THE PREOPERATIVE MANAGEMENT OF MALIGNANT BONE TUMORS
G. E.
HAGGART AND FRANK
L.
SHIPP
IN 1921 the Registry of Bone Sarcoma was established by the American College of Surgeons. Since that time our knowledge of malignant bone tumors has increased tremendously. Nevertheless, the management of these cases has failed to keep pace with the advances thus made. This failure has resulted primarily from errors or delays in diagnosis, either of which can preclude any hope of success. This latter, if iUs to be achieved, requires early recognition and ·prompt treatment. Bone tumors are believed to comprise approximately 1 per cent of all malignancies. Since the skeleton is of mesenchymal origin, the growths are sarcomatous in type. The principal lesions encountered are osteogenic sarcoma, Ewing's tumor, multiple myeloma and metastatic carcinoma. To the physician in general practice, who is called upon daily to treat individuals with symptom/> referable to the musculoskeletal system, these patients can present major diagnostic problems, because the possibility of malignancy must always be kept in mind. All too many of these patients subsequently are referred or find their way to orthopedic surgeons after long periods either without treatment or, what is sometimes worse, after palliative treatment for "rheumatism," "arthritis" or similar complaints. If such tragedies are to be avoided in the future, the general practitioner should adopt a more inquisitive attitude, particularly in regard to pain in the extremities. Unexplained, persistent bone pain should be considered as caused by a malignant bone ~mor until proven otherwise. Every such patient demands a comI1~te diagnostic survey. The following case reports illustrate some of the pitfalls which may be encountered .
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REPORT OF CASES CASE I.-An ll-year-old girl was referred to the clinic for consultation in June 1935. Her principal complaint was persistent, intense pain in the right shoulder, which had become apparent gradually since August 1934. The patient had noticed increasing cyanosis and sweating of the right hand three months before examination. Numbness of the hand on dependency had occurred for the first time two months later. The clinical diagnosis was Ewing's tumor of the humerus. The child showed marked atrophy of the shoulder, with loss of muscle power in the shoulder and elbow. Exquisite tenderness was elicited over the upper shaft of the humerus, but no definite mass could be defined. Radiologically, a diffuse zone of cortical sclerosis, 6.0 cm. in length, was visualized, involving the proximal humeral metaphysis. This surrounded an irregular area of medul-
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lary destruction. The lungs and the other bones showed no evidence of metastasis. Laboratory examination was negative. A tentative diagnosis of sclerosing osteomyelitis was made and exploration was advised. It should be noted that the child was employed as a photographer's model. Her parents objected strenuously to any suggestion that an operation was necessary, on the grounds that it
Fig. 266 (Case 1).- Necrosis of bone resulting from excessive irradiation.
a, June 1935. Sclerosis of cortex with early periosteal reaction. Central area of rarefaction. b, November 1941. Epiphysis now fused. Shaft and head markedly sclerotic. Rarefaction increasing. No periosteal reaction. c, December 1942. Progressive sclerosis of upper shaft with gross sequestration. Aseptic necrosis now evident in head, with early involvement of glenoid. d, November 1946. Upper shaft has been resected. Head appears revascularized. Note destruction of glenoid. might prejudice their daughter's appearance. Instead, they sought treatment elsewhere. This treatment took the form of intensive x-ray therapy, on a presumptive diagnosis of Ewing's tumor, employing only a single, anterior portal over the upper arm. Symptoms were at first relieved; but within a year the reaction to
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excessive local x-ray therapy caused sloughing of the skin and of the shoulder musculature, as well as necrosis of the upper humerus (Fig. 266). In the twelve years since that time, the patient has repeatedly returned to the clinic for reparative surgery, including partial resection of the humerus, debridement of sioughing tendons and multiple skin grafts. Thoracic sympathectomy has been employed in a further endeavor to promote healing. Nevertheless, 'one -of the results has been a flail shoulder. Amputation is now under consideration.
