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Plexiform Neurofibroma

A type of neurofibroma manifesting as a diffuse overgrowth of subcutaneous tissue, usually involving the face, scalp, neck, and chest but occasionally occurring in the abdomen or pelvis. The tumors tend to progress, and may extend along nerve roots to eventually involve the spinal roots and spinal cord. This process is almost always a manifestation of NEUROFIBROMATOSIS 1. (From Adams et al., Principles of Neurology, 6th ed, p1016; J Pediatr 1997 Nov;131(5):678-82)
Also Known As:
Neurofibroma, Plexiform; Tumor Royale; Elephantiasis Neuromatoses; Neurofibromas, Plexiform; Neuroma, Plexiform; Pachydermatoceles; Plexiform Neurofibromas; Plexiform Neuroma; Plexiform Neuromas; Elephantiasis Neuromatosis; Neuromas, Plexiform; Pachydermatocele
Networked: 266 relevant articles (21 outcomes, 53 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neurofibromatosis 1 (Neurofibromatosis Type I)
2. Neoplasms (Cancer)
3. Neurofibromatoses (Neurofibromatosis)
4. Plexiform Neurofibroma
5. Neurofibrosarcoma (MPNST)

Experts

1. Widemann, Brigitte C: 24 articles (11/2022 - 01/2006)
2. Dombi, Eva: 21 articles (11/2022 - 05/2007)
3. Wolkenstein, Pierre: 16 articles (10/2022 - 12/2003)
4. Laurendeau, Ingrid: 11 articles (10/2022 - 06/2004)
5. Friedrich, Reinhard E: 11 articles (03/2022 - 02/2004)
6. Clapp, D Wade: 11 articles (01/2021 - 07/2010)
7. Bièche, Ivan: 10 articles (10/2022 - 06/2004)
8. Fisher, Michael J: 9 articles (11/2022 - 04/2008)
9. Wolters, Pamela L: 9 articles (11/2022 - 05/2007)
10. Ortonne, Nicolas: 9 articles (10/2022 - 01/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Plexiform Neurofibroma:
1. AZD 6244IBA
2. Mitogen-Activated Protein Kinase Kinases (MEKs)IBA
3. Imatinib Mesylate (Gleevec)FDA Link
4. Mitogen-Activated Protein KinasesIBA
5. EverolimusFDA Link
6. Bevacizumab (Avastin)FDA Link
7. nilotinibFDA Link
8. InterferonsIBA
9. Doxycycline (Periostat)FDA LinkGeneric
10. calcipotriene (calcipotriol)FDA LinkGeneric

Therapies and Procedures

1. Therapeutics
2. Gas Lasers
3. Laminectomy
02/01/2003 - "A biopsy of an enlarged lumbar root obtained during decompressive laminectomy was interpreted as consistent with a plexiform neurofibroma. "
11/01/2022 - "Plexiform neurofibromas were most commonly treated with resection and laminectomy; some patients reported tumor enlargement after intervention. "
01/01/2022 - "Ventral prevertebral plexiform neurofibroma Neck pain Weak arms Cervical myelopathy C4-5 kyphosis Cord draped over C4-5 Enhanced prevertebral tumor C4-5 60° Partial Yes 4 days #21 1996 6 years M NF1 Age 6 mo had C1-3 laminectomies elsewhere Progressive kyphosis Quadriparesis C3-5 plexiform neurofibromas C2-4 kyphosis C3-4 45° No No #22 1993 11 years M "Fibromatosis" Neck pain Gag ↓ Right hemiparesis C2 body and odontoid curved dorsally C2-3 kyphosis C2 40° No Yes 3 days #23 2007 13 F Mid-cervical kyphosis Neck pain Unable to move neck C3-4 kyphosis C3-4 45° Yes Halo vest elsewhere 6 weeks Repeat traction on referral #24 1998 12 years M Chiari I Syringohydromyelia Difficulty swallowing Quadriparesis Previous posterior fossa and C1-3 decompression Basilar invagination C3-4 kyphosis C3-4 50° Yes Halo traction 3 days #25 1994 16 years M Chiari I. SHM Difficult speech Quadriparesis Previous posterior fossa and C1-4 laminectomies C3-4 kyphosis Basilar invagination C3-4 55° Yes Halo traction 3 days #26 2002 11 years M Chordoma C3-5 Initial quadriparesis improved after posterior decompression then worse Dorsal and lateral tumor C3-4 C3-4 20° Yes Traction 3 days #27 2006 13 years M C4 lamina Aneurysmal bone cyst Neck and shoulder pain C4 laminectomy for tumor resection Worse 4 months later C4-5 kyphosis C3-4 40° Yes No Table 3 Pediatric cervical kyphosis-postoperative evaluations Case ID Diagnosis Treatment-operation Complication PO orthosis F/U time Fusion status Preop Cobb Postop Cobb Preop JOA Postop JOA Comments Syndromic #1 SED Crown halo traction 1. Median mandibular glossotomy. "
4. Aftercare (After-Treatment)
5. Decompression
11/01/2015 - "Management of an expansile orbital mass: Plexiform neurofibroma decompression by orbitozygomatic approach."
01/01/2022 - "Ventral prevertebral plexiform neurofibroma Neck pain Weak arms Cervical myelopathy C4-5 kyphosis Cord draped over C4-5 Enhanced prevertebral tumor C4-5 60° Partial Yes 4 days #21 1996 6 years M NF1 Age 6 mo had C1-3 laminectomies elsewhere Progressive kyphosis Quadriparesis C3-5 plexiform neurofibromas C2-4 kyphosis C3-4 45° No No #22 1993 11 years M "Fibromatosis" Neck pain Gag ↓ Right hemiparesis C2 body and odontoid curved dorsally C2-3 kyphosis C2 40° No Yes 3 days #23 2007 13 F Mid-cervical kyphosis Neck pain Unable to move neck C3-4 kyphosis C3-4 45° Yes Halo vest elsewhere 6 weeks Repeat traction on referral #24 1998 12 years M Chiari I Syringohydromyelia Difficulty swallowing Quadriparesis Previous posterior fossa and C1-3 decompression Basilar invagination C3-4 kyphosis C3-4 50° Yes Halo traction 3 days #25 1994 16 years M Chiari I. SHM Difficult speech Quadriparesis Previous posterior fossa and C1-4 laminectomies C3-4 kyphosis Basilar invagination C3-4 55° Yes Halo traction 3 days #26 2002 11 years M Chordoma C3-5 Initial quadriparesis improved after posterior decompression then worse Dorsal and lateral tumor C3-4 C3-4 20° Yes Traction 3 days #27 2006 13 years M C4 lamina Aneurysmal bone cyst Neck and shoulder pain C4 laminectomy for tumor resection Worse 4 months later C4-5 kyphosis C3-4 40° Yes No Table 3 Pediatric cervical kyphosis-postoperative evaluations Case ID Diagnosis Treatment-operation Complication PO orthosis F/U time Fusion status Preop Cobb Postop Cobb Preop JOA Postop JOA Comments Syndromic #1 SED Crown halo traction 1. Median mandibular glossotomy. "