Neurofibromas grow from the sheath around nerves. A single one is usually simple to remove. The diffuse plexiform type weaves through tissue planes and around nerves, and surgery there is about reducing bulk and protecting function rather than clearance.
Neurofibromas are benign tumours arising from nerve sheath tissue. A discrete cutaneous neurofibroma can usually be excised completely under local anaesthetic. A plexiform neurofibroma infiltrates widely, is very vascular, and is usually debulked in stages to improve appearance and function rather than removed entirely. Most plexiform lesions occur in neurofibromatosis type 1.
A discrete cutaneous neurofibroma is a soft nodule in or under the skin. It has a reasonable edge, it can be excised with the skin closed directly, and that is usually the end of it. People with neurofibromatosis may have many, and they are removed selectively where they are painful, catching on clothing or cosmetically troubling.
A plexiform neurofibroma is a different problem altogether. It grows along a nerve and out through the tissue around it, without a capsule or a clean plane to dissect. It can involve skin, fat, muscle and the nerve itself over a large area, and it is remarkably vascular: bleeding is the main technical difficulty and the main risk of surgery.
Because of this, complete removal is frequently impossible without sacrificing the nerve and a great deal of surrounding tissue. The realistic aim is staged debulking: reducing bulk, restoring contour and relieving pressure or functional restriction, while accepting that the lesion remains and may regrow.
A change in a long standing plexiform lesion matters. New or increasing pain, rapid growth or a hard area within a previously soft lesion can indicate malignant change, which is uncommon but important, and needs imaging and biopsy rather than reassurance.
The plan depends entirely on which kind of lesion it is, and imaging is needed before any plexiform lesion is touched.
MRI defines the extent of a plexiform lesion and its relationship to nerves and vessels. Surgery on these is not planned without it.
A realistic area is agreed for this operation, with blood cross matched, rather than attempting everything at once.
A discrete lesion is excised with its capsule. A plexiform lesion is reduced in volume, working with meticulous control of bleeding.
Functioning nerve is identified and preserved wherever possible, accepting that tumour will be left behind around it.
Skin is redraped and trimmed to restore contour, closed directly or with a local flap.
Drains may be used after larger debulking. Swelling and bruising are expected. Wound checked for collection of blood.
Wounds heal. Swelling settles gradually, and the true contour result only becomes apparent towards the end of this period.
Scars mature. Any further stage is planned once the tissue has settled.
Regular review for regrowth and for any change suggesting malignancy.
Discrete neurofibromas are usually removed completely with a good result. For plexiform lesions the expectation must be different: improvement in contour and function rather than cure. Regrowth is common, sometimes over years, and repeat debulking is often planned from the outset rather than being a sign of failure. Blood loss can be significant, transfusion is sometimes needed, and some sensory loss in the territory of the involved nerve is frequently unavoidable.
Bleeding dominates the risk profile in plexiform surgery, and it is the reason these operations are planned carefully.
Most of the aftercare is about the wound and about watching for change in what remains.
A discrete lesion usually can. A plexiform one infiltrates through tissue and around nerves without an edge, and complete removal would often cost the nerve and a great deal of surrounding tissue.
Debulking is deliberately incomplete. Regrowth over years is expected in plexiform disease, which is why staged surgery is discussed at the outset.
Neurofibromas are benign. Malignant change is uncommon, but it does happen, which is why a change in pain or growth in a long standing lesion is taken seriously.
Plexiform surgery needs planning: imaging, blood availability, and a realistic staged plan agreed beforehand. Attempting complete clearance of an infiltrating vascular lesion in one sitting is how people end up with nerve loss and a transfusion.
Surgery for symptomatic neurofibroma, and for plexiform lesions causing functional or structural problems, is commonly covered by health insurance and by government schemes. Removal of asymptomatic lesions purely for appearance may not be. Staged procedures are estimated as a plan rather than individually.
Most of these come from families living with neurofibromatosis.
Ask your question →If it is a discrete neurofibroma, usually yes. If it is plexiform, usually not, because it grows through the tissue around the nerve without a clear edge. In that case the aim is to reduce bulk and improve function and appearance.
Discrete lesions that are fully excised rarely recur. Plexiform lesions that have been debulked frequently regrow over years, and further stages are often planned in advance rather than being a surprise.
Neurofibromas are benign. A small proportion of plexiform lesions can undergo malignant change, which is why new pain, rapid growth or a hard area within a soft lesion should be reported and investigated rather than watched.
Plexiform lesions are unusually vascular and do not have a plane that can be dissected bloodlessly. Blood is cross matched beforehand and the operation is planned in stages partly for this reason.
Often some, in the area supplied by the involved nerve, because tumour and nerve are intertwined. Preserving function is prioritised over completeness of removal, but some sensory loss is frequently unavoidable.
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