Malignant transformation of fibrous dysplasia to chondrosarcoma is rare. We report a case in which malignancy developed in an area of fibrous dysplasia.
CHONDROSARCOMA A CASE
IN
FIBROUS
REPORT
AND
MAHER
REVIEW
HALAWA,
From
Mount
DYSPLASIA OF
AHMAD
Gould
THE
ABDEL
Hospital,
OF
THE
PELVIS
LITERATURE AZIZ
Plymouth
Malignant transformation of fibrous dysplasia to chondrosarcoma is rare. We report a case in which malignancy developed in an area of fibrous dysplasia in the ilium. We believe this to be the second reported case at this site. Treatment was by excision of the hemipelvis including the ala of the sacrum. A review of the literature is presented. Malignant change in fibrous dysplasia is rare. It has been estimated that it occurs in less than 1% of cases (Immenkamp 1975). The most common malignant tumour is osteosarcoma followed by fibrosarcoma and then chondrosarcoma. We report a case of chondrosarcoma arising in an area of fibrous dysplasia in the left iliac bone. The only other lesion of fibrous dysplasia in this patient was in the upper end of the left femur. Treatment was by partial resection of the left innominate bone and part of the left ala of the sacrum. CASE
REPORT
A 10-year-old boy presented in 1958 with pain in the left knee. The pain seemed to be referred from the hip, and radiographs showed fibrous dysplasia involving the left iliac bone and the proximal metaphysis of the left femur. This diagnosis was confirmed by biopsy of the femoral lesion (Fig. 1), which was bone grafted three weeks later. Two years later the patient fell at school, sustaining a fracture of the shaft of the femur at the lower limit of the lesion. This fracture was treated by Kuntscher nailing, and six months later more bone graft was placed around the nail. The nail was removed two years after this and replaced by a Capener nail-plate. The femoral lesion eventually consolidated (Fig. 2), though with coxa vara and 11 inches of shortening. Review was continued until 1971, when the 23-year-old patient was discharged from follow-up. Review of the radiographs from 1958 to 1971 revealed no significant change in the appearance of the fibrous dysplasia in the ilium. In 1983, at the age of 35 years, the patient again developed pain in the left knee, and his general
M.
Halawa,
paedic
MChOrth,
FRCS,
FRCSEd
(Orth),
Consultant
practitioner discovered a pelvic mass. This mass was huge but painless, occupying the iliac fossa, the gluteal region and the loin on the left side. It was hard, not tender, and fixed to the pelvis. The skin over the mass was mobile and showed a few dilated veins. The femoral artery could not be felt in the groin because of the encroaching tumour. There was no gap between the costal margin and the tumour mass. The left hip joint had painfree flexion to 1 10#{176} but abduction was limited to 10#{176}. The left knee had a full painless range of movement. There were no abnormal neurological signs in the leg, and he had full power of the muscles of the left knee, ankle and foot. The dorsalis pedis and the posterior tibial pulses were present. A radiograph is shown in Figure 3 ; a chest radiograph was clear. CT scans (Figs 4 to 10) showed that the lesion did not involve the floor of the acetabulum, but that the left ala of the sacrum was affected ; this was thought to be involvement by fibrous dysplasia and not by tumour
OrthoFig. 1
Surgeon
Mount
Gould Hospital, Plymouth PL4 7QD, England. A. A. Aziz, MScOrth, MD, Lecturer in Orthopaedic Surgery Cairo University Hospital, Cairo, Egypt. Requests for reprints should be sent to Mr M. Halawa.
pathologist’s
© 1984 British
very
030l-620X/84/5133
760
Editorial Society $2.00
of Bone and Joint
Surgery
Micrograph
of the original report
stated
biopsy
of the upper
: “The
lesion
left femur
is a loose,
partly
in 1958. The fibrous
and
partly vascular mass and the vessels are mainly lymphatic. In one fragment of tissue there is osteoblastic activity of the kind seen in fibrous dysplasia. There is one small island of cartilage, but it could well
be reactive.
fibrous
It is an uncommon
dysplasia
THE
but
for cartilage
JOURNAL
well-recognised
variant
to be present.”
OF BONE
AND
JOINT
SURGERY
of
CHONDROSARCOMA
Fig.
Figure
2-Fibrous
dysplasia
of the
IN
VOL.
66-B,
showing
No. 5, NOVEMBER
OF
THE
761
PELVIS
Fig.
ilium and the upper femur. Figure 3-Chondrosarcomatous
The femur has consolidated after change of the lesion in the ilium.
