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)

We here present a review of ten pediatric patients with rib tumors that were treated in our hospital since 1970: a juvenile bone cyst, an aneurysmal bone cyst, an eosinophilic granuloma, a Ewing sarcoma, a metastasis of a neuroblastoma, an osteochondroma, a hemangiopericytoma, and a callous tumor at the site of a previous single rib fracture. The relative frequency of rib tumors is similar to that of bone tumors in other locations. A biopsy of the affected bone should be the first step of the operative intervention. As demonstrated in a case of eosinophilic granuloma, the indication for subperiosteal rib resection can be defined generously. A completed regrowth of the rib defect can be expected. Even the loss of a complete rib is generally well tolerated, as shown in an infant with an aneurysmal bone cyst, a rare disease in this age group. Malignant tumors of the chest wall, however, require special experience in thoracic surgery and necessitate close interdisciplinary cooperation. Complete primary resection of a malignant rib tumor and its plastic reconstruction is demonstrated in a case of a Ewing sarcoma in childhood. In children and adolescents a vicryl net should generally be used to reconstruct extended chest wall defects reliably and functionally stable.
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PMID:[Tumors of the ribs in children]. 208 62

Ninety-one patients with parasellar lesions proved by pathology and surgery from July 1982 to May 1987 and 22 patients misdiagnosed clinically or by CT are reported. The authors found that: 1. Besides the main group of parasellar lesions such as pituitary tumor, meningioma, and craniopharyngioma, some rare disease like glioma, pituitary carcinoma, chordoma, olfactory neuroblastoma, Rathke's pouch and tuberculoma of optic nerve were also found which comprised 12% (11/91) in this series. They should be considered in differential diagnosis; 2. Correct diagnosis was made when CT findings conformed well with the clinical features. Over-emphasis of clinical features or neglect of CT findings should be discouraged as they may lead to erroneous diagnosis; 3. In this series, the highest correct diagnosis rates were: pituitary tumor (95%), parasellar meningioma (78%) and craniopharyngioma (50%). It is difficult to make a correct diagnosis for these rare disease entities before operation; and 4. CT scan cannot completely replace angiography and ventriculography when they are needed for differential diagnosis.
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PMID:[CT scan and clinical diagnosis of parasellar lesions--analysis of 91 cases]. 262 13

The authors report a new case of infantile myoclonic encephalopathy with opsoclonus and neuroblastoma in a 14 months-old infant. Some immunologic abnormalities were found at the initial course of the disease. The review of 110 cases of "dancing-eyes syndrome" permit them to specify clinical features and prognosis but cannot explain the pathogenesis of this rare disease.
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PMID:[Opsomyoclonus syndrome in children. A new case. Review of the literature (110 cases)]. 383 36

The neuroblastoma in the adult is a rare disease which has a bad prognosis. Until now, there are no generally accepted therapy conceptions. The clinical symptoms of the patient whose case is presented here were above all pains in the pelvic region. The histologic diagnosis was difficult and could be proved only by additional examinations of other metastases. Although the primary tumor was searched for intensively, is was only found 20 months later. Especially radiotherapy, but also chemotherapy (CYVADIC regimen) have proved to be effective. Above all, the quality of life could be largely maintained over a period of 26 months.
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PMID:[Diagnosis and radio-chemotherapy of the neuroblastoma in adults]. 619 Feb 73

Infant botulism is a rare disease caused by the release of toxin produced in the intestinal tract by Clostridium botulinum. The disease primarily affects infants under 1 year of age. We report a 3-year-old child with stage IV neuroblastoma who developed symptoms of progressive motor weakness, bulbar palsy and respiratory failure 42 days after autologous BMT. The diagnosis of infant botulism was established by identifying botulism toxin in the stool. Human botulism immune globulin (HBIG) was administered. Following the diagnosis, the patient made significant recovery over the next 7 weeks and was successfully extubated from mechanical ventilation. However, her neuroblastoma eventually recurred and she subsequently died of progressive disease. Although the etiology of the development of infant botulism in this case following autologous BMT still remains unclear, alteration of the intestinal microbial environment from gut sterilization and laminar airflow room isolation or, alternatively, immune suppression during pre- and post-autologous BMT and activation of endogenous spores may have contributed to the development of this disease. The use of HBIG in children with botulism over 1 year of age may be beneficial.
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PMID:Development of infant botulism in a 3-year-old female with neuroblastoma following autologous bone marrow transplantation: potential use of human botulism immune globulin. 819 79

