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Unresectable Plexiform Neurofibroma

MedGen UID:
1792120
•Concept ID:
C5555679
•
Neoplastic Process

Definition

Plexiform neurofibroma that is not amenable to surgical resection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnresectable Plexiform Neurofibroma

Professional guidelines

PubMed

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356

Recent clinical studies

Etiology

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Plexiform Neurofibroma Without Neurofibromatosis Type 1.
Wollina U, Schönlebe J
Acta Dermatovenerol Croat 2020 Dec;28(7):245-246. PMID: 33835000
Genetic basis of neurofibromatosis type 1 and related conditions, including mosaicism.
Legius E, Brems H
Childs Nerv Syst 2020 Oct;36(10):2285-2295. Epub 2020 Jun 29 doi: 10.1007/s00381-020-04771-8. PMID: 32601904
Phase II trial of pegylated interferon alfa-2b in young patients with neurofibromatosis type 1 and unresectable plexiform neurofibromas.
Jakacki RI, Dombi E, Steinberg SM, Goldman S, Kieran MW, Ullrich NJ, Pollack IF, Goodwin A, Manley PE, Fangusaro J, Allen R, Widemann BC
Neuro Oncol 2017 Feb 1;19(2):289-297. doi: 10.1093/neuonc/now158. PMID: 27510726Free PMC Article
Interferon-α for unresectable progressive and symptomatic plexiform neurofibromas.
Kebudi R, Cakir FB, Gorgun O
J Pediatr Hematol Oncol 2013 Apr;35(3):e115-7. doi: 10.1097/MPH.0b013e318270cd24. PMID: 23042022

Diagnosis

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Plexiform Neurofibroma Without Neurofibromatosis Type 1.
Wollina U, Schönlebe J
Acta Dermatovenerol Croat 2020 Dec;28(7):245-246. PMID: 33835000
Genetic basis of neurofibromatosis type 1 and related conditions, including mosaicism.
Legius E, Brems H
Childs Nerv Syst 2020 Oct;36(10):2285-2295. Epub 2020 Jun 29 doi: 10.1007/s00381-020-04771-8. PMID: 32601904
Surgical management of abdominal manifestations of type 1 neurofibromatosis: experience of a single center.
Cavallaro G, Basile U, Polistena A, Giustini S, Arena R, Scorsi A, Zinnamosca L, Letizia C, Calvieri S, De Toma G
Am Surg 2010 Apr;76(4):389-96. PMID: 20420249
Resection of benign sciatic notch dumbbell-shaped tumors.
Spinner RJ, Endo T, Amrami KK, Dozois EJ, Babovic-Vuksanovic D, Sim FH
J Neurosurg 2006 Dec;105(6):873-80. doi: 10.3171/jns.2006.105.6.873. PMID: 17405258

Therapy

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Phase 1 dose-escalation study to evaluate the safety, tolerability, pharmacokinetics, and anti-tumor activity of FCN-159 in adults with neurofibromatosis type 1-related unresectable plexiform neurofibromas.
Hu X, Li W, Zeng K, Xu Z, Li C, Kang Z, Li S, Huang X, Han P, Lin H, Hui AM, Tan Y, Diao L, Li B, Wang X, Wu Z, Lin X
BMC Med 2023 Jul 3;21(1):230. doi: 10.1186/s12916-023-02927-2. PMID: 37400844Free PMC Article
Combined Cyclin-Dependent Kinase Inhibition Overcomes MAPK/Extracellular Signal-Regulated Kinase Kinase Inhibitor Resistance in Plexiform Neurofibroma of Neurofibromatosis Type I.
Wang W, Cui XW, Gu YH, Wei CJ, Li YH, Ren JY, Chung MH, Aimaier RH, Zhang HB, Li QF, Wang ZC
J Invest Dermatol 2022 Mar;142(3 Pt A):613-623.e7. Epub 2021 Sep 15 doi: 10.1016/j.jid.2021.07.164. PMID: 34534577
Phase II trial of pegylated interferon alfa-2b in young patients with neurofibromatosis type 1 and unresectable plexiform neurofibromas.
Jakacki RI, Dombi E, Steinberg SM, Goldman S, Kieran MW, Ullrich NJ, Pollack IF, Goodwin A, Manley PE, Fangusaro J, Allen R, Widemann BC
Neuro Oncol 2017 Feb 1;19(2):289-297. doi: 10.1093/neuonc/now158. PMID: 27510726Free PMC Article
Interferon-α for unresectable progressive and symptomatic plexiform neurofibromas.
Kebudi R, Cakir FB, Gorgun O
J Pediatr Hematol Oncol 2013 Apr;35(3):e115-7. doi: 10.1097/MPH.0b013e318270cd24. PMID: 23042022