Fig. 5
Fig. 7 scans
DYSPLASIA
2
Fig. 4
CT
FIBROUS
extent
1984
of the lesion,
the
lack
Fig. 9 of involvement
nailing
and
bone
Fig. 6
Fig. 8 the
3
biopsy,
of the
acetabulum
Fig. and
the
changes
in the ala of the
10 sacrum.
grafting.
762
M.
HALAWA,
tissue. The diagnosis of chondrosarcoma was based on the characteristic radiographic appearance, and it was decided to perform a left hemipelvectomy, including the left ala of the sacrum, but preserving the left lower limb. Operation. The patient was placed on his right side on a table which was broken as for a kidney operation to create a gap between the tumour mass and the costa! margin. An inverted U-incision, started upwards from the mid-inguinal point, curved above the swelling, then back to the paraspinal muscles and ended over the back of the sacrum. The tumour was then cleared on all its aspects except the medial one. The sciatic nerve was exposed and the short rotator and abductor muscles cut to allow dislocation of the hip. The obturator foramen and the obturator nerve were exposed below the acetabulum and the superior pubic ramus was divided with a saw. The ischium could then be divided at the lesser sciatic notch, taking care to avoid injury to the pudendal nerve and vessels. The dissection of the nerve roots from within the sacrum was difficult. The posterior sacral foramina were
A.
A.
AZIZ
exposed and the bars between the foramina were excised using rongeurs. The superior surface of the ala of the sacrum was divided while protecting the lumbosacral nerve trunk. With the anterior primary rami of the sacral nerves exposed, the transverse bars between the sacral foramina were divided anteriorly and the mass excised in toto. Biopsy specimens from the remaining sacrum were taken for histological examination, though macroscopically it was clear that the tumour did not involve this bone. At the end of the operation the femoral nerve, the sciatic nerve, the lateral cutaneous nerve of the thigh, the obturator nerve and the femoral vessels were seen to be intact, but it was discovered that the pudendal nerve had been divided. The removal ofthe mass left a large defect in relation to the abdominal wall, particularly after excision of the glutea! muscles. The defects were partially closed by suturing the abdominal muscles to the paraspinal muscles and to the adductors and flexors of the hip. The hip was found to be completely flail, but the head of the femur could not be pushed above the level of the sacrum. After operation the leg was supported by pillows. A lateral popliteal nerve paresis eventually recovered and the wound healed satisfactorily after some delay. After six weeks of bed rest, the patient sat out and had good knee control. About two months after operation he stood and began to put some weight on the leg, using his original shoe-raise and a pair of crutches. At first the leg was flail but the hip gradually became more stable and could eventually take considerable weight. Walking with crutches is now satisfactory. The postoperative radiograph shows the extent of the resection (Fig. 1 1). Histology of the tumour (Fig. 12) was reported to show a low-grade chondrosarcoma. DISCUSSION
Fig.
Radiograph
of the
pelvis
after
Fig.
Histological section sarcoma oflow-grade
11
operation resection.
to show
the extent
of the
12
of the tumour. This was reported as a chondrohistological type, composed ofcalcifying cartilage, much of which is mature.
Malignant transformation in fibrous dysplasia is rare; only 69 cases have been reported. This total includes 37 cases of osteosarcoma, 21 of fibrosarcoma and 1 1 of chondrosarcoma. The references for these cases are listed in Table I. In these collected cases the average age at the diagnosis of malignant change to osteosarcoma was 33 years, to fibrosarcoma 37 years and to chondrosarcoma 30 years. There was no sex predominance, with 33 men and 36 women. The sites at which malignant change occurred are listed in Table II. Of the reported cases, 38 occurred in patients with polyostotic fibrous dysplasia and 29 in monostotic disease, while the status of the other two cases is not clear from the references. Twenty-one patients had received radiotherapy as treatment for fibrous dysplasia. In the group developing osteosarcoma 24 of the 37 patients had polyostotic fibrous dysplasia ; 15 had received radiotherapy, 13 of whom had polyostotic disease. These figures suggest an increased probability for malignant transformation to osteosarcoma after the treatment of po!yostotic fibrous dysplasia by radiation. THE
JOURNAL
OF BONE
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JOINT
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CHONDROSARCOMA Table
I. Cases
listed
IN FIBROUS
Table fibrous
Anastovos et a/. 1977 Bell and Hinds 1967 Dc Marchi 1956
and Ley 1950 et a!. 1962
Harris Huvoseta/. Jager 1962 Kiehn
1972
et a/. 1961
Perkinson and Higinbotham Pons et a!. 1974 Prake et a/. 1968 Riddell 1964 Rozeeta/. 1967 Sabanas et a/. 1956 Schwartz and Alpert 1964 Sethi et a!. 1962 Slow
1955
1971
1956
Gimeseta!. 1970 Gross and Montgomery 1967 Hall et a!. 1955 Harris et a!. 1962 Hobbs et a/. 1956 Immenkamp 1975 Johnson et a/. 1979 Mogensen 1958 Pons et a/. 1974 Schwartz and Alpert 1964 Tanner et a!. 1961 Turbinkovv and Skoblin 1958 Wanke 1927
Anastovos
1972
Heilner 1953 Huvoseta/. 1972 Jaffe 1958 Unni and Dahlin 1979
metatarsus. and
Dahlin
(1979)
and
lead
to a mistaken
diagnosis
Femur
22
Maxilla
18
Tibia
8
Pelvis
3
Humerus
4
Mandible
4
Ribs
4
Pelzmann,
Nagel
and
be large amounts dysplasia without This could well
of malignancy.
