The treatment is conducted on the same lines as sarcoma in other situations; the insertion of a tube of radium after removal of the tumour diminishes the tendency to recurrence; a portion of the nerve-trunk being sacrificed, means must be taken to bridge the gap. In inoperable cases it may be possible to relieve pain by excising a portion of the nerve above the tumour, or, when this is impracticable, by resecting the posterior nerve roots and their ganglia within the vertebral canal.
The so-called amputation neuroma has already been referred to ([p. 344]).
Diffuse or Generalised Neuro-Fibromatosis—Recklinghausen's Disease.—These terms are now used to include what were formerly known as “multiple neuromata,” as well as certain other overgrowths related to nerves. The essential lesion is an overgrowth of the endoneural connective tissue throughout the nerves of both the cerebro-spinal and sympathetic systems. The nerves are diffusely and unequally thickened, so that small twigs may become enlarged to the size of the median, while at irregular intervals along their course the connective-tissue overgrowth is exaggerated so as to form tumour-like swellings similar to the trunk-neuroma already described. The tumours, which vary greatly in size and number—as many as a thousand have been counted in one case—are enclosed in a capsule derived from the perineurium. The fibromatosis may also affect the cranial nerves, the ganglia on the posterior nerve roots, the nerves within the vertebral canal, and the sympathetic nerves and ganglia, as well as the continuations of the motor nerves within the muscles. The nerve fibres, although mechanically displaced and dissociated by the overgrown endoneurium, undergo no structural change except when compressed in passing through a bony canal.
The disease probably originates before birth, although it may not make its appearance till adolescence or even till adult life. It is sometimes met with in several members of one family. It is recognised clinically by the presence of multiple tumours in the course of the nerves, and sometimes by palpable enlargement of the superficial nerve-trunks ([Fig. 86]). The tumours resemble the solitary trunk-neuroma, are usually quite insensitive, and many of them are unknown to the patient. As a result of injury or other exciting cause, however, one or other tumour may increase in size and become extremely sensitive; the pain is then agonising; it is increased by handling, and interferes with sleep. In these conditions, a malignant transformation of the fibroma into sarcoma is to be suspected. Motor disturbances are exceptional, unless in the case of tumours within the vertebral canal, which press on the spinal medulla and cause paraplegia.
Fig. 86.—Diffuse enlargement of Nerves in generalised Neuro-fibromatosis.
(After R. W. Smith.)
Neuro-fibromatosis is frequently accompanied by pigmentation of the skin in the form of brown spots or patches scattered over the trunk.
The disease is often stationary for long periods. In progressive cases the patient becomes exhausted, and usually dies of some intercurrent affection, particularly phthisis. The treatment is restricted to relieving symptoms and complications; removal of one of the tumours is to be strongly deprecated.