CHONDROSARCOMA IN FIBROUS DYSPLASIA ... - Semantic Scholar

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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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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

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