Comment. The exact diagnosis in this case will always remain in doubt; but the fact that the patient received only local irradiation and yet survived suggests that the original lesion was benign ratheI than malignant. The tragic results of ill-advised and inept radio. therapy are obvious. These might have been averted, or at least minimized, by early exploration and biopsy, followed by treatment based on an accurate diagnosis. CASE 2.-This young man, 17 years old, was referred to the clinic in November 1946, because of nocturnal pain about the left knee and lower thigh. The patient stated that he had fallen on the left knee in September 1945, causing pain and swelling in the lower thigh, neither of which was disabling. Roentgeno grams of the left knee, taken at that time, were reported to be negative. The swelling had persisted and had increased slowly in size. The pain, originally mild and related to activity, had become more severe and nocturnal in occurrence. In addition, there had been a weight loss of 12 pounds in the two months pre. ceding examination. Physical examination disclosed that the patient was an asthenic youth in obvious pain, who walked with a left knee limp. Active movements of the hips and knees were painless; there was no excess synovial fluid. The left lower thigh was found to be 3.0 cm. greater in circumference than the right, while the over lying skin was dusky in hue. A diffuse, indurated mass, which was not tender was found involving the lower femoral metaphYSis and the overlying soft tissues Roentgenologic examination revealed markedly increased density in the lowel femoral shaft, with an extracortical cuff of bony overgrowth, arranged in a typi cal "sunburst" fashion (Fig. 267). There was no evidence of metastases. Labora tory examination disclosed only a mild secondary anemia. The patient was admitted to the hospital for biopsy which yielded a diagnosi~ of osteogenic sarcoma. A high thigh amputation was performed.
Comment. The original x-ray films of September 1945, obtained fOJ comparison, revealed an osteolytic lesion involving the lower metaph. ysis of the left femur, with bony spicules extending medially into the overlying soft parts even at that time. The price paid by this patient for the subsequent delay of fourteen months remains to be seen; a guarded prognosis has been given. CASE 3.-This patient, a 20-year-old girl, had gradually developed aching pain about the left patella six weeks before admission in November 1946. There was no history of antecedent injury. The severity of this complaint, soon after its onset, had led to her hospitalization elsewhere. Examination at that time, which included even oxygen arthrography, appears to have been confined to the knee joint; the findings are said to have been negative. It was noted, however, that
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the patient did exhibit a low-grade fever and mild leukocytosis. The case remained undiagnosed; the patient was reassured and discharged unimproved. When the patient later came to the clinic, she stated that the pain had persisted, was increasing in severity and had become maximal by night. Further questioning disclosed that cellulitus of the left knee had developed in 1938, which had necessitated incision and drainage. In addition, she was said to have suffered
Fig. 267 (Case 2) .-Osteogenic sarcoma. a, September 1945. Slight rarefaction in lower diaphysis. Very early radial spiculation visible along medial border. b, November 1946. Marked thickening of shaft. Obliteration of medullary canal. Characteristic "sunburst" appearance. from "rheumatic fever" with joint involvement in 1941. She had been free from symptoms in the interim. The patient appeared healthy, although she was found still to be running a daily fever of 99.6° F. Tenderness was elicited medially over the lower shaft of the left femur, where some thickening of the soft parts was palpable; no local mass was demonstrable, but a slight effusion was noted in the knee joint. There were no localizing signs otherwise in either knee or hip. The inguinal lymph
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nodes were enlarged bilaterally but were not tender. Roentgenologic examination proved equivocal, showing a destructive lesion of the shaft of the left femur posteriorly at the junction of the middle and lower thirds, with local disruption of the cortex and proliferation of the periosteum (Fig. 268). No soft tissue mass could be visualized. The lungs were clear. The sedimentation rate was 50 mm. in one hour, and white blood count, 9900. The findings were believed compatible with either Ewing's tumor or osteomyelitis. The patient was, therefore, hospitalized for a therapeutic trial on penicillin, the dosage being 40,000 units every three hours for five days. Since there was no appreciable response, operation was thought necessary. The involved bone was explored through a lateral approach, using localizing roentgenograms taken in
Fig. 268 (Case 3) .-Osteomyelitis. November 1946. Elevation and thickening of periosteum, slight thickening of cortex. Medullary canal irregular, with two central areas of rarefaction. the operating room. Drilling of the cortex allowed the escape of thick, greenish pus. Culture of this material subsequently' yielded Staphylococcus aureus haemolyticus of a penicillin-resistant strain. The routine treatment of osteomyelitis, decompression and primary closure was followed by sulfonamide therapy and resulted in complete relief of symptoms.
Comment. The clinical history and physical findings, which had been interpreted elsewhere as iqdicative of a knee joint affection, were not conclusive. The original radiographic examination had been restricted to a circumscribed area about the knee joint, with the result that the osteolytic lesion in the femoral shaft had not been visualized. Chemotherapy was finally selected as a differential measure, in prefer-
G. E. HAGGART, FRANK L. SHIPP
,enee to radiotherapy, because the latter was known to be capable of regression of symptoms in both osteomyelitis and Ewing's tUmor. Penicillin therapy proved ineffectual only because of the !lpecific nature of the causative organism. Hence, the weight of clinical and radiographic evidence favored malignancy. Once again, exploration was necessary before a conclusive diagnosis could be established and efficient treatment instituted.
