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Textbook of Radiology and Imaging David Sutton

7th Edition, Vol.2, Section 5 . Chapter 40. Page 1287-1288

CHAPTER 40: TUMOURS AND TUMOUR-LIKE


CONDITIONS OF BONE (2)

DESMOPLASTIC
FIBROMA

Presented By : dr. Rizki Amallia


Consultant : dr. Amri Wicaksono Pribadi, Sp.Rad
Presentation Date : July 31 th, 2018
SECTION 5. SKELETAL SYSTEM : SOFT TISSUE

CHAPTER 40 : Tumours and


tumour-like condition of bone (2)
• Fibrous cortical defect
• Non-ossifying fibroma
• Desmoplastic fibroma…
• Fibrosarcoma
• Malignant fibrous histiocytoma
• Adamantinoma of long bone
INTRODUCTION
Known as : desmoid tumor of bone, osteofibroma, osseous
desmoplastic fibroma

Rare bone tumour, benign lesion, do not metastasis, but may be locally aggressive,
destructive growth, high reccurence rate after local resection

Exhibits dense fibrous tissue 2

A bony / intraosseus counterpart of soft tissue desmoid tumours, histologically


identical3

Can affect any bone. Majority arises in Metadiaphysis of long bone1 . Mandible, pelvis,
spine : other sites predilection 2,3

1. Taconis WK, Schutte HE, Heul RO. Desmoplastic fibroma of bone: a report of 18 cases. Skeletal Radiology. 1994: 23 ; 4 : 238-8. http://www.semanticscholar.org/paper/Desmoplastic-fibroma-of-bone%3A-a-report-
of-18-cases-Taconis-Schutte
2. Sutton, D. Textbook Of Radiology And Imaging. 7th Edition, Vol.2. London: Elsevier, 2003. P. 1287-1288
3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded from https://radiopaedia.org/articles/desmoplastic-fibroma (June 24th 2018)
EPIDEMIOLOGY

0.11 % incidence of all Occur at any age. Usually in


primary bone tumours4 young adults2 . Mean age at
0.3 % of benign bone presentation is 21, Regress
tumors. with increasing age 3

Rare childhood condition


with congenital multiple
fibromatosis (involvement There is no sex predilection3
muscle, heart & lung)2

2. Sutton, D. Textbook Of Radiology And Imaging. 7th Edition, Vol.2. London: Elsevier, 2003. P. 1287-1288
3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded in https://radiopaedia.org/articles/desmoplastic-fibroma (June 24th 2018)
4. Evans S, Ramasamy A, Jeys LM, Grimer R. Desmoplastic fibroma of bone : a rare bone tumour. J Bone Oncol. 2014;3(3-4): 77-79
- Metaphysis of long
bone (56%) 5
- Mandible (22%)
- Pelvis (14%)

Fig 2. Anatomical distribution of DF in patients


(cranium, mandibula, humerus, radius, pelvis, femur)1
1. Taconis WK, Schutte HE, Heul RO. Desmoplastic fibroma of bone: a report of 18 cases. Skeletal Radiology. 1994: 23 ; 4 : 238-8. http://www.semanticscholar.org/paper/Desmoplastic-fibroma-of-
bone%3A-a-report-of-18-cases-Taconis-Schutte
5. Ma XN, Qiang S, Liu TY,et al. Massive rare desmoplastic fibroma of the ilium and ischium in a young adult. Medicine. 2017 : 96 : 48: 1-4.
6. Shahzad A. Benign tumors & tumors like lesions . http: // www.slideshare.net/mobile/AtifShahzad8/benign-tumors-and-tumor-like-lesions. July 18 th, 2018
PATHOGENESIS

 Remains unclear.
 Implicate trauma and hormonal imbalance
 Genetic factors
- Trombettaet al , reported recurrent chromosome aberration in 11q
and 19p after short-term culturing of the tumor.11
- By cytogenetic analysis, some cases of DF revealed a t (2; 11) (q31;
q12) reciprocal translocation, which would result in fusion genes
and encode novel fusion proteins or a normal but ectopically
expressed protein.

