Soft tissue
Peripheral nerve
Pigmented neurofibroma

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 19 September 2016, last major update November 2012

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Pigmented neurofibroma

Definition / General
Sites
  • Head & neck, buttock or leg are most common sites
Clinical Features
  • Variable association with neurofibromatosis
  • Median age 28 years, range 2 - 61 years
Case Reports
Treatment
  • Excision, do not recur
Clinical Images

Images hosted on other servers:

Above eyelid

Gross Description
  • No gross pigmentation
Micro Description
  • Classic features of neurofibroma with loosely arranged tumor cells containing scant cytoplasm, elongated nuclei; also melanin pigment
  • Shows differentiation toward mature melanin production, but melanin synthesis appears to be impaired (Hum Pathol 2005;36:871)
Micro Images

Images hosted on other servers:

Above eyelid

Dermal spindle cell tumor

Loosely arranged tumor cells; pigment in inset



in NF1

S100+

MelanA +

Positive Stains
Negative Stains
Differential Diagnosis
  • Cellular blue nevus: not associated with neurofibroma; cells have nevoid features, not neural features
  • Congenital neuronevus: has junctional activity and nevoid component
  • Melanotic schwannoma: not associated with neurofibroma; biphasic, rounded nuclei, diffusely S100+
  • Neurocristic hamartoma: complex proliferation of nevomelanocytes
  • Pigmented dermatofibrosarcoma protuberans: more extensive storiform pattern, CD34 (strong), S100 negative