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)

Patients with adrenal tumours were identified (n = 412). Among them, 43% (176 patients) had primary and 57% (236 patients) had secondary tumours. Of the primary tumours, 71% were adenomas, but adrenal cortical carcinoma 6.8% (12 cases), phaeochromocytoma 9.7% (17 cases), neuroblastoma 6.2% (11 cases), ganglioneuroma 1.1% (2 cases) and myelolipoma 4% (7 cases) were also seen. Rare tumours like lipoma and haemangioma were also found. Most of the metastatic tumours were carcinomas (88.2%), mainly from lung (33.2%), stomach (15.9%) and oesophagus (17.3%).
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PMID:Adrenal tumours in Chinese. 163 45

Chromosome abnormalities found in pediatric solid tumors include deletions, translocations, homogeneously staining regions (hsr)/double minutes (dms), and ploidy abnormalities. The discovery of a 13q14 deletion found in lymphocytes of patients with retinoblastoma and developmental delay has led to the cloning of the retinoblastoma gene. Likewise the discovery of an 11p13 deletion in lymphocytes of patients with Wilms' tumor and aniridia has led to the cloning of the Wilms' tumor gene. Chromosome deletions found in tumor cells are considered to play a role on the homologous deletion of cancer suppressor genes. Recently, various translocations have been found mostly in soft tissue sarcomas; i.e. t(11;22) in Ewing's sarcoma, t(2;13) in alveolar rhabdomyosarcoma, t(3;8) in pleomorphic adenoma, t(3;12) in lipoma, t(12;16) in liposarcoma, t(12;14) in leiomyosarcoma, and t(X;18) in synovial sarcoma. These translocations provide important information on the difficult diagnosis of soft tissue sarcomas, and on the selection of chemotherapy protocol. Tumor cells in advanced stage neuroblastomas often show hsr/dms, in which N-myc amplification occurs. While near triploidy was regularly found in early-stage neuroblastomas, near-diploidy or near-tetraploidy was usually found in advanced stage tumors. Among various prognostic factors, N-myc copy numbers and tumor cell ploidies had the largest influence on the prognosis of neuroblastoma patients. Cytogenetic and molecular genetic analyses on tumor cells are becoming increasingly important for the diagnosis of pediatric solid tumors, and the prediction of the patients' prognosis.
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PMID:[Cytogenetics in pediatric solid tumors]. 217 98

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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PMID:Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. 631 Feb 27

Lesions from the SC region of children examined histologically at the RAHC were: 1. Malformations almost always associated with spina bifida aperta or occulta: 183 myelomeningocele (MM), 32 meningocele (M), 35 lipoMM and lipoma, 19 dermoid cyst, six occult meningocele, two Pacinian hamartoma, one short filum, four hindgut cysts or sinuses, two tailgut cysts, and two epithelial heterotopia. 2. Neoplasms, usually without spina bifida: 56 teratomas (11 malignant), five ependymomas (two purely subcutaneous), and 14 miscellaneous primary malignancies, (most neuroblastoma and rhabdomyosarcoma). Distinction between MM with glial tissue and M without glial tissue is important as M had a much better prognosis, less than a third developing hydrocephalus, and 77% walking unaided. Of those with glial tissue, the eight without Arnold-Chiari malformation were myelocystocele associated with cloacal exstrophy (six), caudal regression syndrome (one), and microcephaly (one). Postsacral glial tissue without paraplegia may occur with a subcutaneous vestige of filum terminale, or with herniation of the nonfunctioning half of a diplomyelia. Of postsacral "lipomas" and dermoids, 70% had an intraspinal connection through an occult spina bifida. This posterior vertebral defect is easily overlooked as the arches normally may not ossify until after 6 years. Therefore, the pathologist receiving a postsacral specimen may wish to alert the clinician to the high incidence of late effects from an occult intraspinal component or tethering of the spinal cord. Transsacral hindgut herniations and cysts probably result from ectoendodermal adhesions. Presacral multicystic malformations with mixed squamous and mucus cell lining are probably tailgut remnants or anorectal duplications, and may be mistaken for dermoid or teratoma. In SC teratoma in infants, contrary to some reports on ovarian teratoma in adults, immature tissues do not indicate a worse prognosis. Malignancy is virtually confined to teratomas including a carcinomatous or "yolk sac" component. It is more common in predominantly presacral examples and rare before the age of 4 months. SC ependymoma differs from ependymoma elsewhere in that it may be primary outside the craniospinal cavity (presacral or postsacral), may have a myxopapillary pattern special to the region, and although low-grade and slow growing, is more likely to metastasize beyond the central nervous system. Postsacral examples arise from vestiges of the filum terminale which are normal in the subcutis there. Combinations of all these lesions occur with vertebral defects and with each other.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Sacrococcygeal developmental abnormalities and tumors in children. 636 33

