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BONE SARCOMA DIAGNOSED AT THE TIMEOF RECONSTRUCTIVE HIP SURGERY

Paul A. Dowdy, MD;*Anthony M. Griffin, BSc;† Lawrence M. White, MD;‡ Jay S. Wunder, MD;† Robert S. Bell, MD†

From the *Department of Orthopedic Surgery, University of Western Ontario, London, Ont., the †University Musculoskeletal Oncology Unit, Mount Sinai Hospital and theUniversity of Toronto, and the ‡Department of Radiology, Mount Sinai Hospital, Toronto, Ont.

Accepted for publication Oct. 22, 1997

Correspondence to: Dr. Robert S. Bell, University Musculoskeletal Oncology Unit, Suite 476E, Mount Sinai Hospital, 600 University Ave., Toronto ON M5G 1X5; tel. 416 586-8607; fax 416 586-8397; [email protected]

© 1998 Canadian Medical Association (text and abstract/résumé)

OBJECTIVE: To describe the clinical course of a group of patients in whom sarcoma of the proximal femurwas diagnosed at the time of reconstructive hip surgery.DESIGN: A retrospective case series.SETTING: Final management of all patients took place at a tertiary care centre.PATIENTS AND INTERVENTIONS: Six consecutive patients with sarcoma of the proximal femur diagnosed atthe time of reconstructive hip surgery. The mistaken diagnoses made before surgery were benign tumour(2 patients), avascular necrosis (2 patients), subtrochanteric fracture due to metastasis (1 patient) and gran-uloma from a loose hip implant (1 patient). The final diagnosis was osteosarcoma in 3 patients and chon-drosarcoma in 3. Three patients with high-grade sarcoma received neoadjuvant chemotherapy followed byfemoral or pelvic resection, or both, and reconstruction. Two patients with chondrosarcoma underwentwide excision of the tumour with allograft or modular implant reconstruction. One patient with wide-spread metastasis received only palliative chemotherapy.MAIN OUTCOME MEASURES: Overall survival with respect to oncologic and functional results of treatment.RESULTS: Two patients (1 who received only palliative chemotherapy) died after 5 and 21 months’ follow-up, respectively. Average follow-up for the remaining 4 patients was 65.2 months (range from 51 to 75months). They were disease free at latest follow-up. One patient required amputation for septic complica-tions related to the reconstruction.CONCLUSIONS: Limb salvage surgery for sarcoma of the proximal femur is challenging when the diagnosis ismade at the time of reconstructive surgery rather than through an appropriately planned biopsy. However,this series suggests that limb preservation is feasible and that hindquarter amputation is not the onlysolution.

OBJECTIF : Décrire l’évolution clinique d’un groupe de patients chez lesquels on a diagnostiqué un sarcome àla face proximale du fémur au moment d’une intervention chirurgicale de reconstruction de la hanche.CONCEPTION : Série de cas rétrospective.CONTEXTE : Le traitement final de tous les patients a eu lieu à un centre de soins tertiaires.PATIENTS ET INTERVENTIONS : Six patients consécutifs qui avaient un sarcome à la face proximale du fémurdiagnostiqué au moment d’une intervention chirurgicale de reconstruction de la hanche. Avant l’interven-tion, on avait diagnostiqué par erreur une tumeur bénigne (2 patients), une nécrose vasculaire (2 patients),une fracture sous-trochantérienne causée par des métastases (1 patient) et un granulome causé par un im-plant lâche de la hanche (1 patient). On a diagnostiqué finalement un ostéosarcome chez trois patients etun chondrosarcome chez trois autres. Trois patients atteints d’un sarcome avancé ont reçu une chimio-thérapie néoadjuvante suivie d’une résection fémorale ou pelvienne, ou des deux, et d’une reconstruction.Deux patients atteints d’un chondrosarcome ont subi une excision large de la tumeur suivie d’une allogr-effe ou d’une reconstruction modulaire de l’implant. Un patient atteint de métastases généralisées n’a reçuqu’une chimiothérapie palliative.PRINCIPALE MESURE DE RÉSULTATS : Survie globale en ce qui a trait aux résultats oncologiques et fonction-

