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Initial Symptoms and Clinical Features in Osteosarcoma and Ewing Sarcoma*
Björn Widhe, M.B.†; Torulf Widhe, M.D.†
View Disclosures and Other Information
Investigation performed at Huddinge University Hospital, Huddinge, Sweden
*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Funds were received in total or partial support of the research or clinical study presented in this article. The funding source was The King Oscar II and Queen Sofia Golden Wedding Anniversary Foundation, Stockholm, Sweden.
†Department of Orthopedics, Huddinge University Hospital, S-141 86 Huddinge, Sweden. E-mail address: h95bwi@student.ki.se (Björn Widhe). E-mail address: torulf.widhe@karo.ki.se (Torulf Widhe).

The Journal of Bone & Joint Surgery.  2000; 82:667-667 
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Abstract

Background: The time between the initial symptoms of osteosarcoma and Ewing sarcoma and the correct diagnosis and treatment is long. Over the last two decades, the prognosis for patients with these diseases has dramatically improved due to a new chemotherapy regimen. As a consequence, a limb-sparing operation has become an alternative to amputation. The aim of this study was to establish the initial symptoms and physical signs of osteosarcoma and Ewing sarcoma from the records of the first medical visit and to identify early characteristics of the diseases to shorten the delay to diagnosis.

Methods: A group of patients with osteosarcoma or Ewing sarcoma was identified from the Swedish Cancer Register of patients thirty years old and younger. Records from the first medical visit due to symptoms related to the bone tumor were obtained for 102 patients with osteosarcoma and forty-seven patients with Ewing sarcoma.

Results: Pain related to strain was reported by eighty-seven (85 percent) of the patients with osteosarcoma and thirty (64 percent) of those with Ewing sarcoma, but only twenty-one (21 percent) of the patients with osteosarcoma and nine (19 percent) of those with Ewing sarcoma reported pain at night. Forty-eight (47 percent) of the patients with osteosarcoma and twelve (26 percent) of those with Ewing sarcoma related the onset of symptoms to minor trauma occurring around the same time. A palpable mass was noted in forty (39 percent) of the patients with osteosarcoma and sixteen (34 percent) of those with Ewing sarcoma at the first visit, and in most cases the tumor diagnosis was suspected. There was a broad spectrum of misdiagnoses; the most common was tendinitis, which was the initial diagnosis in thirty-two (31 percent) of the patients with osteosarcoma and ten (21 percent) of those with Ewing sarcoma. The doctor's delay (the period from the first medical visit due to the symptoms to the correct diagnosis) was longer for Ewing sarcoma than for osteosarcoma (nineteen weeks and nine weeks, respectively; p < 0.0001).

Conclusions: An initial symptom of both osteosarcoma and Ewing sarcoma was pain, which was intermittent and often related to strain but not frequently felt at night. A history of trauma was common, but the clinical course often diverged from what was expected from trauma. The clinical course of osteosarcoma and particularly of Ewing sarcoma was not steadily progressive but intermittent, which often misled the doctor into believing that the condition was temporary. The most important clinical feature was a palpable mass, which was noted in more than one-third of the patients at the first visit. This finding emphasizes that a thorough physical examination is absolutely necessary.

Figures in this Article
    Primary malignant bone tumors are rare. The two most common types, osteosarcoma and Ewing sarcoma, have a peak incidence in the second decade of life and an annual incidence of two and 0.8 per million population, respectively7,13.
    These tumors are so rare that most doctors will see only a few patients with symptoms from an undiagnosed primary bone tumor during their whole working life. Most general practitioners therefore have little or no experience with these primary malignant bone tumors, but they are the first doctors consulted in the initial phase of the disease.
    Numerous articles have focused on the treatment and prognosis of primary malignant bone tumors but only a few have described the initial symptoms of the disease. Sweden and other countries have centers specializing in bone tumors. The physicians at these clinics meet the patient when the tumor is diagnosed or when it is strongly suspected. The recollection of the patient, or his or her parent, of when and under what circumstances the disease started may at that time be influenced by the threat of a diagnosis of cancer.
    Early recognition and treatment is important in all malignant diseases. In recent decades there has been a remarkable increase in survival rates of both patients with osteosarcoma and those with Ewing sarcoma1. Early diagnosis of a bone tumor may increase not only the chance of survival but also the possibility of performing a limb-sparing resection.
    The purpose of this study was to identify the early symptoms of osteosarcoma and Ewing sarcoma by analyzing the initial symptoms and physical findings as described in the report from the patient's first visit to a physician for symptoms that could be related to the bone neoplasm. Furthermore, it was intended to establish whether something in the patient's history or in the physical findings was characteristic or divergent and should have made the doctors aware of a malignant bone tumor.
     
