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)

The authors present the case history of a child with neuroblastoma and early infiltration of the orbit accompanied by bilateral blindness. The typical clinical signs developed later. The thickening of the periosteum infiltrated by the tumor in the lateral wall of the orbit was demonstrated by computerized tomography. This may be regarded as an early sign of infiltration of the orbit by the neuroblastoma. In the case reported here a severe inflammatory reaction developed in the left orbit and anterior segment during massive chemotherapy and melted the cornea.
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PMID:[Pediatric neuroblastoma with early bilateral blindness]. 335 84

Malignant small cell tumor of the thoracopulmonary region (MSCT) was first described in 1979 and has been referred to as the Askin tumor. This malignant neoplasm is a member of the peripheral primitive neuroectodermal tumor (PPNET) family and typically involves the periosteum, soft tissue, and extrapulmonary tissue of the thoracic wall. MSCT may also involve the lung parenchyma by local extension or may arise de novo in peripheral lung tissue. Local recurrence, abdominal involvement by tumor extravasation across the diaphragm, and skeletal metastatic disease are relatively common. However, metastasis to the head and neck region and in particular to the oral cavity is extremely rare. We present a recurrent intrapulmonary MSCT with metastasis to the oral cavity in an adolescent Hispanic boy, and review the literature regarding this member of the PPNET family. Differentiation from neuroblastoma may be made based on immunoreactivity for beta 2 microglobulin and HBA71 and lack of immunoreactivity for chromogranin in PPNET and MSCT. Ultrastructural features commonly seen in MSCT and PPNET are round to ovoid tumor cells with occasional cytoplasmic processes with relatively few pleomorphic dense core granules. These tumors lack the gangliocytic and Schwann cell differentiation that is characteristic of neuroblastoma. MSCT and PPNET have a common reciprocal cytogenetic translocation [t(11;22)q(24;q12)], which is shared with Ewing's sarcoma. Prognosis in MSCT is quite dismal, with a 2-year survival of 38% and a 6-year survival of only 14%.
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PMID:Recurrent intrapulmonary malignant small cell tumor of the thoracopulmonary region with metastasis to the oral cavity: review of literature and case report. 757 Oct 88

A combination of computed tomography (CT) and magnetic resonance imaging (MRI) is now established as the optimum assessment of sinonasal malignancy. CT and MRI are of particular value in assessing the skull base, orbit and pteryo-palatine and infratemporal fossae. Although MRI offers better differentiation of tumour from surrounding tissue and fluid, coronal CT is still required for the demonstration of bone erosion particularly in the region of the cribriform plate. Thus the extent of local tumour spread may be determined with a degree of accuracy in excess of 98 per cent. However, the final determinant of penetration of the dura and orbital periosteum requires per-operative frozen section assessment. A knowledge of the tissue characteristics and site of origin can be of value in distinguishing some of the commoner sinonasal malignancies such as squamous cell carcinoma, adenocarcinoma, adenoid cystic carcinoma, olfactory neuroblastoma and chondrosarcoma. Imaging, particularly MRI also plays an important role in the post-therapeutic follow-up of patients, indicating areas of residual or recurrent disease, defining suspicious areas for biopsy. Post-operative surveillance is best achieved with three planar T1-weighted MRI, with, and without, gadolinium and axial T2-weighted sequences. The subtraction of the T1 pre- and post gadolinium T1 sequences can be of particular value in delineating recurrence.
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PMID:Optimum imaging for sinonasal malignancy. 1099 46