loading...| Preferred Name |
Neurofibrosarcoma |
| Synonyms |
Sarcoma, Neurogenic |
| Definitions |
A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with NEUROFIBROMATOSIS 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72) |
| ID |
http://purl.bioontology.org/ontology/MESH/D018319 |
| altLabel |
Sarcoma, Neurogenic Neurilemoma, Malignant Malignant Neurilemmoma Neurogenic Sarcoma MPNST Neurilemmosarcomas Neurilemmosarcoma Malignant Neurilemomas Neurogenic Sarcomas Neurofibrosarcomas Malignant Neurilemoma Malignant Peripheral Nerve Sheath Tumors Malignant Neurilemmomas Peripheral Nerve Sheath Tumors, Malignant Neurilemmoma, Malignant Malignant Peripheral Nerve Sheath Tumor Malignant Schwannomas Malignant Schwannoma Schwannoma, Malignant Sarcomas, Neurogenic |
| AN |
coordinate IM with precoordinated organ/neoplasm term (IM) |
| AQL |
BL BS CF CH CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SC SU TH UL UR VE VI |
| cui |
C0206729 C0751690 |
| DC |
1 |
| definition |
A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with NEUROFIBROMATOSIS 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72) A malignant neurilemmoma with nerve sheath differentiation. It is often associated with NEUROFIBROMATOSIS 1 and RHABDOMYOSARCOMA. |
| DX |
19940101 |
| HN |
1994; for Malignant Peripheral Nerve Sheath Tumors, use Neurilemoma 2000-2018 |
| Inverse of AQ |
http://purl.bioontology.org/ontology/MESH/Q000401 http://purl.bioontology.org/ontology/MESH/Q000150 http://purl.bioontology.org/ontology/MESH/Q000188 http://purl.bioontology.org/ontology/MESH/Q000517 http://purl.bioontology.org/ontology/MESH/Q000737 http://purl.bioontology.org/ontology/MESH/Q000201 http://purl.bioontology.org/ontology/MESH/Q000145 http://purl.bioontology.org/ontology/MESH/Q000556 http://purl.bioontology.org/ontology/MESH/Q000503 http://purl.bioontology.org/ontology/MESH/Q000453 http://purl.bioontology.org/ontology/MESH/Q000208 http://purl.bioontology.org/ontology/MESH/Q000134 http://purl.bioontology.org/ontology/MESH/Q000821 http://purl.bioontology.org/ontology/MESH/Q000382 http://purl.bioontology.org/ontology/MESH/Q000601 http://purl.bioontology.org/ontology/MESH/Q000235 http://purl.bioontology.org/ontology/MESH/Q000648 http://purl.bioontology.org/ontology/MESH/Q000523 http://purl.bioontology.org/ontology/MESH/Q000276 http://purl.bioontology.org/ontology/MESH/Q000191 http://purl.bioontology.org/ontology/MESH/Q000652 http://purl.bioontology.org/ontology/MESH/Q000662 http://purl.bioontology.org/ontology/MESH/Q000151 http://purl.bioontology.org/ontology/MESH/Q000628 http://purl.bioontology.org/ontology/MESH/Q000098 http://purl.bioontology.org/ontology/MESH/Q000196 http://purl.bioontology.org/ontology/MESH/Q000534 http://purl.bioontology.org/ontology/MESH/Q000266 http://purl.bioontology.org/ontology/MESH/Q000532 http://purl.bioontology.org/ontology/MESH/Q000139 http://purl.bioontology.org/ontology/MESH/Q000378 http://purl.bioontology.org/ontology/MESH/Q000175 http://purl.bioontology.org/ontology/MESH/Q000000981 http://purl.bioontology.org/ontology/MESH/Q000097 http://purl.bioontology.org/ontology/MESH/Q000473 http://purl.bioontology.org/ontology/MESH/Q000451 http://purl.bioontology.org/ontology/MESH/Q000209 |
| Machine permutation |
1994; for Malignant Peripheral Nerve Sheath Tumors, see Neurilemoma 2000-2018 |
| MDA |
19930625 |
| MMR |
20180308 |
| MN |
C10.551.775.500.750.750 C04.557.450.565.590.350.590 C10.668.829.725.500.600.600 C04.557.580.600.580.795 C04.557.450.795.350.590 |
| notation |
D018319 |
| prefLabel |
Neurofibrosarcoma |
| TERMUI |
T371151 T781858 T000937762 T054650 T371147 T371150 T839571 T371156 T371161 T028143 |
| TH |
NLM (1998) NLM (2000) NLM (1994) NLM (2014) NLM (2012) ORD (2010) NLM (2019) |
| tui |
T191 |
| subClassOf |