5. Ma XN, Qiang S, Liu TY,et al. Massive rare desmoplastic fibroma of the ilium and ischium in a young adult. Medicine. 2017 : 96 : 48: 1-4.
7. Prabowo Y, Hidayah MRN. Case report : Desmoplastic Fibroma of Proximal Tibia Mimicking Malignant Tumor. Journal of Indonesian Orthopaedic & Traumatology, Volume 1, Number 1, April
2018 : 22 - 26
CLINICAL FINDINGS
Some patients may be asymptomatic, other present
with nonspecific variety of symptoms, such as :
 Pain, is a constant presenting feature2
 Lesion, is often tender

 Swelling of the affected area 4


 Deformity or loss of function
 Patologic fractures
(9-15% of cases)

2. Sutton, D. Textbook Of Radiology And Imaging. 7th Edition, Vol.2. London: Elsevier, 2003. P. 1287-1288
4. Evans S, Ramasamy A, Jeys L, Grimer R. Desmoplastic fibroma of bone : a rare bone tumour. J Bone Oncol. 2014;3(3-4): 77-79
7. Prabowo Y, Hidayah MRN. Case report : Desmoplastic Fibroma of Proximal Tibia Mimicking Malignant Tumor. Journal of Indonesian Orthopaedic & Traumatology, Volume 1, Number 1, April 2018 : 22 - 26
HISTOPATHOLOGY

- identical to soft tissue desmoid tumours, with abundant collagenous stroma & little
cellularity or pleomorphism, No giant cells or hemosiderin deposits will be observed
- the main cell types that are seen include : fibroblasts, myofibroblasts, &
undifferentiated mesenchymal cells 3

3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded in https://radiopaedia.org/articles/desmoplastic-fibroma (June 24 th 2018)
Cut surface shows a lobulated mass3

3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded in https://radiopaedia.org/articles/desmoplastic-fibroma (June 24 th 2018)
RADIOLOGICAL FINDINGS:
X-RAY
 Oval
 Size 7- 10 cm
 Solitary
 Expansive multilocular radiolucency with
ill-defined border, very coarse, thick
internal septa
 Destructive (lytic bone lesion) / Osteolysis
with ridges
 Endosteal scalloping
 Cortical thinning
 Expanded & irreguler sclerotic margin,
often with a trabeculated pattern
 Lobulated margins
 No periosteal reaction
 Soft tissue expansion of the lesion may
occur
 No matrix mineralisation
2. Sutton, D.Textbook of Radiology and Imaging. 7th edition, Vol.2, London: Elsevier, 2003. p. 1187
3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded in https://radiopaedia.org/articles/desmoplastic-fibroma (June 24 th 2018)
5. Evans S, Ramasamy A, Jeys L, Grimer R. Desmoplastic fibroma of bone : a rare bone tumour. J Bone Oncol. 2014;3(3-4): 77-79
3 A. 44 ys old male with DF of the 3 B. A 26 ys old female with a 3 C. A 57 ys old male with
right proximal femur. Geographic lesion in the proximal right lesion in the distal right
pattern of destruction with cortical humerus. Geographic pattern humerus. Geographic pattern
breakthrough of destruction in epi-,meta- of destruction with ridges;
and diaphysis; some regular destruction of lateral
endosteal ridges are visible cortex; some ballooning and
lamellar periosteal bone
formation is present

1. Taconis WK, Schutte HE, Heul RO. Desmoplastic fibroma of bone: a report of 18 cases. Skeletal Radiology. 1994: 23 ; 4 : 238-8. http://www.semanticscholar.org/paper/Desmoplastic-
fibroma-of-bone%3A-a-report-of-18-cases-Taconis-Schutte
RADIOLOGICAL FINDINGS:
CT SCAN
Preferred imaging technique for both the diagnosis of intra
osseus tumor extension & assessment of cortical involvement8,9

CT Axial (bone window setting) of the left


tibia, shows

 A solid
 Mildly hyperdense
 Well circumscribed
 Eccentric lesion in the distal
metaphysis of the left tibia
 Not eroding the cortex (note the distal
part of biopsy needle inside the lesion)
10

8.Nedopil A, Raab P, Rudert M. Desmoplastic Fibroma: A Case Report with Three Years of Clinical and Radiographic Observation and Review of the Literatur. Open orthop J. 2013; 8: 40-46. (Feb 13rd, 2013)
9.Levrini G, Pattacini P. Desmoplastic fibroma of the distal tibia : A case report of a minimally invasive histological diagnosis. Mol Clin Oncol. 2016; 5(5) : 537-9
RADIOLOGICAL FINDINGS:
MRI
Is favored for the assessment of exra osseus tumor growth and pre operative planning9. There is
considerable overlap with other bony lesions on MR appearances

