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)

Cutaneous ganglioneuroma is rare. Only five cases have been reported, and in all patients the lesions developed after birth. We describe a congenital cutaneous ganglioneuroma. The differential diagnosis includes well-differentiated cutaneous metastases of neuroblastoma and ganglion cells entrapped by a neurofibroma in patients with neurofibromatosis.
J Am Acad Dermatol 1996 Aug
PMID:Primary congenital cutaneous ganglioneuroma. 869 26

Cutaneous metastases of internal malignancies are very rare in children. In this group, neuroblastoma, leukaemia and lymphoma are the most common malignancies that may develop metastases or neoplastic infiltrates to the skin. Carcinomas have infrequently been reported in children, and cutaneous metastases from carcinoma in this group have not been described. A 10-year-old girl presented with an erythematous plaque on the left hemithorax. Histopathological findings revealed grouped signet-ring cells within the lumina of lymphatic vessels in the dermis. Immunohistochemical examination confirmed the epithelial origin of the tumour. Despite an exhaustive search, the primary site could not be determined. This exceptional observation is, to the best of our knowledge, the first report of cutaneous metastasis from occult carcinoma in a child.
Br J Dermatol 1998 Jan
PMID:Cutaneous metastasis from a presumed signet-ring cell carcinoma in a 10-year-old child. 953 39

Neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.
Br J Dermatol 2000 Mar
PMID:Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma. 1073 67

Mast cells are suggested to participate in regenerative processes, but their influence on epithelialization and wound healing has not been well studied. Since mast cells can be found in contact with epidermis in chronic inflammatory skin diseases and venous ulcers, the effect of mast cells on keratinocyte growth was studied. Keratinocytes were cultured in serum-free conditions with (complete medium) or without (basal medium) epidermal growth factor (EGF) and bovine pituitary extract (BPE) to reach subconfluence in a 24-well plate, and the cells were treated with different mast cell mediators histamine, heparin and tryptase, or lysate from HMC-1 cells, a human leukemic mast cell line. Whole skin cultures were used as a model for in vitro wounds to study the effect of mast cells on epithelial outgrowth from skin specimens. Histamine inhibited 3H-thymidine incorporation of keratinocytes dose-dependently by 29% at 1 mM, and 89% at 5 mM histamine. In whole skin culture, histamine inhibited epithelial outgrowth dose-dependently by 64% already at 0.1 mM histamine and maximally (91%) at 1 mM histamine. Heparin inhibited 3H-thymidine incorporation dose-dependently by up to 33% at 2 microg/ml in the absence, but not in the presence, of EGF/BPE. In contrast, in whole skin culture, heparin first inhibited the epithelial outgrowth by up to 27% at 2 microg/ml, but then reversed the inhibition to 30% stimulation at 200 microg/ml. Skin tryptase (0.0285 to 2.85 microg/ml) with or without heparin (0.5 to 20 microg/ml) did not affect thymidine incorporation in keratinocytes. Lysate from HMC-1 cells, but not that from control, neuroblastoma cells, inhibited 3H-thymidine incorporation in keratinocytes dose-dependently, and maximal (47%) inhibition was reached with 16,700 lysed HMC-1 cells/ml. In whole skin culture, HMC-1 lysate inhibited the epithelial outgrowth by up to 36% at 67,000 lysed cells/ml. The results show that mast cells and their mediators are inhibitory to keratinocyte 3H-thymidine incorporation and epithelial outgrowth in vitro, although, the inhibitory effect of histamine was seen at high concentrations suggesting a requirement for close morphologic vicinity of mast cells to keratinocytes. Thus, mast cells are assumed to control epidermal regeneration and to impair epithelialization of chronic ulcers.
Exp Dermatol 2001 Jun
PMID:Inhibition of keratinocyte growth in cell culture and whole skin culture by mast cell mediators. 1138 Jun 14

The hair cycle is an extraordinarily complex process relying on spatially and temporally coordinated integration of intercellular signaling, cell division and death, cell migration, and gene expression. The hairless gene (hr) is expressed with hair-cycle-dependent kinetics, and pathogenic mutations in hr are responsible for the hairless and rhino phenotypes in mice and atrichia with papular lesions in humans. In addition to its expression in the skin and hair follicle, hr is also highly expressed in the brain, yet the factors governing its differential cell-type-specific expression have not yet been defined. A thyroid hormone responsive element was previously identified in the rat hr promoter which confers thyroid hormone (T3) responsiveness to heterologous promoter constructs; however, prior studies have not focused on the hr promoter itself. The hairless promoter was cloned, and it is shown that the hr promoter is transactivated by T3 in neuroblastoma cells but not in keratinocytes. Therefore, while T3 has a significant role in the regulation of neuronal expression of hairless, its upregulation in keratinocytes is T3 independent. Furthermore, hr is subject to cell-type-specific negative autoregulation, inhibiting the activity of its own promoter in keratinocytes but not neuroblastoma cells. These findings illustrate a molecular distinction between the regulation of hr expression in defined cell populations.
Exp Dermatol 2004 Apr
PMID:The hairless promoter is differentially regulated by thyroid hormone in keratinocytes and neuroblastoma cells. 1508 42

