Fibromata, Part 2

Anomalies and Curiosities of Medicine
by George M. Gould, M.D., Walter L. Pyle, M.D.

(Page 19 of 36)

Nine months after this accident there was noticed an enlargement of the middle joint of the little finger, and about the same time an enlargement on the middle finger. Gradually all the joints of the right hand became involved. The enlargement increased so that at the age of twelve they were of the size of walnuts, and at this time the patient began to notice the same process developing in the left hand. The growths continued to develop, new nodules appearing, until the fingers presented the appearance of nodulated potatoes.

One of the most frequent of the fibro-cartilaginous tumors is the "mixed cartilaginous" tumor of Paget, which grows in the interstitial tissues of the parotid gland, and sometimes attains enormous size. Matas presented the photograph of a negress having an enormous fibroma growing from the left parotid region; and there is a photograph of a similar case in the Mutter Museum of the College of Physicians, Philadelphia.

The hyaline enchondroma is of slow growth, but may at times assume immense proportions, as is shown in the accompanying illustration, given by Warren, of a patient in whom the growth was in the scapula.

In 1824 there is quoted the description of a peculiar growth which, though not definitely described, may be spoken of here. It was an enormous encysted tumor, springing from the clavicle of a Veronese nobleman. Contrary to general expectations it was successfully removed by Portalupi, a surgeon of Venice. It weighed 57 pounds, being 20 1/2 inches long and 30 inches in circumference. It is said this tumor followed the reception of a wound.

Among the benign bone tumors are exostoses - homologous outgrowths differing from hypertrophies, as they only involve a limited part of the circumference. When developmental, originating in childhood, the outgrowths may be found on any part of the skeleton, and upon many and generally symmetric parts at the same time, as is shown in Figure 248.

Barwell had a case of a girl with 38 exostoses. Erichsen mentions a young man of twenty-one with 15 groups of symmetric exostoses in various portions of the body; they were spongy or cancellous in nature.

Hartmann shows two cases of multiple exostoses, both in males, and universally distributed over the body.

Macland of the French navy describes an affection of the bones) of the face known as anakhre or goundron (gros-nez). It is so common that about one per cent of the natives of certain villages on the Ivory Coast, West Africa, are subject to it. As a rule the earliest symptoms in childhood are: more or less persistent headache, particularly frontal, sanguineous and purulent discharge from the nostrils, and the formation of symmetric swellings the size of an almond in the region of the nasal processes of the superior maxilla. The cartilage does not seem to be involved, and, although it is not so stated, the nasal duct appears to remain intact.

The headache and discharge continue for a year, and the swelling continually increases through life, although the symptoms gradually disappear, the skin not becoming involved, and no pain being present. It has been noticed in young chimpanzees. The illustration represents a man of forty who suffered from the disease since puberty. Pressure on the eyeball had started and the native said he expected that in two years he would lose his sight. Figure 251 shows an analogous condition, called by Hutchinson symmetric osteomata of the nasal processes of the maxilla. His patient was a native of Great Britain.

Among neuromata, multiple neurofibroma is of considerable interest, chiefly for the extent of general involvement. According to Senn, Heusinger records the case of a sailor of twenty-three in whom all the nerves were affected by numerous nodular enlargements. Not a nerve in the entire body was found normal. The enlargement was caused by increase in the connective tissue, the axis-cylinders being normal. In this case there was neither pain nor tenderness.

Prudden reports the case of a girl of twenty-five who, during convalescence from variola, became paraplegic, and during this time multiple neuromata appeared. At the postmortem more than a thousand tumors were found affecting not only the peripheral branches and the sympathetic, but also the cranial nerves and the pneumogastric. Under the microscope these tumors showed an increase in the interfascicular as well as perivascular fibers, but the nerve-fibers were not increased in size or number. Virchow collected 30 cases of multiple neurofibromata. In one case he found 500, in another from 800 to 1000 tumors.

Plexiform neuroma is always congenital, and is found most frequently in the temporal region, the neck, and the sides of the face, but almost any part of the body may be affected. Christot reports two cases in which the tumors were located upon the cheek and the neck. Czerny observed a case in which the tumor involved the lumbar plexus. Quoted by Senn, Campbell de Morgan met with a plexiform neuroma of the musculo-spiral nerve and its branches. The patient was a young lady, and the tumor, which was not painful, had undergone myxomatous degeneration.

Neuroma of the vulva is a pathologic curiosity. Simpson reports a case in which the tumor was a painful nodule situated near the urinary meatus. Kennedy mentions an instance in which the tumor appeared as extremely tender tubercles.

Tietze describes a woman of twenty-seven who exhibited a marked type of plexiform neurofibroma. The growth was simply excised and recovery was promptly effected.

Carcinomatous growths, if left to themselves, make formidable devastations of the parts which they affect. Warren pictures a case of noli-me-tangere, a destructive type of epithelial carcinoma. The patient suffered no enlargement of the lymphatic glands. The same absence of glandular involvement was observed in another individual, in whom there was extensive ulceration. The disease had in this case originated in the scar of a gunshot wound received during the Civil War, and had destroyed the side of the nose, the eye, the ear, the cheek, including the corresponding half of the upper and lower lips.