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    +Fig. 1:Bar graph showing the normal distribution of female age (mean and standard deviation, 15.1 6.0 years) in the series.
     
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    +Fig. 2:Bar graph showing the normal distribution of male age (mean and standard deviation, 16.0 5.0 years) in the series.
     
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    +Fig. 3:Illustration showing the anatomical distribution of the Ewing sarcomas and osteosarcomas in the series. The numbers represent the numbers of patients.
     
    Anchor for JumpAnchor for JumpTable I:  Reasons for Consulting a Doctor
    *The values are given as the numbers of patients.
    Osteosarcoma*Ewing Sarcoma*
    Regional pain71 (70%)34 (72%)
    Palpable mass4 (4%)  5 (11%)
    Regional pain and palpable mass26 (25%)  7 (15%)
    Regional pain and paresthesia1 (1%)1 (2%)
     
    Anchor for JumpAnchor for JumpTable II:  Physical Findings at the First Medical Visit
    *The values are given as the numbers of patients.†Tumors in the upper extremity were excluded.
    Osteosarcoma*Ewing Sarcoma*
    Local tenderness94 (92%)33 (70%)
    Palpable mass40 (39%)16 (34%)
    Painful movement of joint40 (39%)16 (34%)
    Restricted movement of joint 23 (23%)  8 (17%)
    Fever (reported or measured)3 (3%)14 (30%)
    Atrophy of muscle5 (5%)0 (0%)
    Limp† (noted or reported)28 (31%)19 (40%)
     
    Anchor for JumpAnchor for JumpTable III:  Initial Diagnosis at the First Medical Visit
    *The values are given as the numbers of patients.
    Osteosarcoma*Ewing Sarcoma*
    Tendinitis32 (31%)10 (21%)
    Suspected bone tumor32 (31%)  9 (19%)
    Uncertain12 (12%)  9 (19%)
    Sciatica0 (0%)  5 (11%)
    Coxitis simplex1 (1%)4 (9%)
    Osteomyelitis1 (1%)3 (6%)
    Chondromalacia patellae5 (5%)0 (0%)
    Fracture3 (3%)1 (2%)
    Osgood-Schlatter disease2 (2%)1 (2%)
    Hematoma3 (3%)0 (0%)
    Pathological fracture2 (2%)0 (0%)
    Dislocation of shoulder2 (2%)0 (0%)
    Muscle rupture2 (2%)0 (0%)
    Meniscal lesion2 (2%)0 (0%)
    Varicocele0 (0%)1 (2%)
    Lipoma0 (0%)1 (2%)
    Inguinal hernia0 (0%)1 (2%)
    Psychological explanation0 (0%)1 (2%)
    Legg-Calv笐erthes disease1 (1%)0 (0%)
    Bursitis1 (1%)0 (0%)
    Postinfection pain1 (1%)0 (0%)
    Common cold0 (0%)1 (2%)
    Total10247
     
