Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuroblastoma is a common solid tumor of infancy and childhood. From 1967 to 1986 we evaluated and treated 58 children with neuroblastoma; in ten (17%) of these children, symptomatic hepatic metastasis developed. The ten children ranged in age from 2 days to 2 years 3 months. The most common symptoms attributable to hepatic metastasis were abdominal enlargement, abdominal pain, respiratory difficulty due to upward pressure on the diaphragm, and obstruction of the inferior vena cava. At the time of initial diagnosis, two children had stage III disease, three had stage IV disease, and five had stage IV-S disease. Six were initially given chemotherapy; all six of these patients required radiation therapy when hepatic enlargement progressed. In the other four cases, radiation therapy was used alone or in combination with chemotherapy. Irradiation or irradiation plus chemotherapy produced complete resolution of local symptoms in seven cases, and a partial response in one case. The seven children who had a complete response are alive without evidence of recurrent disease; the remaining three children died of their tumor. The roles of chemotherapy, surgery, and radiation therapy in the management of symptomatic hepatic metastasis from neuroblastoma are discussed.
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PMID:Hepatic metastasis from neuroblastoma. 368 39

The clinical characteristics of 43 patients (pts) and the biological features of their non-stage 4 neuroblastoma (11, 3, 15, 7 and 7 with stages 1, 2A, 2B, 3 and 4S, respectively) all managed initially without cytotoxic therapy at Memorial Sloan-Kettering Cancer Center are summarised. We staged patients by the International Neuroblastoma Staging System and measured their urine and serum tumour markers. Tumour MYCN copy number, chromosomal ploidy, chromosome 1p deletion, Shimada histopathology, trk-A and CD44 expression were analysed. Among patients with localised tumour (n = 36), 13 had residual disease after initial surgery, 19 had regional lymph node invasion and 6 had epidural involvement (2 of 6 being paraplegic). All 7 stage 4S patients had liver tumours, 3 had bone marrow involvement and 3 had lymph node involvement. The most common adverse biological markers were unfavourable histopathology (9/40 evaluable tumours) and diploidy (7/39 tumours tested). At a median follow-up of 50+ months, 42 patients are alive and well (5 with evidence of disease), and 1 patient in remission died of encephalopathy. Progressive/recurrent disease occurred in 12 patients, 1 stage 2A, 2 stage 2B, 4 stage 3 and 5 stage 4S. Chemotherapy was eventually used in 4 patients: a 3-year-old stage 2B patient who developed stage 4; a 2-year-old whose recurrent tumour had poor-risk biological markers; a 1-year-old whose recurrent stage 3 disease infiltrated a vertebral body and a stage 4S infant with respiratory impairment from progressive hepatomegaly. Three of the treated patients had diploid tumours. We conclude that non-stage 4 is of itself a strong predictor of a favourable outcome. Diploidy, unfavourable histopathology and unresectable tumours were associated with disease progression. However, evolution of local-regional tumour into distant metastatic stage 4 disease is not typical of neuroblastoma.
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PMID:Survival from non-stage 4 neuroblastoma without cytotoxic therapy: an analysis of clinical and biological markers. 951 65

Neuroblastoma is a common solid tumor of childhood that can involve the abdomen, thorax, pelvis, or the head and neck. The clinical manifestations are dependent on the widespread distribution of neural crest tissue and the length of the sympathetic chain involvement. Abdominal pain and hypertension may occur as a result of renal vasculature compression; respiratory distress may be evident in thoracic tumors; and Homer's syndrome or heterochromia of the iris may manifest from neuroblastoma of the head and neck. In addition, symptoms of cord compression and back pain may result from spinal cord compromise due to epidural invasion. Metastatic involvement of the liver, skin, periorbital regions, or bone may cause hepatomegaly, skin nodules, proptosis, or bone marrow failure, respectively. Clinical findings along with tumor metastasis may be studied by various imaging modalities to assess the nature and extent of the tumor. Diagnostic tests include plain radiography, ultrasonography, CT scanning, and MR imaging. Bone marrow studies, bone scans, and scintigraphy with 131I-metaiodobenzylmandelic may be utilized for metastatic evaluation. By using these imaging studies to detect the nature and behavior of neuroblastoma, early intervention may indeed improve patient survival.
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PMID:Imaging neuroblastoma in children. 1262 83

Neuroblastoma, the most common malignant tumor in the neonatal period is metastatic in 25 to 50% of cases. While the prognosis of antenatal neuroblastoma is often considered as favorable, included in the most common metastatic stage (Stage IV S), it can lead to fetal or neonatal death. We report a case of a fetus with a stage IV neuroblastoma who died in utero. The most important prognostic factor is tumor stage, making sonographic detection of metastasis essential. Nevertheless, accurate staging remains difficult by sonography. When metastatic neuroblastoma is suspected, sonographic survey has to be reinforced, and if serious criteria such as massive hepatomegaly, placentomegally or hydrops appear, delivery must be considered.
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PMID:[Antenatal metastatic neuroblastoma: prognostic criteria. A case report]. 1506 99

Neuroblastoma (NBL) is a neuroectodermal tumor derived from neural crest cells. The biological and clinical behavior of NB is extremely heterogenous. We here report a newborn who presented as 4S NBL with a massive hepatomegaly resulting in IVC syndrome.
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PMID:Neonatal neuroblastoma with inferior vena cava syndrome. 2404 May 88

Neuroblastoma (NBL) stage 4s is an incompletely understood phenomenon with variable clinical course. While the majority of patients may undergo spontaneous regression and achieve complete resolution without intensive therapy, a small proportion is at increased risk of developing secondary complications. One such situation is liver insufficiency due to diffuse metastases. We report a patient suffering from NBL 4S who required double lifesaving liver transplantation. Abdominal and respiratory complications due to hepatomegaly are crucial determinants for treatment intensity and duration in 4S NBL [1,2] . We provide an algorithm in order to facilitate the clinical decision when dealing with similar potentially life-threatening events.
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PMID:Liver transplantation as a potentially lifesaving measure in neuroblastoma stage 4S. 2808 36