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Sarcomas of bone are rare tu-mours; most orthopedic sur-geons will see only a few exam-

ples during their career.1 The initialdiagnostic work-up of this malignanttumour is an important aspect of plan-ning successful limb salvage therapy,and an appropriate biopsy is consid-

ered fundamental to limb preserva-tion.1–3 Extensive surgery performed atthe site of an unrecognized bone sar-coma compromises the eventual sur-gical resection of the lesion by spread-ing disease into the surroundingtissues.1,3 Hindquarter amputation(hemipelvectomy) probably offers the

most straightforward option for localdisease control in a patient referredwith a diagnosis of sarcoma found atthe time of reconstructive surgery.However, after extensive manipula-tion of a tumour in the proximal fe-mur, even hemipelvectomy may notremove all contaminated tissues.

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nels du traitement.RÉSULTATS : Deux patients (un qui n’a reçu qu’une chimiothérapie palliative) sont morts après 5 et 21mois de suivi respectivement. Le suivi moyen chez les quatre autres patients s’est établi à 65,2 mois(fourchette de 51 à 75 mois). Ils étaient libres de toute maladie au dernier suivi. Un patient a dû subir uneamputation à cause de complications septiques liées à la reconstruction.CONCLUSIONS : L’intervention chirurgicale de conservation du membre en cas de sarcome de la face proxi-male du fémur représente un défi lorsque le diagnostic est posé au moment de l’intervention chirurgicale dereconstruction plutôt qu’au moyen d’une biopsie planifiée comme il se doit. Cette série indique toutefoisqu’il est possible de préserver le membre et que l’amputation trans-pelvienne n’est pas la seule solution.

Table I

Chondrosarcoma2º (grade 2/3)

Osteosarcoma(grade 3/3)


Dedifferentiatedchondrosarcoma

Chondrosarcoma(grade 2/3)


Final diagnosis

Summary of Diagnosis, Treatments and Final Outcome in 6 Patients Who Had Bone Sarcoma Diagnosed at the Time of Reconstructive Surgery

Resection of pelvictumour, proximalfemur, reconstructionwith hemipelvicallograft, proximalfemoral allograftimplant

Neoadjuvantchemotherapy, wideresection of proximalfemur, wide resection ofpelvis, reconstruction ofpelvis and proximalfemur with allograftimplant

Patientno.

Palliative chemotherapy

Neoadjuvantchemotherapy: 3courses of cisplatin/doxorubicin followed byresection of entirefemur, total femoraltumour prosthesis

1

Resection of proximalfemur, proximal femoraltumour prosthesis

Neoadjuvantchemotherapy,resection of proximalfemur with allograftimplant

2

Final treatment

3

4

Amputation forsepsis causedby bowelperforation 61mo postop.

Disease free,transient sciaticnerve palsy

5

Died ofdisseminateddisease

Died ofdisseminateddisease

Disease free,functioningwell, walkingwith 1 cane

Disease free,functioningwell, walkingunassisted

Outcome

64

75

4

21

51

71

Follow-up, mo

F/40

M/51

M/60

F/74

M/35

Sex/age,yr

AVN of righthip

AVN of left hip

Pathologicsubtrochantericfracture of rightfemur

Enchondromaof left proximalfemur

GCT rightproximal femur

Initialdiagnosis

Hip screw and platefollowed byattempted THA

Left THA

Reconstruction withnailing

Curettage and bonegrafting with pinand plate fixation

Curettage and bonegrafting

Initial treatment

6 F/61 Loose left THA Acetabular revision

GCT = giant cell tumour, AVN = avascular necrosis, THA = total hip arthroplasty

In this paper we present a consecu-tive series of 6 patients referred to atertiary care musculoskeletal oncologyunit after a primary bone sarcoma hadbeen discovered during reconstructivehip surgery. We describe the initial er-rors made in diagnosis of the bone sar-coma and the principles of subsequentmanagement.