    Anchor for JumpAnchor for JumpTable IV:  Patient's, Doctor's, and Total Delays
    *The values are given as the mean number of weeks, with the range in parentheses.
    Osteosarcoma*Ewing Sarcoma*P Value
    Patient's delay  6 (1-26)  15 (1-100)    0.0002
    Doctor's delay  9 (1-52)19 (1-72)<0.0001
    Total delay15 (2-75)  34 (3-150)<0.0001
    A listing of all patients diagnosed with osteosarcoma or Ewing sarcoma in Sweden between the beginning of January 1983 and the end of December 1995 was obtained from the Swedish Cancer Register at the National Swedish Board of Health, Stockholm, Sweden. The study was limited to patients who were thirty years old or younger, and all tumors except those located in the skull or the ribs were included. The Swedish Cancer Register is a population-based register for all malignant tumors diagnosed in Sweden. A double-reporting system is used so that every physician and pathologist has to report all patients with a malignant tumor, and therefore almost all cases in Sweden are reported. Each patient's data from the Swedish Cancer Register includes information about the clinic where and the date when the accurate diagnosis was established, the histological findings, the location of the tumor, and the day of death.
    Files from the clinic where the tumor diagnosis was initially made were used to find information about when and where the patient first consulted a doctor for symptoms that could be related to the primary malignant bone tumor. Records from earlier visits were requested until the medical file from the first visit was obtained.
    The data recorded at the first medical visit included pain at night; pain related to strain; intermittent pain at rest; swelling; trauma; physical activity; duration of symptoms; periods of fever; physical signs such as a palpable mass, tenderness, limping, painful movements of the joint, restricted movements of the joint, and atrophy of muscle; and the doctor's prescription and initial diagnosis.
    Patient's delay was defined as the period from when the patient first noted symptoms until the patient first consulted a doctor, and doctor's delay was defined as the period from the first medical visit for these symptoms until the date that an accurate diagnosis was established.
    The Student t test, chi-square test, and analysis of variance were used for statistical analysis. The significance level was set at p < 0.05.
    There were 102 patients with osteosarcoma; sixty-one were male and forty-one, female. Forty-seven patients had Ewing sarcoma; twenty-eight were male and nineteen, female. A predilection for the male gender of 1.5:1 was shown for both osteosarcoma and Ewing sarcoma. The mean age of the patients with osteosarcoma was 15.8 years (range, 5.5 to 29.5 years) compared with 15.4 years (range, 2.5 to 26.0 years) for the patients with Ewing sarcoma (Fig. 1 and Fig. 2). Seventy-five (74 percent) of the osteosarcomas and eleven (23 percent) of the Ewing sarcomas were located around the knee (Fig. 3).

    First Medical Visit

    Eighty-six (58 percent) of the patients first consulted a district general practitioner for symptoms related to the primary malignant bone tumor, and forty-two (28 percent) consulted a doctor at an emergency ward. Eleven (7 percent) of the patients, who were young, saw their school doctor, and eight (5 percent) of the patients, who were in the military service, consulted a military doctor. Only two patients (1 percent) consulted a private practitioner. Thus, 72 percent of the patients first consulted a general practitioner for symptoms related to the primary malignant disease.

    Reasons for Consultation

    Regional pain alone or in combination with a palpable mass were the two main reasons that patients consulted a doctor (Table I). Seventy-one (70 percent) of the patients with osteosarcoma and thirty-four (72 percent) of the patients with Ewing sarcoma consulted a doctor about regional pain alone. Twenty-six (25 percent) of the patients with osteosarcoma and seven (15 percent) of those with Ewing sarcoma consulted a doctor about regional pain and a palpable mass. Only four (4 percent) of the patients with osteosarcoma and five (11 percent) of those with Ewing sarcoma did not report pain at the first medical visit; these patients all had a palpable mass only. One patient (1 percent) with osteosarcoma and one (2 percent) with Ewing sarcoma had regional pain and paresthesia (Table I).

    Pain

    Only twenty-one (21 percent) of the patients with osteosarcoma and nine (19 percent) of those with Ewing sarcoma had pain at night; however, eighty-seven (85 percent) of the patients with osteosarcoma and thirty (64 percent) of those with Ewing sarcoma reported pain related to strain. Intermittent pain at rest, which was equally common in patients with osteosarcoma and those with Ewing sarcoma, was reported by fifty-seven (56 percent) and twenty-seven (57 percent), respectively.