Neoplastic
change in areas of fibrous dysplasia has the same prognosis as the same malignancy arising elsewhere, and should have the same treatment. Partial or complete resection of the hemipelvis is a less mutilating procedure than hindquarter amputation, avoiding the problems of a hindquarter prosthesis and
histological
sections.
We
also
wish
to thank
1
Ulna
1
Scapula
1
Mrs
bone
1
C. Postle-Hacon
I
the psychological trauma of losing one quarter of the body. Enneking (1966) and Enneking and Dunham (1978) described partial and complete resections of the innominate bone for malignancy ; they believe that involvementofthe sciatic nerve is not a contra-indication, since the resection can include this nerve. Steel (1978) reported five such resections on patients with chondrosarcoma involving different parts of the innominate bone. None of his patients had local recurrence or metastasis after follow-up for three to six years. It is vital to know the exact extent of the lesion before such an operation. Computerised tomography is effective and accurate in this respect. Involvement of the entire hemipelvis does not contra-indicate excision, though this becomes more difficult (Steel 1978). In the case we report, resection of the ala of the sacrum was necessary, with care to preserve the sciatic nerve. A biopsy from the retained part of the sacrum was free from involvement. Sweetnam (1983) used the term hemipelvectomy to describe excision ofthe innominate bone while preserving the lower limb. He confirmed that the operation is no less radical
than
hindquarter
amputation
proximal level of bone division, be at or, as in our case, beyond
for typing
the
Hospital, Royal
in
1984
terms
of
since bone resection the sacro-iliac joint.
the
can
Professor P. Byers, Professor of Morbid Devon and Exeter Hospital, for reporting
manuscript.
References.on
VOL. 66-B, No. 5, NOVEMBER
in
1
Fibula
The authors would like to thank Dr Roger Drury, Consultant Pathologist, Plymouth General Anatomy, Royal National Orthopaedic Hospital and Dr Peter Antony, Consultant Pathologist, on the
bones
Metatarsus
The group developing fibrosarcoma included 21 patients, 12 with monostotic fibrous dysplasia, 8 with polyostotic disease and one of unreported type. Only five patients had received radiotherapy and three of these originally had monostotic involvement. Of 1 1 patients in the chondrosarcoma group, six had polyostotic disease, four had monostotic involvement and one type was not reported. Only one patient in this group had received radiotherapy (Feintuch 1973). The bones reported to be involved by chondrosarcomatous transformation were the femur in four cases, the pelvis in four (ilium two, ischium one and pubis one), and one case each involving the humerus, the maxilla, and the Unni
change
(n=69)
Frontal
1973
Salyer (1980) reported that there may of atypical cartilage in areas of fibrous definite chondrosarcomatous change.
malignant
et a!. 1977
Bejui-Thivolet et a!. 1982 Cabitza 1951 Dabska and Buraczewski Dc Smet et a!. 1981 Feintuch
of
Site
Orbital
Chondrosarcoma
Sutro 1951 Tannereta/. 1961 Vakhurkina 1958 Van Horn et a/. 1963 Yannopoulos et a/. 1964
II. Sites dysplasia
Cases
Fibrosarcoma
Abelanet et a/. 1974 Balcells-Gorina et a!. 1963 Belloni and Zanetti 1949 Coley and Stewart 1945 Dabska and Buraczewski 1972
Euler
763
OF THE PELVIS
in the literature
Osteosarcoma
Dustin
DYSPLASIA
the next
page
764
M. HALAWA,
A. A. AZIZ
REFERENCES Abelanet
R, Forest
revue
M, Meary
R, Languepin
de Ia litt#{233}rature. Bu//
Anastovos
K, Popov
Balcells-Gorina
K, Kostov
Cancer
(Paris)
P. Malignant
A, Bondia
B. Sarcomes :443-56.
degeneration M, Moreno
Garcia-Puente 1963;40: 7-11.