The authors would like to recount and discuss the case of a 14 months old child which presented a dumbbell dorsal lipoma, purely extradural with no congenital anomalies. The warning signal was the discovery of paraparesis with the regression of walking ability. The hypothesis of medullary compression by a dumbbell dorsal tumor was quickly dismissed with the discovery of a mediastinal mass on the pulmonary X-Ray. A diagnosis of the pre-operational neuroblastoma was carried out because of detection of dorsal epidural medullary compression revealed by the myelography. The testing was completed with a vertebral computed tomography, which clearly showed the extent of medullar compression, and the mediastinal tumoral development under the paravertebral muscular masses. The child was operated on at 2 fronts, vertebrally and thoraxically for an encapsulated, perfectly separable and non hemorrhaging tumor. The anatomopathological examination confirmed the diagnosis of the lipoma. It is in the resumption of the computed tomographic examination that the tumoral density measures will confirm fatty tissue with numbers of density--50. In light of the literature, we are discussing the appearance of the lipoma, entirely exceptional because of its dorsal location, its extradural and certainly its dumbbell position, with large thoraxic development. It is necessary to stress the importance of the meticulous neurological examination of the child, the diagnostical necessity of the CT scan and the importance of the avoidance of the creation of an instable vertebral lesion at the time of laminotomy. In this case only the early double surgical operation permitted the recovery of the child.
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PMID:[Dorsal extradural hourglass lipoma in children]. 673 27

Eighteen patients (3 men and 15 women; mean age 63 years) with right-sided tumors were evaluated by both transthoracic and transesophageal echocardiography from 1989 to 1996. The indications for echocardiographic studies included evaluation for a presumed mass and further evaluation of ventricular function and valvular function. Fifteen patients had right atrial tumors. These included 5 hypernephromas, 4 myxomas, 2 angiosarcomas, 1 lipoma, 1 cavernous hemangioma, 1 hepatoma, and 1 chondrosarcoma. Three patients had right ventricular (RV) tumors: 1 metastatic olfactory neuroblastoma, a leiomyosarcoma, a chondrosarcoma, and a fourth patient had infiltration of the RV free wall of unknown etiology. Biopsy of either right atrial or RV masses was performed with transesophageal echocardiographic guidance in 2 patients, and allowed histologic diagnosis before surgical resection. These findings indicate that tumors are more often found in the right atrium than in the right ventricle, and females predominate. Most tumors arising within the right atrium are benign, whereas those extending into the right atrium from outside are malignant. RV tumors are rarely encountered; when present, they are likely to be malignant.
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PMID:Right-sided cardiac tumors detected by transesophageal echocardiography and its usefulness in differentiating the benign from the malignant ones. 907 May 59

Cervicothoracic lesions are not uncommon in children. All cervicothoracic lesions except superficial lesions extend from the neck to the thorax through the thoracic inlet. Evaluation of this area involves multiple imaging modalities: plain radiography, ultrasonography, nuclear medicine, computed tomography, and magnetic resonance (MR) imaging. However, MR imaging is the method of choice for assessing the full extents of cervicothoracic lesions and their relationships to neurovascular structures. Cervicothoracic lesions can be classified as congenital lesions, inflammatory lesions, benign tumors, malignant tumors, and traumatic lesions. Lymphangioma is the most common cervicothoracic mass in children; other congenital lesions include hemangioma, thymic cyst, and vascular anomalies. Inflammatory adenopathy reactive to tuberculosis, mononucleosis, tularemia, cat-scratch fever, infection with human immunodeficiency virus, or other upper respiratory tract infections can manifest as cervicothoracic lesions; tuberculous abscesses and abscesses of other origins can also be seen. Lipoma, lipoblastoma, aggressive fibromatosis, and nerve sheath tumors (either isolated lesions or those associated with neurofibromatosis) can also occur as cervicothoracic masses. Malignant cervicothoracic tumors include lymphoma, thyroid carcinoma, neuroblastoma, and chest wall tumors (rhabdomyosarcoma, Ewing sarcoma, and neuroectodermal tumor). Traumatic cervicothoracic lesions include pneumomediastinum of traumatic origin, traumatic pharyngeal pseudodiverticulum, esophageal foreign-body granuloma, and cervicothoracic hematoma.
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PMID:Cervicothoracic lesions in infants and children. 1033 90