    Trauma

    Forty-eight (47 percent) of the patients with osteosarcoma and twelve (26 percent) of those with Ewing sarcoma related the onset of symptoms to trauma occurring at about the time that the symptoms began. Adolescents commonly engage in athletic activity; 58 percent of the male patients and 35 percent of the female patients participated in sports, and the majority of the traumatic incidents reported by the patients were of a similar type and magnitude as those regularly experienced by participants in common sports.

    Physical Findings at the First Medical Visit

    According to the records, none of the 149 patients had a physical examination that revealed no remarkable findings at the first visit. Forty (39 percent) of the patients with osteosarcoma and sixteen (34 percent) of those with Ewing sarcoma already had a palpable mass at the tumor site at the first medical visit. As many as ninety-four (92 percent) of the patients with osteosarcoma and thirty-three (70 percent) of those with Ewing sarcoma had tenderness at the tumor location (Table II).

    Initial Diagnosis

    Osteosarcoma

    At the first medical visit, a bone tumor was suspected in thirty-two (31 percent) of the patients with osteosarcoma. There was a significant difference, with regard to suspicion of a tumor, between male patients (21 percent) and female patients (46 percent) (p < 0.05). Tendinitis was the most common misdiagnosis and was the initial diagnosis for thirty-two (31 percent) of the osteosarcomas (twenty-five [41 percent] in male patients and seven [17 percent] in female patients). The doctor was uncertain about the diagnosis for twelve (12 percent) of the patients. Only two patients were initially diagnosed as having a pathological fracture (Table III).

    Ewing Sarcoma

    At the first medical visit, a bone tumor was suspected in nine patients (19 percent) who had Ewing sarcoma. Tendinitis was the most common misdiagnosis and was the initial diagnosis for ten (21 percent) of the patients. The doctor was uncertain about the diagnosis for nine (19 percent) of the patients. Misdiagnosis of Ewing sarcoma of the pelvis or the proximal aspect of the femur was not uncommon. The most frequent misdiagnoses for young patients were coxitis simplex and osteomyelitis, whereas tendinitis and sciatica were the most frequent misdiagnoses for older patients (Table III).

    Radiographic Examination

    Sixty-eight (67 percent) of the patients with osteosarcoma and twenty-eight (60 percent) of those with Ewing sarcoma had a radiograph made at the first medical visit. Radiographic examination was more often carried out for young or female patients or if there was a palpable mass or a history of limping or trauma (p < 0.05). However, the correct diagnosis was not established for all patients who had a radiograph. The radiograph was misinterpreted by the radiologist as normal or inconclusive for six (9 percent) of the sixty-eight patients with osteosarcoma and for twelve (43 percent) of the twenty-eight patients with Ewing sarcoma.

    Analgesics and Physiotherapy Prescribed at the First Visit

    Nonsteroidal anti-inflammatory drugs were prescribed for twenty patients. Fourteen of them were diagnosed with tendinitis; two, with sciatica; and one each, with a hematoma and a muscle rupture. The diagnosis was uncertain for the remaining two patients. All of these patients reported pain related to strain. Thirteen patients were referred for physiotherapy, ten were diagnosed as having tendinitis, and three were diagnosed as having sciatica.

