Intraspinal pigmented schwannoma with malignant progression

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Abstract
A case with recurrent pigmented intraspinal tumour with malignant progression is presented. The primary tumour grew around the nerve roots T9 and T10, was attached to dura and infiltrated the vertebral bone tissue. On light microscopy it was comprised of monomorphic cells with large amount of cytoplasmic pigment and many large pigmented globoid bodies. Mitoses were not observed. On electron microscopy, in addition to cytoplasmic melanosomes of regular size, macromelanosomes were numerous. The tumour cells were surrounded partially by basement membrane like material. On these bases a histological diagnosis of benign pigmented tumour of neural crest origin was suggested (a possible pigmented meningioma or pigmented schwannoma). The patient got a recurrence one year after the primary operation. Biopsy from the re-operation showed histologically the same type of tumour with more pleomorphic cells. Subsequently, the tumour grew progressively and metastases were observed in the lungs and in the skin. The patient died two years after the primary operation. The malignant progression of the tumour and other reports on similar tumours was most consistent with a diagnosis of malignant pigmented schwannoma and this was confirmed later on with immunohistochemical staining showing positive staining for basement membrane components, collagen type IV and laminin as well as a positive staining for S-100 protein. The present findings show that despite benign histological features these tumours can behave very aggressively and stress the need of more information on this type of tumour.

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