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)

The results of management of 14 patients with Stage IV-S neuroblastoma are reported. The treatment policy, although not consistent over this time span, in general used a combination of radiotherapy and chemotherapy or infrequently one modality alone. Twelve of 14 (86%) survived more than 6 years. One patient, with a solitary mediastinal primary tumor, died of rapidly progressive disease at three months. The other death occurred in a 4.5-year-old presenting with hepatomegaly at diagnosis followed by skeletal dissemination 2.5 years later. Thirteen of the patients were younger than 1 year of age. Of the 11 patients that received radiotherapy, 4 experienced mild asymptomatic scoliosis or kyphoscoliosis at 3 to 12 years after initial therapy. A review of the literature indicates that spontaneous regression in this tumor is very frequent; therefore, it is recommended that for the common presentation of massive hepatomegaly in an infant, close observation is warranted, unless life threatening complications occur. However, initial therapeutic intervention may be indicated in those patients with life threatening presentations. This data did not substantiate the necessity for complete surgical excision of the primary tumor, as has been suggested by others.
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PMID:Stage IV-S neuroblastoma. Results with definitive therapy. 670 96

The clinical features of 30 children with IV-S neuroblastoma have been reviewed. They were treated at 11 Italian pediatric institutions in the period 1970-1981. Patients with IV-S neuroblastoma comprise 6.1% of all the neuroblastoma cases observed during that period. The age at diagnosis ranged from birth to 11 months (mean, 3 months). The distribution of "primary" lesions was of interest. The adrenal gland was affected most often (57% of patients), but tumors of the chest and neck were also encountered. No specific "primary" lesion was identified in ten children. The most frequent site of widespread disease was the liver (87%). Neither the size of the "primary" tumor, nor the number or size of subcutaneous nodules was of prognostic significance. Unfavorable features were age less than 2 months and clinical signs of pulmonary, renal, or hepatic embarrassment. These data confirm the need for prompt intervention in the infant who is suffering from compression of these vital structures by the enlarged liver. Radiation therapy is to be preferred for this purpose, because of the often severe toxicity that is associated with chemotherapy in infants. One, and possibly two babies in this series died of chemotherapy-related complications.
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PMID:IV-S neuroblastoma: a cooperative study of 30 children. 672 72

An 8-week-old girl was seen because of firm, blue cutaneous nodules. A biopsy showed neuroblastoma. When rubbed, the nodules blanched and were surrounded with a halo of erythema. She had Horner's syndrome, hepatomegaly, bone marrow and shaft invasion, and a high thoracic mass with signs of spinal cord compression. Blanching cutaneous nodules are a unique finding in neuroblastoma and may be the first sign of this disease.
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PMID:Infantile neuroblastoma presenting with cutaneous blanching nodules. 706 66

Acute renal failure developed in an infant aged 30 days during chemotherapy and irradiation for hepatic neuroblastoma (IV-S). Acute hemodialysis via a profunda femoris - long saphenous vein shunt was accomplished on 9 occasions in this 4 kg infant. The complications of septicemia and acute respiratory embarrassment caused by hepatomegaly were treated by appropriate antibiotic treatment, creation of a temporary ventral hernia and assisted ventilation. Careful attention to correct pediatric hemodialysis technique, as described, can permit long-term survival if the underlying disorder is also amenable to treatment.
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PMID:Acute renal failure in an infant with congenital neuroblastoma - successful treatment by hemodialysis. 714 95

An apparently normal 10-day-old girl had, since birth, a gray-white vascularized tumor in the inferior quadrants of the right anterior chamber. General physical examination revealed abdominal distension, hepatomegaly, splenomegaly, and a deeply located abdominal mass. Multiple subcutaneous nodules scattered all around the body's surface could be palpated. Surgical exploration confirmed the presence of a left adrenal neuroblastoma. Six months after a complete resection of the abdominal tumor and general radiotherapy and chemotherapy, the right anterior chamber mass regressed almost completely, leaving only a calcified residue. The child is living and well six years after the diagnosis of neuroblastoma. This is the first case known to us in the literature of a congenital anterior chamber metastasis, as well as the first case reported of an anterior segment metastasis from a neuroblastoma.
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PMID:Anterior chamber metastasis from neuroblastoma. 715 21

