Lipofibromatosis (original) (raw)
La lipofibromatosi infantile è una rara neoplasia pediatrica dei tessuti molli con una tendenza distintiva a contenere grasso. Questa malattia è stata recentemente descritta come una distinta entità clinica. Istologicamente, è caratterizzata da tessuto adiposo abbondante con elementi fibroblastici. Nonostante sia del tutto benigna, tale neoplasia è stata segnalata per avere un alto tasso di recidività locale.
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| dbo:abstract | Lipofibromatosis (LPF) is an extremely rare soft tissue tumor which was first clearly described in 2000 by Fetsch et al as a strictly pediatric, locally invasive, and often recurrent (at the site of its surgical removal) tumor. It is nonetheless a non-metastasizing, i.e. benign, tumor. While even the more recent literature has sometimes regarded LPF as a strictly childhood disorder, rare cases of LPF has been diagnosed in adults. The diagnosis of lipofibromatosis should not be automatically discarded because of an individual's age. Based primarily on histopathologic (i.e. microscopic appearance of specially prepared tissue) analyses, lipofibromatosis was initially regarded as either a type of, or very similar to, aponeurotic fibroma (also termed calcifying aponeurotic fibroma), fibrous hamartoma of infancy, EWSRI-SMAD3-rearranged fibroblastic tumor (also termed EWSR1-SMAD3-positive fibroblastic tumor), or infantile digital fibromatosis. However, further analyses of these tumors' various differences, particularly in the gene abnormalities that their neoplastic cells express, led the World Health Organization, 2020, to classify LPF and each of the four other tumors as distinctly different forms in the category of fibroblastic and myofibroblastic tumors. Lipofibromatosis-like neural tumor is a very recently defined disorder which initial studies regarded to be a variant of lipofibromatosis. However, more recent studies have emphasized critical differences in the clinical presentations and gene abnormalities between these two tumors. Here, lipofibromatosis-like neural tumor is considered to be a distinct tumor form with its own Wikipedia page. (en) La lipofibromatosi infantile è una rara neoplasia pediatrica dei tessuti molli con una tendenza distintiva a contenere grasso. Questa malattia è stata recentemente descritta come una distinta entità clinica. Istologicamente, è caratterizzata da tessuto adiposo abbondante con elementi fibroblastici. Nonostante sia del tutto benigna, tale neoplasia è stata segnalata per avere un alto tasso di recidività locale. (it) |
| dbo:treatment | dbr:Radical_surgery |
| dbo:wikiPageID | 68497913 (xsd:integer) |
| dbo:wikiPageLength | 16312 (xsd:nonNegativeInteger) |
| dbo:wikiPageRevisionID | 1042649535 (xsd:integer) |
| dbo:wikiPageWikiLink | dbr:Benign_tumor dbr:Epidermal_growth_factor dbr:Epidermal_growth_factor_receptor dbr:FN1_(gene) dbr:SPARC_(gene) dbr:Aponeurotic_fibroma dbr:Pediatric_surgery dbr:Debulking dbr:Dermatology dbr:Dermis dbr:EGR1 dbr:Infantile_digital_fibromatosis dbr:Orbit_(anatomy) dbr:Ligand_(biochemistry) dbr:S100_protein dbr:PI3K/AKT/mTOR_pathway dbr:Receptor_tyrosine_kinases dbr:MUC1 dbr:Deletion_(genetics) dbr:Pediatrics dbr:BRAF_(gene) dbr:CD34 dbr:Actin dbr:Adipocyte dbr:Adjuvant_therapy dbc:Benign_neoplasms dbr:Fusion_gene dbr:GRIA1 dbr:Lipoblast dbr:Lipofibromatosis-like_neural_tumor dbr:Adipose_tissue dbr:Chromosomal_inversion dbr:Chromosomal_translocation dbr:Biopsied dbr:Fluorescence_in_situ_hybridization dbr:RET_proto-oncogene dbr:Radical_surgery dbr:Real-time_polymerase_chain_reaction dbr:Histology dbr:Histopathological dbr:EGFR_(gene) dbr:EGF_(gene) dbc:Dermal_and_subcutaneous_growths dbc:Connective_and_soft_tissue_neoplasms dbr:Heparin-binding_EGF-like_growth_factor dbr:Mitotic_index dbr:CD99 dbr:Fibroblast dbr:Fibroblastic_and_myofibroblastic_tumors dbr:Fibrous_hamartoma_of_infancy dbr:Fine-needle_aspiration dbr:HBEGF_(gene) dbr:FN1 dbr:Metastasis dbr:ROS1_(gene) dbr:Skin dbr:Immunohistochemistry dbr:Soft_tissue dbr:Myofibril dbr:NTRK1_(gene) dbr:TGF_alpha dbr:PDGFRB_(gene) dbr:PDGFRB dbr:Tumor_marker dbr:RET_(gene) dbr:Muscle_cells dbr:Transforming_growth_factor_alpha dbr:RNA_sequencing dbr:VCL_(gene) dbr:Histopathologic |
| dbp:field | dbr:Pediatric_surgery dbr:Dermatology dbr:Pediatrics |
| dbp:frequency | Very rare (en) |
| dbp:name | Lipofibromatosis (en) |
| dbp:prognosis | Excellent (en) |
| dbp:treatment | dbr:Radical_surgery |
| dbp:wikiPageUsesTemplate | dbt:Infobox_medical_condition_(new) dbt:Reflist |
| dct: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 | La lipofibromatosi infantile è una rara neoplasia pediatrica dei tessuti molli con una tendenza distintiva a contenere grasso. Questa malattia è stata recentemente descritta come una distinta entità clinica. Istologicamente, è caratterizzata da tessuto adiposo abbondante con elementi fibroblastici. Nonostante sia del tutto benigna, tale neoplasia è stata segnalata per avere un alto tasso di recidività locale. (it) Lipofibromatosis (LPF) is an extremely rare soft tissue tumor which was first clearly described in 2000 by Fetsch et al as a strictly pediatric, locally invasive, and often recurrent (at the site of its surgical removal) tumor. It is nonetheless a non-metastasizing, i.e. benign, tumor. While even the more recent literature has sometimes regarded LPF as a strictly childhood disorder, rare cases of LPF has been diagnosed in adults. The diagnosis of lipofibromatosis should not be automatically discarded because of an individual's age. (en) |
| rdfs:label | Lipofibromatosi infantile (it) Lipofibromatosis (en) |
| owl:sameAs | wikidata:Lipofibromatosis dbpedia-it:Lipofibromatosis https://global.dbpedia.org/id/gfNT |
| prov:wasDerivedFrom | wikipedia-en:Lipofibromatosis?oldid=1042649535&ns=0 |
| foaf:isPrimaryTopicOf | wikipedia-en:Lipofibromatosis |
| foaf:name | Lipofibromatosis (en) |
| is dbo:wikiPageWikiLink of | dbr:Lipofibromatosis-like_neural_tumor dbr:Fibroblastic_and_myofibroblastic_tumors |
| is foaf:primaryTopic of | wikipedia-en:Lipofibromatosis |