Subfrontal schwannoma is a rare disease, which can be mis-diagnosed as an olfactory meningioma or a neuroblastoma, because of similar clinical symptoms and signs and neuroradiological features. Especially for young subjects, olfactory neuroblastoma should be carefully differentiated, since the management strategies for those lesions are significantly different. The craniofacial approach is often needed for the resection of a neuroblastoma. We report a case of 14-year old boy in which olfactory neuroblastoma was suspected prior to surgery, but turned out to be a schwannoma histologically. Molecular genetic examination revealed neither NF2 gene mutation nor loss of heterozygosity of chromosome 22q, unlike common schwannomas.
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PMID:Subfrontal schwannoma. 1582 81

The ganglioneuroma is a benign neuroblastal tumor. All neuroblastomas and ganglioneuromas derive from immature cells of the sympathetic nerve system. The ganglioneuroma is a very rare disease and effects newborns and infants more often than adolescents and adults. The benign tumors are relatively difficult to diagnose since they usually are asymptomatic. A 5-year old girl with persistent bladder wall thickening and prominent course of the prevesical ureter presents for diagnosis and therapy. Sonography demonstrated a space-occupying lesion of the left kidney. Subsequent MRI raises the suspicion of a retroperitoneal neuroblastoma. The tumor could be removed in two surgical sessions. The ganglioneuroma is a benign tumor with symptoms depending on location and with a relatively good prognosis. Its low incidence and resultant limited experience often leads to delayed diagnosis, potentially determining therapy and diagnosis.
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PMID:[Retroperitoneal ganglioneuroma]. 1611 Apr 9

Adrenal abscess is a rare disease in the neonatal period. The classical symptoms are abdominal mass, anaemia and prolonged jaundice which are associated with fever, vomiting and feeding difficulties. The authors present the histories of two mature, male newborns with adrenal hemorrhage, where the size of the masses increased progressively and finally adrenal abscesses were developed. The first case showed the classical symptoms of adrenal abscess, however, the antibiotic treatment led to complete remission of the symptoms and laboratory infectious markers, therefore the baby was discharged. In a few day later he became septic and was admitted again. This time he was operated on and a large abscess was evacuated, which contained about 60 ml of pus. After this intervention multi-organ failure developed, but due to the intensive therapy the baby recovered. In the second newborn the increasing adrenal hemorrhage was accompanied by elevated infectious markers despite of the different antibiotic treatments, however, he showed no clinical signs of infection. Because of the discrepancies between the clinical and laboratory findings and also the imaging studies the possibility of neuroblastoma has also arisen. Urinary vanillylmandelic acid excretion was normal, but elevated neuron-specific enolase levels were measured. Surgical exploration was performed, pus was drained from the mass and anaerobic Bacteroides fragilis was cultured, therefore metronidazole therapy was started. After that the baby fully recovered.
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PMID:[Different clinical forms of adrenal abscess in two newborns]. 1698 18

Here we report a patient with stage-4 bilateral cystic adrenal neuroblastomas with disseminated liver metastases and consumption coagulopathy who underwent liver transplant. Our patient was initially diagnosed with infantile hepatic hemangioendothelioma and bleeding into the adrenal glands secondary to consumption coagulopathy (Kasabach-Merritt syndrome). Liver transplant was performed as a life-saving procedure under this diagnosis. We discuss this unique patient because of the diagnostic pitfalls of this rare disease and the successful clinical outcome after LT and subsequent chemotherapy for neuroblastoma.
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PMID:Liver transplant in an infant with bilateral cystic neuroblastoma complicated by hepatic metastases and life-threatening consumption coagulopathy. 1833 88

Carcinomas of the head and neck occur in both children and adults, but notable differences exist in their relative frequency, pathologic subtypes, etiologies, presenting symptoms, and late effects. In contrast, treatment strategies are similar depending on the disease type and distribution at the time of diagnosis. Thus, in adult patients, squamous cell carcinomas or one of its variants are the most common malignancies in the head and neck. However, in children, cancers of the head/neck are most commonly rhabdomyosarcomas, lymphomas, including Hodgkin's lymphoma, lymphoblastic lymphomas, and Burkitt's lymphoma or neuroblastoma. Epithelial cancers are unusual in the pediatric population, with the exception of nasopharyngeal carcinoma. Although nasopharyngeal carcinoma is a rare disease in children, representing less than 1% of childhood cancers, it does constitute 20%-50% of pediatric malignancies of the nasopharynx. This is one of the few malignant tumors in children that arise from the epithelium. Despite the differences between the diseases in children from that in adults, the management strategy has been based largely on the experience in adults. This review will describe the epidemiology, etiology, management, and late effects in children and adults, and offer explanations for both the similarities and differences.
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PMID:Head and neck carcinomas across the age spectrum: epidemiology, therapy, and late effects. 1995 31


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