The records of 207 neuroblastoma patients seen at the Children's Hospital of Philadelphia between 1944 and 1977 were reviewed to study some of the features associated with the unusually good prognosis found in patients with Stage IV-S neuroblastoma. Initially, 22 patients appeared to fit the criteria of small primary tumor and distant disease in liver, skin, and/or marrow without evidence of bone metastases; 5 patients were subsequently rejected as being incorrectly staged. The remaining 17 patients had abdominal primary tumors and hepatic disease; in 12 of the 17, an enlarged liver was the presenting sign. Six patients had skin lesions, 4 had disease in the marrow on routine smear, and additional sites of spread were pancreas and bowel serosa. The treatment given was not systematic, and it was not possible to correlate any specific form of therapy with a satisfactory outcome. Eleven of 17 patients survived; 6 of 11 survivors had spontaneous regression of all or part of their diseases, 5 of 6 who died received irradiation, chemotherapy, or both. Death usually occurred in the first month as a complication of the local disease; 1 patient succumbed to radiation nephritis. This study establishes that the special pattern of widespread neuroblastoma termed Stage IV-S does exist, and that is associated with a good prognosis. Careful consideration should be given before selecting treatment for the Stage IV-S child because spontaneous regression is likely to occur in most of them. In patients with rapidly enlarging livers, renal or pulmonary complications may develop because of liver bulk or coagulopathies. Treatment should be directed to the liver in these cases because distant metastases seldom supervene. Low-dose irradiation, mild chemotherapy, and possibly surgical release of intraabdominal pressure using a silastic patch have all been effective. Unfortunately, patients occasionally succumb to local disease in spite of these and more aggressive measures.
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PMID:A review of 17 IV-S neuroblastoma patients at the children's hospital of philadelphia. 726 Aug 34

Stage IV-S neuroblastoma has a relatively favorable outcome. However, urgent surgical treatment may be necessary for management of life-threatening complications related to massive hepatomegaly caused by metastatic tumor infiltration. The enlarged liver often becomes of primary concern because diaphragmatic elevation results in life-threatening respiratory embarrassment. An external SILAS-TIC dome has been used as a temporizing procedure to decrease the intraabdominal pressure. The authors developed a modification of this approach using an internal polytetrafluoroethylene patch to create a ventral hernia. This technique decreases the potential complications of prosthetic material use, namely, risk of infection. The graft may be left in place for an extended period and removed in staged operations as the bulk of the metastatic tumor regresses.
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PMID:Abdominal expansion as a bridging technique in stage IV-S neuroblastoma with massive hepatomegaly. 784 23

Two cases of bilateral adrenal neuroblastoma in infants are reported. The first patient presented with abdominal distension due to metastatic hepatomegaly and the second patient was found because of elevated urinary VMA by VMA mass-screening system. Both were under one year of age and were classified as stage IVs neuroblastoma. In each case unilateral adrenalectomy was performed and the contra-lateral adrenal tumor was observed. The remaining tumors slowly decreased in size and clinical progress was good. A review of published cases of bilateral adrenal neuroblastoma suggests that the characteristics of this disease are age less than 1 year, stage IVs, and good prognosis.
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PMID:Bilateral adrenal neuroblastoma. 819 31

Infants with neuroblastoma (NBL) frequently present as stage 4s and overall, such patients have a good prognosis. However, not all survive, and neonates with hepatomegaly are particularly at risk. We therefore reviewed our 4s experience, the objective being to identify lethal patterns of disease progression. The specific aims of this work were (1) to develop a semiquantitative scoring system based on the severity of signs and symptoms that alone or in combination presaged a fatal outcome, and (2) to determine if early intervention could reverse life-threatening disease. Thirty-five patients were seen over a period of 50 years. The signs and symptoms of organ distress caused by hepatomegaly occurred in the lungs, kidneys, gastrointestinal tract (GI), the inferior vena cava (IVC), and the liver. A scoring scale reflecting organ compromise was developed, the scores ranging from 0 (0 compromise) to 10 (all 5 systems showing evidence of impairment). Scores were derived for 32 of 35 patients; 13 were 4 weeks old or under (neonates) when first seen, and 19 were aged 1-12 months (infants). Neonates were more likely than infants to develop increasing symptomatology (50% versus 25%) and were more likely to die when a score of 2 or more developed. None of the 6 neonates who did so survived despite treatment, compared with three of four infants. Early intervention is recommended: (1) for 4s neonates who develop a score of 1 and (2) for older infants with a score > or = 2.
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PMID:Hepatomegaly in neuroblastoma stage 4s: criteria for treatment of the vulnerable neonate. 888 11

Despite intensified chemotherapy protocols, including autologous bone marrow transplantation (ABMT), stage IV neuroblastoma has a poor prognosis, and modern therapeutic trends are aimed at the eradication of minimal residual disease, which is though to be the main factor leading to relapse. In this pilot study, we report the systemic administration of high doses of interleukin-2 after ABMT in four patients. Five day cycles of IL-2 at a dose of 18 x 10(6) IU/m2/day were administered at variable time intervals as frequent as it was necessary to maintain the levels of natural killer (NK) cytotoxic activity higher than the median control value (40 LU/ml blood) throughout 1 year from the start of first IL-2 treatment. After IL-2 infusion, NK and LAK activities increased significantly (median 742 x 10(-3) LU/ml blood and 186.8 x 10(-3) LU/ml blood, respectively). Toxicities were transient and no life-threatening complications were observed. Fever, anorexia, skin rash and enlarged liver were always present. Anaemia, thrombocytopenia, leukocytosis, lymphocytosis and and eosinophilia occurred following most of the IL-2 courses. Although the small number of patients does not allow an estimation of the immunomodulatory-antineoplasic effects of IL-2, the results seem promising for the management of neuroblastoma patients.
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PMID:High-dose systemic interleukin-2 therapy in stage IV neuroblastoma for one year after autologous bone marrow transplantation: pilot study. 888 13


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