Plexiform Neurofibroma

The patient is a 33 yo man seen for evaluation of a large tumor involving the left thigh.  He lives in a rural area and his last medical visit was at age sixteen.  Sadly, he was without medical insurance until recently.  He lived with his father until recently when the latter died, and now he has been taken in by a family in his community.

The examination showed a pleasant man with a sunny disposition and a broad smile.  While mildly retarded he answers questions appropriately.  His care-giver accompanied him and it is clear that she and her family have welcomed him.

The patient has the stigmata of neurofibromatosis (NF1) with scores of cafe au lait spots and neurofibromas.  Most compelling is a large plexiform NF of the right thigh.  He has other plexiform NFs of the buttock and left chest, but these are smaller.

We will attempt to have him evaluated and treated at a center which specializes in the care of patients with NF.  This post will be updated periodically.

Photos:  Left thigh, anterior and posterior views

 Your comments will be appreciated.

References: 

1. Type 1 NF  eMedicine.  Full text online.

2, KT Power, et. al. Management of Massive Lower Limb Plexiform Neurofibromatosis – When to Intervene?  Ann R Coll Surg Engl. 2007 November; 89(8): 807  Free Full Text Online