(Barce/ona)
A, Tomeno 1974;61
offibrous de Vega
Bejui-Thivolet F, Patricot LM, Vauzelle JL. Transformation Hop Paris 1982,58(21): 1329-35. Bell H, Hinds EC. Fibrosarcoma complicating polyostotic
Belloni Cabitza Coley
L, Zanetti E. Osteodisplasia A. Contributo allo studio BL,
Dabska De
Stewart
FW.
Bone
di Jaffe
sarcoma
R. Sulla transformazione 1956; 11:639-49.
fibrous
transformation
in fibrous
della
Ora/
dysplasia
Surg
dysplasia fibrosa
fibreuse
of bone.
1967 ; 23 : 299-3 Ric
Onco/ogy
59
Sci
forme
complexe
h#{233}mim#{233}lique et
: 92-6 sarcomatous
: a propos
1945 ; 121 : 872-8
monostotica
:
with
degeneration.
d’un cas, revue
Med
de la litterature.
C/in Sem
10. 1949;
di Jaffe-Lichtenstein).
Surg
d’une
1977
dysplasia
sarcomatosa.
(malattia Ann
(Mosk)
fibrous
sur dysplasie
ad evoluzione
dysplasia.
des os : a propos
Stomato/ogiia
dysplasia.
localizzate
fibrous
fibreuse
of polyostotic
sarcomateuse
fibrose
in polyostotic sarcomatosa
dysplasia.
V. A case
e Lichtenstein
delle osteodisplasie
J. On malignant
M, Buraczewski
Marchi Med
fibrosa
sur dysplasie
19:
Chir
13 17-23.
Organi
Mov
1951
;36:
8-24.
1. 26 : 369-83. di un caso a localizzazione
1972;
: a proposito
mandibolare.
Friu/i
AA, Travers H, NeffJR. Chondrosarcoma occurring in a patient with polyostotic fibrous dysplasia. Skeletal Radiology 1981 ;7 : 197-201. P Jr, Ley RA. Contribution a l’#{233}tude des dysplasies osseuses : description anatomo-clinique d’un cas d’ost#{233}osarcome polymorphe chez un enfant atteint de fibro-xanthomatose osseuse avec pr#{233}maturation sexuelle. Rev Be/g Pathol 1950 ; 20 : 52-72. Euler H. Zur Frage der Osteodystrophia fibrosa localisata. Dtsch Zahnaerzt/ Mund-und Kieferheik 1956 ; 24: 3-9. Enneking WF. Local resection of malignant lesions ofthe hip and pelvis. J Bone Joint Surg [Am] l966;48-A : 991-1007. Enneking WF, Dunham WK. Resection and reconstruction of primary neoplasms involving the innominate bone. J Bone Joint Surg [Am] 1978; 60-A : 73 1-46. Femtuch TA. Chondrosarcoma arising in a cartilaginous area of previously irradiated fibrous dysplasia. Cancer 1973 ; 31 : 877-81. Gimes B, Thaisz E, Feh#{233}rL. Beitrag zur malignen Entartung der fibr#{246}sen Knockendysplasie. Fortschr Geb Rontgenstr Nuk/earmed 1970; 113: De Smet
Dustin
211-5.
Gross
CW,
Montgomery
Hall
A, Bersack 1019-27.
Hams
WH, Dudley Joint Surg [Am]
Heilner
H. Die
WW.
SR,
Vitolo
Fibrous RE.
HR Jr, Barry 1962
Osteofibrosis
44-A
dysplasia
Fibrosarcoma Ri. The : 207-33.
deformansjuvenilis
natural
and
malignant
arising history und
ihre
degeneration.
in an apparently of fibrous
dysplasia
Differential-diagnose.
Arch
benign
Otolarvngol
fibrous
lesion
: an orthopaedic, Langenbecks
1967;85:653-7. of bone. J Bone pathological,
Arch
Chir
AA Jr, Fisher WC, Beck RE. Fibrous dysplasia ofthe skull with sarcoma. AJR 1956,76: 320-3. Huvos AG, Higinbotham NL, Miller TR. Bone sarcomas arising in fibrous dysplasia. J Bone Joint Surg Immenkamp M. Die Maligne Entartung bei fibr#{246}serDysplasie. Z Orthop 1975 ; 113 : 33 1-43.
and 1953
;277:
Joint
Surg
[Am]
roentgenographic
1955 study.
