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Discussion of the diagnosis and x-ray treatment of malignant disease of bone
NEW SERIES, VOL. XVIII
DECEMBER,
No. 3
1932
DISCUSSION OF THE DIAGNOSIS AND X,RAY TREATMENT
MALIGNANT
OF
DISEASE OF BONE*
SHERWOOD MOORE, M.D. ST. LOUIS,
UCH of the substance of this paper is at variance with accepted views and wiI1 be disagreed with by many readers as it departs from orthodoxy at severa points. The subject of bone tumors is far too obscure and unnecessariIy so. It stands greatIy in need of simpIification. The skeIeton is a pecuIiarIy suitabIe structure for investigation by x-ray methods. Since their advent into medicine, knowIedge of the skeIeton, its anatomy, physioIogy and pathoIogy has grown enormousIy. The skeIeton is far better understood than connective tissue, cartiIage, adipose tissue, muscIe, etc. Of the diseases of bone, which have been most extensiveIy studied, both on account of the great aid afforded by x-ray methods and because of their highIy IethaI nature, tumor has attracted much attention. Bone tumor has been for the past severa years a popuIar subject for investigation and pubIication. Impetus to this arises from the work of the Registry of Bone Sarcoma of the American CoIIege of Surgeons. Credit for the notabIe accompIishment of this body shouId be given to Codman. There are such authoritative pubIications on malignant bone tumors that any prospect of adding anything origina to that which has ah-eady been written is aImost out of the question. However, many physicians are unacquainted with
M
* From the Department
of Radiology,
MO.
this work. It wiI1 be the endeavor of this paper to direct attention to and popuIarize some of the exceIIent existing pubIications on maIignant bone tumors with the idea of simpIifying this obscure subject. These pubIications are the studies of the materia1 in the Registry of Bone Sarcoma of the American CoIIege of Surgeons by Codmanl and KoIodny,2 various pubIications by BIoodgood,3 Ewing,4 and Connor, and the monograph of Geschickter and CopeIand.6AII of these are masterIy. NevertheIess, it seems to a radioIogist that there are certain points concerning bone tumors that these writers fai1 to deveIop. I wish to caI1 attention to a significant fact, i.e., that these pubIications, with the exception of those of Ewing, were written by surgeons, and they necessarily Iack the genera1 view of the subject of gross bone pathoIogy which one acquires in the radioIogicaI or pathoIogica1 Iaboratory. This appIies equaIIy to matters of incidence, differentia1 diagnosis and treatment. INCIDENCE The incidence of maIignant bone tumors is not commensurate with the amount that has been written on the subject. The writings incIine one to believe that maIignant disease of bone, unfortunateIy too common as it is, is commoner than is actuaIIy the case. Furthermore, the more
Washington University SchooI of Medicine, Institute of RadioIogy. 403
and the Edward MaIIinckrodt
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important pubhcations have been made by men who have had an immense amount of accummated materia1 with which to work.
Disease
DECEMBER, 19x2
mahgnant bone tumors, and hence where many patients appIy for treatment, their occurrence is fortunately rather rare. In
FIG.
2. Section of tissue near metacarpa1 taken at operation. Shows soIid invasion of soft tissue by tumor ceIIs. There is no interceIIuIar substance and there is a tendency toward peritheIia1 arrangement. There are severa large bIood spaces Iined with tumor cells.
FIG. I. Radiograph
of hand made Nov. 5, x928. Characteristic endotheIia1 myeIoma of third metacarpa1 bone. Excision of metacarpat.
One rest& of this condition has been the eIaboration of cIassifications, etioIogicaI theories, and the promuIgation of certain ideas in regard to treatment of bone tumors which are of theoretica rather than practical use. UnfortunateIy, the task of the physician or radiorogist dealing with the occasiona case of mahgnant disease of bone has not been made proportionately easier. After all is said and done, the greater proportion of individuals with malignant condition in the skeIeton wiII have to be cared for by what may be termed the casual radioIogist or surgeon. AI1 such cases cannot be put under the care of what may be termed speciahsts in the fieId of maIignant bone disease. Except in those centers where specia1 attention is paid to
the hospitaIs allied to the Washington University SchooI of Medicine, the Barnes HospitaI, the St. Louis ChiIdren’s Hospital, the St. Louis Maternity Hospital, in a period of eighteen years and in 123,285 hospital admissions there have been 220 cases of sarcoma with g6 exampIes of primary maIignant bone tumors. Over the same period there were in the Barnes HospitaI, 277 cases ofsecondary or metastatic maIignant invoIvement of the skeIeton in 5883 cases of carcinoma. The other tumors capabIe of skeIetaI metastasis have been too few in number and uncertain in diagnosis to warrant the Iabor of segregating them and forming an additiona cIassification. The commonest sites of origin of these carcinomata were : AIimentary tract.. . . Uterus.......................... Breast.. . . . ... Prostate........................
. . . . . . . . . . . 1214 ..
.. ....
865 352 234
The remaining cases were wideIy scattered among other epitheIia1 structures.
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The ratio of incidence of metastatic to primary bone tumor in the preceding statistics is 2.9 of the former to I of the Iatter. High as the figure is for metastatic bone tumor, I doubt if it shows the true incidence of that compIication as such skeIeta1 foci are not sought for as intensively as are the primary bone tumors. Systematic skeIeta1 radiography in cancer wouId undoubtedIy greatIy increase the foregoing ratio. Our views of the secondary and remote manifestations of cancer are predicated on the findings in the autopsy room. These views shouId be modernized and brought into correct reIationship with the facts as deveIoped by radiography. It is not suffrcientIy we11 known how searching is radiography nor the extent to which it is capabIe of supplementing the knowIedge attainabIe from the postmortem examination. In the vast majority of exampIes of metastatic bone tumors this unfortunate happening is of Iater occurrence in the course of cancer. With the increasing efficiency of treatment of cancer Iate manifestations are becoming more of a probIem than ever before. DEFINITIONS
OF
TERMS
AND
CLASSIFICATIONS
In view of this, some definitions of terms and a cIassification are in order, to secure an inteIIigent approach to the probIem of differentiation of primary and metastatic skeIeta1 invoIvement in malignant disease. This is an essentia1 preIiminary to the correct treatment of the cases with skeIeta1 neopIastic disease. The cIassification and terms appIied to all bone lesions by the Registry Committee of the American CoIIege of Surgeons is as foIIows:* I. Metastatic tumors primary in tissues other than bone 2. PeriosteraI fibrosarcoma 3. Osteogenic tumors: (a) benign, and (b) maIignant 4. Inff ammatory conditions 3. Benign giant ceI1 tumors * Tagen from KoIodny, A., Bone Sarcoma. Chicago, SurgicaI
Pub. Co., 1927.
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6. Angiomata: (a) maIignant 7. Ewing’s tumor
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benign,
and
(6)
FIG. 3. Radiograph ot spine of Feb. 26, 1932. Arrow indicates destructive lesion in vertebra. Since first observation forearm amputated for IocaI return of tumor.
