Definition – A comparatively rare, connective tissue new-growth, presenting tumors of various size, shape and color and occurring primarily or secondarily in the skin and subcutaneous tissue.
Symptoms – Sarcoma may appear primarily in the skin or develop secondarily to the same process in some other organ or tissue. It may be studied in three divisions : (1) primary pigmented; (2) primary non-pigmented; (3) idiopathic multiple pigmented.
Primary pigmented sarcoma or melanosarcoma is the most malignant and most common form and may develop from an irritated nevus or other pigmentation of the skin, or arise independent of any previous lesion. It begins as a single, small,hard, oval or spheroid growth of a dark blue or blackish color which tends to enlarge from the size of a pea to a nut or egg. It is usually sessile but may be pedunculated, remain solitary and stationary for a long time or multiplies by the appearance of new lesions at near or distant points; one or more may become active, ulcerate or even disappear without a break in the surface. By coalescence of adjacent lesions they may assume the shape of a large lobulated growth. When ulcers form they are irregular, with uneven floors and discharge a blackish substance of variable consistency or sometimes pus. Through the lymphatics some vital organ is soon involved and death is not long delayed. Melanotic whitlow is an insidious form described by Hutchinson which develops as a chronic onychitis with slight pigmentation at the border of the nail like a silver nitrate stain. By gradual evolution it forms a fungating tumor without much change in color; the nail is shed and the disease soon becomes generalized.
Fig. 189 – Sarcoma cutis, secondary to the same disease in the viscera.
Primary non-pigmented or non-Melanotic sarcoma may be localized or generalized. The single or localized form is quite rare and relatively less malignant than any other form of sarcoma. Like the pigmented variety it often originates in an irritated nevus or warty lesion and is usually situated on the extremities. The nodule which forms is firm, whitish, the color of the normal skin or rarely red, and may reach the size of an orange, remain stationary for a variable period, then ulcerate and become fungoid in character. Generalization follows in time and may be accidentally excited by interference.
The generalized form presents multiple lesions on the extremities and chest, rarely on other regions, in the form of pea-to egg-sized, smooth, round, lobulated or irregular tumors, which at first may be subcutaneous, unattached to the superimposed epidermis and whitish or normal in color. They develop rapidly and, before long, the skin between the tumors becomes infiltrated, hard, uneven, glossy, of a bluish-red or purple color, ulcerates at some points and discharges an offensive product. The nodules become attached to the skin and parts beneath, coalesce in a few weeks, soften and form ulcers. Finally death occurs from exhaustion or from involvement of the mucous membranes or viscera. This variety of sarcoma runs an acute course and is very malignant in character.
Fig. 190 – Primary pigmented sarcoma in a native of India. Initial lesion appeared on the left ear after it had been pierced for ear-rings. Other lesions in the same locality by coalescence caused the large lobulated mass so noticeable (courtesy of Dr. Tengshe).
Idiopathic multiple pigmented (hemorrhagic) sarcoma first described by Kaposi, occurs chiefly in male adults between the ages of forty and sixty and usually begins upon the feet, hands and face. In two or three years time the disease may extend by discrete growths along the limbs to the trunk. Edema and pruritus precede the appearance of bluish or purplish spots, caused by cutaneous hemorrhage, on which develop pin-head- to bean-sized, tender nodules of the same color. These gradually increase in size although they may remain discrete, and by multiplication progressively cause an infiltration of the skin, often elephantiac in appearance. The nearer the location of these growths to the distal end of the limb, the greater their number and the larger their size. These tumors may remain stationary for a long time, may disappear by absorption, rarely ulcerate and are often the seat of cutting or lancinating pains. Complete recovery may take place or visceral involvement or death ensue in a period ranging from four to ten years.
Fig. 191 – Primary cutaneous sarcoma, showing the initial lesion on the forehead and the first secondary tumor on the chest.
Fig. 192 – A later illustration of Fig. 191, showing the development of multiple tumors, scattered over a large portion of the surface.
(Courtesy of Dr. Horace Packard.)
Etiology and Pathology. – The causes are practically unknown beyond the fact that local irritation plays an important part in the development of the single, pigmented and non-pigmented forms. Multiple pigmented sarcoma is almost wholly found in males after the age of forty. However, some of the most malignant forms have been observed in children and it is at times congenital, although it may occur at any age. As has been mentioned, sarcoma cutis may be secondary to the disease in some deeper part of the body. It is a connective tissue growth made up of round, mixed round, and spindle cells. The mixed forms, fibro-, angio-, and lymphosarcoma are occasionally found. The pigmented sarcoma which is the commonest type, is believed by many to be carcinomatous, developed from pigmented moles or warts.
Diagnosis of sarcoma is generally not difficult but, in case of doubt, a microscopical examination of the growth should be made. The pigmented forms may be recognized by their color and history. Non-pigmented sarcoma may need to be distinguished from fibroma and epithelioma. The single variety lacks the induration with characterizes epithelioma. The multiple forms are firmer than fibromata, less often pedunculated and frequently undergo degeneration which never occurs in the latter. Sarcoma would hardly be confounded with syphilitic gumma or the nodules of lupus vulgaris, leprosy or mycosis fungoides.
Prognosis and Treatment – A fatal termination may be expected in the great majority of cases. When the growths are single, few or localized, operative measures are indicated but in the other forms, surgery has not only failed but in some instances has promoted generalization. Arsenic is about the only remedy which has been reported as curative and it is best given in hypodermic form (Fowler’s solution). The X-rays are always indicated and may be used in connection with the surgical treatment or alone. Radium has likewise benefited a few, a very few cases. Injections of Coley’s fluid consisting of the combined toxins of the streptococcus and the bacillus prodigiosus, as well as the inoculations with the streptococcus toxin used alone, have not given satisfactory results at my hands, although a few cases have been reported by others as benefited by these agents.