THE CLASSIC: Sarcoma of the Long Bones

Abstract

Samuel Weissel Gross (1837–1889) of Philadelphia was lecturer in surgical pathology at the Jefferson Medical College during the latter half of the 19th century. Although around the mid-century (1945) Boyer had named these tumors sarcoma and they had been studied by Nelaton and others, Garrison considered this extensive study by Gross the “first comprehensive work on bone sarcoma.” In referring to the author of this paper, one must be wary of confusing him with his father, Samuel D. The elder Gross started in Lexington, Kentucky, but spent most of his important years as Samuel W.’s predecessor at the Jefferson, and was one of the leading surgeons and surgical pathologists of his era. ARTICLE I In the last edition of his work on “Surgical Pathology,” Professor Billroth states that “the subdivisions, made according to the histological peculiarities of the various sarcomata, are of no great value during life”; but being convinced, from personal observation, that clinical differences exist among the divisions of sarcoma as marked as are met with among the varieties of carcinoma, I have ventured to differ from so distinguished an investigator, and have studied these growths, as all morbid processes should be studied, with the view of determining the relations subsisting between their minute structure and their clinical characters. To attain this end I have carefully analyzed 165 cases, many of which are original, and the number might have been very materially increased had I included examples of mixed sarcomas. I have limited my investigations, however, to those cases alone in which the histological features of each form of sarcoma were typical, and I have used none except a few of osteoid sarcoma, about the true nature of which there could be no doubt, that were not confirmed by microscopical examination. The primary neoplasms of the long bones, exclusive of the clavicle, the metatarsal and metacarpal bones, and the phalanges, which I shall not consider, are, in the order of the frequency of their occurrence, sarcomas, osteomas, chondromas, osteoid chondromas, fibromas, and myxomas. Vascular and cystic growths, as I shall have occasion to show hereafter, are, for the most part, nothing more than metamorphosed sarcomas, while it is extremely doubtful whether primary carcinoma ever occurs in the osseous system. Since sarcomas affect the long bones more frequently than all the other tumors combined, and as they constitute an important group of morbid growths which are not thoroughly understood, and are described under a great number of different and confusing names, I shall devote this and a succeeding paper to a description of their histology, general pathology, symptomatology, diagnosis, prognosis, and treatment, covering, as far as possible, the entire field, although in doing so, I will touch upon some points which may be considered elementary, but which I trust may prove to be instructive to those not entirely familiar with the subject. The term sarcoma, which is of very ancient date, and was employed by Abernethy to designate tumors “having a firm and fleshy feel,” was revived by Virchow, and is now employed by him and other pathologists and most surgeons to indicate a new formation of the connective tissue series, which is composed almost entirely of unripe, transitional, or embryonic cells, which serve to distinguish it, on the one hand, from the perfected tumors of that system, and, on the other, from the carcinomas. From the excessive preponderance, development, and mode of arrangement of its cellular constituents, which endow it with its peculiar characters, and form their indisposition to pass into higher tissues, they constitute a structure which is unlike any natural structure, and may, therefore, be regarded as an atypical production. The cells are contained in an intercellular substance, which is hyaline, granular, fibrillated, or alveolar, which, along with the various degenerations to which these tumors are liable, give rise to certain subdivisions. The principal varieties of sarcoma of the bones, which are determined by the prevailing form and size of their cellular elements, are the round-celled, the spindle-celled, and the giant-celled, the cells themselves merely representing different stages of development. In accordance with the dimensions of the cells, they are, moreover, separated into the small-celled, and large-celled, a distinction which is most useful, not only because the size of the cells influences the consistence of the tumors, but from the fact that it has a special bearing upon the prognosis. Certain subdivisions of sarcoma are constituted by the nature of their matrix or intercellular substance, by their transformations, and by their vascularity. Thus, when fibrous tissue replaces in great part the ordinary intercellular substance, the growth is called a fibrous sarcoma. When the basis substance has undergone mucoid transformation, it is a myxomatous sarcoma; when it presents the appearance of the reticulum of the lymph glands, it is known as lymphadenoid sarcoma; when it forms an alveolar structure, it is an alveolar sarcoma; when the cells have undergone fatty degeneration and granules of fat and free oil are disseminated in the intercellular substance, it is a lipomatous sarcoma; the presence of certain other constituents, as