Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The answer to the question posed in the title, "Small Round Cell Neoplasms: Can Electron Microscopy and Immunohistochemical Studies Accurately Classify Them?", is obviously "yes"; but a qualified yes--generally yes, perhaps with expertise usually yes, but never just plain yes. Some cases certainly will defy the best attempts even of the most expert in the application of these "special" techniques. And embarrassing as it may be for those of us infatuated with the latest technology to admit, it is with the difficult case especially that old-fashioned technology so often must be depended upon. In his excellent recent appraisal of the role of a variety of special techniques in this application, Triche offers the following comment: "Overall, electron microscopy is probably the most universally useful of all diagnostic techniques other than light microscopy in round cell tumors." The data from our studies certainly point to the same conclusion. With each of the tumors, electron microscopy demonstrated itself to be more reliable than immunohistochemistry. Electron microscopy offers not only greater sensitivity and specificity, but also greater versatility. Immunohistochemistry allows hypothesis testing only. Electron microscopy, on the other hand, can provide answers even when the right questions are not being asked. For example, if a particular small round cell tumor under investigation happens in actuality to represent something other than the neuroblastoma which it is being considered (e.g., a granulocytic sarcoma, liposarcoma, Wilm's tumor, etc.), electron microscopy can reveal this fact, but a neuron-specific enolase stain cannot. Parenthetically, it should also be said that electron microscopy has proven particularly well suited to the examination of fine-needle aspiration specimens. The two spare many patients in our institution the need for a major operative procedure to establish a secure tissue diagnosis. Immunohistochemistry does have a role to play but it is, at least in our opinion, clearly secondary to that of electron microscopy. The concept of replacing electron microscopy with a battery of immunostains has often been advocated as an economic measure, but this argument begins quickly to lose its weight as the number stains included in the battery is increased to cover the diagnostic possibilities. Giving consideration to the capriciousness of some of these stains, there exists with this also an increasing possibility of a spurious or misinterpreted result leading to an errant diagnosis.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Small round cell neoplasms: can electron microscopy and immunohistochemical studies accurately classify them? 241 63

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

In the present study we review ENT tumor pathology in childhood. Only the most salient aspects are emphasized and the variety of entities reviewed was restricted. Molecular biology techniques reveal infection by human papilloma virus (types 6 and 11) in 50 % of papillomas, while immunohistochemical techniques are less effective in papilloma virus detection. The myofibroblastic nature of nasal angiofibroma has been demonstrated and its incidence is 25 times more frequent in patients with familial polyposis of the colon. Overexpression of p53 occurs in the initial stages of nasopharyngeal carcinoma, while overexpression of c-myc is correlated with an unfavorable prognosis. Recently, olfactory neuroblastoma has been shown not to express the protein product of the MIC-2 gene (antibody 12E7), thus the hypothesis that it could be a member of the Ewing tumor family (neuroectodermal peripheral tumors) has not been confirmed, although it is a primitive neural tumor. The head and neck rhabdomyosarcoma with the best prognosis is that located in the orbit, and cytogenetic studies have shown chromosomic translocation t(2;13) in 50 % of these childhood tumors when they are of the alveolar-type, while trisomy of chromosome 2 or 20 is more characteristic of the embryonic-type. Currently, any classifying features of ENT lymphomas must be based on the Revised European-American Classification of Lymphoid Neoplasms (REAL). Papillary and medullary carcinomas are the most common histological types of thyroid carcinoma in childhood. Alterations in ret/PTC play a significant role in the pathogenesis of both.
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PMID:[Advances in the diagnosis of ENT tumors in childhood]. 1272 79

Neoplastic abdominal tumours, particularly those originating from embryonal tissue (such as hepatoblastoma and nephroblastoma) and neural crest cells (such as neuroblastoma), are well-documented in young children. Neoplasms of adulthood, most commonly carcinoma of different visceral organs, are also well-documented. Abdominal tumours in adolescence constitute a distinct pathological group. The radiological features of some of these tumours have been described only in isolated reports. The purpose of this pictorial essay was to review the imaging findings of various kinds of abdominal tumours in adolescent patients (with an age range of 10-16 years) who presented to the Children Cancer Center of our institution in the past 15 years. Some tumours, though rare, have characteristic imaging appearances (especially in CT) that enable an accurate diagnosis before definite histological confirmation.
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PMID:A pictorial review of imaging of abdominal tumours in adolescence. 2060 98

Neoplasms of the sacrum are rare. Given the non-specific imaging findings in sacral lesions, the imaging-based differential diagnosis is always difficult. This case is about an adult with primary sacral neuroblastoma and we have discussed imaging and histopathological findings of this rare tumor.
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PMID:Primary osseous sacral neuroblastoma in an adult. 3026 6