexus of the large bowel and ileum, whereas the submucosal plexus was spared. At this time, antineuronal nuclear antibodies (ANNA-1, anti-Hu) were found at high titer in the serum of the patient. One and a half years earlier, a paravertebral ganglioneuroblastoma had been removed. Histologic examination had shown undifferentiated neuroblasts and morphologically mature ganglion cells with both cell types embedded in an inflammatory infiltrate morphologically similar to the lymphoplasmocytic infiltration seen in the myenteric plexus. The patient's serum was found to bind to nuclei of mouse intestinal tract neurons, thus fulfilling defining criteria for ANNA-1. The serum also reacted with antigens of defined molecular weight in a Western blot, thus fulfilling defining criteria for anti-Hu. Expression of the Huantigen could be visualized in the nuclei of the patient's tumor cells by immunohistochemistry. These tests showed that an antitumor inflammatory response was the cause of the bowel disease. This is the first report of a tumor from the neuroblastoma group that caused paraneoplastic intestinal pseudoobstruction. Ganglionitis and subsequent aganglionosis are the hallmark of the morphologic diagnosis which cannot be obtained by suction biopsy in patients with intact submucosal plexus. Instead, serum testing for autoantibodies can reveal the etiology.
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PMID:Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction. 1032 94

The oral alkylating agent, temozolomide (Temodal: TMZ), is the only anticancer drug that has been shown in a phase III study to improve survival in glioblastoma (GBM) when administered with concomitant radiotherapy. Pharmacokinetic studies have documented relatively high concentrations of TMZ in brain tumors and cerebrospinal fluid (20-40% of the area under the plasma concentration curve), and other studies have demonstrated that TMZ is effective for treatment of various brain tumors, including recurrent and newly diagnosed glioma, primary CNS lymphoma, metastatic melanoma, and neuroblastoma. Molecular markers that predict a favorable response to TMZ plus concomitant radiotherapy include methylguanine methyltransferase (MGMT) promoter methylation patients with GBM and chromosome 1p/19q deletion in patients with anaplastic oligodendroglioma or low-grade glioma. Myelosuppression, nausea and constipation are relatively frequent in patients undergoing treatment with TMZ, and prophylaxis against Pneumocystis carinii pneumonia should be instituted. This article will summarize and discuss these issues as well as review ongoing and anticipated studies of TMZ in combination with other anti-cancer therapies.
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PMID:[Temozolomide: Temodal]. 1834 14

The anti-Hu syndrome is a well-known paraneoplastic syndrome and may be rarely seen in patients with neuroblastoma. However, it is relatively unknown that anti-Hu antibodies can cause gastro-intestinal signs and symptoms. We report on a child with neuroblastoma who presented with gastro-intestinal disturbances as a result of the anti-Hu syndrome and summaries two similar case reports reported in literature. Neuroblastoma patients with gastro-intestinal disturbances, ranging from constipation to a paralytic ileus, might suffer from the gastro-intestinal anti-Hu syndrome. The causative antibodies can be determined to diagnose or exclude this syndrome, and successful treatment is possible.
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PMID:Paraneoplastic gastro-intestinal anti-Hu syndrome in neuroblastoma. 1898 84

A babygirl, aged six weeks, was hospitalized for rectal prolapse and isolated constipation. The investigation revealed a neuroblastoma (NB) inducing a medullar compression responsible for the sphincter disorders. NB is second among pediatric solid tumors, but is the most frequent cancer among infants. Its diagnosis is difficult because of its rarity and the variety of its symptoms. A new staging, based on imaging, has recently been proposed by the International Neuroblastoma Risk Group. With the exception of its localized, easily resectable forms, NB is best treated by chemotherapy.
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PMID:[Atypical clinical presentation of a neuroblastoma in an infant]. 2346 84

Mesenteric fibromatosis (MF) is a locally aggressive proliferative spindle cell lesion of the mesentery. A 34-year-old male presented with increasing abdominal pain and constipation. On workup, patient was found to have a large pelvic mass on CT A/P concerning for cancer. The patient underwent surgical excision of >15 cm intra-abdominal tumor along with adherent small bowel section. Histology of the tumor showed a spindle cell lesion consistent with MF. Previous reports have shown association of MF with Gardner syndrome and familial adenomatous polyposis. We present the first reported case of MF in a patient with previous neuroblastoma.
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PMID:Mesenteric fibromatosis in a patient with a history of neuroblastoma: a case report. 3018 89

The aim of this study was to assess the colonic transit in children and teenagers with chronic constipation. Twenty patients from 1.5 to 16 years old were included (mean age = 6.9 years). Chronic constipation etiologies were as follows: congenital megacolon in 6; surgical treatment in 5 (imperforate anus 2, hip dysplasia 1, sacral teratoma 1, and paraspinal neuroblastoma 1); idiopathic chronic constipation in 5; sacral myelomeningocele in 3; and intestinal duplication in 1. Static images on the anterior projection of the abdomen were performed 1, 6, 24, 48, and 72 h after the radiotracer oral administration. Doses were 3.7 MBq of 67Ga-citrate. The images were visually analyzed and classified by the observers as normal, diffuse slow transit, right slow transit, and left slow transit. Patients' dosimetric estimation was performed also. There were four cases of diffuse slow transit that responded well to the clinical treatment, 3 of them being chronic idiopathic constipation. From five patients with right slow transit, 4 were submitted to appendicostomy (Malone surgery) with good results. There were 11 cases of left slow transit (ten at the rectosigmoid level). Five of the 6 patients with congenital megacolon had left slow transit. Patients' dose estimation was 1.9 mSv to the whole body, 1.8 mSv to the ovaries, and 1.4 mSv to the testicular. This method is simple, safe, noninvasive, provides helpful functional information, and allows therapeutic decision regarding chronic constipation.
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PMID:Scintigraphic evaluation of colonic transit in children with constipation using 67Ga-citrate. 3050 22

Chronic abdominal pain is a very common complaint in the population of children and adolescents. In most cases, the usual cause are functional gastrointestinal disorders. However, in a few percent of children, the reason for persistent chronic stomach pain are organic diseases occurring in the gastrointestinal tract, as well as parenteral diseases, including uro-genital tract abnormalities, inflammation of the lower respiratory tract and cancer processes. Among organic causes, in addition to those commonly encountered, such as: intolerances and food allergies, gastroesophageal reflux disease, chronic gastritis or duodenitis, or urinary tract infections, the diagnosis should also include very rare causes, for example, neoplastic diseases, among them tumors of the abdominal cavity. In the case described in the present article, a 6-year-old girl with chronic abdominal pain, symptoms of gastro-oesophageal reflux and constipation, and previously diagnosed food allergy and lactose intolerance, was referred for widening the diagnosics due to the occurrence of alarm symptoms. The nodule revealed in the chest X-ray, in CT scan, turned out to be a paravertebral tumor with the specific features of neuroblastoma. After a macroscopically complete tumor resection based on the result of histopathological examination, the diagnosis of ganglineuroblastoma was established. The presence of alarm symptoms in anamnesis and physical examination in children with abdominal pain suggests a higher probability of the organic origin of the disease and should always lead to extended diagnostics. Ganglioneuroblastoma is a very rare disease, in most cases is located primarily in the abdominal cavity, and the most common associated symptom is abdominal pain.
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PMID:[Ganglioneuroblastoma in a child with chronic abdominal pain - a case report]. 3063 34