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Fig. 141.—Multiple Cartilaginous Exostoses in a man æt. 27, the same as in Fig. 140.
Diffuse Osteoma, Leontiasis Ossea.—This rare affection was described by Virchow, and named leontiasis ossea because of the disfigurement to which it gives rise. It usually commences in adolescence as a diffuse overgrowth first of one and then of both maxillæ; these bones are enlarged in all directions and project on the face, and the nasal fossæ and the maxillary and frontal sinuses become filled up with bone, which encroaches also on the orbital cavities. In addition to the hideous deformity, the patient suffers from blocking of the nose, loss of smell, and protrusion of the eyes, sometimes followed by loss of sight. The condition is liable to spread to the zygomatic and frontal bones, the vault of the skull, and to the mandible. The base of the skull is not affected. The disease is of slow progress and may become arrested; life may be prolonged for many years, or may be terminated by brain complications or by intercurrent affections. In certain cases it is possible to remove some of the more disfiguring of the bony masses.
A less aggressive form, confined to the maxilla on one side, is sometimes met with, and, in a case of this variety under our own observation, the disfigurement, which was the only subject of complaint, was removed, after reflecting the soft parts, by paring away the excess of bone; this is easily done as the bone is spongy, and at an early stage, imperfectly calcified.
A remarkable form of unilateral hypertrophy and diffuse osteoma of the skull, following the distribution of the fifth nerve, has seen described by Jonathan Hutchinson and Alexis Thomson.
Chondroma.—Cartilaginous tumours, apart from those giving rise to multiple exostoses, grow from the long bones and from the scapula, ilium, ribs, or jaws. They usually project from the surface of the bone, and may attain an enormous size; sometimes they grow in the interior of a bone, the so-called enchondroma.
Fig. 142.—Multiple Chondromas of Phalanges and Metacarpals in a boy æt. 10 (cf. Fig. 143).
The hyaline cartilage composing the tumour frequently undergoes myxomatous degeneration, resulting in the formation of a glairy, semi-fluid jelly, and if this change takes place throughout the tumour it comes to resemble a cyst. On the other hand, the cartilage may undergo calcification or ossification. The most important transition of all is that into sarcoma, the so-called malignant chondroma or chondro-sarcoma, which is associated with rapid increase in size, and parts of the tumour may be carried off in the blood-stream and give rise to secondary growths, especially in the lungs.
Fig. 143.—Skiagram of Multiple Chondromas shown in Fig. 142.
Cases have been met with in which certain parts of the skeleton—only those developed in cartilage—were so uniformly permeated with cartilage that the condition has been described as a “chondromatosis” and is regarded as dating from an early period of fœtal life. Unlike the condition known as multiple cartilaginous exostoses, it is a malignant disease.
The chondroma is met with as a slowly growing tumour which is specially common in the bones of the hand, often in a multiple form (Figs. 142 and 144). The surface is smooth or lobulated, and in consistence the tumour may be dense and elastic like normal cartilage, or may present areas of softening, or of bony hardness. The skin moves freely over it, except in relation to the bones of the fingers, where it may become adherent and ulcerate, simulating the appearance of a malignant tumour. Large tumours growing from the bones of the extremities may implicate the main vessels and nerves, either surrounding them or pressing on them.
Portions of a chondroma, which have undergone calcification or ossification, throw a dark shadow with the X-rays; unaltered cartilage and myxomatous tissue appear as clear areas.
Fig. 144.—Multiple Chondromas in Hand of boy æt. 8
Treatment.—It is necessary to remove the whole tumour, and in chondromas growing from the surface of the bone, especially if they are pedunculated, this is comparatively easy. When a bone, such as the scapula or mandible, is involved, it is better to excise the bone, or at least the part of it which bears the tumour. In the case of central tumours the shell of bone is removed over an area sufficient to allow of the enucleation of the tumour, or the affected portion of bone is resected. Should there be evidence of malignancy, such as increased rate of growth, a tube of radium should be inserted, and in advanced cases with destruction of tissue, amputation may be called for.
In multiple chondromas of the hand in young subjects, it was formerly the custom to amputate the limb; an attempt should be made to avoid this by shelling out the larger tumours individually, and persevering with the application of the X-rays or of radium to inhibit the growth of the smaller ones.
Chondromas springing from the pelvic bones usually arise in the region of the sacro-iliac joint; they project into the pelvis and press on the bladder and rectum, and on the sciatic and obturator nerves; sometimes also on the iliac veins, causing Ĺ“dema of the legs. They are liable to take on malignant characters, and rarely lend themselves to complete removal by operation.
Fig. 145.—Radiogram of Myeloma of Humerus.
(Mr. J. W. Struthers' case.)
Fibroma is met with chiefly as a periosteal growth in relation to the mouth and pharynx, the simple epulis of the alveolar margin and the naso-pharyngeal polypus being the most common examples. We have met with a fibroma in the interior of the lower end of the femur of an adult, causing expansion of the bone with decided increase in girth and liability to pathological fracture; it is possible that this represents the cured stage of osteomyelitis fibrosa.