A. The coronal T1-weighted images


(An isotense lesion compared to
the adjacent normal muscle
within the proximal tibia
metadiaphysis with cortical
destruction & soft tissue
extension)
B. The axial T2-weighted imaged
(the area T2 low signal is more
extensive than the hyperintese
medullary area)
C. The axial T1-weighted image
after gadolinium contrast adm
(the hyperintense area on the T2-
weighted image (*) is more
intensely enhanced than the area
with T2 low signal) 11

8. Nedopil A, Raab P, Rudert M. Desmoplastic Fibroma: A Case Report with Three Years of Clinical and Radiographic Observation and Review of the Literatur. Open orthop J. 2013; 8: 40-46. (Feb 13rd, 2013)
10. Lee SY, Ha D, Kim DW, et al. Desmoplastic fibroma of bone: case report. J Korean Soc Radiol. 2011: 64: 491-6
CASE 1
22 ys old male, a lesion in the proximal
left radius

A. Geographic pattern of
destruction, with cortical
breakthrough and ballooning;
many ridges

B, C. MRI. T1 weighted
sagittal and axial images with
inhomogeneous low to
intermediate signal intensity

D. MRI. T2 weighted axial


image with predominantly
low signal intensity

1. Taconis WK, Schutte HE, Heul RO. Desmoplastic fibroma of bone: a report of 18 cases. Skeletal Radiology. 1994: 23 ; 4 : 238-8. http://www.semanticscholar.org/paper/Desmoplastic-
fibroma-of-bone%3A-a-report-of-18-cases-Taconis-Schutte
CASE 2
29 ys old man, felt minor pain in the right
hip region, first noted pain ± 2
ys ago, no history of trauma.

4. Ma XN, Qiang S, Liu TY,et al. Massive rare desmoplastic fibroma of the ilium and ischium in a young adult. Medicine. 2017 : 96 : 48: 1-4.
CASE 3

11. Bohm P, Krober S, Greschniok A. Desmoplastic Fibroma of the Bone.A Report of Two Patients, Review of the Literature, and Therapeutic Implications. American Cancer Society.
1996 : 1011 - 1023
DIFFERENTIAL DIAGNOSIS
Similarity with Benign Lesion

 Osteolytic lesion with


trabeculated, honeycombed, or
bubbly app. - Non Ossifying
 Expansile Fibroma
 Geographic pattern
 Narrow zone of transition - Giant Cell
 Eccentrically, multilocular app./ Tumor
ridges in the bony wall
 No matrix calcification
 Usually seen in femur, tibia

Similarity with Maligna Lesion Low Grade of


 Cortical destruction Osteosarcoma
 Soft tissue mass involved

The diagnosis is very difficult on


imaging alone as many tumours
have similar radiological
appearances. Biopsy is the gold
standard

3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded in https://radiopaedia.org/articles/desmoplastic-fibroma (June 24th 2018)
6. Shahzad A. Benign tumors & tumor like lesions. Http://www.slideshare.net/mobile/atifshahzad8/benign-tumors-and-tumor-lie-lesions.July,18TH , 2018
12. Kremli M. Bone tumors and tumor like conditions. Downloaded from : http://slideplayer.com/slide/10836593/
Differentiation Desmoplastic Fibroma NOF (Non-ossifying fibroma) GCT (Giant Cell Tumour) Low Grade of Osteosarcoma

Age 15 mo – 75 ys Peak incidence at 10-15 ys old 20 – 50 ys 10 – 20 ys


35% of children Peak 20 – 30 ys

Incidence of all 0.11 % 3 unknown 4 – 9,5 % 20 %


primary bone
tumours

Sex Male = female Male 2x > Female Female > male Male > female

Size 7 – 10 cm > 3 cm 2,5 – 12 cm

Predilection Metadiaphyseal of long bone Metaphyseal of long bone Metaphysis extend to Metadiaphysis of tubular bones
epiphysis

