A RARE BONE TUMOUR OSTEOFIBROUS DYSPLASIA

1. Dr Ramachandra Reddy
Under the guidance of
Prof. A. Devadoss
Dr. Sathish Devadoss

2. 5yrs old boy
Informant : mother
Presented with c/o swelling in left leg distal
third region incidentally noticed by mother
2yrs ago
Swelling gradually progressive in nature
not a/w pain

3. No other swelling in body/sudden increase
in swelling.
No significant family history
No other treatment history
No h/o pain, night cries

4.  O/E:swelling present
over distal 3rd part of leg
lateral aspect
 Skin normal over
swelling
 No engorged veins
/deformity
 O/p:
 No local rise of
temperature/tenderness
 swelling fixed to bone
 No DNVD

5.  Immature skeletal bone
 It shows well defined
expansile lytic bone
lesion in diaphysis of tibia
and fibula
 No break in cortex
 No matrix calcification

6. Fibrous dysplasia
Osteofibrous dysplasia
Adamantinoma of long bones
Osteofibrous dysplasia like adamantinoma
Neurofibromatosis of long bones

7. Planned for en bloc excision of fibular and
tibial lesion with fibular bone graft for tibia

11. EXCISION OF TUMOUR AFTER EN BLOC EXCISION

12. AUTOGENOUS NON
VASCULAR FIBULAR BONE
GRAFT
GRAFT PLACEMENT

13. AUTOGENOUS FIBULA
GRAFT IN SITU
PERIOSTEUM CLOSURE

14. Gross exmn.:
 Well circumscribed
grey white mass of
size 6*4cm of distal
3rd fibula

15. Cut surface shows:
 Grey white mass
 Soft rubbery mass in
medullary canal of
fibula & tibia
 No hemorrhagic
lesion/cysts.

17. Wound inspection on post op day 2 with
a/k slab
Suture removal on 12th post op day and AK
cast for 1mth with non weight bearing
walking.
Further follow up yet to be awaited to see
radiological union of graft in tibia.

19. We diagonsed this condition as
osteofibrous dysplasia (a rare condition)

20. A rare lesion occurs exclusively in tibia &
fibula.
In 1976, Campanacci introduced the term
Defn: Is a disorder c/by presence of fibrous
tissue & bone trabeculae rimmed by
osteoblasts.
Predominantly affects infants & children.

21. Its incidence is reported to be 0.2% of all
primary bone tumours.
Typically occurs in first 2 decades of life
male preponderance.
The etiology is unknown.
Osteofibrous dysplasia has been
postulated to arise from a fibrovascular
abnormality.

22. Due to high incidence (60-100%) of
recurrence, any surgical procedure is
recommended only after skeletal maturity.
Surgical options include extra periosteal
resection, autologous graft, limb
lengthening procedures etc.

23. Sakamoto et al. found that GSα mutation
at the Arg201 was seen in fibrous
dysplasia but this did not occur in
Osteofibrous dysplasia, and they
concluded that this could be useful for
distinguishing between the two lesions.

24.  Histologically OFD is
characterized by a loose,
often storiform fibrous
background containing
spicules of woven bony
trabeculae that are lined
by a layer of osteoblasts.
 fibrous dysplasia typically
lacks the distinctive
osteoblastic rimming of
the bony trabeculae.

25. OSTEOFIBROUS
DYSPLASIA ADAMANTINOMA
 benign lesion composed
primarily of fibro-osseous
tissue.
 slow-growing, low-grade,
malignant biphasic tumor
with intermingled epithelial &
fibro-osseous components.
 aggressive tumor that is
locally invasive and can
metastasize.

26. Osteofibrous dysplasia–like adamantinoma
(a/k as differentiated or regressing
adamantinoma) is a benign lesion like
osteofibrous dysplasia but has features of
both osteofibrous dysplasia and
adamantinoma.
It may progress and become a malignant
adamantinoma.

29. As the lesion was in diaphyseal region of
tibia & fibula we suspected lesion to be
fibrous dysplasia/neurofibromatosis of long
bone so we did en bloc excision biopsy
and it turned out to be osteofibrous
dysplasia.
Waiting for further follow up

30. Thank you….
Happy doctors
day