Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinicopathologic, immunocytochemical, and electron microscopic features of a case of meningeal melanocytoma arising in the phylum terminale are reported. Meningeal melanocytoma is an uncommon tumor that must be distinguished from metastatic or primary malignant melanoma, meningeal melanocytic nevi, pigmented meningioma, pigmented schwannoma or neurofibroma, and pigmented primitive neuroectodermal tumor. This is a difficult differential diagnosis that can be best archived by complementing histological examination with a selected panel of antibodies and, most important, electron microscopic study. The distinctive ultrastructural appearance of most of these lesions point to this technique as the gold standard in pigmented proliferations of the nervous system.
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PMID:Meningeal melanocytoma: a case report and literature review. 980 60

Tumor-specific immunohistochemical markers are valuable in the differential diagnosis of malignant small round cell tumors (MSRCTs). The cone-rod homeobox-containing gene (CRX) is a transcription factor that is preferentially expressed in retinal photoreceptor cells. It has been shown that the CRX antibody is a good immunohistochemical marker to differentiate retinoblastoma from other intracranial MSRCTs. Outside of the central nervous system, however, the usefulness of CRX immunohistochemistry in establishing a diagnosis of metastatic retinoblastoma is uncertain, as the expression of CRX in primitive neuroectodermal tumor/Ewing sarcoma (PNET/ES), neuroblastoma, and other MSRCTs is unknown. Archival specimens from resections, core biopsies, and bone marrow biopsies of 41 neuroblastomas, 24 PNET/ES, 19 embryonal rhabdomyosarcomas, 17 alveolar rhabdomyosarcomas, 17 Wilms tumors, 14 desmoplastic small round cell tumors, 20 medulloblastomas, 9 pineal tumors, 17 melanocytic tumors (compound and Spitz nevi), and 8 retinoblastomas were immunostained for CRX. All retinoblastomas had strong diffuse nuclear immunoreactivity for CRX; 8 of the 20 medulloblastomas showed strong nuclear immunoreactivity either in occasional clusters of tumor cells or in rare single scattered tumor cells; 3 of the 9 pineal tumors showed strong nuclear immunoreactivity in approximately 40% to 50% of the tumor cells. Neuroblastomas, PNET/ES, embryonal rhabdomyosarcomas, alveolar rhabdomyosarcomas, Wilms tumors, desmoplastic small round cell tumors, and melanocytic tumors were all negative. Scant nonspecific cytoplasmic staining was observed in some tumors, mostly PNET/ES. These findings suggest that CRX is a useful marker to discriminate metastatic retinoblastoma from other, more common, MSRCTs of childhood.
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PMID:Immunohistochemical expression of CRX in extracranial malignant small round cell tumors. 2279 Aug 57