Myxoma, lipoma, and angioma of bone are all rare.
Myeloma.—The myeloid tumour, which is sometimes classified with the sarcomas, contains as its chief elements large giant cells, like those normally present in the marrow. On section these tumours present a brownish-red or chocolate colour, and, being highly vascular, are liable to hæmorrhages, and therefore also to pigmentation, and to the formation of blood cysts. Sometimes the arterial vessels are so dilated as to impart to the tumour an aneurysmal pulsation and bruit. The enlargement or “expansion” of the bone results in the cortex being represented by a thin shell of bone, which may crackle on pressure—parchment or egg-shell crackling.
The myeloma is most often met with between the ages of twenty-five and forty in the upper end of the tibia or lower end of the femur. It grows slowly and causes little pain, and may long escape recognition unless an examination is made with the X-rays. Although these tumours have been known to give rise to metastases, they are, as a rule, innocent and are to be treated as such. When located in the shaft of a long bone, pathological fracture is liable to occur.
Diagnosis and X-ray Appearances of Myeloma.—The early diagnosis of myeloma is made with the aid of the X-rays: the typical appearance is that of a rounded or oval clear area bounded by a shell of bone of diminishing thickness (Fig. 145). The inflammatory lesions at the ends of the long bones—tubercle, syphilitic gumma, and Brodie's abscess, that resemble myeloma, are all attended with the formation of new bone in greater or lesser amount. The myeloma is also to be diagnosed from chondroma, from sarcoma, and from osteomyelitis fibrosa cystica.
Treatment.—In early cases the cortex is opened up to give free access to the tumour tissue, which is scraped out with the spoon. Bloodgood advises the use of Esmarch's tourniquet, and that the curetting be followed by painting with pure carbolic acid and then rinsing with alcohol; a rod of bone is inserted to fill the gap. In advanced cases the segment of bone is resected and a portion of the tibia or fibula from the other limb inserted into the gap; a tube of radium should also be introduced.
Fig. 146.—Periosteal Sarcoma of Femur in a young subject.
The coexistence of diffuse myelomatosis of the skeleton and albumosuria (Bence-Jones) is referred to on p. 474. Myeloma occurs in the jaws, taking origin in the marrow or from the periosteum of the alveolar process, and is described elsewhere.
Sarcoma and endothelioma are the commonest tumours of bone, and present wide variations in structure and in clinical features. Structurally, two main groups may be differentiated: (1) the soft, rapidly growing cellular tumours, and (2) those containing fully formed fibrous tissue, cartilage, or bone.
Fig. 147.—Periosteal Sarcoma of Humerus, after maceration.
(Anatomical Museum, University of Edinburgh.)
(1) The soft cellular tumours are composed mainly of spindle or round cells; they grow from the marrow of the spongy ends or from the periosteum of the long bones, the diploë of the skull, the pelvis, vertebræ, and jaws. As they grow they may cause little alteration in the contour of the bone, but they eat away its framework and replace it, so that the continuity of the bone is maintained only by tumour tissue, and pathological fracture is a frequent result. The small round-celled sarcomas are among the most malignant tumours of bone, growing with great rapidity, and at an early stage giving rise to secondary growths.
(2) The second group includes the fibro-, osteo-, and chondro-sarcomas, and combinations of these; in all of them fully formed tissues or attempts at fully formed tissues predominate over the cellular elements. They grow chiefly from the deeper layer of the periosteum, and at first form a projection on the surface, but later tend to surround the bone (Fig. 150), and to invade its interior, filling up the marrow spaces with a white, bone-like substance; in the flat bones of the skull they may traverse the diploë and erupt on the inner table. The tumour tissue next the shaft consists of a dense, white, homogeneous material, from which there radiate into the softer parts of the tumour, spicules, needles, and plates, often exhibiting a fan-like arrangement (Fig. 151). The peripheral portion consists of soft sarcomatous tissue, which invades the overlying soft parts. The articular cartilage long resists destruction. The ossifying sarcoma is met with most often in the femur and tibia, less frequently in the humerus, skull, pelvis, and jaws. In the long bones it may grow from the shaft, while the chondro-sarcoma more often originates at the extremities. Sometimes they are multiple, several tumours appearing simultaneously or one after another. Secondary growths are met with chiefly in the lungs, metastasis taking place by way of the veins.
Clinical Features.—Sarcoma is usually met with before the age of thirty, and is comparatively common in children. Males suffer oftener than females, in the proportion of two to one.
In periosteal sarcoma the presence of a swelling is usually the first symptom; the tumour is fusiform, firm, and regular in outline, and when it occurs near the end of a long bone the limb frequently assumes a characteristic “leg of mutton” shape (Fig. 146). The surface may be uniform or bossed, the consistence varies at different parts, and the swelling gradually tapers off along the shaft. On firm pressure, fine crepitation may be felt from crushing of the delicate framework of new bone.
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