the mineral salts, bone, or cartilage, produces respectively calcifying, ossifying, and chondroid sarcomas; when they have undergone cystic degeneration, they are known as cystic sarcomas; excessive vascularity gives rise to telangiectatic sarcoma; and effusions of blood, to hemorrhagic sarcoma. All these subdivisions, pure or variously intermixed, are met with in the sarcomas of the long bones, the only one that is missing being pigmented or melanotic sarcoma, of which I have been unable to find a single recorded example. Sarcoma of the bones is generally denominated osteosarcoma, but the latter term, which is employed by clinicians, should be dropped, since, from a histological standpoint, it signifies a sarcoma, whether it be seated in or on a bone, or in the soft parts, containing osseous tissue of new formations, and is synonymous with osteoid or ossifying sarcoma. As an evidence of the confusion that has arisen from retaining that name, I may mention that Wilks and other English writers call the periosteal osteoid sarcomas osteosarcomas, while many German pathologists employ the term to designate myeloid tumors, which are always central. According to their point of departure they may be separated into the peripheral, periosteal, or periosseous, and the central, endosteal, intraosseous, or myelogenic, a division which is not only convenient as designating their seat, but which is also justified by the fact, that, in general, they present marked differences in their histological construction, and are of different prognostic import. Thus, the majority of the peripheral growths, which proceed from the layer of embryonic cells, or osteoblasts, which separates the periosteum from the bone, and is variously known as the osteogenic substance of Ollier, the ossification blastema of Kölliker, the proliferating layer of the periosteum of Virchow, and the cambium layer of the bone of Max Schultze, are osteoid spindle-celled growths, while the central tumors, which develop from the medulla, and from the osteoblastic layer which lines the interior of the medullary canal, are, as a rule, giant-celled sarcomas. In their macroscopic or gross appearances these two divisions of sarcoma present some points of difference which may be utilized for diagnostic and prognostic purposes, in the absence of microscopical examination. The central tumors, particularly those of the giant-celled variety, are inclosed in a capsule or case, which may be entirely bony, but which is usually partly bony and partly membranous. The periosteal formations, on the other hand, are limited by the outer fibrous layer of that membrane, or, in the event of its participation in the proliferation, by a capsule of connective tissue. In either event, osseous tissue is never found in the investing membrane, so that the presence or absence of bone in the capsule is of great value in the determination of the original seat of the new growth. Although the sarcomas are very liable to certain transformations which tend to obscure their diagnosis from their naked-eye appearances, yet, in their pure forms, their structure is so dissimilar, that we may judge pretty accurately of their nature by their tints and consistence. Thus a tumor which presents a dark red or maroon color can be nothing else than a giant-celled sarcoma. Specimens which are characterized by a firm, glistening, grayish-white tissue come under the subdivision of large spindle-celled sarcoma. When there is an admixture of bony spicules and soft tissue, there need be no doubt as to its osteoid nature; should the soft portion be dense and firm, it may be termed an osteoid fibrous sarcoma; if it be soft and medullary, it will probably turn out to be an osteoid round-celled tumor; while, if it be semifluid and gelatinous, it forms a myxomatous osteoid sarcoma. Tumors which have the consistence and appearance of the brain of a newly-born child may be ranked among the medullary or encephaloid sarcomas of the small round-celled type, although the soft, small spindle-celled tumors present precisely similar features. When the tissue looks like a recent coagulum, or contains one or more cysts filled with blood, we will be right in assuming that we are dealing with a highly vascular medullary growth. These conclusions are based upon the combined clinical and microscopical characters of sarcomas, and may be held to be as true for this group of neoplasms as it is true that the gross characters of scirrhous carcinoma, for example, correspond to a known type of structure. In their configuration, sarcomas of the long bones vary in accordance with their situation. Central tumors, whether they occupy the epiphyses or diaphyses, are generally spherical. Periosteal tumors are long, oval, or fusiform, when they occupy the shafts; while they are pyriform, with their base towards the articulation, when they surround the articular ends of the long bones; or conoidal, when they develop from a limited portion of the joint-end. Their surface is usually smooth and even, although it may be tuberous, bossed, or lowly-lobed, which is particularly liable to be the case when their investing capsules are implicated in the new formation, and when they have softened, and are about to become the seat of ulceration. Their volume varies very greatly. They may be no larger than a walnut, or they may attain huge dimensions, as, for