    Delay in Diagnosis

    The patient's delay (p = 0.0002), doctor's delay (p < 0.0001), and total delay (p < 0.0001) were all significantly longer for the patients with Ewing sarcoma than for the patients with osteosarcoma (Table IV). A palpable mass, the ordering of a radiograph, and a history of trauma were all factors that reduced the time to the correct diagnosis significantly. When the patients had a palpable mass at the first medical visit, the doctor's delay averaged five weeks compared with sixteen weeks for those without a mass (p < 0.0001). When a radiograph was made at the first visit, the doctor's delay averaged eight weeks compared with nineteen weeks when a radiograph was not made (p = 0.0001). Patients who reported a traumatic incident at the onset of symptoms had a mean total delay of sixteen weeks compared with twenty-five weeks for patients who did not report such an incident (p < 0.01).
    Seventy-six (75 percent) of the patients with osteosarcoma had a doctor's delay that was shorter than two months, and only ten (10 percent) had a doctor's delay exceeding six months. However, nineteen (40 percent) of the patients with Ewing sarcoma had a doctor's delay of less than two months, and thirteen (28 percent) had a doctor's delay exceeding six months.
    There were only small differences in delay between male and female patients. Female patients with osteosarcoma had a longer mean patient's delay (eight weeks compared with five weeks for male patients; p < 0.05), but the doctor's delay did not differ between the sexes.
    In this investigation, we intended to establish the initial symptoms and physical signs of osteosarcoma and Ewing sarcoma by analyzing the records from the first visit to a physician for symptoms that could be related to the bone neoplasm. No previous studies on this subject have, to our knowledge, utilized this source of information; instead, investigators have relied upon data given at admission to the tumor clinic, when the diagnosis was known or strongly suspected10,15. We compared the patient history that was recorded at the first visit with that recorded at admission to the tumor center. The discrepancy between the data in those files demonstrated how vital it is to use primary medical records in a study on the initial symptoms of bone tumors. All but three of the records used in the present study were typewritten, and nearly all had a clear description of symptoms and physical signs at the first medical visit.
    The predilection for the male gender (1.5:1) and the anatomical distribution of the osteosarcomas (distal aspect of the femur, forty-five patients; proximal aspect of the tibia, twenty-five; and proximal aspect of the humerus, ten) observed in the present study are characteristic of and in accordance with previous findings in large studies of primary bone tumors1-3. The present series is therefore representative of these neoplasms.
    As expected, most patients first sought medical attention from the district general practitioner. Most general practitioners presumably had little experience with bone tumors and not infrequently described the clinical picture as unfamiliar. They were often doubtful about the diagnosis and ordered an early check-up. Many patients attributed the onset of symptoms to a traumatic incident and therefore went to the emergency ward, as would be expected. Most patients who visit an emergency room after a traumatic episode expect a radiograph to be made, and radiographs were made for twenty-three (79 percent) of the twenty-nine patients with osteosarcoma and ten (77 percent) of the thirteen with Ewing sarcoma who visited an emergency room in our study.

    Symptoms and Physical Findings

    Pain that is more severe at night than during the day is generally accepted as a typical symptom of a primary malignant bone tumor. Letson et al.8 stated: "In contrast, pain from a musculoskeletal malignancy is often continuous and dull. It is usually severe at rest and is characteristically worse at night." Simon and Finn9 stated that "unremitting pain or pain that is more severe at rest or at night than during the day is often considered as a symptom of a malignant disease." The present study showed that pain at night was not a dominant initial symptom of malignant bone tumors as only twenty-one (21 percent) of the osteosarcomas and nine (19 percent) of the Ewing sarcomas caused pain at night. However, pain related to strain and intermittent pain at rest were frequent; as these symptoms are also common in several benign musculoskeletal disorders, they can easily lead to an inaccurate diagnosis.
    Consistent with previous studies6,13, fourteen (30 percent) of the patients with Ewing sarcoma had unexplained periods of fever. A combination of this fever with pain and an inconclusive radiograph led the doctor to suspect osteomyelitis, which has also been reported earlier5,14. This initial misdiagnosis delayed correct treatment for several months.
    A palpable mass, the physical finding that most strongly indicates a bone tumor, was reported, at the first visit, in forty (39 percent) of the patients with osteosarcoma and sixteen (34 percent) of those with Ewing sarcoma. Vlasak and Sim13 reported that approximately 70 percent of patients with Ewing sarcoma had a palpable mass at the time of diagnosis, but they made those observations at a later stage of the disease. In the present study, not all patients with a palpable mass were suspected to have a malignant bone tumor but they had a significantly shorter doctor's delay. Twenty-three (72 percent) of the thirty-two patients with osteosarcoma and all nine of the patients with Ewing sarcoma who had a preliminary diagnosis of a bone tumor at the first visit had a palpable mass. This prevalence emphasizes both that a palpable mass is one of the strongest indications of a primary malignant bone tumor and that a thorough and extensive physical examination of every patient is very important.
    Limping by children is in most cases a self-limiting, benign condition; however, some rare serious conditions must be excluded11, as limping has been reported as a symptom of primary malignant bone tumors. Our study showed that it was common, with twenty-eight of the patients with osteosarcoma and nineteen of the patients with Ewing sarcoma limping in the initial phase of the disease (Table II).