PATIENTS AND METHOD

Six consecutive patients ranging inage from 35 to 74 years (average 54years) were referred to our institutionafter having undergone inappropriatereconstructive surgery on the hip forsuspected benign disease (5 patients)or metastatic carcinoma (1 patient),which eventually turned out to bebone sarcoma. The initial diagnosiswas benign tumour in 2 patients, sub-trochanteric fracture secondary tometastatic carcinoma in 1 patient,avascular necrosis of the femoral headin 2 patients and granuloma due to a

loose total hip arthroplasty in 1 pa-tient (Table I).The final diagnosis was osteosar-

coma in 3 patients and chrondrosar-coma in 3. We reviewed the charts ofthese patients to determine what stepscould have been taken to avoid the ini-tial inappropriate surgery, what subse-quent therapy they received after refer-ral to the oncology unit and what werethe oncologic and functional results oftreatment. The duration of follow-upranged from 4 to 71 months.

RESULTS

Two patterns of inappropriate man-agement were recognized in the initialcare of these patients: (a) the surgeonrecognized that there was a tumourpresent in the proximal femur but didnot establish the appropriate histologicdiagnosis; (b) the surgeon failed to re -cognize that a tumour was present.In 3 patients the surgeon identified

a tumour using plain radiographs and

cross-sectional imaging. In none ofthese 3 was a biopsy specimen orfrozen section obtained before pro-ceeding with definitive management.In 2 patients (nos. 1 and 2 in Table I)the surgeon decided that the lesionwas benign (giant cell tumour and en-chondroma) without benefit of anypathological evaluation (Fig. 1). These2 patients underwent curettage andbone grafting of the “benign” tumour;in one of them (no. 2) a hip screw andside plate were also inserted (Fig. 2).In a third patient (no. 3), the initialsurgeon assumed that a pathologicsubtrochanteric fracture of the proxi-mal femur was due to metastatic carci-noma and performed closed reduction,inserting a locking nail without obtain-ing a biopsy specimen for diagnosis(Fig. 3). Several days later the tumourwas recognized as a de-differentiatedchondrosarcoma. In none of these 3patients was a frozen section obtainedearly in the operation. The diagnosisof sarcoma was recognized only from

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FIG. 1. Case 1. Anteroposterior (left) and lateral (right) conventional radiographs of the right hip illustrate a lytic lesion within the anteroinferior aspect of the rightfemoral head, with extension into the anterior aspect of the femoral neck, thought to be a benign giant cell tumour. On histologic examination after curettage andautografting, the mass was found to be an osteosarcoma.

the permanent specimens reportedseveral days after the initial procedurehad been completed.In the remaining 3 patients, the sur-

geon mistakenly diagnosed avascularnecrosis preoperatively in 2 patients(nos. 4 and 5). In both, plain radi-ographs initially showed mottled scle-rosis and lucency in the femoral headwithout subchondral crescentic lu-cency or joint collapse (Fig. 4). Nu-clear medicine bone scanning demon-strated marked increased uptake.Neither patient underwent magneticresonance imaging before the total hiparthroplasty. In patient 4, the sarcomawas not recognized on review of thepathological appearance of the re-

sected femoral head. The diagnosiswas made 2 months later after re- exploration of the hip for presumedsepsis (Fig. 5). By this time the tumourwas unresectable even by amputationand the patient died rapidly frommetastasis. In patient 5 sarcomatoustissue was recognized intraoperativelyafter removal of the femoral head andreaming of the acetabulum (Fig. 6).In the final patient in this series (pa-

tient 6), a large mass developed in thesoft tissues surrounding a hip with aloose total joint replacement (Fig. 7).This was assumed to be a granulomarelated to implant loosening. At thetime of revision, the acetabular com-ponent was found to be loose, and ex-

tensive membrane was removed fromthe site. Within 3 months the massreappeared and grew (Fig. 8). Biopsydemonstrated chondrosarcoma.The final diagnoses and presenting

symptoms are summarized in Table I.After initial consultation, each patientwas advised to undergo hindquarteramputation to achieve the best chancefor local disease control. Every patientrejected this advice.After review of the pathological find-