example, three feet in circumference. In the latter event, it may be assumed that the growth is the seat of interstitial hemorrhage or cystic transformation. So long as they are circumscribed by their investing capsules, sarcomas merely stretch and push aside the surrounding tissues; but when their capsules take part in the cell proliferation, or are perforated, they evince a locally infectious nature, and become diffused, either through the conversion of the cells of the tissues into like elements, or through the continuous multiplication of the tumor elements. In the peripheral sarcomas, the bone generally passes through the growth unchanged. In some instances the cortex of the compact substance is the seat of condensing sclerosis; in others, it is eroded, pitted, or, as it were, worm-eaten. In others, infection of the compact tissue ensues without changes in its outer surface, through which nodules are formed in it, and give rise to what may be termed interstitial sarcoma, although I have never known a tumor to originate primarily in this situation. Again, the disease may extend along the Haversian system to infect the medulla. Finally, the tumor may occupy all these localities, so that we may even find a sarcoma which has originated in the periosteum constituted as follows: masses in the medulla, interstitial deposits in the compact tissue, a large tumor around the shaft, and, finally, one growing from the fibrous layer of the periosteum, the last two of which are merely separated and cut up into lobules by the remains of that membrane. In the central sarcomas, the original bone is usually destroyed to such an extent that not a vestige of it remains. Next to the carcinomata of the soft parts, the sarcomata of the bones are the most malignant of all neoplasms, as is evinced by the local infection of the adjacent tissues, the occasional implication of the associated lymphatic glands, and the occurrence of secondary deposits in the viscera and tissues belonging to the same series. From their tendency to infiltrate the surrounding structures, they show a great disposition to recur locally after extirpation, so that in the performance of an operation for their removal, the rule should be to amputate as far as possible from the seat of the disease. Unlike the carcinomata, the lymphatic glands are rarely the seat of primary contamination. Thus, of 133 cases in which this point is noted, these organs were enlarged in only In of these the lymphatic tumor was merely to as was by the fact, that in some cases it after the of the primary while, in others, minute to elements. In cases, the of the glands was not while, in only were they into a tissue similar to the growth. These are of as they in the that of the lymphatic glands should not be regarded as a to and, that infection generally through the and not through the lymph The general of sarcoma, or the occurrence of secondary growths in the viscera and in other tissues, is one of the most marked of this of morbid Thus I find that of all cases are characterized by this clinical the tumors being found most in the lymphatic glands, and other of the osseous system. In this it should be that the secondary deposits are with the writers a case, for example, as one of sarcoma of the with secondary deposits in the in the latter not in their structure from the original sarcoma always its a carcinoma its a its so that the peculiarities of a to as to the nature of the original tumor in the event of the latter been by some other point of distinction between the secondary and growths, as of the is the presence of in the and its absence in the it is true that the sarcomas of the bones are to be regarded as malignant the of that is to infection of the tissues and local to the lymphatic glands, and multiplication in are not in the same in the different varieties of sarcoma, as is by the an of the cases of sarcomas, they may be in the order of their as follows: Periosteal spindle-celled, periosteal round-celled, periosteal central round-celled, central spindle-celled, and central the periosteal forms being more malignant by as will be out the of the central tumors may, to a certain be to the disposition which they evince to extend their bony or to the sarcomas composed of cells and they are synonymous with the tumors of it will be that they are with the of general to an so that the term as designating the in their is in to of the long bones evince a great for their articular and the majority develop in the substance of the of central or peripheral they are liable, even in the of the most to be with other such as periosteal and of the bones and articular have rise to the number of They may be from by the and from by the of the in to the gives rise to of the soft structures, and is with and, now and with From of the long bones, if there is such an and its occurrence is by and the distinction is by no always particularly in to the there is a as in instances to which I shall call In the majority of sarcomas there is no or, if it be it is not very in sarcoma, is not found from the very is it and in some cases it is not the tumor to as is are extremely the while of the sarcomas that Finally, when a growth is from the of a large as, for example, in the of the it is far more to be a sarcoma than an is very liable to occasion in the diagnosis, and this is particularly true of those cases in which