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

The clinical manifestations of 253 neuroblastoma cases in childhood, treated in Denmark from 1943 to 1980, were reviewed. Most striking was the vagueness of symptoms in the majority of patients, only a few of whom exhibited the symptoms strongly suggestive of a neuroblastoma (i.e. the Horner syndrome, the watery diarrhoea syndrome, the dancing eye syndrome). The vagueness of the symptoms might have led to fatal procrastination of the diagnosis. The diagnostic delay has, however, no independent prognostic significance for survival in our patients (p = 0.09). The maximal tumour spread was recorded for all 253 patients, and the distribution of metastases was in accordance with the "soil-seed" hypothesis. The tumour spreads with equal frequency by local growth, by lymphatic vessels to distant lymph nodes, and by blood to bone. Only in widely disseminated tumours are metastases to the lungs, the meninges, the brain, and the reproductive organs seen to occur. Eighty-five percent of the patients, for whom data were available, excreted VMA above the normal value for their age, and 43% excreted Norepinephrine + Epinephrine (N + E) above normal levels. The excretion of both VMA and N + E was significantly correlated to stage, and thus to prognosis. Neither the level of VMA excretion nor the level of N + E had any bearing on the survival when age and stage were adjusted for. Serial VMA and N + E determinations show that patients with normal values for these parameters had significantly better prognosis than patients with elevated values during the first, second, third and fourth trimesters after the initiation of treatment. Increasing values in the individual patient were associated with a poor prognosis. We found no correlation between the initial leucocyte count and survival when age and stage were adjusted for.
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PMID:Signs, symptoms, metastatic spread and metabolic behavior of neuroblastomas treated in Denmark during the period 1943-1980. 363 7

Neuroblastoma is one of the commonest childhood malignancies. The most important prognostic factor is age at diagnosis; early diagnosis, when the tumor is still localized and surgically resectable, is second in importance. On retrospective review of children seen at the Hospital for Sick Children, ophthalmic involvement was seen in 80 of 405 (20%). The three major eye signs of neuroblastoma, proptosis, Horner's syndrome and opsoclonus, are closely related to the site, stage of tumor, and outcome of the patient. Proptosis or periorbital ecchymosis due to orbital metastases was present in 60 of 80 children (bilaterally in 33). The 3-year survival rate was 11.2%. In 53 of 60 cases with orbital metastases the neuroblastoma originated in the abdomen. Unilateral Horner's syndrome occurred in 14 children, as the presenting sign in 9, related to localized disease in 11 and in a favorable location (cervical or thoracic neuroblastoma) in 8. The 3-year survival rate was 78.6%. Opsoclonus-myoclonus was the presenting sign of occult, localized neuroblastoma in all 9 children in whom it occurred. The 3-year survival rate was 100%. For all presentations, girls had a significantly better survival rate than boys (48.7% vs. 22.4%). Children presenting with any of these ophthalmological signs should undergo thorough and repeated investigations searching for neuroblastoma.
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PMID:Ocular involvement in neuroblastoma: prognostic implications. 649 2

A case of heterochromia iridis and Horner's syndrome is reported in a 7-year old girl with paravertebral neurilemmoma. These clinical findings can be useful in the early diagnosis of mediastinal tumors in the paravertebral axis. While typically associated with neuroblastoma, these findings can be due to tumors which are inately benign--in this case neurilemmoma. The mechanism for heterochromia is briefly discussed.
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PMID:Heterochromia iridis and Horner's syndrome due to paravertebral neurilemmoma. 682 13

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

Cervical neuroblastoma is relatively rare and shows very good prognosis. We reported a case of surgically resected cervico-mediastinal neuroblastoma. A 7-month-old boy was pointed out to have Horner syndrome (right ptosis and anhidrosis) and an abnormal shadow at right cervico-mediastinum on chest X-ray film during his stay in another hospital because of acute bronchitis. On examination after admission in our hospital, 3 x 4 cm size mass was palpated at the right neck and urine VMA and HVA level were elevated. The close examination demonstrated no sign of tumor in any other organ and tissue, which suggested that the tumor was cervical origin. 9 x 5, 5 x 3 cm tumor was almost removed and patient received chemotherapy. He is now free from disease at 1 year after operation.
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PMID:[Experience with a operated cervico-mediastinal neuroblastoma in infant]. 830 10

Cervical neuroblastoma, a disease primarily of infants, has a favorable prognosis. Eleven patients are reported. Clinical presentations (other than mass) included stridor and swallowing problems. Masses when felt were commonly mistaken for infectious adenitis. Imaging studies (US, CT, MRI) showed solid masses with vascular displacement and narrowing; intraspinal extension was absent though extension into the adjacent sites of mediastinum and skull occurred. Horner syndrome was seen in five patients with accompanying heterochromia iridis in one. Five tumors had calcification. A high index of suspicion will lead to biopsy and less delay in diagnosis once a mass is felt or imaged.
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PMID:Cervical neuroblastoma in eleven infants--a tumor with favorable prognosis. Clinical and radiologic (US, CT, MRI) findings. 841 48

