A female skull dating from 1829 with the bony skeleton of a large facial tumour (possibly caused by neurofibromatosis) involving the right side of the face. The tumour arose in the right antrum, and during five years’ growth destroyed the right malar bone, the palate, and the maxilla. Specimen from the Hunterian Museum of the Royal College of Surgeons, London.
DEFINITION: Neurofibromatosis is a genetically-inherited disorder in which the nerve tissue grows tumors (i.e., neurofibromas) that may be harmless or may cause serious damage by compressing nerves and other tissues. The disorder affects all neural crest cells (Schwann cells, melanocytes, endoneurial fibroblasts). Cellular elements from these cell types proliferate excessively throughout the body forming tumors and the melanocytes function abnormally resulting in disordered skin pigmentation. The tumors may cause bumps under the skin, colored spots, skeletal problems, pressure on spinal nerve roots, and other neurological problem. [Wikipedia]
DESCRIPTION: [Facial tumour possibly caused by neurofibromatosis] ‘An old Gentleman, a Servant to the King below [the] stairs, brought his Son to me, an infirm Youth of about thirteen years of age, having a large Tumour behind his right Ear arising from a Crude Matter. It had been of some years growth. I applied [an] Emplastr. ad strumas cum stercore columbino… but it heated: whereupon I applied a Cataplasm…then opened it by Caustick [a substance which causes the skin to corrode], and discharged a mixt crude Matter [by scraping the inside with a stone]. The Ulcer being sordid, I laid it open more, and dressed it’. [Richard Wiseman, Eight Chirurgicall Treatises (1676), p. 51.]