8. MyeIoma. (Among other omissions fracture and metaboIic disease of bone!) It should be said that in the radioIogica1 Iaboratory infectious processes, or their resuIt in bone, offer great diffrcuIty in differentia1 diagnosis. Such differentiation is too Iengthy to be incIuded in the Iimits of this paper. KoIodny modifies the foregoing cIass&cation as far as it appIies to primary malignant bone tumors as fohows: I. Osteogenic sarcoma 2. Ewing’s sarcoma 3. MyeIoma 4. A group of unclassified sarcoma, including among others angioendotheIioma and extraperiosteal sarcoma. This cIassification is admirabIe and it is especiaIIy appIicabIe in the x-ray Iaboratory. It is aIso usefu1 in the treatment of the diseases under discussion. The writer differs with such an authority as KoIodny as to the classification of endotheIioma of bone or Ewing’s sarcoma, believing a that with ” 1 ~~ it shouId be ProuDed
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Disease
the myelomas. Two of the cases observed by the author (Figs. I-7), indicate by their course and spread that the tendency of
throughout the marrow system, and the tumors not attaining great size, does not come to the notice of the surgeon and
FIG. 4. Lateral radiograph of right Ieg of second case, April 3, 1929.
FIG. 5. LateraI radiograph of right leg, Aug. 23, rgzg, showing characteristic effect of irradiation of Ewing’s tumor.
endbtheIia1 myeIoma is to the progressive involvement of the entire marrow with the formation of Iarge osteolytic centraI tumors in muItipIe regions of the skeIeton. In the writer’s view they progressively involve the marrow system equaIIy with the other myelomas. It wouId seem that the chief difference between endotheIia1 myeIoma and those of other type is that in the former the primary tumor attains a Iarger size at an earher period than is the case in the Iatter, and, hence, attention is caIIed to it sooner. In the pubIished cases of endothehal myeIoma there is remarkabIe paucity of reports as to the condition of the remainder of the skeIeton at the time of discovery of the tumor. There is aIso a great Jack of knowIedge of the progression or course of the disease in a Iarge group of cases. MyeIoma of other type deveIoping rapidIy
radiologist unti1 it is widespread. ApparentIy the reverse is true in endothelial myeloma; it is discovered earlier in its life cycIe than the other myeIomas. In spite of the great merit of KoIodny’s cIassification of the maIignant bone tumors, it has seemed to the writer that this diffrcuIt subject couId be further simpIified by the foIIowing cIassification : I. Tumors which originate from the tissues housed in bone. These are the various types of myeloma incIuding endotheIia1 myeIoma (Fig. 8). EpitheIial tumors of denta origin in the maxiIIae beIong in this heading but are onIy mentioned. There must necessarily be a few rare tumors of blood vesseI origin and maIignant nature, angioendothelioma, etc., which beIong in this cIass.
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2. Bone tumor proper. Osteogenic sarcoma (Fig. 9). 3. Tumors originating from the invest-
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METASTASIS
Some remarks on the metastasis and spread of primary bone tumors are perti-
FIG. 7. Section from rib removed at autopsy. Pathological picture cIoseIy resembles that of Figure z
FIG. 6. Anteroposterior radiograph of Ieft knee joint of Oct. 8, rgzg. Arrows indicate bone defects in femur and tibia. At this period there was a huge defect in the skuI1.
ments of bone, the periostea1 fibrosarcoma, parostea1 sarcoma, capsuIar sarcoma (Fig. IO). These Iast named are in no sense true bone tumors, except from the accident of juxtaposition. OnIy rareIy do they invoIve, extend to, or infiltrate bone. They may, however, on occasion, cause resorption of bone which seems to be a pressure effect arising from their increase in size.
FIG. 8. Lateral radiograph of foot with characteristic changes in pIasma ceI1 myeIoma in distal portion of a limb.
nent at this point. In spreading, sarcomas foIIow the path of Ieast resistance, and those of the skeIeton are no exception to this ruIe. Osteogenic
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sarcoma extends far beyond the Iimits which are demonstrabre by x-ray or other methods of examination of the affected
Disease Osteogenic sarcoma is rapidIy invasive of surrounding soft tissues. EndotheIiaI myeloma and myeIoma are onIy sIightIy inva-
FIG. g.
bone. It has been shown that it may extend far up or down the marrow cavity. Large bIood spaces deveIop in them, and these become invaded by tumor ceIIs. Osteogenic sarcomas aIso foIIow to the Iimits of their extent the Ioose areoIar tissue spaces in intermuscuIar septa, around bIood vesseIs, etc. In the extremities it doubtIess earIy reaches the point where the septa are fused into the dense fascias at the termination of the segments of Iimbs. When metastasis takes place, it is by a bIood stream distribution of the metastatic materia1. Therefore, puImonary metastases are the first to occur. This occurrence is of such constancy that it can be stated as a genera1 ruIe in sarcoma. Sarcoma does not travel through the hence regionaI Iymph nodes Iymphatics, ,I 3 are usually spared.
FIG. IO. Radiograph of leg of Dec. 24, rg3o. Large soft tissue tumor. Bone is unaffected.
sive when they break through the bone, and spread to adjacent tissues, the former more so than the Iatter. PuImonary metastases are not found in the author’s cases. UsuaIIy in the case of $1 types of myeIoma, IittIe if any of the tumor is found beyond the confines or immediate vicinity of the bone. EndotheIiaI myeloma
FIG. I I. Radiograph of femora. Arrows indicate destruction of bone by metastatic nodules of hypernephroma.
FIG.
12. Radiograph of findings of metastatic
chest with pulmonary
characteristic sarcoma.
FIG. 14. Radiograph of chest of March 4, 19x2. Arrows indicate areas of infikration in Iung thought to be a metastatic sarcoma from tibia.
FIG. 13. LateraI radiograph of Ieg of Nov. II, 1931. Case of Paget’s disease. Arrow indicates destructive process at upper end of tibia.
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whiIe it may Iong remain a singIe focus is often said to metastasize to other bone. In the writer’s opinion this mereIy repre-
FIG. 13. Photograph of gross materia1 removed from tibia. White zone indicated by arrow was adenocarcinoma mocroscopicalIy confirmed.
>
Disease a metastasis in the sense of emboIic transport of maIignant materia1 from one point through the bIood or Iymph circuIation to more remote ones eIsewhere in the marrow. ExtraperiosteaI sarcoma, periostea1 fibrosarcoma and capsuIar sarcoma are Iess distinctive in their radioIogica1 manifestations than osteogenic sarcoma. Their spread and metastasis apparentIy are the same as that of the soft tissue sarcomas which arise whoIIy independent of bone. I would condense the facts of the spread and metastasis of these tumors in the foIlowing way : Osteogenic sarcoma spreads rapidIy into bIood spaces and vesseIs (which it may form) aIong the areoIar tissues, and for distances which cannot be suspected. It metastasizes by blood stream distribution and does not invoIve the Iymphatics. The metastases are puImonary. EndotheIiaI myeIomas and the other myeIomas spread more sIowIy. Of these two groups onIy endotheIia1 myeIoma invades surrounding tissue to any great extent. AI1 types of myeIoma tend to invoIve uItimateIy the entire marrow system, with varying degrees of destruction of the containing bone. Extreme destruction of the marrow seems
“PERIOSTEiL LIPPING”
COHBINA’TlON OF THREE FOREGOING FIG. 16.
sents a remote deveIopment of the same process in the marrow at a point distant from the first recognized growth. It is not
to be the cause of death. MyeIomas do not metastasize as a genera1 ruIe, certainIy this is not observed in the x-ray Iaboratory.
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Sarcomas, either osteogenic or of other origin, onIy exceptionaIIy extend to, that is, incItrate adjacent bones or metastasize
FIG. 17. ExampIe of tumor and pathoIogica1 in which amputation is indicated.