Low-affinity nerve growth factor receptor (p75) is a member of the tumor necrosis factor receptor family. It may modulate the binding of nerve growth factor (NGF) to the functional high-affinity receptor tyrosine kinase (trk) A. NGF is thought to be responsible for growth, apoptosis, and function of the nervous system. The presence of this receptor (p75) was determined in a large group of neural and nonneural tumors and fetal and adult tissues. One thousand one hundred fifty tumors were analyzed with monoclonal antibody for p75, along with selected normal fetal and adult tissues. Immunoreactivity for p75 was present in adult pericytes, perivascular fibroblasts, basal cells of several types of epithelia, perineurial cells, and dendritic reticulum cells. Additionally, a wide zone of subepithelial mesenchyme and skeletal muscle were positive in the first-trimester fetus, but were diminished or negative in the adult. Consistently positive nonneural mesenchymal tumors included dermatofibrosarcoma protuberans (DFSP), embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and spindle cell hemangio(endotheli)oma. Schwann cell tumors, ganglioneuroma, granular cell tumor, and malignant peripheral nerve sheath tumor (MPNST) were also p75 positive. Mesenchymal nonneural tumors that were variably positive (32% to 69%) for p75 included fibrosarcoma variants, solitary fibrous tumor, hemangiopericytoma, spindle cell lipoma, Ewing's sarcoma, mesenchymal chondrosarcoma, and malignant melanoma. Nervous system tumors such as paragangliomas, neuroblastoma, meningioma, and perineurioma and nonneural mesenchymal tumors, including extraskeletal osteosarcoma, benign fibrous histiocytomas, fibromas, alveolar soft part sarcoma, epithelioid sarcoma, smooth muscle and gastrointestinal stromal tumors, and angiosarcomas, were almost always negative for p75. Epithelial tumors that were consistently positive included mixed tumor and adenoid cystic carcinoma, whereas mesothelioma, adenocarcinomas, and most squamous cell carcinomas were negative. p75 is not a specific marker for nerve sheath tumors. It is present in a variety of other mesenchymal tumors including synovial sarcoma and in CD34-positive tumors such as DFSP, spindle cell lipoma, and hemangiopericytoma. The presence of p75 in nonneural tumors such as DFSP and rhabdomyosarcoma mimic its presence in early fetal mesenchyme and skeletal muscle, suggesting oncofetal expression in these tumors. p75 may be useful to distinguish DFSP from benign fibrous histiocytoma.
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PMID:Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues. 1156 28

Chest wall lesions in childhood include a wide range of pathologies. Benign lesions include lipoma, neurofibroma, lymphangioma, haemangioma and mesenchymal hamartoma. Malignant lesions include neuroblastoma, rhabdomyosarcoma, Ewings sarcoma, Askin tumour and primitive neuroectodermal tumours. Manifestations of systemic diseases such as leukaemia, lymphoma, Langerhans cell histocytosis and infections such as tuberculosis and actinomycosis may also cause chest wall lesions. The imaging characteristics of the above are reviewed but only a minority of lesions show diagnostic imaging characteristics. Most lesions require biopsy and histopathological examination for definitive diagnosis. The role of different imaging modalities is discussed, with an emphasis on magnetic resonance imaging for demonstrating lesion morphology and local spread, with computed tomography and nuclear medicine being used mainly to assess remote disease.
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PMID:Chest wall lesions. 1245 4

This study was conducted to determine the outcome of patients who develop a second neoplasm after radiotherapy (RT) for a childhood solid tumor. From 1956 to 1998, 429 children with a malignant solid tumor were treated at a single radiation oncology facility. The medical records and radiotherapy charts were reviewed to determine if the patient developed a secondary neoplasm after treatment for malignancy. Twenty-three (5.4%) patients developed a secondary neoplasm. There were 12 males and 11 females with a median age at RT of 6.6 years (range, 2 months to 20 years). There were 14 malignant neoplasms in 13 (3.0%) and 14 benign neoplasms in 11 patients (2.6%). The types of initial solid tumors treated with RT were Ewing sarcoma in 6, Wilms tumor in 6, medulloblastoma in 5, neuroblastoma in 3, and other in 3. Median RT dose was 45 Gy (range, 12.3 to 60 Gy) using 4 MV in 9, 1.25 MV in 8, 250 KV in 4, and 6 MV photons in 1 patient. One child was treated using 15-MeV electrons. Fourteen had chemotherapy. Median follow-up was 23.2 years (range, 5.3 to 44.4 years). For the 14 malignant neoplasms, the median time interval from initial tumor to second malignancy was 10.1 years. The 14 second malignant neoplasms (SMN) were osteosarcoma in 3, breast carcinoma in 2, melanoma in 2, malignant fibrous histiocytoma in 1, dermatofibrosarcoma in 1, leiomyosarcoma in 1, mucoepidermoid carcinoma in 1, colon cancer in 1, chronic myelogenous leukemia in 1, and basal cell carcinoma in 1. Ten of the 14 SMN (71%) were at the edge or inside the RT field. The 5- and 10-year overall survival rate after diagnosis of an SMN was 69.2%; it was 70% for children with a SMN at the edge or inside the RT field and 66.7% for those outside of the RT field. The 14 benign neoplasms appeared at a median time of 16.9 years and included cervical intraepithelial neoplasia in 3, osteochondroma in 3, thyroid adenoma in 1, duodenal adenoma in 1, lipoma in 1, cherry angioma in 1, uterine leiomyoma in 1, ovarian cystadenofibroma in 1, and giant cell tumor in 1. Only 5 (36%) of the 14 benign tumors occurred in the RT field, with osteochondroma being the most common. Of 189 deaths occurring in 429 patients, only 3 (1.6%) were secondary to radiation-induced malignancy. Not all SMN in children receiving RT occur in the irradiated field. More than two-thirds of children with a radiation-induced malignancy are alive 10 years after the diagnosis of a SMN.
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PMID:Secondary neoplasms after radiotherapy for a childhood solid tumor. 1580 94


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