Lipofibromatosis-like neural tumor (original) (raw)
Lipofibromatosis-like neural tumor (LPF-NT) is an extremely rare soft tissue tumor first described by Agaram et al in 2016. As of mid-2021, at least 39 cases of LPF-NT have been reported in the literature. LPF-NT tumors have several features that resemble lipofibromatosis (LPF) tumors, malignant peripheral nerve sheath tumors, spindle cell sarcomas, low-grade neural tumors, peripheral nerve sheath tumors, and other less clearly defined tumors; Prior to the Agaram at al report, LPF-NTs were likely diagnosed as variants or atypical forms of these tumors. The analyses of Agaram at al and subsequent studies uncovered critical differences between LPF-NT and the other tumor forms which suggest that it is a distinct tumor entity differing not only from lipofibromatosis but also the other tumor fo
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| dbo:abstract | Lipofibromatosis-like neural tumor (LPF-NT) is an extremely rare soft tissue tumor first described by Agaram et al in 2016. As of mid-2021, at least 39 cases of LPF-NT have been reported in the literature. LPF-NT tumors have several features that resemble lipofibromatosis (LPF) tumors, malignant peripheral nerve sheath tumors, spindle cell sarcomas, low-grade neural tumors, peripheral nerve sheath tumors, and other less clearly defined tumors; Prior to the Agaram at al report, LPF-NTs were likely diagnosed as variants or atypical forms of these tumors. The analyses of Agaram at al and subsequent studies uncovered critical differences between LPF-NT and the other tumor forms which suggest that it is a distinct tumor entity differing not only from lipofibromatosis but also the other tumor forms. LPF-NTs are locally invasive, are commonly treated by surgical excision, and have a relatively high rate of local recurrence if their surgical excisions are incomplete. They are generally considered to be benign, non-metastasizing (i.e. not spreading to other parts of the body) tumors. However, one case of LFT-NT reported by Agaram et al was associated with metastasis, apparently as a result of the tumor's cells transformation into a malignant sarcoma. Further studies are needed to determine the frequency of such cases and the overall metastatic potential of LPF-NT. LPF-NTs were given the "neural tumor" terminology because in at least some cases: 1) their tumor cells express S100 and CD34 but not SOX10 proteins, a pattern that is often found in neural and neuroectodermal tumor cells; and 2) their histopathology consists of tumor cell infiltrations into adipose tissues in a pattern that is very similar to that found in some low grade malignant peripheral nerve sheath tumors. (en) |
| dbo:medicalCause | dbr:NTRK1 |
| dbo:treatment | dbr:Radical_surgery |
| dbo:wikiPageID | 68573171 (xsd:integer) |
| dbo:wikiPageLength | 19640 (xsd:nonNegativeInteger) |
| dbo:wikiPageRevisionID | 1054168245 (xsd:integer) |
| dbo:wikiPageWikiLink | dbr:Benign_tumor dbr:Sarcoma dbr:MAPK/ERK_pathway dbr:Dermatofibrosarcoma_protuberans dbr:Dermatology dbr:Tropomyosin_3 dbr:Nucleoli dbr:Pediatric dbr:S100_protein dbr:Gene_expression dbr:Nervous_tissue dbr:Neuron dbr:PI3K/AKT/mTOR_pathway dbr:Entrectinib dbr:Antibody dbr:Malignant_peripheral_nerve_sheath_tumor dbr:Clinical_trial dbr:Deletion_(genetics) dbr:Fusion_genes dbr:Surgery dbr:CD34 dbr:Adjuvant_therapy dbc:Benign_neoplasms dbr:Aggressive_infantile_fibromatosis dbr:Fusion_gene dbr:Fusion_protein dbr:H&E_stain dbr:Karyotype dbr:Lipofibromatosis dbr:Locus_(genetics) dbr:Adipose_tissue dbr:Fibroma dbr:Fibrosarcoma dbr:Food_and_Drug_Administration dbr:Cell_division dbr:Cell_nucleus dbr:Cell_signaling dbr:Chromosomal_inversion dbr:Chromosomal_translocation dbr:Chromosome_1 dbr:Grading_(tumors) dbr:Histopathology dbr:Radical_surgery dbr:SOX10 dbc:Dermal_and_subcutaneous_growths dbc:Connective_and_soft_tissue_neoplasms dbr:LMNA dbr:Larotrectinib dbr:Trk_receptor dbr:Mitotic_index dbr:Metastasizing dbr:Necrosis dbr:Solitary_fibrous_tumor dbr:Neuroectoderm dbr:Tropomyosin_receptor_kinase_A dbr:Immunohistochemistry dbr:Soft_tissue dbr:Malignant_transformation dbr:Spindle_cell_sarcoma dbr:PLCG1 dbr:Peripheral_nerve_sheath_tumor dbr:Malignant_peripheral_nerve_sheath_tumors dbr:Chimeric_protein dbr:Lumbar_spine dbr:NTRK1 |
| dbp:causes | An NTRK1- containing fusion gene in ~80% of cases (en) |
| dbp:field | Pediatric or adult dermatology and surgery (en) |
| dbp:frequency | Very rare (en) |
| dbp:name | Lipofibromatosis-like neural tumor (en) |
| dbp:onset | 1.1991888E9 |
| dbp:prognosis | Good (en) |
| dbp:treatment | dbr:Radical_surgery |
| dbp:wikiPageUsesTemplate | dbt:Infobox_medical_condition_(new) dbt:Reflist |
| dcterms:subject | dbc:Benign_neoplasms dbc:Dermal_and_subcutaneous_growths dbc:Connective_and_soft_tissue_neoplasms |
| rdf:type | owl:Thing wikidata:Q12136 dbo:Disease |
| rdfs:comment | Lipofibromatosis-like neural tumor (LPF-NT) is an extremely rare soft tissue tumor first described by Agaram et al in 2016. As of mid-2021, at least 39 cases of LPF-NT have been reported in the literature. LPF-NT tumors have several features that resemble lipofibromatosis (LPF) tumors, malignant peripheral nerve sheath tumors, spindle cell sarcomas, low-grade neural tumors, peripheral nerve sheath tumors, and other less clearly defined tumors; Prior to the Agaram at al report, LPF-NTs were likely diagnosed as variants or atypical forms of these tumors. The analyses of Agaram at al and subsequent studies uncovered critical differences between LPF-NT and the other tumor forms which suggest that it is a distinct tumor entity differing not only from lipofibromatosis but also the other tumor fo (en) |
| rdfs:label | Lipofibromatosis-like neural tumor (en) |
| owl:sameAs | wikidata:Lipofibromatosis-like neural tumor https://global.dbpedia.org/id/G5hS1 |
| prov:wasDerivedFrom | wikipedia-en:Lipofibromatosis-like_neural_tumor?oldid=1054168245&ns=0 |
| foaf:isPrimaryTopicOf | wikipedia-en:Lipofibromatosis-like_neural_tumor |
| foaf:name | Lipofibromatosis-like neural tumor (en) |
| is dbo:wikiPageWikiLink of | dbr:Lipofibromatosis dbr:Fibroblastic_and_myofibroblastic_tumors |
| is foaf:primaryTopic of | wikipedia-en:Lipofibromatosis-like_neural_tumor |