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Metastatic hypernephromas cause much confusion in the diagnosis of bone Iesions, and, hence, in treatment. They may form
fracture
to remote portions of the skeIeton. When bone metastases appear to be present the probabiIities are that the tumor is of some other nature than sarcoma. Giant ceI1 sarcoma must be referred to here because it has been so Iong put in the same category as the sarcomas. This disease can be considered as a maIignant one onIy when it is negIected. Correct treatment removes it from the cIass of maIignant diseases. Giant ceI1 tumor is onIy sIightIy invasive of surrounding parts, does not metastasize and it onIy rareIy recurs after excision or amputation if properly done, and does not directIy destroy Iife. It is to be borne in mind, however, that maIignant bone tumors sometimes contain giant ceIIs, and Iead to mistakes in diagnosis. The giant ceI1 tumors wiI1 be referred to again when the therapy of maIignant bone tumors is considered.
FIG. 18. Huge giant ceI1 tumor too large for treatment other than amputation.
a singIe focus in a singIe bone, or they may have a wide distribution throughout the skeIeton (Fig. I I) and at the same time the Iungs may be the site of metastases. As far as radioIogica1 methods are concerned, they may simuIate osteogenic sarcoma preciseIy both in their supposed primary focus in bone and in puImonary distribution. They can also arise in younger persons which is not usuaIIy the case with the majority of the metastatic skeIeta1 tumors. However, if there are multipIe skeIeta1 foci, and characteristic findings in the Iungs, one may be safe in concIuding that the case is not a bone sarcoma, but a hypernephroma.
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to bone, EpitheliaI tumors, metastatic have certain characteristics. Recognition of these aids materiahy in their separation
Disease a tumor found in these Iocations must be primary in the bones involved and is either an osteogenic tumor or a myeloma. I wiI1
FIG. lg. Large parostea1 sarcoma in which amputation is indicated. There is some evidence of pressure effect on posterior aspect of bone.
from primary malignant bone tumors. ObviousIy, the first point in separating primary bone tumors from epitheIia1 metastases is that, in general, epitheIia1 tumors wiI1 be found at a later age than is the case with sarcomas. If an epithelia1 tumor is metastatic to bone it is more Iikely to be muItipIe as to the bones involved. This is so contrary to the rule for bone tumor that it is a safe statement that muItip1e foci in bone eIiminate primary bone tumor from consideration. EpitheIial tumors so rareIy metastasize distaI to the most outIying Iymph-node distribution, nameIy those at the eIbow and knee joints, that it can be said that metastasis beyond these two joints is virtuahy never observed. Hence, a tumor arising in these dista1 segments of the Iimbs wiI1 not be a metastatic epitheIia1 tumor. This particuIar behavior is aIso true of hypernephroma. Therefore,
FIG. 20.
digress enough at this point to say that in the examination of cases with actua1 or suspected bone tumor or metastasis that the entire skeIeton be radiographed. This is a ruIe unfortunateIy more often observed in the breach than in performance. A radioIogica1 study in suspected cases of this nature is incompIete unIess the chest is also examined. Our materia1 indicates that it is aImost axiomatic that carcinomata do not metastasize to the Iungs with the same manifestations as are found in sarcomas. That metastasis of the epithehal tumors to the lungs frequentIy occurs anyone with the
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Ieast knowIedge of autopsy findings in carcinoma is we11 aware. This is most often by way of Iymphatic permeation which
FIG.
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infrequent in the materia1 studied to permit of any extended statement. MeIanomas have never been noted metastatic in bone.
Radiograph of knee joint, April 7, 1927. A giant cc11 tumor of inner condyle of femur.
21.
does not produce the large globular growths (Fig. 12) in the parenchyma of the Iungs usuaIIy found in metastatic sarcoma, or hypernephroma. Bone sarcoma can occur Iate in life, but KoIodny (Zoc. cit.) is authority for the statement that 60 per cent of the cases reported have arisen on the basis of an existing Paget’s disease. An exampIe of such a case has been encountered which demonstrates that even so important a rule as this and the one of non-spread of epitheIia1 tumors beyond knee and eIbow joints, does admit of exception. (Figs. 13, 14 and 15.) The spread and secondary manifestation of the other type of tumors in the skeIeton and in the Iungs have been too
FIG, 22. Radiograph of same knee joint as in Figure 21, Dec. 7, rg2q. Marked restitution and good function. Result. of intensive s-my treatment. Patient in satisfactory condition.
The majority of those arising in the skin seem to behave much as epithehal tumors do, and extend via the lymphatic system. Those from the nail bed and from the choroid seem to favor a blood-stream distribution to the parenchymatous organs. Tumors of the ovary and testes have been too infrequently noted in the x-ray laboratory and in character are too protean in their man’festation to permit of any genera1 statement to be made concerning them
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except that they destroy invoIve the skeIeton.
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life before
they
RADIOLOGY
It seems that those who write on bone tumors do so as if the subject were in a finished and compIete state. The naturaI history of the disease, so to speak, appears to escape them. Because of this, overemphasis is put on certain things beyond their innate importance, whiIe others are underestimated. A radioIogist can draw one conclusion in regard to much that has been written about bone tumors by those who are not radioIogists. It is that instead of radioIogica1 findings contributing something essentia1 to the fina concIusion as to the condition in hand, that the history, gross pathoIogy, and microscopic pathoIogy are used to expIain the x-ray findings. This is unfortunate, as unfortunate in fact as the case of the pathoIogist, who, given pathological materia1, must know the history, clinica course, etc., before he can venture upon an opinion as to the pathoIogica1 materia1. Neither the pathoIogist nor the radioIogist makes the fina diagnosis. Their work is remarkabIy aIike. Their service is to cIarify and make certain, points in diagnosis and their function is to observe and interpret objectiveIy materia1 submitted to them for opinion. When the radioIogist must have the compIete cIinica1 history before him, I am constrained to fee1 that his usefulness to those who have caIIed upon him is at an end. Writers on bone tumors are much given to stating that certain changes are pathognomonic of this, that or the other type of bone tumor. A smaI1 triangIe of dense bone at the expanding portion of an osteogenic sarcoma has great importance paid to it as a diagnostic sign. “Arborization,” i.e., the formation of fine Iines of caIcific materia1 extending into the soft tissues, their course being perpendicuIar to the shaft of the bone, is aIso supposed to be pathognomonic of osteogenic sarcoma. The Iaminated structure in endo-
Disease theIia1 myeIoma or “onionization” is aIso thought to be highIy diagnostic (Fig. 16). The fact of the matter is, any of these three signs can accompany and do accompany conditions other than bone tumor. Single pathognomonic x-ray signs in bone tumors, either primary or secondary, are, in the writer’s experience, non-existent. He feeIs that in the majority of instances an experienced radioIogist can state the nature of a bone Iesion with a correctness equa1 to that found in other medica opinion. However, he reaIizes his own incapacity to describe radiographic findings and Iikewise doubts the abiIity of others to do so. Identihcation of these conditions rests on the recognition of a congeries of physica changes in the invoIved bone, which eIude description in words. DIFFERENTIAL
DIAGNOSIS
UnIess the severa maIignant affections of the skeIeton can be diagnosticaIly differentiated, no inteIIigent method of deaIing with them can be evoIved. When confronted with the probIem of a bone tumor it must be ascertained lirst whether it is primary or secondary. When this is achieved, what method of treatment is to be empIoyed? I. If the patient is a young person with involvement of the bones dista1 to the knee or eIbow, and the tumor is singIe, we can assume that it is a primary tumor. 2. In the last condition, i.e., a singIe bone focus, proxima1 to knee or eIbow joints, in a young person, and metastasis to the Iungs, the condition may be either a primary bone tumor or a hypernephroma. 3. If there is a diffuse bone Iesion invoIving many bones and indicating a centrifuga1 spread, one is not deaIing with a primary bone tumor. 4. If one has a solitary bone Iesion, even in view of a history of carcinoma or other maIignant tumor, and there are round circumscribed areas of density in the parenchyma of the Iungs, one is deaIing with an osteogenic sarcoma or hypernephroma with metastasis in the Iungs.