ings, all patients with high-grade sarco-mas were advised to receive neoadju-vant (i.e., preoperative) chemotherapy,which was subsequently given to 2 pa-tients with osteosarcoma (patients 1and 5) and 1 patient with de-differenti-ated chondrosarcoma (patient 3). Thechemotherapy consisted of cisplatin anddoxorubicin.4–9 In patient 4 (with unre-sectable osteosarcoma) the disease pro-gressed despite chemotherapy and nosurgery was performed.The definitive surgical manage-

ment of patients with a sarcoma whohave undergone prior surgery involvesthe removal of all tumour and poten-tially contaminated tissue at the localsite.1 The patients in this series re-quired extensive resection in order toachieve this goal since their initial re-constructive procedures violated anumber of tissue planes and involvedacetabular reaming in 2 patients (nos.5 and 6). In these patients, most ofthe musculature about the hip andthigh required resection, as well as anyneurovascular structures that werecontaminated with tumour.Staging studies of the local site of

tumour origin were difficult to inter-pret in 4 of the 6 patients since metal-lic devices had been inserted in thehip. Despite these limitations, bothMRI and CT provided useful informa-tion (Figs. 5 and 8). Pathological re-view of the resected tumours revealedresection margins that were free ofmalignant cells in every case.

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FIG. 2. Case 2. A plain radiograph of the left hipillustrates fracture of a prosthesis in a patienttreated by curettage, autografting and screw/plate fixation for what was thought to be an en-chondroma. The lesion was found to be a chon-drosarcoma (grade 2/3).

FIG. 3. Case 3. An anteroposterior plain radi-ograph of the right hip after closed reduction andinsertion of an intramedullary locking nail forwhat was presumed to be a pathologic fracturethrough metastatic carcinoma. The lesion wasfound to be a dedifferentiated chondrosarcoma.

In 2 patients, the pelvis was conta-minated with tumour; this occurred in1 patient from tumour extension andin the other from acetabular reamingat the time of attempted total hiparthroplasty (Fig. 6). These 2 patientsrequired en bloc pelvic and femoral re-section. Reconstruction was achievedusing hemipelvic and proximal femoralallografts with a long-stemmed totalhip arthroplasty (Figs. 9 and 10).10,11

The remaining 3 patients had prox-imal femoral tumours without acetab-ular extension. They underwent wideresection of their tumours with recon-struction of the femoral defect usingeither an allograft-implant (patient 1)or a modular endoprosthesis (patients2 and 3) (Figs. 11 and 12).Two patients died of disseminated

disease during the follow-up period;the one who received palliativechemotherapy only and the one witha dedifferentiated chondrosarcomawho had a total femoral replacement.The average follow-up for the re-

maining 4 patients was 65.2 months(range from 51 to 75 months). Onepatient with osteosarcoma had a soli-tary pulmonary metastasis resected 24months postoperatively.12 She was freeof disease, both systemically and locally,75 months postoperatively. There wereno local tumour recurrences.

Of the 4 patients who underwentlimb salvage and were alive at follow-up,3 were functioning well. Two patients(nos. 2 and 5) were walking with a sin-gle cane and 1 patient (no. 1) was walk-ing with no assistance. All 3 were per-forming activities of daily living withoutdifficulty. Two of the 3 patients whowere working preoperatively had re-turned to work at the time of follow-up.One patient (no. 6) suffered a

spontaneous bowel perforation 8months after pelvic allograft replace-ment. Subsequently, chronic disloca-tion of her prosthesis developed andeventually she required hindquarteramputation 5 years postoperatively forrecurrent abdominal sepsis associatedwith her pelvic allograft.

