Round-Celled

Sarcomatosis of the skin, except with melanotic growths, is very unusual. When this condition does occur, it is usually with the small round-celled type of neoplasm, for this is the most malignant variety. The following may be taken as a typical example: Patient was a white man aged 41, who had always enjoyed the best of health until six months previously, when small nodules began to appear upon his scalp, shortly followed by their development upon trunk and limbs. Examination revealed a healthy looking, strongly built man who looked the picture of health. All over his body there were, however, at least two hundred small cutaneous and subcutaneous nodules, usually about 1 cm. in diameter, but a few were 4 to 5 cm. in diameter. There was no ulceration of the skin over them. Tumors were freely movable in the skin, and were rather firm to the touch. There was no marked dilatation of the surface vessels. In the groin there were a number of enormously enlarged glands. Excision of one of the small growths showed a typical small round-celled sarcoma. In other instances the tumors are much fewer in number and may be comparatively large. It is rare to find a spindle-celled sarcomatosis of the skin except in tumors of the multiple hemorrhagic variety. The outlook is hopeless; neither Coley's serum nor x-ray has any effect, and patients usually die within a year.

Melanotic

The multiple melanotic growths, so frequently called melanotic sarcomata, have already been described in another chapter.

Pigmented Hemorrhagic (Kaposi)

The multiple pigmented hemorrhagic sarcoma of Kaposi* is rather a curious lesion in many respects, for it lacks the malignancy of a true spindle-celled sarcoma, although composed of that variety of tissue; at times it seems to heal spontaneously, and may be related to the sarcoid group of tumors.

Incidence

The disease is rather rare in both England and America, although cases in the latter country have been described by Fordyce,* Hyde,* Hartzell,* and others. In continental Europe it is much commoner. Kaposi has had over thirty cases, De Amicis* in Italy over fifty, and many have been reported from Austria and Russia. In America it occurs most commonly in Jewish immigrants, which might seem to speak for its infective nature.

*Kaposi: Kaposi and Hebra, Dis. of Skin,* New Sydenham Soc. Translation, London, 1875.

*Fordyce: Jour. Cut. and Gen. Urin. Dis., 1891, ix, 1.

*Hyde: Quoted by Crocker. "Hartzell: Jour. Cut. Dis., 1908, xxvi, 97. "De Amicis: II Morgagni, 1872.

Clinical Course

The affliction always begins on either the hands or feet, usually the dorsal surface. At first an edema is noted, then bluish spots appear, in the center of which are nodosities. The tumors slowly increase in both size and number, usually traveling up the limb. The neoplasms are either round or flat, are dark-red at first, then purple, and then brown. The color is due to hemorrhage and the deposit of blood pigment. They vary in number from twenty to several hundred, and in size from 1 cm. to 15 cm. (Fig. 57). They may be hard or soft, and usually bleed readily when punctured. The general health is not affected for several years, and in the early stages there is no glandular involvement. The patients ultimately die from general sarcomatosis. Mariani* has given the best survey of the subject, including literature, yet extant. It is to be noted that these tumors grade off into the Spiegler-Fendt type, usually called sarcoids, and also described by Polland as comparatively benign sarcomata.

Pathology

Histologically, there may be seen numerous large cavities filled with blood, the space between the cavities being composed of parallel rows of spindle cells, between which is deposited much pigment. Traces of hemorrhages may be seen in places. This picture has been interpreted by some as the result of infection- that is, that the tumor is in reality a granuloma-and by others as a pure sarcoma of a special type, the latter view predominating.

Treatment

Treatment is not satisfactory. According to Kob-ner,* Sherwell,* and De Amicis, arsenic will cure some cases if given in large amounts and over a considerable space of time, Fowler's solution being the form generally employed. Possibly cacodylate of soda, used intramuscularly, might produce a better effect, and yet one must be inclined to agree with Fox and Wile, who believe that arsenic cures only sarcoids and not true sarcomata. X-ray is useful in certain cases, though probably not for any great length of time. One observer has reported the disappearance of the lesions following the local application of bichloride solutions.