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TREATMENT
In any bone Iesion which is muItiple, treatment resoIves itseIf into the appIication of paIliative measures. It is as a palhative measure that radiation therapy is of the most use. However, as it tends to Iose its eff&iency the Ionger it is appIied, it should not be resorted to unti1 symptoms are present. In other words, it should not be used as a pIacebo. Any skeIeta1 Iesion of the extremities which is definiteIy osteogenic sarcoma and not yet metastatic shouId in the opinion of this writer be treated with radica1 surgery, that is to say, that if the tumor is dista1 to the knee or eIbow, amputation shouId be done, but, above the proxima1 joints. Osteogenic sarcoma of the humerus or shouIder girdIe shouId have a shouIder girdIe amputation. Since the spread of sarcoma may be of the nature pointed out earlier in this paper amputation at the hip joint for osteogenic sarcoma does not seem feasible, unIess it be of the scIerosing type. The same statement is true of osteogenic tumors of the trunk, and in this fieId the writer beIieves that radiation therapy is the treatment of choice. Routine preoperative or postoperative radiation as a prophylactic measure in sarcoma has never had any specia1 appea1 to the writer and has onIy been given when requested by the surgeon. In addition to the favorabIe response of endotheIia1 myeIoma to radiation and to the fact that there is ground for beIieving that it is a type of systemic disease it shouId be treated by radiation alone. Where there are widespread metastases in osteogenic sarcoma, great pain, cachexia, etc., radiation shouId be appIied. PathoIogica1 fractures of the extremities or great tumefaction, fungus formation, and uIceration shouId have in addition, and as a paIIiative measure, amputation or excision as the case may be, to be foIlowed by radiation. (Figs. 17, 18, 19.) Surgery of this type has immense possibiIities of affording symptomatic reIief. It is not onIy justifiable but in the writer’s opinion it is
Disease mandatory in those who suffer greatIy. Not enough surgery of this type is done. Secondary invoIvement of the skeIeton with maIignant disease presupposes, of course, widespread dissemination which makes surgica1 intervention quite out of the question on the face of it, unless it is paIIiative. These cases should be, and are, fit subjects for irradiation. It is the agency which gives them more reIief, proIongs their Iives, and contributes some degree of comfort. In the case of the spine there often is uncontroIIabIe pain, even with ExampIes of reIief from such morphine. pain by irradiation are within the knowIedge of anyone with any material experience with the treatment of this type of case. Irradiation then is the symptomatic paIIiative treatment for widespread secondary tumors of the skeIeton. There have been enough exampIes of cure of giant ceI1 tumor by irradiation that this agency shouId be the first choice in the treatment of these cases with certain exceptions. If irradiation after a reasonabIe tria1 faiIs to produce the desired resuIt then one may resort to surgery. The exceptions are those where the tumor is of such great size and Iocation that repair cannot be hoped for (Fig. 20). Irradiation shouId not be employed if a joint has been destroyed by the giant ceI1 tumor and if recovery wouId resuIt in a stiff ffai1 or useIess Iimb. In these Iast named conditions amputation is to be preferred. AdmirabIe resuIts are secured in giant ceI1 tumor by irradiation aIone (Figs. 21 and 22). It shouId be recaIIed that this tumor is not a common one, if it were there wouId be more cures reported. The resuIts reported by CoIey following the use of his toxin treatment convince the writer that the toxin treatment of malignant bone disease shouId aIways be combined with the other methods of treatment. What is to be expected from the treatment of tumors in bone? Of the myeIomas with which the writer has had to dea1 a smaI1 amount of reIief of pain has been
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obtained aImost immediately folIowed by deveIopment of secondary tumors elsewhere in the skeIeton. The onIy exampIe of osteogenic sarcoma that is known by the writer to have survived any method of treatment is one that he irradiated. This individua1 is aIive nearly ten years after the deveIopment of the tumor. She was treated wrongIy in every way. Amputation was recommended and refused. Biopsy was done, radium directly impIanted into the growth, a proceeding at the time thought by many to be tantamount to destroying the patient. The implantation was foIIowed by heavy x-radiation at intervaIs for months. The patient is we11 at the present writing. The case is reported eIsewhere. AI1 other cases of osteogenic sarcoma have been treated either in conjunction with surgery or the treatment started Iate in the disease, and a11 the patients of whom we have knowIedge are dead. It is possibIe that in some instances the reputed cures of Ewing’s tumor by irradiation have been exampIes of osteogenic sarcoma. CoIor is Ient to this beIief by the
Disease fact that favorabIe irradiation response is often the basis for diagnosis of Ewing’s tumor and, hence, biopsy is omitted. None of the cases of metastatic tumor of the skeIeton which we have treated has had a Iong surviva1. However, enough reIief of symptoms has been contributed to make this procedure we11 worth whiIe. The first requirement for the successfu1 treatment of a primary tumor of the skeIeton is that a11 the tumor shouId be excised or a11 irradiated to secure a favorabIe result. Therein Iies the expIanation of al1 faiIures of treatment whether surgical or by irradiation. If the surgeon excises a11 of the disease the patient wiI1 be weI1. If the correct amount of x-ray be distributed throughout the tumor the patient wiI1 aIso get weI1. The fact that a tumor is secondary in one Iocation, that is, metastatic, means that it probabIy has other undiscoverable extensions in other Iocations which it is simpIy impossibIe to recognize and to irradiate or remove. This is the chief reason for not irradiating secondary tumor of the skeIeton in the absence of symptoms.
REFERENCES I. CODMAN, E. A. Registry of Bone Sarcoma; 25 criteria for establishing diagnosis of osteogenic sarcoma; 13 registered cases of “five-year cures” anaIyzed according to these criteria. Surg., Gynec., Oh., 42: 381-383, 1926. 2. KOLODNY, A. Bone Sarcoma. Chicago, SurgicaI Pub. Co., 1927. 3. BLOODG~OD, J. C. Bone sarcoma periostea1 and diffuse type, and their diagnosis from benign Iesions. J. Bone w Joint Surg., 8: 727-749, 1926. Bone tumors: sarcoma, periostea1 group, scIerosing type, osteogenic, methods of diagnosis and treatment. J. Radiol., vo1. 4: 46-51, 1923.
osteomyelitis Bone diseases: non-suppurating (GarrC) infectious ossifying periostitis. J. Radial., 3: 310-316, 1922. Myxoma, centraI and periosteal. Ann. Surg., 72: 712, 1920. 4. EWING, J. A review and classihcation of bone sarcomas. Arch. Surg., 4: 485-533, 1922. Neoplastic Diseases. Ed. 3. PhiIa., Saunders, 1931. 5. CONNOR, C. L. EndotheIiaI myeloma, Ewing; report of 54 cases. Arch. Surg., 4: 485-533, 1922. 6. GESCHICKTER, C. F., and COPELAND, M. M. Tumors of bone. Am. J. Cancer, 1931.
