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: 134 relevant articles (3 outcomes, 24 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neoplasms (Cancer)
2. Pain (Aches)
3. Neurofibromatosis 1 (Neurofibromatosis Type I)
4. Neurofibroma
5. Nerve Sheath Neoplasms (Nerve Sheath Neoplasm)


1. Widemann, Brigitte C: 9 articles (04/2015 - 01/2006)
2. Wolkenstein, Pierre: 9 articles (06/2014 - 12/2003)
3. Dombi, Eva: 8 articles (04/2015 - 05/2007)
4. Friedrich, Reinhard E: 7 articles (12/2015 - 02/2004)
5. Valeyrie-Allanore, Laurence: 6 articles (06/2014 - 04/2010)
6. Kluwe, Lan: 6 articles (01/2014 - 08/2004)
7. Leroy, Karen: 6 articles (01/2013 - 06/2004)
8. Vidaud, Dominique: 6 articles (01/2013 - 06/2004)
9. Bièche, Ivan: 6 articles (01/2013 - 06/2004)
10. Laurendeau, Ingrid: 6 articles (01/2013 - 06/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Plexiform Neurofibroma:
1. InterferonsIBA
2. imatinib (Gleevec)FDA Link
3. Sirolimus (Rapamycin)FDA Link
4. AntibodiesIBA
5. Farnesyltranstransferase (Farnesyltransferase)IBA
6. tipifarnib (R115777)IBA
7. pirfenidoneIBA
8. Neurofibromin 1 (Neurofibromin)IBA
04/01/2014 - "Malignant peripheral nerve sheath tumors (MPNSTs) are Schwann cell-derived malignancies that arise from plexiform neurofibromas in patients with mutation of the neurofibromin 1 (NF1) gene. "
04/01/2006 - "We have previously observed activation of the Hh signalling pathway in Schwann cells of plexiform neurofibroma in neurofibromatosis type 1 (NF1), which results from functional loss of the NF1 encoding protein, neurofibromin. "
02/01/2001 - "Dermal and plexiform neurofibromas are peripheral nerve sheath tumors that arise frequently in neurofibromatosis type 1. The goal of the present study was to examine the tumorigenic properties of neurofibromin-deficient human Schwann cells (SCs) that were found to represent a subset of SCs present in approximately half of the total neurofibromas examined. "
02/01/2013 - "Since the bi-allelic inactivation of both neurofibromin 1 (NF1) gene alleles (NF1(-/-)) in Schwann cells (SCs) is common in both benign plexiform neurofibromas (PNs) and malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1 (NF1), other genetic alterations in SCs may be required for tumor progression of PNs to MPNSTs. "
05/01/2012 - "These models have been designed to address key questions including: (1) whether NF1 loss in the Schwann cell lineage is essential for tumorigenesis; (2) what cell type(s) in the Schwann cell lineage gives rise to dermal neurofibromas, plexiform neurofibromas and MPNSTs; (3) how the tumor microenvironment contributes to neoplasia; (4) what additional mutations contribute to neurofibroma-MPNST progression; (5) what role different neurofibromin-regulated Ras proteins play in this process and (6) how dysregulated growth factor signaling facilitates PNS tumorigenesis. "
9. Fluorodeoxyglucose F18 (Fludeoxyglucose F 18)FDA Link
10. Stem Cell FactorIBA

Therapies and Procedures

1. Gas Lasers
2. Transplants (Transplant)
3. Aftercare (After-Treatment)
4. Heterologous Transplantation (Xenotransplantation)
5. Facial Transplantation