http://dbpedia.org/ontology/abstract
|
Lipofibromatosis-like neural tumor (LPF-NT … 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. malignant peripheral nerve sheath tumors.
|
http://dbpedia.org/ontology/medicalCause
|
http://dbpedia.org/resource/NTRK1 +
|
http://dbpedia.org/ontology/treatment
|
http://dbpedia.org/resource/Radical_surgery +
|
http://dbpedia.org/ontology/wikiPageID
|
68573171
|
http://dbpedia.org/ontology/wikiPageLength
|
19640
|
http://dbpedia.org/ontology/wikiPageRevisionID
|
1054168245
|
http://dbpedia.org/ontology/wikiPageWikiLink
|
http://dbpedia.org/resource/Category:Connective_and_soft_tissue_neoplasms +
, http://dbpedia.org/resource/Antibody +
, http://dbpedia.org/resource/Pediatric +
, http://dbpedia.org/resource/Immunohistochemistry +
, http://dbpedia.org/resource/Chromosomal_inversion +
, http://dbpedia.org/resource/Malignant_peripheral_nerve_sheath_tumor +
, http://dbpedia.org/resource/Trk_receptor +
, http://dbpedia.org/resource/Neuron +
, http://dbpedia.org/resource/NTRK1 +
, http://dbpedia.org/resource/H&E_stain +
, http://dbpedia.org/resource/Chromosome_1 +
, http://dbpedia.org/resource/Larotrectinib +
, http://dbpedia.org/resource/Soft_tissue +
, http://dbpedia.org/resource/Malignant_peripheral_nerve_sheath_tumors +
, http://dbpedia.org/resource/Adjuvant_therapy +
, http://dbpedia.org/resource/Fusion_gene +
, http://dbpedia.org/resource/Mitotic_index +
, http://dbpedia.org/resource/Metastasizing +
, http://dbpedia.org/resource/Fusion_protein +
, http://dbpedia.org/resource/Clinical_trial +
, http://dbpedia.org/resource/Aggressive_infantile_fibromatosis +
, http://dbpedia.org/resource/Radical_surgery +
, http://dbpedia.org/resource/PI3K/AKT/mTOR_pathway +
, http://dbpedia.org/resource/Category:Dermal_and_subcutaneous_growths +
, http://dbpedia.org/resource/PLCG1 +
, http://dbpedia.org/resource/Neuroectoderm +
, http://dbpedia.org/resource/Chromosomal_translocation +
, http://dbpedia.org/resource/Grading_%28tumors%29 +
, http://dbpedia.org/resource/Category:Benign_neoplasms +
, http://dbpedia.org/resource/Karyotype +
, http://dbpedia.org/resource/Fibrosarcoma +
, http://dbpedia.org/resource/Spindle_cell_sarcoma +
, http://dbpedia.org/resource/Entrectinib +
, http://dbpedia.org/resource/Tropomyosin_3 +
, http://dbpedia.org/resource/Surgery +
, http://dbpedia.org/resource/Nervous_tissue +
, http://dbpedia.org/resource/Chimeric_protein +
, http://dbpedia.org/resource/Adipose_tissue +
, http://dbpedia.org/resource/Histopathology +
, http://dbpedia.org/resource/Benign_tumor +
, http://dbpedia.org/resource/Fibroma +
, http://dbpedia.org/resource/Cell_signaling +
, http://dbpedia.org/resource/Locus_%28genetics%29 +
, http://dbpedia.org/resource/Food_and_Drug_Administration +
, http://dbpedia.org/resource/SOX10 +
, http://dbpedia.org/resource/Dermatology +
, http://dbpedia.org/resource/Nucleoli +
, http://dbpedia.org/resource/Sarcoma +
, http://dbpedia.org/resource/S100_protein +
, http://dbpedia.org/resource/Tropomyosin_receptor_kinase_A +
, http://dbpedia.org/resource/Malignant_transformation +
, http://dbpedia.org/resource/MAPK/ERK_pathway +
, http://dbpedia.org/resource/Fusion_genes +
, http://dbpedia.org/resource/Necrosis +
, http://dbpedia.org/resource/Dermatofibrosarcoma_protuberans +
, http://dbpedia.org/resource/Lipofibromatosis +
, http://dbpedia.org/resource/Solitary_fibrous_tumor +
, http://dbpedia.org/resource/Lumbar_spine +
, http://dbpedia.org/resource/Gene_expression +
, http://dbpedia.org/resource/Cell_division +
, http://dbpedia.org/resource/CD34 +
, http://dbpedia.org/resource/LMNA +
, http://dbpedia.org/resource/Peripheral_nerve_sheath_tumor +
, http://dbpedia.org/resource/Cell_nucleus +
, http://dbpedia.org/resource/Deletion_%28genetics%29 +
|
http://dbpedia.org/property/causes
|
An NTRK1- containing fusion gene in ~80% of cases
|
http://dbpedia.org/property/field
|
Pediatric or adult dermatology and surgery
|
http://dbpedia.org/property/frequency
|
Very rare
|
http://dbpedia.org/property/name
|
Lipofibromatosis-like neural tumor
|
http://dbpedia.org/property/onset
|
1.1991888E9
|
http://dbpedia.org/property/prognosis
|
Good
|
http://dbpedia.org/property/treatment
|
http://dbpedia.org/resource/Radical_surgery +
|
http://dbpedia.org/property/wikiPageUsesTemplate
|
http://dbpedia.org/resource/Template:Reflist +
, http://dbpedia.org/resource/Template:Infobox_medical_condition_%28new%29 +
|
http://purl.org/dc/terms/subject
|
http://dbpedia.org/resource/Category:Connective_and_soft_tissue_neoplasms +
, http://dbpedia.org/resource/Category:Benign_neoplasms +
, http://dbpedia.org/resource/Category:Dermal_and_subcutaneous_growths +
|
http://www.w3.org/ns/prov#wasDerivedFrom
|
http://en.wikipedia.org/wiki/Lipofibromatosis-like_neural_tumor?oldid=1054168245&ns=0 +
|
http://xmlns.com/foaf/0.1/isPrimaryTopicOf
|
http://en.wikipedia.org/wiki/Lipofibromatosis-like_neural_tumor +
|
http://xmlns.com/foaf/0.1/name
|
Lipofibromatosis-like neural tumor
|
owl:sameAs |
http://dbpedia.org/resource/Lipofibromatosis-like_neural_tumor +
, https://global.dbpedia.org/id/G5hS1 +
, http://www.wikidata.org/entity/Q108486055 +
|
rdf:type |
http://www.wikidata.org/entity/Q12136 +
, http://dbpedia.org/ontology/Disease +
|
rdfs:comment |
Lipofibromatosis-like neural tumor (LPF-NT … 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 fopofibromatosis but also the other tumor fo
|
rdfs:label |
Lipofibromatosis-like neural tumor
|