DECEMBER,
No. 3
1932
DISCUSSION OF THE DIAGNOSIS AND X,RAY TREATMENT
MALIGNANT
OF
DISEASE OF BONE*
SHERWOOD MOORE, M.D. ST. LOUIS,
UCH of the substance of this paper is at variance with accepted views and wiI1 be disagreed with by many readers as it departs from orthodoxy at severa points. The subject of bone tumors is far too obscure and unnecessariIy so. It stands greatIy in need of simpIification. The skeIeton is a pecuIiarIy suitabIe structure for investigation by x-ray methods. Since their advent into medicine, knowIedge of the skeIeton, its anatomy, physioIogy and pathoIogy has grown enormousIy. The skeIeton is far better understood than connective tissue, cartiIage, adipose tissue, muscIe, etc. Of the diseases of bone, which have been most extensiveIy studied, both on account of the great aid afforded by x-ray methods and because of their highIy IethaI nature, tumor has attracted much attention. Bone tumor has been for the past severa years a popuIar subject for investigation and pubIication. Impetus to this arises from the work of the Registry of Bone Sarcoma of the American CoIIege of Surgeons. Credit for the notabIe accompIishment of this body shouId be given to Codman. There are such authoritative pubIications on malignant bone tumors that any prospect of adding anything origina to that which has ah-eady been written is aImost out of the question. However, many physicians are unacquainted with
M
* From the Department
of Radiology,
MO.
this work. It wiI1 be the endeavor of this paper to direct attention to and popuIarize some of the exceIIent existing pubIications on maIignant bone tumors with the idea of simpIifying this obscure subject. These pubIications are the studies of the materia1 in the Registry of Bone Sarcoma of the American CoIIege of Surgeons by Codmanl and KoIodny,2 various pubIications by BIoodgood,3 Ewing,4 and Connor, and the monograph of Geschickter and CopeIand.6AII of these are masterIy. NevertheIess, it seems to a radioIogist that there are certain points concerning bone tumors that these writers fai1 to deveIop. I wish to caI1 attention to a significant fact, i.e., that these pubIications, with the exception of those of Ewing, were written by surgeons, and they necessarily Iack the genera1 view of the subject of gross bone pathoIogy which one acquires in the radioIogicaI or pathoIogica1 Iaboratory. This appIies equaIIy to matters of incidence, differentia1 diagnosis and treatment. INCIDENCE The incidence of maIignant bone tumors is not commensurate with the amount that has been written on the subject. The writings incIine one to believe that maIignant disease of bone, unfortunateIy too common as it is, is commoner than is actuaIIy the case. Furthermore, the more
Washington University SchooI of Medicine, Institute of RadioIogy. 403
and the Edward MaIIinckrodt
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important pubhcations have been made by men who have had an immense amount of accummated materia1 with which to work.
Disease
DECEMBER, 19x2
mahgnant bone tumors, and hence where many patients appIy for treatment, their occurrence is fortunately rather rare. In
FIG.
2. Section of tissue near metacarpa1 taken at operation. Shows soIid invasion of soft tissue by tumor ceIIs. There is no interceIIuIar substance and there is a tendency toward peritheIia1 arrangement. There are severa large bIood spaces Iined with tumor cells.
FIG. I. Radiograph
of hand made Nov. 5, x928. Characteristic endotheIia1 myeIoma of third metacarpa1 bone. Excision of metacarpat.
One rest& of this condition has been the eIaboration of cIassifications, etioIogicaI theories, and the promuIgation of certain ideas in regard to treatment of bone tumors which are of theoretica rather than practical use. UnfortunateIy, the task of the physician or radiorogist dealing with the occasiona case of mahgnant disease of bone has not been made proportionately easier. After all is said and done, the greater proportion of individuals with malignant condition in the skeIeton wiII have to be cared for by what may be termed the casual radioIogist or surgeon. AI1 such cases cannot be put under the care of what may be termed speciahsts in the fieId of maIignant bone disease. Except in those centers where specia1 attention is paid to
the hospitaIs allied to the Washington University SchooI of Medicine, the Barnes HospitaI, the St. Louis ChiIdren’s Hospital, the St. Louis Maternity Hospital, in a period of eighteen years and in 123,285 hospital admissions there have been 220 cases of sarcoma with g6 exampIes of primary maIignant bone tumors. Over the same period there were in the Barnes HospitaI, 277 cases ofsecondary or metastatic maIignant invoIvement of the skeIeton in 5883 cases of carcinoma. The other tumors capabIe of skeIetaI metastasis have been too few in number and uncertain in diagnosis to warrant the Iabor of segregating them and forming an additiona cIassification. The commonest sites of origin of these carcinomata were : AIimentary tract.. . . Uterus.......................... Breast.. . . . ... Prostate........................
. . . . . . . . . . . 1214 ..
.. ....
865 352 234
The remaining cases were wideIy scattered among other epitheIia1 structures.
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The ratio of incidence of metastatic to primary bone tumor in the preceding statistics is 2.9 of the former to I of the Iatter. High as the figure is for metastatic bone tumor, I doubt if it shows the true incidence of that compIication as such skeIeta1 foci are not sought for as intensively as are the primary bone tumors. Systematic skeIeta1 radiography in cancer wouId undoubtedIy greatIy increase the foregoing ratio. Our views of the secondary and remote manifestations of cancer are predicated on the findings in the autopsy room. These views shouId be modernized and brought into correct reIationship with the facts as deveIoped by radiography. It is not suffrcientIy we11 known how searching is radiography nor the extent to which it is capabIe of supplementing the knowIedge attainabIe from the postmortem examination. In the vast majority of exampIes of metastatic bone tumors this unfortunate happening is of Iater occurrence in the course of cancer. With the increasing efficiency of treatment of cancer Iate manifestations are becoming more of a probIem than ever before. DEFINITIONS
OF
TERMS
AND
CLASSIFICATIONS
In view of this, some definitions of terms and a cIassification are in order, to secure an inteIIigent approach to the probIem of differentiation of primary and metastatic skeIeta1 invoIvement in malignant disease. This is an essentia1 preIiminary to the correct treatment of the cases with skeIeta1 neopIastic disease. The cIassification and terms appIied to all bone lesions by the Registry Committee of the American CoIIege of Surgeons is as foIIows:* I. Metastatic tumors primary in tissues other than bone 2. PeriosteraI fibrosarcoma 3. Osteogenic tumors: (a) benign, and (b) maIignant 4. Inff ammatory conditions 3. Benign giant ceI1 tumors * Tagen from KoIodny, A., Bone Sarcoma. Chicago, SurgicaI
Pub. Co., 1927.
Disease
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6. Angiomata: (a) maIignant 7. Ewing’s tumor
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benign,
and
(6)
FIG. 3. Radiograph ot spine of Feb. 26, 1932. Arrow indicates destructive lesion in vertebra. Since first observation forearm amputated for IocaI return of tumor.