    Initial Diagnosis

    A history of minor trauma, pain related to strain, and regional tenderness generated a broad spectrum of possible diagnoses (Table III). Although tendinitis is an uncommon condition in such a young population, it was the most frequent misdiagnosis in this study. In many cases, both the history and the physical signs were inconsistent with this diagnosis. These patients were ordered to refrain from physical activity for two to three weeks. During this period, the pain subsided and both the patient and the doctor were led to believe that the condition was temporary and would resolve spontaneously. However, when the patient resumed physical activity, the pain returned and often became more severe.
    Age naturally had an influence on the possible misdiagnoses: children with hip pain were diagnosed as having coxitis, osteomyelitis, or Legg-Calv笐erthes disease, but young adults with the same symptoms and physical findings were diagnosed as having tendinitis and sciatica.

    Radiographs

    As many as sixty-eight (67 percent) of the patients with osteosarcoma and twenty-eight (60 percent) of those with Ewing sarcoma had a radiograph made at the first medical visit. This high proportion of the patients who were examined radiographically might be explained by the fact that many patients related the onset of symptoms to a traumatic episode; a radiograph was made to exclude the possibility of a fracture. The initial radiographs were misinterpreted by the radiologist as normal or inconclusive for six (9 percent) of the sixty-eight patients with osteosarcoma and twelve (43 percent) of the twenty-eight patients with Ewing sarcoma. Amazingly, radiographs were not made at the first visit for ten patients who had a palpable mass. Five of these patients had a misdiagnosis of tendinitis; one each, a misdiagnosis of muscle rupture, sciatica, and hematoma; and two, an uncertain diagnosis.