DISCUSSION

The topic of unrecognized primarymalignant bone tumour discovered atthe time of reconstructive hip surgeryhas not been discussed in the litera-ture. Cases of sarcoma arising from atotal hip arthroplasty or a metallic im-plant have been reported.13–17 It is pos-sible that in some of these patients (es-pecially those in which the periodbetween the hip arthroplasty and dis-covery of the tumour was short) thetumour was present and missed at the

time of initial arthroplasty.The diagnosis of a primary bone

tumour can be difficult,1,2 and cer-tainly the appropriate diagnosisshould be sought by a consultativeprocess involving the pathologist, ra-diologist and orthopedic surgeon. Inthe patients we have presented, theprocess of diagnosis failed because ei-ther the surgeon recognized a proxi-mal femoral tumour but thought thatthe lesion was not a primary sarcomaor the surgeon failed to recognize thepresence of a tumour.Mankin, Lange and Spanier3 have

discussed the hazards of taking abiopsy in patients with primary bonetumours. For patients with these raretumours who are treated by non- experts, the risks of inappropriate di-agnosis or management are high. Thepresent series emphasizes a furtherdanger: definitive treatment of a bonetumour before a definitive diagnosishas been made.In this review, 2 patients were

treated for presumed benign lesions ofthe femoral neck. The risk of fractureafter biopsy of a femoral neck lesion iscertainly worrisome, and reconstruc-tion of the proximal femur at the timeof biopsy may be warranted if a definitediagnosis of benign tumour is obtainedfrom a frozen section of the biopsy

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FIG. 4. Case 4. Plain radiographs showing mottled sclerosis and lucency in the left femoral head without a subchondral crescentic lucency or joint collapse.

sample. However, a definitive diagno-sis may be difficult to achieve with im-mediate frozen sections, and it is criti-cal that expert pathologists, radiologistsand clinicians agree with all aspects ofthe diagnosis before the patient re-ceives further treatment. We thereforeadvise that these patients be referred toa centre with subspecialty expertise inmusculoskeletal pathology before thebiopsy is undertaken. We recognizethat bone biopsy at most skeletal sitesis performed at primary or secondarycare hospitals in Canada. However, thedifficulty in planning and interpretingprimary tumour bone biopsies from theproximal femur is such that these pa-tients warrant referral.In this review, patients 1 and 2

were treated with curettage and bonegrafting (as well as insertion of fixationdevices in patient 2). The treatment

would likely have been easier and theoutcome better had the initial surgeondelayed definitive management andperformed only a biopsy.Patient 3 demonstrates the error of

assuming that all pathologic fracturesin adults are due to metastatic cancer.This patient presented with a sub-trochanteric fracture through an obvi-ous lytic lesion. Although there wasno history of tumour elsewhere, thesurgeon assumed that this lesion mustbe metastatic from another site andproceeded to fixate the fracture withan intramedullary device therebyspreading tumour from the femoralhead to the knee and extensively con-taminating the abductor muscles. To-tal femoral resection was required toremove the sarcoma. In older patientspresenting with a solitary pathologicfracture the surgeon should always

search for evidence (by history, physi-cal examination and appropriate inves-tigations [i.e., total body bone scan-ning]) of a primary tumour that mayhave metastasized to the bone. Evenif a primary cancer is identified, thesurgeon should proceed with cautionand ensure that a pathologist is pre-sent to interpret a frozen section at thetime of biopsy before proceeding withdefinitive fixation. If the frozen - section findings are inconclusive thepatient should be placed in tractionuntil a reliable diagnosis is made.Reaming the fracture site in order topass an intramedullary device is partic-

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FIG. 6. Case 5. Postoperative radiograph of theleft hip after removal of the femoral head andreaming of the acetabulum. Osteosarcoma wasrecognized intraoperatively during attemptedtotal hip arthroplasty for presumed avascularnecrosis. Old screw tracts are seen within theproximal femur from initial treatment with a hipscrew and plate.