Prognosis

Management of Central and Peripheral Nervous System Tumors in Patients with Neurofibromatosis.
Brown R
Curr Oncol Rep 2023 Dec;25(12):1409-1417. Epub 2023 Oct 31 doi: 10.1007/s11912-023-01451-z. PMID: 37906356
Genetic basis of neurofibromatosis type 1 and related conditions, including mosaicism.
Legius E, Brems H
Childs Nerv Syst 2020 Oct;36(10):2285-2295. Epub 2020 Jun 29 doi: 10.1007/s00381-020-04771-8. PMID: 32601904
Phase II trial of pegylated interferon alfa-2b in young patients with neurofibromatosis type 1 and unresectable plexiform neurofibromas.
Jakacki RI, Dombi E, Steinberg SM, Goldman S, Kieran MW, Ullrich NJ, Pollack IF, Goodwin A, Manley PE, Fangusaro J, Allen R, Widemann BC
Neuro Oncol 2017 Feb 1;19(2):289-297. doi: 10.1093/neuonc/now158. PMID: 27510726Free PMC Article
Interferon-α for unresectable progressive and symptomatic plexiform neurofibromas.
Kebudi R, Cakir FB, Gorgun O
J Pediatr Hematol Oncol 2013 Apr;35(3):e115-7. doi: 10.1097/MPH.0b013e318270cd24. PMID: 23042022
Resection of benign sciatic notch dumbbell-shaped tumors.
Spinner RJ, Endo T, Amrami KK, Dozois EJ, Babovic-Vuksanovic D, Sim FH
J Neurosurg 2006 Dec;105(6):873-80. doi: 10.3171/jns.2006.105.6.873. PMID: 17405258

Clinical prediction guides

Phase 1 dose-escalation study to evaluate the safety, tolerability, pharmacokinetics, and anti-tumor activity of FCN-159 in adults with neurofibromatosis type 1-related unresectable plexiform neurofibromas.
Hu X, Li W, Zeng K, Xu Z, Li C, Kang Z, Li S, Huang X, Han P, Lin H, Hui AM, Tan Y, Diao L, Li B, Wang X, Wu Z, Lin X
BMC Med 2023 Jul 3;21(1):230. doi: 10.1186/s12916-023-02927-2. PMID: 37400844Free PMC Article
Plexiform Neurofibroma Without Neurofibromatosis Type 1.
Wollina U, Schönlebe J
Acta Dermatovenerol Croat 2020 Dec;28(7):245-246. PMID: 33835000
Genetic basis of neurofibromatosis type 1 and related conditions, including mosaicism.
Legius E, Brems H
Childs Nerv Syst 2020 Oct;36(10):2285-2295. Epub 2020 Jun 29 doi: 10.1007/s00381-020-04771-8. PMID: 32601904
Dual mTORC1/2 inhibition induces anti-proliferative effect in NF1-associated plexiform neurofibroma and malignant peripheral nerve sheath tumor cells.
Varin J, Poulain L, Hivelin M, Nusbaum P, Hubas A, Laurendeau I, Lantieri L, Wolkenstein P, Vidaud M, Pasmant E, Chapuis N, Parfait B
Oncotarget 2016 Jun 14;7(24):35753-35767. doi: 10.18632/oncotarget.7099. PMID: 26840085Free PMC Article
Surgical management of abdominal manifestations of type 1 neurofibromatosis: experience of a single center.
Cavallaro G, Basile U, Polistena A, Giustini S, Arena R, Scorsi A, Zinnamosca L, Letizia C, Calvieri S, De Toma G
Am Surg 2010 Apr;76(4):389-96. PMID: 20420249

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