    Delay in Diagnosis

    Sneppen and Hansen10 reported a mean total delay in the diagnosis of osteosarcoma of twenty-eight weeks compared with fifteen weeks in the present study. This discrepancy in delay has two possible explanations. First, Sneppen and Hansen calculated the total delay on the basis of information given at the time of the patient's admission to the tumor center, whereas in the present study we used the records from the first medical visit. The difference in population between the studies is also important. The present study was limited to patients who were no older than thirty years of age because osteosarcoma has a different etiology and pattern in older patients. In contrast, Sneppen and Hansen included patients of all ages.
    Frassica et al.6 reported a mean total delay in diagnosis of thirty-two weeks for patients with Ewing sarcoma of the pelvis, and Damron et al.4 reported a mean total delay in diagnosis of thirty-seven weeks for patients with Ewing sarcoma of the proximal aspect of the femur. This data is in accordance with that in the present study, in which the mean total delay in the diagnosis of Ewing sarcoma was thirty-four weeks.
    There might be several different reasons why the patient's and doctor's delays in the diagnosis of Ewing sarcoma were longer than the delays in the diagnosis of osteosarcoma. First, malignant diseases are generally believed to have a steadily progressive course. However, patients with Ewing sarcoma often reported relapsing fever and periods of pain that were followed by periods with no or only few symptoms. The intermittent clinical course misled the doctor into believing that the patient's symptoms were temporary and that the condition was resolving spontaneously. Furthermore, it was not uncommon for the initial radiograph to be inconclusive or normal. It often takes a long time before a doctor will reconsider a diagnosis when the radiograph is normal. Thus, the doctor's delay exceeded five months for nine of twelve patients with Ewing sarcoma who had a normal or inconclusive initial radiograph. Finally, only eleven (23 percent) of the Ewing sarcomas were located around the knee compared with seventy-five (74 percent) of the osteosarcomas. Tumors located in the spinal and pelvic regions are more difficult to diagnose because they must grow larger before they are palpable and are visible on plain radiographs15.
    Patient's delay differed according to gender; female patients with osteosarcoma waited for a longer period of time before consulting a doctor. However, the doctor already suspected a bone tumor at the first visit in 46 percent of the female patients compared with 21 percent of the male patients. The fact that male patients more often participate in sports gave both the doctor and the patient a conceivable explanation for the symptoms. The fact that female patients waited for a longer period before consulting a doctor and the fact that they were less muscular made it easier for the doctor to notice a palpable mass. These factors may explain the observed difference between the sexes with regard to the delay in diagnosis.
    The delay between the time when the patient first noted symptoms and the first medical visit (the patient's delay) may be impossible to change, as in the present study there were no evident obstacles that prevented the patient from seeing a doctor. The only way to reduce the total delay may be to shorten the period from the first medical visit to the establishment of the accurate diagnosis (the doctor's delay). In this study, the doctor's delay in the diagnosis of osteosarcoma was generally short; it did not exceed two months for seventy-six (75 percent) of the osteosarcomas. The patients with Ewing sarcoma generally had a long doctor's delay; thirteen patients (28 percent) had a delay that was longer than six months. All except one of the thirteen patients consulted a doctor more than six times before the tumor was diagnosed, and one patient visited the hospital sixteen times and the district general practitioner seven times about symptoms related to the bone tumor. Three patients with Ewing sarcoma of the pelvis had intermittent low-back pain. After several weeks of conservative treatment with analgesics and physiotherapy, a radiograph of the lumbar spine was ordered. However, the correct diagnosis was further delayed because the radiograph did not include the pelvis.
    Our data showed that a long doctor's delay was due to three main factors. First, tumors of the spinal and pelvic regions were difficult to visualize on the plain radiographs. Second, a radiograph showing normal findings was believed to exclude a diagnosis of a malignant tumor and the interpretation was accepted for too long before repeat radiographs were made. Finally, treatment for other diagnoses proceeded for too long despite the divergence of the clinical picture from what could be expected from the original (incorrect) diagnosis.
    Three factors were associated with a short doctor's delay: the presence of a palpable mass, which emphasizes the importance of a thorough and extensive physical examination; the ordering of a radiograph, although in most cases the radiographic examination was conducted to exclude the possibility of a fracture without any suspicion of a bone neoplasm; and the age of the patient, with children having a shorter doctor's delay because the doctor's uncertainty concerning the diagnosis resulted in an early check-up and more frequent radiographs.
    Another factor that should raise the doctor's suspicion of a malignant tumor is the clinical course after a minor traumatic episode. Forty-eight (47 percent) of the patients with osteosarcoma and twelve (26 percent) of those with Ewing sarcoma related the onset of symptoms to a traumatic incident occurring at a similar time, findings that are consistent with those of previous reports10,12. Primary malignant bone tumors are rare, whereas children and young adults are frequently seen by a doctor after minor trauma. However, the clinical course of the patients in our study often diverged from what is expected after minor trauma. The patients had temporary pain connected to the trauma, but the symptoms resolved in a day or two. After a short period (days or weeks) of little or no pain, the pain returned and became more intense and severe with time.
    In conclusion, an initial symptom of the primary bone tumors was intermittent pain that was often related to strain but was not frequently felt at night. A history of trauma was common, but the clinical course often diverged from what should be expected from such an etiology. The clinical course was not always steadily progressive but was often intermittent, misleading the doctor to believe that the condition was temporary. The most important clinical feature was a palpable mass, which was noted at the first visit in more than one-third of the patients. This finding emphasizes that a thorough physical examination is absolutely necessary.
    Arndt, C. A. S., and Crist, W. M.: Common musculoskeletal tumors of childhood and adolescence. New England J. Med.,341: 342-352, 1999.341342  1999 
     
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    Damron, T. A.; Sim, F. H.; O'Connor, M. I.; Pritchard, D. J.; and Smitson, W. A.: Ewing's sarcoma of the proximal femur. Clin. Orthop.,322: 232-244, 1996.322232  1996  [PubMed]
     