FIG. 5. Case 4. Coronal T1-weighted magnetic resonance image of the pelvis 4 months after total hiparthroplasty was performed for avascular necrosis. The implant was removed 2 months after the ini-tial procedure and an osteosarcoma was identified. A large extraosseous mass of low T1-weightedsignal intensity is seen centred over the region of the left hip (arrow). Additionally, loss of normal T1-weighted marrow fat signal is seen within the residual proximal femur, presumably secondary tomarrow edema or intramedullary tumour extension. The patient was given palliative chemotherapyand soon died from systemic metastasis.

ularly contraindicated if the lesion is asarcoma since tumour implants mayembolize into the venous circulation.These misdiagnosed cases of proxi-

mal femoral lesions suggest that refer-ral to a subspecialty unit was indicatedbefore biopsy. Subspecialty manage-ment is not necessary for most patientswith metastatic disease in the proximalfemur. However, those who have soli-tary lesions (especially if there is nohistory of malignant disease else-where) should probably be referredbefore a biopsy is done.In the first 3 cases, recognition of

the tumour could have prompted re-ferral to a surgical oncologist. How-ever, in the latter 3 cases, patientswere thought to have conditions thatare normally managed by reconstruc-tive surgeons. The 2 patients thoughtto have avascular necrosis demon-strated mottled lucency and sclerosisof the femoral head on plain radi-ographs (Fig. 4), but in neither casewas the localized subchondral crescentsign or joint collapse typical of ad-vanced osteonecrosis demonstrated.

To avoid this management error, pa-tients with symptomatic osteonecrosiswho do not show typical changesshould be assessed through MRI.18 Ifthe image obtained is not typical ofosteonecrosis, biopsy should precedeany reconstructive procedure.A similar situation applies in patients

with soft-tissue masses or dramatic bonelysis associated with an implant. Al-though the diagnosis in most of thesecases will be granulomatous pseudotu-mours caused by wear debris from theimplant, the surgeon should not neglectthe possibility of a sarcoma. Before anydefinitive treatment, a biopsy of themass should be taken, and materialfrom cystic lesions should always be sentfor pathological evaluation.Radical surgical treatment of all

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FIG. 8. Case 6. Computed tomography scan through the proximal thighs performed 3 months afteracetabular revision. Despite the scatter from the femoral prosthesis, a large soft-tissue mass can beclearly seen along the medial and posterior aspect of the right femoral diaphysis. Irregular serpigi-nous calcification is seen within the posterolateral aspect of the mass.

FIG. 7. Case 6. A conventional anteroposteriorradiograph of the right hip obtained before ac-etabular revision of the patient’s total hiparthroplasty. A large soft-tissue mass is seenmedial to the proximal femur. The tissue patho-logical findings were not reviewed at the time ofsurgery.

FIG. 9. Case 5. After neoadjuvant chemotherapy,the proximal femur and a portion of the pelviswere resected and reconstructed with an allo-graft implant. This radiograph of the hip was ob-tained 5 years after the reconstruction.

6 cases would have entailed a hind -quarter amputation,1,19 since extensivespread of the tumour around the hiphad resulted from the initial proce-dure. The extent of tissue contamina-tion was difficult to assess in the pa-tients with implanted metallic devicesbecause of inherent imaging artifactsencountered on cross-sectional imag-ing. Generally it was necessary to dis-cuss personally with the surgeon theinitial surgical approach in order toplan the extent of tissue resection nec-essary to achieve local tumour control.In most patients it was necessary to ex-cise critical muscular structuresaround the hip (abductors and hipflexors) that had been contaminatedat the initial operation. In 3 patients,

contamination of the anterior pelvisalso required femoral nerve resectionalong with the hip flexors.After an initial assessment, all pa-

tients were advised to undergo ampu-tation at the level of the sacroiliacjoint to achieve the highest chance oflocal disease control. All patients re-jected this advice in favour of the limbsalvage option even though they rec-ognized the higher risk of local tu-mour recurrence. All patients withhigh-grade bone sarcomas weretreated with adjuvant chemotherapybefore their eventual resection. Limb-salvage surgery with adequate tu-mour-free margins was possible in 5of the 6 patients in this series and thesixth patient could not have beenmanaged adequately even with ahindquarter amputation. There wereno local relapses after a minimum fol-low-up of 2 years in the 4 survivingpatients. These cases represent ex-treme examples of limb salvagesurgery, but there is no question thatthey are functionally better than they

