Ophthalmic nerve malignant nerve sheath tumour

Case contributed by A.Prof Frank Gaillard

Case Discussion

Ophthalmic nerve malignant nerve sheath tumour.


A resection of the left eye including an attached ovoid piece of skin superomedial to the eye.  The eye is 48mm superior to inferior, 42mm medial to lateral and 49mm anterior to posterior.  The ellipse of skin including the eyelashes anteriorly is 43mm medial to lateral and 9mm superior to inferior.  The attached  ovoid piece of skin is 92mm superomedial to inferolateral by 38mm. The medial 20mm of the left eyebrow is 20mm superomedial to the eyelashes.  Lateral to the eyebrow there is either a blood vessel or nerve, 15mm in length and 2-3mm in diameter.

Microscopic description

Sections show a nerve, which is thickened and largely replaced by markedly atypical spindle cells of similar appearance to tumour. 

Sections show a nerve containing occasional clusters of atypical spindle cells and a lymphoplasmacytic infiltrate.

Sections from the nodular and serpiginous lesion deep to the skin and posterior to the eye show a cellular tumour composed of spindle cells arranged in short fascicles and small whorled structures.  Cells have large, hyperchromatic pleomorphic nuclei with irregular nuclear routlines, prominent nucleoli and moderate amounts of elongate eosinophilic cytoplasm.  Mitoses are frequent, including atypical forms.  In areas, the tumour forms small nests of more epithelioid cells.  There is a patchy moderate lymphoplasmacytic infiltrate.  Some areas contain thick collagen fibres, probably entrapped reticular dermis.  Tumour is centred on the subcutis.  Tumour extensively involves adjacent nerves.  Some nerves appear completely replaced by tumour cells, with other nerves showing perineural spread.  In areas, the architecture is plexiform.  Tumour extensively involves the extrinsic nerves of the eye, posterior and medially, but is clear of the optic nerve.  Tumour involves the margin posterior to the eye, and involves the margin of the nerve described as lateral to the eyebrow. Tumour is 0.1mm from the lateral skin margin and 0.4mm from the deep skin margin.  The superior and medial skin margins appear clear.  No lymphatic or vascular space invasion is identified.

By immunohistochemistry, tumour cells are positive for S100, weakly positive for smooth muscle actin and negative for MelanA, HMB45, AE1/3 and desmin.

COMMENT: The plexiform nature of the tumour, and associated plexiform neurofibroma are highly suggestive of Neurofibromatosis Type 1. 

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Case information

rID: 10181
Published: 19th Jul 2010
Last edited: 20th Nov 2015
System: Head & Neck
Tag: orbit
Inclusion in quiz mode: Included

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