Although neuroblastoma is a relatively common malignancy of childhood, it is rarely of cervical origin. We encountered two cases of primary cervical neuroblastoma in infants. The first case was in a newborn who presented with noisy breathing, dyspnea, and choking on feeding soon after birth. Physical examination disclosed a left retropharyngeal mass near the midline causing aerodigestive compromise. Magnetic resonance imaging showed a well-defined hyperintense mass in the corresponding region. The infant underwent complete surgical excision of the mass 1 month after birth. The pathology report confirmed neuroblastoma. Horner's syndrome developed soon after surgery but resolved spontaneously within 6 months. No recurrence was noted during a 3-year follow-up period. The second patient, a 5-month-old female, presented with stridor and shortness of breath of 2-weeks' duration due to a right cervical mass. Magnetic resonance imaging showed a parapharyngeal mass suggestive of neuroblastoma. Complete surgical excision was done at the expense of sacrificing the sympathetic nerve trunk. The pathologic diagnosis was moderately differentiated neuroblastoma. No recurrence was noted when the patient was seen 2 years after surgery, although right-sided Horner's syndrome persisted. Surgical intervention can result in a good prognosis for low stage cervical neuroblastomas; however, long-term follow-up is important.
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PMID:Primary cervical neuroblastoma in infants. 926 64

Neuroblastoma is one of the most intensely studied solid malignancies that affect the pediatric age groups; its clinical presentation, treatment strategies and ultimate prognosis vary greatly. The biologic and genetic character of each tumor has an important impact on disease behavior, and clinical staging now incorporates these factors to generate an overall therapy plan. The clinical presentation of neuroblastoma is related to primary tumor location, production of metabolically active substances, and the presence of metastatic disease. There are also prognostically important associated syndromes including opsoclonus-myoclonus, Horner's syndrome, neurofibromatosis, and a variety of other neurocristopathies. The histologic features of the tumor are of prognostic significance and are utilized in treatment stratification. The International Neuroblastoma Staging System (INSS) has unified classic clinical staging. Features at diagnosis and those determined by initial operation are combined with biologic prognostic factors to achieve risk group assignment for virtually all patients. There are groups of children in which limited therapy is curative and intermediate-risk situations where standard multimodality treatment provides favorable outcomes. Unfortunately, there are many patients with high-risk disease that require intensive strategies, but success is still limited. It is in these most resistant patients that innovative approaches are being undertaken and novel strategies are being investigated.
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PMID:Current aspects of biology, risk assessment, and treatment of neuroblastoma. 998 66

Cervical neuroblastoma (CNB) is relatively rare, accounting for less than 5% of these tumors. Because it arises from the cervical sympathetic chain, complete resection will leave the child with Horner's syndrome in a high proportion of cases. Advances in technology have allowed for the development of diagnostic and imaging modalities more specific to the disease. One of these has been the advent of radiolabeled meta-iodobenzylguanidine (MIBG) to assess the primary tumor and focal metastatic involvement. This nuclide is also taken up by normal salivary-gland tissue; this may be altered, however, in the presence of sympathetic denervation. We present a case of a primary CNB associated with Horner's syndrome, which led to confusion in interpretation of the subsequent MIBG scan. We alert the reader to potential pitfalls in the use of this examination in this disease entity.
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PMID:Pitfalls in the interpretation of MIBG scans in cervical neuroblastoma. 1095 91

Neuroblastic tumors are the third most common cause of solid tumors in early childhood. Cervical tumors account for only 5% of cases. In this report, we describe a series of four pediatric neuroblastic tumors of the neck. The histological diagnosis was ganglioneuroblastoma in three cases and neuroblastoma in one case. Presenting signs were solitary cervical mass in two cases and respiratory distress in association with Claude-Bernard Horner's syndrome in two cases. Mean age at presentation was 15 months. Cervical computed tomography scan and/or magnetic resonance imaging depicted calcifications within the tumor in 50% of cases and allowed accurate assessment of extension. Increased urine catecholamine levels were observed only in the patient with neuroblastoma. Scintigraphy with [131]iodine-methyliodobenzylguanidine demonstrated selective uptake by the tumor in two cases. Amplification of N-myc oncogene, a documented unfavorable prognostic sign, was not found in any case. Surgical treatment was performed in all patients. Neoadjuvant chemotherapy was performed in one case. All patients underwent regular surveillance. No evidence of recurrence has been observed with a mean follow-up period of 7 years.
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PMID:Primary pediatric neuroblastic tumors of the neck. 1151 94


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