8. MyeIoma. (Among other omissions fracture and metaboIic disease of bone!) It should be said that in the radioIogica1 Iaboratory infectious processes, or their resuIt in bone, offer great diffrcuIty in differentia1 diagnosis. Such differentiation is too Iengthy to be incIuded in the Iimits of this paper. KoIodny modifies the foregoing cIass&cation as far as it appIies to primary malignant bone tumors as fohows: I. Osteogenic sarcoma 2. Ewing’s sarcoma 3. MyeIoma 4. A group of unclassified sarcoma, including among others angioendotheIioma and extraperiosteal sarcoma. This cIassification is admirabIe and it is especiaIIy appIicabIe in the x-ray Iaboratory. It is aIso usefu1 in the treatment of the diseases under discussion. The writer differs with such an authority as KoIodny as to the classification of endotheIioma of bone or Ewing’s sarcoma, believing a that with ” 1 ~~ it shouId be ProuDed
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Disease
the myelomas. Two of the cases observed by the author (Figs. I-7), indicate by their course and spread that the tendency of
throughout the marrow system, and the tumors not attaining great size, does not come to the notice of the surgeon and
FIG. 4. Lateral radiograph of right Ieg of second case, April 3, 1929.
FIG. 5. LateraI radiograph of right leg, Aug. 23, rgzg, showing characteristic effect of irradiation of Ewing’s tumor.
endbtheIia1 myeIoma is to the progressive involvement of the entire marrow with the formation of Iarge osteolytic centraI tumors in muItipIe regions of the skeIeton. In the writer’s view they progressively involve the marrow system equaIIy with the other myelomas. It wouId seem that the chief difference between endotheIia1 myeIoma and those of other type is that in the former the primary tumor attains a Iarger size at an earher period than is the case in the Iatter, and, hence, attention is caIIed to it sooner. In the pubIished cases of endothehal myeIoma there is remarkabIe paucity of reports as to the condition of the remainder of the skeIeton at the time of discovery of the tumor. There is aIso a great Jack of knowIedge of the progression or course of the disease in a Iarge group of cases. MyeIoma of other type deveIoping rapidIy
radiologist unti1 it is widespread. ApparentIy the reverse is true in endothelial myeloma; it is discovered earlier in its life cycIe than the other myeIomas. In spite of the great merit of KoIodny’s cIassification of the maIignant bone tumors, it has seemed to the writer that this diffrcuIt subject couId be further simpIified by the foIIowing cIassification : I. Tumors which originate from the tissues housed in bone. These are the various types of myeloma incIuding endotheIia1 myeIoma (Fig. 8). EpitheIial tumors of denta origin in the maxiIIae beIong in this heading but are onIy mentioned. There must necessarily be a few rare tumors of blood vesseI origin and maIignant nature, angioendothelioma, etc., which beIong in this cIass.
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2. Bone tumor proper. Osteogenic sarcoma (Fig. 9). 3. Tumors originating from the invest-
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METASTASIS
Some remarks on the metastasis and spread of primary bone tumors are perti-
FIG. 7. Section from rib removed at autopsy. Pathological picture cIoseIy resembles that of Figure z
FIG. 6. Anteroposterior radiograph of Ieft knee joint of Oct. 8, rgzg. Arrows indicate bone defects in femur and tibia. At this period there was a huge defect in the skuI1.
ments of bone, the periostea1 fibrosarcoma, parostea1 sarcoma, capsuIar sarcoma (Fig. IO). These Iast named are in no sense true bone tumors, except from the accident of juxtaposition. OnIy rareIy do they invoIve, extend to, or infiltrate bone. They may, however, on occasion, cause resorption of bone which seems to be a pressure effect arising from their increase in size.
FIG. 8. Lateral radiograph of foot with characteristic changes in pIasma ceI1 myeIoma in distal portion of a limb.
nent at this point. In spreading, sarcomas foIIow the path of Ieast resistance, and those of the skeIeton are no exception to this ruIe. Osteogenic
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sarcoma extends far beyond the Iimits which are demonstrabre by x-ray or other methods of examination of the affected
Disease Osteogenic sarcoma is rapidIy invasive of surrounding soft tissues. EndotheIiaI myeloma and myeIoma are onIy sIightIy inva-
FIG. g.
bone. It has been shown that it may extend far up or down the marrow cavity. Large bIood spaces deveIop in them, and these become invaded by tumor ceIIs. Osteogenic sarcomas aIso foIIow to the Iimits of their extent the Ioose areoIar tissue spaces in intermuscuIar septa, around bIood vesseIs, etc. In the extremities it doubtIess earIy reaches the point where the septa are fused into the dense fascias at the termination of the segments of Iimbs. When metastasis takes place, it is by a bIood stream distribution of the metastatic materia1. Therefore, puImonary metastases are the first to occur. This occurrence is of such constancy that it can be stated as a genera1 ruIe in sarcoma. Sarcoma does not travel through the hence regionaI Iymph nodes Iymphatics, ,I 3 are usually spared.
FIG. IO. Radiograph of leg of Dec. 24, rg3o. Large soft tissue tumor. Bone is unaffected.
sive when they break through the bone, and spread to adjacent tissues, the former more so than the Iatter. PuImonary metastases are not found in the author’s cases. UsuaIIy in the case of $1 types of myeIoma, IittIe if any of the tumor is found beyond the confines or immediate vicinity of the bone. EndotheIiaI myeloma
FIG. I I. Radiograph of femora. Arrows indicate destruction of bone by metastatic nodules of hypernephroma.
FIG.
12. Radiograph of findings of metastatic
chest with pulmonary
characteristic sarcoma.
FIG. 14. Radiograph of chest of March 4, 19x2. Arrows indicate areas of infikration in Iung thought to be a metastatic sarcoma from tibia.
FIG. 13. LateraI radiograph of Ieg of Nov. II, 1931. Case of Paget’s disease. Arrow indicates destructive process at upper end of tibia.
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whiIe it may Iong remain a singIe focus is often said to metastasize to other bone. In the writer’s opinion this mereIy repre-
FIG. 13. Photograph of gross materia1 removed from tibia. White zone indicated by arrow was adenocarcinoma mocroscopicalIy confirmed.
>
Disease a metastasis in the sense of emboIic transport of maIignant materia1 from one point through the bIood or Iymph circuIation to more remote ones eIsewhere in the marrow. ExtraperiosteaI sarcoma, periostea1 fibrosarcoma and capsuIar sarcoma are Iess distinctive in their radioIogica1 manifestations than osteogenic sarcoma. Their spread and metastasis apparentIy are the same as that of the soft tissue sarcomas which arise whoIIy independent of bone. I would condense the facts of the spread and metastasis of these tumors in the foIlowing way : Osteogenic sarcoma spreads rapidIy into bIood spaces and vesseIs (which it may form) aIong the areoIar tissues, and for distances which cannot be suspected. It metastasizes by blood stream distribution and does not invoIve the Iymphatics. The metastases are puImonary. EndotheIiaI myeIomas and the other myeIomas spread more sIowIy. Of these two groups onIy endotheIia1 myeIoma invades surrounding tissue to any great extent. AI1 types of myeIoma tend to invoIve uItimateIy the entire marrow system, with varying degrees of destruction of the containing bone. Extreme destruction of the marrow seems
“PERIOSTEiL LIPPING”
COHBINA’TlON OF THREE FOREGOING FIG. 16.
sents a remote deveIopment of the same process in the marrow at a point distant from the first recognized growth. It is not
to be the cause of death. MyeIomas do not metastasize as a genera1 ruIe, certainIy this is not observed in the x-ray Iaboratory.
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Sarcomas, either osteogenic or of other origin, onIy exceptionaIIy extend to, that is, incItrate adjacent bones or metastasize
FIG. 17. ExampIe of tumor and pathoIogica1 in which amputation is indicated.