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DISCUSSION
In order to avoid mistakes such as these, each such patient should be subjected to a complete diagnostic survey. These procedures have become more and more comprehensive in their scope with further refinements in laboratory technics. Nevertheless, the basis on which every such survey is founded still lies in the clinical history and the physical examination. Pain is, by all odds, the most important single complaint in bone malignancy; but a description of other symptoms, swelling, impairment of function and loss of weight, must be elicited with care. Pain in a malignant bone tumor is usually constant and progressive. It is more noticeable by night, rather than on exertion. Swelling of the part, occurring somewhat later in the course of the disease, is also likely to increase steadily, although the rate may vary with the character of the neoplasm. Function may show impairment early, although this is by no means always the case. The relationship of trauma to the onset of symptoms deserves mention. As was noted several years ago; in osteogenic sarcoma particularly, many patients will give a history of an antecedent injury to the part. Furthermore, in other types of tumor, the patient's attention may first be drawn to the area by reason of an injury, with persistence of the resultant symptoms. Careful inquiry is necessary regarding the onset, extent and severity of such symptoms as ecchymosis, swelling or tenderness. The physician should also note the relationship of these complaints to the present illness. Physical examination, to be dependable, must be thorough and complete. Inspection alone will often disclose deformities of the skull or long bones, of which the patient himself may be ignorant. Evidence may be found of involvement of the nervous or the lymphatic system. Such organs as the thyroid, breast, prostate and kidneys merit special attention for they are known to be favorite sites for tumors metastasizing to bone. Local examination of the affected part may reveal the presence of a visible or palpable swelling. It should be remembered that malignant bone tumors show a predilection for the ends of long bones, near the epiphyseal lines, where complexities of growth and function occur. Hence, the very location of the mass may arouse suspicion, as should such phenomena as increased warmth or superficial venous
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engorgement. The exact site and size of the tumor, its outline, mobility, consistency and its relationship to overlying soft parts are all of importance. In many cases, the diagnosis will by this time be apparent from evaluation of the history and physical findings. The laboratory procedures include radiographic, chemical, serologic and pathologic studies, of which at least the more important are available to the general practitioner. Roentgenologic examination of the involved part necessitates stereoscopic views in at least two planes, while further information may be gleaned from variations in technic. Films of the chest are essential, for we know that bone tumors in general tend to metastasize through the blood stream to the lungs, rather than through the lymphatic system. The skull, spine, pelvis and any other bones indicated are likewise subjected to close scrutiny. It has been our experience that the interpretation of the films in such cases may be exceedingly difficult. Certainly, expert opinion should be sought before making a final diagnosis. Laboratory eXilmination regularly comprises blood counts and serology. Blood chemical estimates in regard to serum calcium, phosphorus, phosphatase or total protein may also be of value. Tests for Bence-Jones proteinuria are carried out if there is anything to indicate multiple myeloma. The final and probably the most reliable procedure at our disposal is microscopic examination of tissue sections. In our experience specimens obtained by aspiration or punch biopsy are generally less dependable than surgical specimens; for this reason, we prefer the open method. The danger of hemorrhage from a highly vascular tumor can be obviated by the use of the electro-desiccating knife. Tourniquets are never employed for the control of bleeding in such a case. In this regard, a word should be said about the approach to long bones for biopsy. Since amputation may prove unnecessary, consideration must be given to the future function of the limb. Trauma to the soft parts and to the tumor itself should be reduced to a minimum for this reason, as well as to avoid the spread of tumor cells. With this in view, the general principles of extensile exposure, first outlined by Henry in 1927, have been followed in this clinic with excellent results. In brief, the value of the information to be gained from. biopsy, in our judgment, far outweighs any risk to the patient. The efficacy of preoperative irradiation of proven bone tumors is still under investigation. SUMMARY
Realizing that these patients may present themselves to any practitioner, it is believed that every individual with persistent, unexplained bone pain should be investigated on a presumptive diagnosis of malignant bone tumor. Under these circumstances, adequate radiographic studies must accompany the history and physical findings. If
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doubt still exists as to the exact diagnosis, further laboratory investigations are necessary, of which by far the most important and most reliable is microscopic examination of tissue obtained at biopsy.. REFERENCES 1. Henry, A. K.: Exposures of long bones and other surgical methods. New York, Wood, 1927, pp. 80. 2. Henry, A. K.: Extensile exposure applied to limb surgery. New York, Wood, 1945, pp. 180.