    Durbin, M.; Randall, R. L.; James, M.; Sudilovsky, D.; and Zoger, S.: Ewing's sarcoma masquerading as osteomyelitis. Clin. Orthop.,357: 176-185, 1998.357176  1998  [PubMed]
     
    Frassica, F. J.; Frassica, D. A.; Pritchard, D. J.; Schomberg, P. J.; Wold, L. E.; and Sim, F. H.: Ewing sarcoma of the pelvis. Clinicopathological features and treatment. J Bone Joint Surg,75-A: 1457-1465, Oct 1993.75-A1457  1993 
     
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    Anchor for JumpAnchor for Jump
    +Fig. 1:Bar graph showing the normal distribution of female age (mean and standard deviation, 15.1 6.0 years) in the series.
    Anchor for JumpAnchor for Jump
    +Fig. 2:Bar graph showing the normal distribution of male age (mean and standard deviation, 16.0 5.0 years) in the series.
    Anchor for JumpAnchor for Jump
    +Fig. 3:Illustration showing the anatomical distribution of the Ewing sarcomas and osteosarcomas in the series. The numbers represent the numbers of patients.
    Anchor for JumpAnchor for JumpTable I:  Reasons for Consulting a Doctor
    *The values are given as the numbers of patients.
    Osteosarcoma*Ewing Sarcoma*
    Regional pain71 (70%)34 (72%)
    Palpable mass4 (4%)  5 (11%)
    Regional pain and palpable mass26 (25%)  7 (15%)
    Regional pain and paresthesia1 (1%)1 (2%)
    Anchor for JumpAnchor for JumpTable II:  Physical Findings at the First Medical Visit
    *The values are given as the numbers of patients.†Tumors in the upper extremity were excluded.
    Osteosarcoma*Ewing Sarcoma*
    Local tenderness94 (92%)33 (70%)
    Palpable mass40 (39%)16 (34%)
    Painful movement of joint40 (39%)16 (34%)
    Restricted movement of joint 23 (23%)  8 (17%)
    Fever (reported or measured)3 (3%)14 (30%)
    Atrophy of muscle5 (5%)0 (0%)
    Limp† (noted or reported)28 (31%)19 (40%)
    Anchor for JumpAnchor for JumpTable III:  Initial Diagnosis at the First Medical Visit
    *The values are given as the numbers of patients.
    Osteosarcoma*Ewing Sarcoma*
    Tendinitis32 (31%)10 (21%)
    Suspected bone tumor32 (31%)  9 (19%)
    Uncertain12 (12%)  9 (19%)
    Sciatica0 (0%)  5 (11%)
    Coxitis simplex1 (1%)4 (9%)
    Osteomyelitis1 (1%)3 (6%)
    Chondromalacia patellae5 (5%)0 (0%)
    Fracture3 (3%)1 (2%)
    Osgood-Schlatter disease2 (2%)1 (2%)
    Hematoma3 (3%)0 (0%)
    Pathological fracture2 (2%)0 (0%)
    Dislocation of shoulder2 (2%)0 (0%)
    Muscle rupture2 (2%)0 (0%)
    Meniscal lesion2 (2%)0 (0%)
    Varicocele0 (0%)1 (2%)
    Lipoma0 (0%)1 (2%)
    Inguinal hernia0 (0%)1 (2%)
    Psychological explanation0 (0%)1 (2%)
    Legg-Calv笐erthes disease1 (1%)0 (0%)
    Bursitis1 (1%)0 (0%)
    Postinfection pain1 (1%)0 (0%)
    Common cold0 (0%)1 (2%)
    Total10247
    Anchor for JumpAnchor for JumpTable IV:  Patient's, Doctor's, and Total Delays
    *The values are given as the mean number of weeks, with the range in parentheses.
    Osteosarcoma*Ewing Sarcoma*P Value
    Patient's delay  6 (1-26)  15 (1-100)    0.0002
    Doctor's delay  9 (1-52)19 (1-72)<0.0001
    Total delay15 (2-75)  34 (3-150)<0.0001
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