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American Journal of Surgery
411
Metastatic hypernephromas cause much confusion in the diagnosis of bone Iesions, and, hence, in treatment. They may form
fracture
to remote portions of the skeIeton. When bone metastases appear to be present the probabiIities are that the tumor is of some other nature than sarcoma. Giant ceI1 sarcoma must be referred to here because it has been so Iong put in the same category as the sarcomas. This disease can be considered as a maIignant one onIy when it is negIected. Correct treatment removes it from the cIass of maIignant diseases. Giant ceI1 tumor is onIy sIightIy invasive of surrounding parts, does not metastasize and it onIy rareIy recurs after excision or amputation if properly done, and does not directIy destroy Iife. It is to be borne in mind, however, that maIignant bone tumors sometimes contain giant ceIIs, and Iead to mistakes in diagnosis. The giant ceI1 tumors wiI1 be referred to again when the therapy of maIignant bone tumors is considered.
FIG. 18. Huge giant ceI1 tumor too large for treatment other than amputation.
a singIe focus in a singIe bone, or they may have a wide distribution throughout the skeIeton (Fig. I I) and at the same time the Iungs may be the site of metastases. As far as radioIogica1 methods are concerned, they may simuIate osteogenic sarcoma preciseIy both in their supposed primary focus in bone and in puImonary distribution. They can also arise in younger persons which is not usuaIIy the case with the majority of the metastatic skeIeta1 tumors. However, if there are multipIe skeIeta1 foci, and characteristic findings in the Iungs, one may be safe in concIuding that the case is not a bone sarcoma, but a hypernephroma.
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to bone, EpitheliaI tumors, metastatic have certain characteristics. Recognition of these aids materiahy in their separation
Disease a tumor found in these Iocations must be primary in the bones involved and is either an osteogenic tumor or a myeloma. I wiI1
FIG. lg. Large parostea1 sarcoma in which amputation is indicated. There is some evidence of pressure effect on posterior aspect of bone.
from primary malignant bone tumors. ObviousIy, the first point in separating primary bone tumors from epitheIia1 metastases is that, in general, epitheIia1 tumors wiI1 be found at a later age than is the case with sarcomas. If an epithelia1 tumor is metastatic to bone it is more Iikely to be muItipIe as to the bones involved. This is so contrary to the rule for bone tumor that it is a safe statement that muItip1e foci in bone eIiminate primary bone tumor from consideration. EpitheIial tumors so rareIy metastasize distaI to the most outIying Iymph-node distribution, nameIy those at the eIbow and knee joints, that it can be said that metastasis beyond these two joints is virtuahy never observed. Hence, a tumor arising in these dista1 segments of the Iimbs wiI1 not be a metastatic epitheIia1 tumor. This particuIar behavior is aIso true of hypernephroma. Therefore,
FIG. 20.
digress enough at this point to say that in the examination of cases with actua1 or suspected bone tumor or metastasis that the entire skeIeton be radiographed. This is a ruIe unfortunateIy more often observed in the breach than in performance. A radioIogica1 study in suspected cases of this nature is incompIete unIess the chest is also examined. Our materia1 indicates that it is aImost axiomatic that carcinomata do not metastasize to the Iungs with the same manifestations as are found in sarcomas. That metastasis of the epithehal tumors to the lungs frequentIy occurs anyone with the
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Ieast knowIedge of autopsy findings in carcinoma is we11 aware. This is most often by way of Iymphatic permeation which
FIG.
Disease
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413
infrequent in the materia1 studied to permit of any extended statement. MeIanomas have never been noted metastatic in bone.
Radiograph of knee joint, April 7, 1927. A giant cc11 tumor of inner condyle of femur.
21.
does not produce the large globular growths (Fig. 12) in the parenchyma of the Iungs usuaIIy found in metastatic sarcoma, or hypernephroma. Bone sarcoma can occur Iate in life, but KoIodny (Zoc. cit.) is authority for the statement that 60 per cent of the cases reported have arisen on the basis of an existing Paget’s disease. An exampIe of such a case has been encountered which demonstrates that even so important a rule as this and the one of non-spread of epitheIia1 tumors beyond knee and eIbow joints, does admit of exception. (Figs. 13, 14 and 15.) The spread and secondary manifestation of the other type of tumors in the skeIeton and in the Iungs have been too
FIG, 22. Radiograph of same knee joint as in Figure 21, Dec. 7, rg2q. Marked restitution and good function. Result. of intensive s-my treatment. Patient in satisfactory condition.
The majority of those arising in the skin seem to behave much as epithehal tumors do, and extend via the lymphatic system. Those from the nail bed and from the choroid seem to favor a blood-stream distribution to the parenchymatous organs. Tumors of the ovary and testes have been too infrequently noted in the x-ray laboratory and in character are too protean in their man’festation to permit of any genera1 statement to be made concerning them
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American Journal of Surgery
except that they destroy invoIve the skeIeton.
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life before
they
RADIOLOGY
It seems that those who write on bone tumors do so as if the subject were in a finished and compIete state. The naturaI history of the disease, so to speak, appears to escape them. Because of this, overemphasis is put on certain things beyond their innate importance, whiIe others are underestimated. A radioIogist can draw one conclusion in regard to much that has been written about bone tumors by those who are not radioIogists. It is that instead of radioIogica1 findings contributing something essentia1 to the fina concIusion as to the condition in hand, that the history, gross pathoIogy, and microscopic pathoIogy are used to expIain the x-ray findings. This is unfortunate, as unfortunate in fact as the case of the pathoIogist, who, given pathological materia1, must know the history, clinica course, etc., before he can venture upon an opinion as to the pathoIogica1 materia1. Neither the pathoIogist nor the radioIogist makes the fina diagnosis. Their work is remarkabIy aIike. Their service is to cIarify and make certain, points in diagnosis and their function is to observe and interpret objectiveIy materia1 submitted to them for opinion. When the radioIogist must have the compIete cIinica1 history before him, I am constrained to fee1 that his usefulness to those who have caIIed upon him is at an end. Writers on bone tumors are much given to stating that certain changes are pathognomonic of this, that or the other type of bone tumor. A smaI1 triangIe of dense bone at the expanding portion of an osteogenic sarcoma has great importance paid to it as a diagnostic sign. “Arborization,” i.e., the formation of fine Iines of caIcific materia1 extending into the soft tissues, their course being perpendicuIar to the shaft of the bone, is aIso supposed to be pathognomonic of osteogenic sarcoma. The Iaminated structure in endo-
Disease theIia1 myeIoma or “onionization” is aIso thought to be highIy diagnostic (Fig. 16). The fact of the matter is, any of these three signs can accompany and do accompany conditions other than bone tumor. Single pathognomonic x-ray signs in bone tumors, either primary or secondary, are, in the writer’s experience, non-existent. He feeIs that in the majority of instances an experienced radioIogist can state the nature of a bone Iesion with a correctness equa1 to that found in other medica opinion. However, he reaIizes his own incapacity to describe radiographic findings and Iikewise doubts the abiIity of others to do so. Identihcation of these conditions rests on the recognition of a congeries of physica changes in the invoIved bone, which eIude description in words. DIFFERENTIAL
DIAGNOSIS
UnIess the severa maIignant affections of the skeIeton can be diagnosticaIly differentiated, no inteIIigent method of deaIing with them can be evoIved. When confronted with the probIem of a bone tumor it must be ascertained lirst whether it is primary or secondary. When this is achieved, what method of treatment is to be empIoyed? I. If the patient is a young person with involvement of the bones dista1 to the knee or eIbow, and the tumor is singIe, we can assume that it is a primary tumor. 2. In the last condition, i.e., a singIe bone focus, proxima1 to knee or eIbow joints, in a young person, and metastasis to the Iungs, the condition may be either a primary bone tumor or a hypernephroma. 3. If there is a diffuse bone Iesion invoIving many bones and indicating a centrifuga1 spread, one is not deaIing with a primary bone tumor. 4. If one has a solitary bone Iesion, even in view of a history of carcinoma or other maIignant tumor, and there are round circumscribed areas of density in the parenchyma of the Iungs, one is deaIing with an osteogenic sarcoma or hypernephroma with metastasis in the Iungs.