would have been with a hindquarteramputation.5,6,9,20–24

CONCLUSIONS

It would be unwise to draw defini-tive conclusions about the course oftreatment in this small series of pa-tients. However, it is fair to commentthat a biopsy should be done in casesof presumed benign lesions of theproximal femur before definitive treat-ment and in cases of solitary bone le-sions in which the tumour is assumedto be metastatic. Cases of avascularnecrosis should be investigated preop-eratively by MRI if subchondral cres-centic lucency and femoral head col-lapse are not evident on conventionalradiographs, and “pseudotumours”presumed to arise from implant weardebris should be viewed with suspi-cion if they are growing rapidly. Thesurgeon should obviously avoid thesituation of discovering a sarcomaduring or after performing a recon-structive procedure. However, the re-

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FIG. 10. Case 6. Radiographs of the proximal right femur (left) and pelvis (right) after hemipelvic and proximal femoral resection and reconstruction witha hemipelvic allograft and proximal femoral allograft implant.

sults in this small series of patientssuggest that hemipelvectomy is notthe only solution when this occurs andthat limb salvage surgery should beconsidered if the patient refuses am-putation.

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FIG. 12. Case 2. A radiograph of the left hip afterlimb salvage surgery shows resection of theproximal femur and reconstruction with a proxi-mal femoral tumour prosthesis.

FIG. 11. Case 1. Anteroposterior view of the righthip after neoadjuvant chemotherapy and limbsalvage surgery shows resection of the proximalfemur and reconstruction with a composite allo-graft implant.

18. Lang P, Genant HK, Jergesen HE,Murray WR. Imaging of the hip joint.Computed tomography versus mag-netic resonance imaging. Clin Orthop1992;274:135-53.

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SESAP Critique / Critique SESAP

ITEM 482Most thymomas are benign. In a collection of 15 series, the rate of malignancy averaged 33%.When muscular weakness (myasthenia gravis) occurs in conjunction with thymoma, the cause is a reduction in

acetylcholine receptors at the neuromuscular junction. Antibodies to the acetylcholine receptors are found in morethan 85% of patients with myasthenia gravis associated with thymoma and it is believed that there is thymic involve-ment in this autoimmune response to the acetylcholine receptors. In addition to myasthenia gravis, many other syn-dromes are associated with thymoma, including connective tissue diseases, blood disorders, and Cushing’s syn-drome. When Cushing’s syndrome is seen, it is secondary to an APUD or “carcinoid” tumor of the thymus thatproduces corticotropin. The other disorders associated with thymic tumors are secondary to autoimmune processes.Any patient with a thymic tumor should undergo operation to establish a diagnosis, to resect a potential malig-

nant tumor, and possibly to palliate myasthenia gravis if it is present. In addition to thymoma, carcinoid, lymphoma,teratoma, and squamous cell cancer can originate in the thymus. Medical therapy, which is moderately successful inalleviating the symptoms of muscle weakness in myasthenia gravis, is aimed at amplifying the muscle response toacetylcholine by using anticholinesterase drugs such as pyridostigmine and neostigmine and in using immunosup-pressive drugs to suppress autoimmune effects. Thymectomy provides long-term improvement in symptoms ofmyasthenia gravis in 57% to 86% of patients with myasthenia gravis associated with or without thymoma. However,improvement after thymectomy may take several years.

B

References482/1. Orringer MB: Chest wall, mediastinum, and pleura, in Greenfield LJ (ed): Surgery: Scientific Principlesand Practice. Philadelphia, JB Lippincott Co, 1993, pp 1332-1333482/2. Papatestas AE, Genkins G, Kornfeld P, et al: Effects of thymectomy in myasthenia gravis. Ann Surg206:79-88, 1987482/3. Rosenow EC, Hurley BT: Disorders of the thymus: A review. Arch Intern Med 144:763-770, 1984482/4. Verley JM, Hollman KH: Thymoma: A comparative study of clinical stages, histologic features, and sur-vival in 200 cases. Cancer 55:1074-1086, 1985

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