Soft tissue
Peripheral nerve
Neurofibroma
Plexiform neurofibroma
Author: Vijay Shankar, M.D.
Topic Completed: 1 November 2012
Minor changes: 1 December 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Plexiform neurofibroma [title]
Table of Contents
Definition / general | Sites | Clinical features | Radiology images | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosisCite this page: Shankar V. Plexiform neurofibroma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueneurofibromaplexiform.html. Accessed October 16th, 2021.
Definition / general
- Benign peripheral nerve sheath tumor that surrounds multiple nerve fascicles
- Has irregularly thickened, distorted, tortuous structure
- Plexiform: complex; in the form of a plexus or network
Sites
- Orbit, face, neck, back, inguinal
Clinical features
- Almost always develops during childhood
- Large tumors are attached to major nerve trunks in neck or extremities
- 5% transform to MPNST, a higher rate than classic neurofibromas
- Part of diagnostic criteria for neurofibromatosis type 1 (NF1, Mod Pathol 1998;11:612), although isolated plexiform neurofibromas may occur without definitive evidence of NF1 (Laryngoscope 2004;114:1410)
- 27% of childhood neurofibroma patients had plexiform neurofibromas in one study (J Pediatr (Rio J) 2007;83:571)
Radiology images
Images hosted on other servers:
Heterogeneous mass of the left submandibular gland
Mass with diffuse heterogeneous enhancement
Case reports
- 5 year old boy with massive penile enlargement (Urol J 2007;4:52)
- 12 year old boy with facial plexiform neurofibroma (J Indian Soc Pedod Prev Dent 2007;25:30)
- 17 year old boy with plexiform neurofibroma of the submandibular gland and von Recklinghausen's disease (Rare Tumors 2011;3:e4)
- 19 year old man with diffuse plexiform neurofibroma of the back (Hawaii Med J 2010;69:191)
Treatment
- Excision for symptomatic relief; often cannot completely excise the tumor
- Recurrence may occur even if completely excised
Clinical images
Contributed by R.F. Chinoy, M.D.
7 year old girl with café au lait spots; mother (right) had small neurofibroma
Contributed by Mark R. Wick, M.D.
NF1 patient
Images hosted on other servers:
Cutaneous neurofibroma various locations
Large mass on back - pre and postop
12 year old boy with swelling of the face and café au lait macules
19 year old man with enlarging gluteal mass and café au lait patches
Left lower limb plexiform neurofibroma
Gross description
- Associated with grossly enlarged and tortuous nerves
- Deep tumors are often large
- Often resembles "bag of worms"
- Highly vascularized and locally invasive
Gross images
Contributed by Mark R. Wick, M.D.
Plexiform
Microscopic (histologic) description
- Nodular or diffuse
- Diffuse cases are also known as elephantiasis neurofibromatosa; characterized by an overgrowth of epidermal and subcutaneous tissue (Dermatol Online J 2009;15:7)
- Hypocellular with myxoid background; contains Schwann cells, fibroblasts and mast cells
- Occasional nuclear palisading
- Rarely is pigmented due to melanocytes (J Am Acad Dermatol 2007;56:862)
- No biphasic pattern of schwannoma
Microscopic (histologic) images
Contributed by R.F. Chinoy, M.D.
Tongue lesion
Contributed by Mark R. Wick, M.D.
NF1
Images hosted on other servers:
H&E, S100
Facial swelling
Positive stains
Differential diagnosis