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TREATMENT
In any bone Iesion which is muItiple, treatment resoIves itseIf into the appIication of paIliative measures. It is as a palhative measure that radiation therapy is of the most use. However, as it tends to Iose its eff&iency the Ionger it is appIied, it should not be resorted to unti1 symptoms are present. In other words, it should not be used as a pIacebo. Any skeIeta1 Iesion of the extremities which is definiteIy osteogenic sarcoma and not yet metastatic shouId in the opinion of this writer be treated with radica1 surgery, that is to say, that if the tumor is dista1 to the knee or eIbow, amputation shouId be done, but, above the proxima1 joints. Osteogenic sarcoma of the humerus or shouIder girdIe shouId have a shouIder girdIe amputation. Since the spread of sarcoma may be of the nature pointed out earlier in this paper amputation at the hip joint for osteogenic sarcoma does not seem feasible, unIess it be of the scIerosing type. The same statement is true of osteogenic tumors of the trunk, and in this fieId the writer beIieves that radiation therapy is the treatment of choice. Routine preoperative or postoperative radiation as a prophylactic measure in sarcoma has never had any specia1 appea1 to the writer and has onIy been given when requested by the surgeon. In addition to the favorabIe response of endotheIia1 myeIoma to radiation and to the fact that there is ground for beIieving that it is a type of systemic disease it shouId be treated by radiation alone. Where there are widespread metastases in osteogenic sarcoma, great pain, cachexia, etc., radiation shouId be appIied. PathoIogica1 fractures of the extremities or great tumefaction, fungus formation, and uIceration shouId have in addition, and as a paIIiative measure, amputation or excision as the case may be, to be foIlowed by radiation. (Figs. 17, 18, 19.) Surgery of this type has immense possibiIities of affording symptomatic reIief. It is not onIy justifiable but in the writer’s opinion it is
Disease mandatory in those who suffer greatIy. Not enough surgery of this type is done. Secondary invoIvement of the skeIeton with maIignant disease presupposes, of course, widespread dissemination which makes surgica1 intervention quite out of the question on the face of it, unless it is paIIiative. These cases should be, and are, fit subjects for irradiation. It is the agency which gives them more reIief, proIongs their Iives, and contributes some degree of comfort. In the case of the spine there often is uncontroIIabIe pain, even with ExampIes of reIief from such morphine. pain by irradiation are within the knowIedge of anyone with any material experience with the treatment of this type of case. Irradiation then is the symptomatic paIIiative treatment for widespread secondary tumors of the skeIeton. There have been enough exampIes of cure of giant ceI1 tumor by irradiation that this agency shouId be the first choice in the treatment of these cases with certain exceptions. If irradiation after a reasonabIe tria1 faiIs to produce the desired resuIt then one may resort to surgery. The exceptions are those where the tumor is of such great size and Iocation that repair cannot be hoped for (Fig. 20). Irradiation shouId not be employed if a joint has been destroyed by the giant ceI1 tumor and if recovery wouId resuIt in a stiff ffai1 or useIess Iimb. In these Iast named conditions amputation is to be preferred. AdmirabIe resuIts are secured in giant ceI1 tumor by irradiation aIone (Figs. 21 and 22). It shouId be recaIIed that this tumor is not a common one, if it were there wouId be more cures reported. The resuIts reported by CoIey following the use of his toxin treatment convince the writer that the toxin treatment of malignant bone disease shouId aIways be combined with the other methods of treatment. What is to be expected from the treatment of tumors in bone? Of the myeIomas with which the writer has had to dea1 a smaI1 amount of reIief of pain has been
416
American
Journal
of Surgery
Moore-Bone
obtained aImost immediately folIowed by deveIopment of secondary tumors elsewhere in the skeIeton. The onIy exampIe of osteogenic sarcoma that is known by the writer to have survived any method of treatment is one that he irradiated. This individua1 is aIive nearly ten years after the deveIopment of the tumor. She was treated wrongIy in every way. Amputation was recommended and refused. Biopsy was done, radium directly impIanted into the growth, a proceeding at the time thought by many to be tantamount to destroying the patient. The implantation was foIIowed by heavy x-radiation at intervaIs for months. The patient is we11 at the present writing. The case is reported eIsewhere. AI1 other cases of osteogenic sarcoma have been treated either in conjunction with surgery or the treatment started Iate in the disease, and a11 the patients of whom we have knowIedge are dead. It is possibIe that in some instances the reputed cures of Ewing’s tumor by irradiation have been exampIes of osteogenic sarcoma. CoIor is Ient to this beIief by the
Disease fact that favorabIe irradiation response is often the basis for diagnosis of Ewing’s tumor and, hence, biopsy is omitted. None of the cases of metastatic tumor of the skeIeton which we have treated has had a Iong surviva1. However, enough reIief of symptoms has been contributed to make this procedure we11 worth whiIe. The first requirement for the successfu1 treatment of a primary tumor of the skeIeton is that a11 the tumor shouId be excised or a11 irradiated to secure a favorabIe result. Therein Iies the expIanation of al1 faiIures of treatment whether surgical or by irradiation. If the surgeon excises a11 of the disease the patient wiI1 be weI1. If the correct amount of x-ray be distributed throughout the tumor the patient wiI1 aIso get weI1. The fact that a tumor is secondary in one Iocation, that is, metastatic, means that it probabIy has other undiscoverable extensions in other Iocations which it is simpIy impossibIe to recognize and to irradiate or remove. This is the chief reason for not irradiating secondary tumor of the skeIeton in the absence of symptoms.
REFERENCES I. CODMAN, E. A. Registry of Bone Sarcoma; 25 criteria for establishing diagnosis of osteogenic sarcoma; 13 registered cases of “five-year cures” anaIyzed according to these criteria. Surg., Gynec., Oh., 42: 381-383, 1926. 2. KOLODNY, A. Bone Sarcoma. Chicago, SurgicaI Pub. Co., 1927. 3. BLOODG~OD, J. C. Bone sarcoma periostea1 and diffuse type, and their diagnosis from benign Iesions. J. Bone w Joint Surg., 8: 727-749, 1926. Bone tumors: sarcoma, periostea1 group, scIerosing type, osteogenic, methods of diagnosis and treatment. J. Radiol., vo1. 4: 46-51, 1923.
osteomyelitis Bone diseases: non-suppurating (GarrC) infectious ossifying periostitis. J. Radial., 3: 310-316, 1922. Myxoma, centraI and periosteal. Ann. Surg., 72: 712, 1920. 4. EWING, J. A review and classihcation of bone sarcomas. Arch. Surg., 4: 485-533, 1922. Neoplastic Diseases. Ed. 3. PhiIa., Saunders, 1931. 5. CONNOR, C. L. EndotheIiaI myeloma, Ewing; report of 54 cases. Arch. Surg., 4: 485-533, 1922. 6. GESCHICKTER, C. F., and COPELAND, M. M. Tumors of bone. Am. J. Cancer, 1931.