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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a group of 97 patients aged from 6 months to 12 years, all with suspected or proven neural crest tumours, metaiodobenzylguanidine (MIBG) scintigraphy was performed at the time of diagnosis and, in some instances, after induction chemotherapy. All the patients underwent a tumour biopsy with cytological and histological analysis, in addition to imaging examinations such as X-rays, ultrasound, computed tomography and magnetic resonance, within a short period before or after scintigraphy. In 82 of 97 cases MIBG was effective in detecting the primary tumour, hence the technique's sensitivity was 84%. A significant different of sensitivity between [131I]MIBG and [123I]MIBG was not demonstrated. As regards metastatic locations, MIBG scans revealed one or more bone metastases in 12 cases, bone marrow involvement (assumed to be present when diffuse and symmetric uptake in the spine, pelvis and possibly other skeletal sites were visualized) in 9 cases, and focal liver metastases or hepatomegaly in 4 cases. Probably owing to the restrictive diagnostic criterion adopted or to the early phase of the bone marrow involvement, the last was found by biopsy but missed by MIBG in 25 cases. The overall sensitivity in detecting metastases was low (48%), but it was much higher if only bone metastases were considered (81%). Twenty-nine patients who had positive scans at diagnosis were checked following 1-2 courses of induction chemotherapy (IC). MIBG scans remained positive in 22 primary tumours, while 7 primary masses were no longer detected. Out of 12 cases showing metastases at diagnosis, two cases with liver lesions became normal and in one case some, but not all, of the bone lesions were not detectable; 4 cases remained abnormal, while in 5 cases bone marrow involvement was not confirmed. Three cases were confirmed to be true negatives; in 4 other cases bone marrow involved not showing at diagnosis was revealed and confirmed by biopsy; 3 cases in which bone marrow involvement was not revealed by MIBG at diagnosis, had normal MIBG and biopsy results after IC; finally, 2 false negative bone marrow cases and 5 true negative cases at diagnosis remained unchanged, but were not checked by biopsy. Performing total body MIBG scintigraphy in childhood neuroblastoma at diagnosis is useful: 1) to predict the nature of the masses detected by other imaging techniques, when biopsy has not yet been performed; 2) for more accurate tumour staging, in addition to standard imaging investigations, MDP scintigraphy and bone marrow aspiration biopsy, thanks to its ability to detect metastatic lesions; 3) to anticipate the decrease in sensitivity of the technique in detecting both the primary mass and the metastases following induction chemotherapy.
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PMID:Radioiodinated meta-iodobenzylguanidine in the diagnosis of childhood neuroblastoma. 900 44

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

The authors report three cases of stage IV-S neuroblastoma in infants aged 4, 6, and 8 weeks, who despite chemo- and radiotherapy required surgical intervention to urgently relieve major thoracoabdominal compression secondary to massive hepatomegaly. The results were successful, with abdominal expansion being achieved by the introduction of a polytetrafluoroethylene prosthesis, which was removed during the 2nd, 3rd and 7th postoperative month, respectively, after tumor regression. Two children were in complete remission 32 and 38 months later, the 3rd died after 16 months of tumor progression.
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PMID:Abdominal expansion using a polytetrafluoroethylene prosthesis in the treatment of Pepper syndrome. 1078 88

We present the case of a newborn with Costello syndrome who died due to heart arrhythmia. In the autopsy, a neuroblastoma was found. The male patient was born at term. During the first hours of life, he developed severe respiratory failure requiring mechanical ventilation. Phenotypic features included cranial and facial dysmorphia, short thorax, tachycardia, heart murmur, abdominal distention, hepatomegaly, short extremities, widespread petechias, diminished muscular tone, ungueal hypoplasia in toes, bilateral cryptorchidia, and generalized redundant skin. In the evolution he presented several sepsis episodes, difficulty for feeding, supraventricular arrhythmia, two heart arrests, and opisthotonos, and died at 65 days of life due to heart arrhythmia. The autopsy revealed hydrocephaly, a neuroblastoma, and a heart without anatomic alterations. Costello syndrome was diagnosed. Costello syndrome is not frequent; in this patient, the diagnosis was suspected in life and was confirmed postmortem, the topic is reviewed, the important aspect in this case is the association with a neuroblastoma.
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PMID:[Costello syndrome associated to a neuroblastoma. Presentation of a case]. 1113 63

Stage 4S neuroblastoma is an unusual malignancy that has an excellent prognosis, except in young infants. A 2-month-old with 4S neuroblastoma complicated by massive hepatomegaly, managed by abdominal decompression surgery and a negative-pressure dressing system is presented. Diffuse alveolar hemorrhage also developed, which was treated with high-dose corticosteroids.
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PMID:Unique management of stage 4S neuroblastoma complicated by massive hepatomegaly: case report and review of the literature. 1199 Jul 2

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

The authors describe a case of hepatoblastoma in a 15-month-old male and discuss the differential diagnosis and electron microscopic features of small round cell tumors. The patient was found to have an enlarged liver and was admitted to the hospital for further investigation. Fine-needle aspiration of the liver revealed small. uniform cells with increased nuclear/cytoplasmic ratio and focal rosette formation. A diagnosis of small blue cell neoplasm favoring hepatoblastoma was made, but neuroblastoma could not be ruled out. Electron microscopic analysis performed on the liver aspirate showed features of hepatic differentiation as well as absence of neuroblastic differentiation. The diagnosis of hepatoblastoma was made. Serum alpha-fetoprotein level of 33,250 mg/L confirmed the diagnosis. Liver biopsy performed subsequently showed tumor cells arranged in nests, acini, and trabeculae with mitotic figures. Electron microscopy showed the same findings as described above. The patient underwent chemotherapy for 4 months and subsequently a partial liver resection was performed. This case illustrates the important role of electron microscopy in evaluating small round cell tumors in children.
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PMID:Hepatoblastoma in a 15-month-old male: cytomorphology, electron microscopy, and differential diagnosis. 1470 29

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

A 2 1/2-month-old infant presented with a massive hepatomegaly. Ultrasound and computerized tomography showed a large cystic lesion in the right adrenal, small cysts in the left adrenal, and multiple cystic liver metastases. The right adrenal cyst, on excision, turned out to be a cystic neuroblastoma with hemorrhage. On follow-up, the cysts in the left adrenal and the liver metastases are regressing.
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PMID:Bilateral cystic adrenal neuroblastoma with cystic metastasis in the liver. 1770 80

The authors report 2 cases of neuroblastoma-associated hepatomegaly, which were treated using a Silastic patch, and discuss in the light of recent reports, the technical aspects and outcome of these children. They were satisfied by the decompression achieved with the patch and believe there is no increased risk in using Silastic rather than other types of material. The outcome for these children depends more on the evolution of the underlying disease than the technical aspects of the abdominal decompression.
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PMID:Surgical management of neuroblastoma-related hepatomegaly: do material and method really count? 1848 75


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