;37-A:
J Bone
160-89.
Hobbs
Jaffe HL. Tumours and J#{227}gerM. Osteoidsarkom
tumorous aufdem
conditions Boden
ofthe bones andjoints. einer fibros-polyostotischen
London : Kimpton, 1958. Dysplasie (Jaffe-Lichtenstein)
[Am]
ZentralblAl/g
1972
;54-A
: 1047-56.
PatholAnat
1962;
103:
291.
EF, Gottlieb LI. Malignant transformation of polyostotic fibrous dysplasia. South Med J 1979 ; 72 : 353-6. Kiehn CL, Des Prez JD, Harris AH. Fibrous dysplasia ofthe facial bones. Am J Surg 1961 ; 102: 835-8. Mogensen EF. Fibrous dysplasia of bone : report of an unusual case with endocrine disorders. Acta Med Scand 1958 ; 161 : 435-8. Pelzmann KS, Nagel DZ, Salyer WR. Case report I 14. Skeletal Radiology 1980;5 : 116-8. Perkinson NG, Higinbotham NL. Osteogenic sarcoma arising in polyostotic fibrous dysplasia : report of a case. Cancer 1955 :8: 396-402. Pons A, Arlet J, AIIbeIIi MJ, et al. Deux cas de d#{233}g#{233}n#{233}rescence maligne osseuse sur dysplasie fibreuse des os. J Radio! E/ectrol Med Nuci 1974;55:268-9. Pra#{235}ke T, Kreji J, Jirava E. K ot#{225}zce sarkomat#{244}zniho zurhnutu fibrozni kostni dysplazie. Acta Chir Orthop Traumatol Cech 1968 ;35 : 9-16. Riddell DM. Malignant change in fibrous dysplasia: report ofa case. JBoneJoint Surg[Br] 1964;46-B:25l-5. Roze R, Mazabraud A, Semet P. Dysplasie fibreuse des os et myxomes des tissus mous : d#{233}g#{233}n#{233}rescence sarcomateuse localis#{233}e. J Radio! Electrol
Johnson
Med
CB,
Gilbert
NucI
l967;48:
527-36.
Sabanas AO, Dahlia DC, Childs DS Jr, Ivins JC. Postradiation sarcoma of bone. Cancer 1956;9: 528-42. Schwartz DT, Alpert M. The malignant transformation of fibrous dysplasia. Am J Med Sci 1964; 247 : 1-20. Sethi RS, Climie ARW, Tuttle WM. Fibrous dysplasia of the rib with sarcomatous change. J Bone Joint Surg [Am] 1962 ;44-A : 183-8. Slow IN. Osteogenic sarcoma arising in preexisting fibrous dysplasia: report ofcase. J OralSurg 1971 ;29: 126-9. Steel HH. Partial or complete resection of the hemipelvis : an alternative to hindquarter amputation for periacetabular chondrosarcoma of the pelvis. J Bone Joint Surg [Am] 1978 ;60-A : 719-30. Sutro CJ. Osteogenic sarcoma of the tibia in a limb affected with fibrous dysplasia. Bull Hosp Joint Dis 1951 ; 12 : 2 17-28. Sweetnam R. Limb preservation in the treatment of bone tumours. Ann R Coil Surg Eng! 1983 ;65: 3-7. Tanner HC Jr, Dahlin DC, Childs DS Jr. Sarcoma complicating fibrous dysplasia : probable role of radiation therapy. Oral Surg 1961 ; 14: 837-46. Turbinkovv F, Skoblin AP. Operezdenii mestnoj fibroznoj osteodystrofic v sarkomu. Ortop Traumatol Protez 1958 ; 17 : 53. Unni KK, Dahlia DC. Premalignant tumors and conditions of bone. Am J Surg Pathol l979;3 : 47-60. Vakhurkina AM. Malignant osteoblastoclastoma developing on the background of fibrous osteodysplasia. Arch Path 1958 ;20: 18-24. Van Horn PE, Johnson EW, Dahlin DC. Fibrous dysplasia ofthe femur with sarcomatous change. Am J Orthop 1963;5: 165. Wanke R. Ostitis fibrosa und Sarkom. Dtsch Z Chir 1927 ; 201 : 358. Yannopoulos J, Bom AF, GrilhIthS CO, Crikelair GF. Osteosarcoma arising in fibrous dysplasia of the facial bones : case report and review of the literature. Am J Surg 1964; 107:556-64.
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