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Central Nervous System Desmoid Fibromatosis

MedGen UID:
1389377
•Concept ID:
C4329628
•
Neoplastic Process
Synonym: Central Nervous System Desmoid-Type Fibromatosis

Definition

Intracranial and/or spinal involvement by desmoid fibromatosis. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCentral Nervous System Desmoid Fibromatosis

Recent clinical studies

Etiology

Safety and efficacy of intralesional steroid injection for aggressive fibromatosis.
Pruksakorn D, Lorsomradee S, Phanphaisarn A, Teeyakasem P, Klangjorhor J, Chaiyawat P, Kosachunhanun N, Settakorn J, Arpornchayanon O
World J Surg Oncol 2017 Nov 2;15(1):195. doi: 10.1186/s12957-017-1262-9. PMID: 29096658Free PMC Article

Diagnosis

Radiation-induced Desmoid Tumor Development in the Radiotherapy Field in a Child With Pineoblastoma: A Case Report.
Isik Bedir S, Karabagli P, Batur A, Ozturk M, Karabagli H, Yavas G, Koksal Y
J Pediatr Hematol Oncol 2023 Jul 1;45(5):e639-e642. Epub 2023 May 12 doi: 10.1097/MPH.0000000000002680. PMID: 37278565
A novel APC gene mutation associated with a severe phenotype in a patient with Turcot syndrome.
Fritch Lilla SA, Yi JS, Hall BA, Moertel CL
J Pediatr Hematol Oncol 2014 Apr;36(3):e177-9. doi: 10.1097/MPH.0000000000000009. PMID: 24309598
Intraoral desmoplastic fibroma: a manifestation of tuberous sclerosis.
Tandon S, Garg RK
Fetal Pediatr Pathol 2012 Aug;31(4):195-201. Epub 2012 Mar 9 doi: 10.3109/15513815.2011.648719. PMID: 22404501
Infantile fibromatosis of the neck with intracranial involvement: MR and CT findings.
Flacke S, Pauleit D, Keller E, Knoepfle G, Textor J, Leutner C, Schild HH
AJNR Am J Neuroradiol 1999 May;20(5):923-5. PMID: 10369367Free PMC Article
Multiple ependymomas in a patient with Turcot's syndrome.
Torres CF, Korones DN, Pilcher W
Med Pediatr Oncol 1997 Jan;28(1):59-61. doi: 10.1002/(sici)1096-911x(199701)28:1<59::aid-mpo11>3.0.co;2-5. PMID: 8950338

Therapy

Safety and efficacy of intralesional steroid injection for aggressive fibromatosis.
Pruksakorn D, Lorsomradee S, Phanphaisarn A, Teeyakasem P, Klangjorhor J, Chaiyawat P, Kosachunhanun N, Settakorn J, Arpornchayanon O
World J Surg Oncol 2017 Nov 2;15(1):195. doi: 10.1186/s12957-017-1262-9. PMID: 29096658Free PMC Article

Prognosis

Safety and efficacy of intralesional steroid injection for aggressive fibromatosis.
Pruksakorn D, Lorsomradee S, Phanphaisarn A, Teeyakasem P, Klangjorhor J, Chaiyawat P, Kosachunhanun N, Settakorn J, Arpornchayanon O
World J Surg Oncol 2017 Nov 2;15(1):195. doi: 10.1186/s12957-017-1262-9. PMID: 29096658Free PMC Article
A novel APC gene mutation associated with a severe phenotype in a patient with Turcot syndrome.
Fritch Lilla SA, Yi JS, Hall BA, Moertel CL
J Pediatr Hematol Oncol 2014 Apr;36(3):e177-9. doi: 10.1097/MPH.0000000000000009. PMID: 24309598
Case report: metachronous central nervous system desmoid tumours and thyroid carcinoma in a young familial adenomatous polyposis patient.
Butel-Simoes GI, Spigelman AD
Fam Cancer 2013 Dec;12(4):647-9. doi: 10.1007/s10689-013-9640-0. PMID: 23604857
Aggressive fibromatosis (fibrosarcoma) of the facial nerve.
Pulec JL
Ear Nose Throat J 1993 Jul;72(7):460-7, 470-2. PMID: 8354157

Clinical prediction guides

Safety and efficacy of intralesional steroid injection for aggressive fibromatosis.
Pruksakorn D, Lorsomradee S, Phanphaisarn A, Teeyakasem P, Klangjorhor J, Chaiyawat P, Kosachunhanun N, Settakorn J, Arpornchayanon O
World J Surg Oncol 2017 Nov 2;15(1):195. doi: 10.1186/s12957-017-1262-9. PMID: 29096658Free PMC Article

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