the adjacent has undergone changes from or has been by the growing when the are so similar that a diagnosis, without is as of the for example, usually occurs in the is from the very which in and so as to the of and his general The is and the are the is the is if not and at great the articulation, moreover, is and and gives to or containing of not In sarcomas, the absence of or the tendency to the formation of is a of great important features of sarcoma are the of the of the although in some cases the is from on and absence of In many cases, the are to about without or In sarcoma, is not to ordinary treatment, moreover, sarcoma from In the latter and along with and of the are of great in the and in the general but in sarcoma, the tumor to the of these and the local is increased by These which have been carefully studied by and when considered in with the will generally to the sarcomas from articular of the long bones are most in those of the the and being in of all Thus of 165 cases which I have the was the seat of the disease in the the the the the the and were the seat of the disease in these in accordance with their histological construction, were examples of giant-celled sarcoma; periosteal osteoid sarcoma; central spindle-celled sarcoma; periosteal round-celled sarcoma; central round-celled sarcoma; periosteal spindle-celled sarcoma. it that the giant-celled tumors are the most as they of the entire will also be that the central tumors are more by than those which in the are more liable to their occurrence by than as is in the of cases in which the is at which they themselves In the of cases in which the is I have separated their occurrence at the different of from which it will be that develop the and after that of the long bones to be in their histological by the at which they the of the central spindle-celled, which are most after the the being the they all develop that the being for the periosteal for the periosteal round-celled, for the periosteal spindle-celled, for the central round-celled, and a for the was the in of the cases in which the is They could be to and other in out of cases of giant-celled sarcoma; of of central round-celled sarcoma; of of central spindle-celled sarcoma; of of periosteal round-celled sarcoma; of of periosteal osteoid sarcoma; of of periosteal spindle-celled sarcoma. to what I to be some of the more important points in the general pathology and diagnosis of these new formations, I will now take up the study of the sarcomas, in which these will more In the of this of tumors, general being no should be in to or Although surgical has been by a of there can be no doubt that it has frequently in local and as as The more the growth of the the more there is for in view of the in determining the true nature of the in its are extremely since, of to with a pure myeloid sarcoma, the disease may prove to be an osteoid sarcoma, or a medullary sarcoma, in which will have been and the have been to the of local infection and general In an operation the rule should be to as far as possible from the seat of without The for this of will be if it be that the soft parts, the bones, and even the may be by of the disease. it is that when have been to the or in to the tumors, the is as, in the event of nothing more can be In the cases of and for example, in which, at the and of of the had been for myeloid tumors of that bone, there was in the and in the in a case in which of the for a as large as the which had its capsule and processes between the of the from the of the and of the were found to be the seat of In all of these cases, tissue had the of the and precisely the same may if a be in its to the morbid growth. From these I the general for in the point at which should be tumors of the epiphyses of the and the when the epiphyses of these bones are through the of the at the of the and of the for disease of the at the for that of the of the the for tumors of the epiphyses of the and through the of the when the of the is at the of the and of the for disease of the of the and at the when the of the is In the event of the being for example, not larger than an it will not be to so a tumor of that size the end of the or the may, with great be through its the other hand, a tumor may the to be made Thus, a as large as the in the of the had be by at the of of an entire or of the along with the shaft, of the more long bones may be particularly if the tumor presents a smooth surface, and is of a firm, dense or is inclosed in an osseous these the surrounding tissues most be found to be free from if the morbid growth is and soft and or the will be to be and should be In cases of doubt, may be and of the be for it, if the capsule of the tumor is to be perforated, and the soft by the morbid the periosteum of the adjacent bone is to the capsule of the of if the bone is eroded, the latter should also be since, in these it is liable to be the seat of From an standpoint, can be in of except when the end of the is in a small and the can be through which the remains In a case of this in his a while in the the were the after is by than after the fact that the was on the bones of the except in two while the was the seat of the majority of the operation be if it be the seat of the the should be to Thus in cases I and of the not than were while in the case of Professor Gross of at the the of was so of the that in two