Previous studies have shown frequent mutations in the BRAF (V-raf murine sarcoma viral oncogene homolog B1) or NRAS (neuroblastoma RAS viral [V-ras] oncogene homolog) genes in cutaneous melanoma, but the relationship between these alterations and tumor cell proliferation has not been examined in human melanoma. In our study of 51 primary nodular melanomas and 18 paired metastases, we found mutations in BRAF (codon 600, previously denoted 599) in 15 primary tumors (29%) and eight metastases (44%). The figures for NRAS mutations were 27% and 22%, respectively. Mutations in BRAF and NRAS genes were mutually exclusive in all but one case, and were maintained from primary tumors through their metastases. Mutations, however, were not associated with tumor cell proliferation by Ki-67 expression, tumor thickness, microvessel density, or vascular invasion, and there were no differences in patient survival. Although BRAF and NRAS mutations are likely to be important for the initiation and maintenance of some melanomas, other factors might be more significant for proliferation and prognosis in subgroups of aggressive melanoma.
J Invest Dermatol 2005 Aug
PMID:BRAF and NRAS mutations are frequent in nodular melanoma but are not associated with tumor cell proliferation or patient survival. 1609 42

Frequent somatic mutation of v-raf murine sarcoma viral oncogene homolog B (BRAF), a downstream effector of the rat sarcoma oncogene (RAS) signaling pathway, is described in melanoma and other tumors. Our analysis of melanoma cell lines suggests that activating mutations in BRAF can occur simultaneously with inactivation of phosphatase and tensin homolog (PTEN), but neuroblastoma RAS (NRAS) mutations are not coincident. We determined the concurrent prevalence of mutations in BRAF and NRAS, and alteration of PTEN expression in 69 primary cutaneous melanomas. BRAF mutations were seen in 57% of cases. NRAS was mutated in 17% of samples, exclusively in exon 2. Two cases showed concurrent BRAF and NRAS mutations. Using immunohistochemistry, PTEN protein expression was lost or greatly reduced in 19% of tumors. Seven tumors with reduced PTEN yielded DNA amenable to sequencing, and three also showed mutation in BRAF but none in NRAS. In all, 11 (85%) of 13 tumors showing reduced PTEN expression were greater than 3.5 mm thick, and the association of increasing Breslow thickness and loss or reduction of PTEN expression was statistically significant (P<0.0001). Mutations in NRAS were not coincident with reduced PTEN expression, and the concurrent mutation of NRAS and BRAF was rare.
J Invest Dermatol 2006 Jan
PMID:Examination of mutations in BRAF, NRAS, and PTEN in primary cutaneous melanoma. 1641 31

The most widely used retinoids include topical tretinoin (Retin-A), adapalene (Differin), topical tazarotene (Tazorac), isotretinoin (Accutane), and acitretin (Soriatane). This article will review new uses and developments in tazarotene (its failure to secure FDA approval in oral form for psoriasis), adapalene (its new 0.3% gel form and use in rosacea), alitretinoin (its use in photoaging), bexarotene (its use for psoriasis and chronic hand dermatitis), isotretinoin (the IPledge program, its use for neuroblastoma and branded formulation pharmacological superiority to generics), and retinoic acid metabolism-blocking agents (RAMBAs) (liarazole use for ichthyosis and psoriasis).
J Drugs Dermatol 2006 Oct
PMID:Schools of pharmacology: retinoid update. 1703 62

We report a 6-year-old girl with a subtle form of hypohidrotic ectodermal dysplasia and a phenotype consisting of curly hair, a round face, a stocky build, and obesity, which was associated with intrathoracic neuroblastoma. Although this new association could be a chance occurrence, its description may alert physicians to look for similar combinations and report these, as it may lead to better syndrome delineation, and patient care.
Pediatr Dermatol
PMID:Hypohidrotic ectodermal dysplasia and intrathoracic neuroblastoma. 1754 78

A subcutaneous mass arising in the right gluteal area of an 11-year-old female shih tzu dog was surgically excised. Histologically, the mass was composed of small round or ovoid neoplastic cells that were arranged in nests of various sizes. The neoplastic cells generally had hyperchromatic nuclei and scanty eosinophilic cytoplasm, and were surrounded by a pale pink fibrillar area. Immunohistochemically, the neoplastic cells were positive for vimentin, S-100 protein, neurone-specific enolase and synaptophysin, but negative for cytokeratin, neurofilament protein, glial fibrillary acidic protein and chromogranin A. On ultrastructural observation, aggregates of thin cytoplasmic processes were frequently seen among the neoplastic cells. Based on these features, the tumour was diagnosed as a neuroblastoma. To the authors' knowledge, this is the first description of a neuroblastoma originating from the skin in an adult dog.
Vet Dermatol 2010 Aug
PMID:Primary neuroblastoma in the skin of an adult shih tzu dog. 2023 May 84


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