Other site Mandible, radius, pelvis proximal fibula distal radius, sacrum, vertebral Coxae, maxilla, vertebrae
predilection body

Periosteal reaction +/- -- +/- +

Sclerotic margin + + - +

Erosion cortex + - +

Soft tissue mass +/- - +/- +


extension

3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded in https://radiopaedia.org/articles/desmoplastic-fibroma (June 24th 2018)
4. Shahzad A. Benign tumors & tumor like lesions. Http://www.slideshare.net/mobile/atifshahzad8/benign-tumors-and-tumor-lie-lesions.July,18TH , 2018
12. Kremli M. Bone tumors and tumor like conditions. Downloaded from : http://slideplayer.com/slide/10836593/
RESUME MIND MAPPING
 Oesmoid tumor of bone, osteofibroma, osseous
desmoplastic fibroma
1. Introduction  Dense fibrous tissue
 Metadiaphysis of long bone

 0,11% of all primary bone tumours


2. Epidemiology  Young adults (21 th)
 No sex predilection

 Pain
3. Clinical  Tender lesion
findings  Swelling

DESMOPLASTIC 4.  Abundant collagenous stroma


FIBROMA  little cellularity/pleomofphism
Histopathology

 X-Ray : oval, solitary, cortical thinning, endosteal scalloping,


lobulated margin,no matrix mineralisation
5. Radiological  CT Scan : solid, midly hyperdense, well circumscribed,
eccentric lesion
Finding  MRI : T1-weighted isointense, / inhomogeneous low to
intermediate signal. T2 weighted low signal

 GCT
6. Differential  NOF

diagnosis Low Grade of
Osteosarcoma
QUIZ

WHAT CAN BE POSSIBLE DIAGNOSIS


BASED ON THIS PICTURE ?

A. CHONDROSARCOMA
B. OSTEOCHONDROMA
C. OSTEOMYELITIS
D. DESMOPLASTIC FIBROMA
E. FIBROUS CORTICAL DEFECT
REFERENCES
1. Taconis WK, Schutte HE, Heul RO. Desmoplastic fibroma of bone: a report of 18 cases. Skeletal Radiology. 1994: 23 ; 4 :
238-8. http://www.semanticscholar.org/paper/Desmoplastic-fibroma-of-bone%3A-a-report-of-18-cases-Taconis-Schutte
2. Sutton, D. Textbook of Radiology and Imaging. 7th edition, Vol.2, London: Elsevier, 2003. p. 1287-1288
3. Knipe H, Weerakkody Y, et al. Desmoplastic Fibroma. 2018. Downloaded in https://radiopaedia.org/articles/desmoplastic-
fibroma (June 24th 2018)
4. Evans S, Ramasamy A, Jeys L, Grimer R. Desmoplastic fibroma of bone : a rare bone tumour. J Bone Oncol. 2014;3(3-4):
77-79
5. Ma XN, Qiang S, Liu TY,et al. Massive rare desmoplastic fibroma of the ilium and ischium in a young adult. Medicine.
2017 : 96 : 48: 1-4.
6. Shahzad A. Benign tumors & tumors like lesions . http: // www.slideshare.net/mobile/AtifShahzad8/benign-tumors-and-
tumor-like-lesions. July 18th, 2018
7. Prabowo Y, Hidayah MRN. Case report : Desmoplastic Fibroma of Proximal Tibia Mimicking Malignant Tumor. Journal of
Indonesian Orthopaedic & Traumatology, Volume 1, Number 1, April 2018 : 22 - 26
8. Nedopil A, Raab P, Rudert M. Desmoplastic Fibroma: A Case Report with Three Years of Clinical and Radiographic
Observation and Review of the Literatur. Open orthop J. 2013; 8: 40-46. (Feb 13rd, 2013)
9. Levrini G, Pattacini P. Desmoplastic fibroma of the distal tibia : A case report of a minimally invasive histological
diagnosis. Mol Clin Oncol. 2016; 5(5) : 537-9
10. Lee SY, Ha D, Kim DW, et al. Desmoplastic fibroma of bone: case report. J Korean Soc Radiol. 2011: 64: 491-6
11. Bohm P, Krober S, Greschniok A. Desmoplastic Fibroma of the Bone A Report of Two Patients, Review of the Literature,
and Therapeutic Implications. American Cancer Society. 1996 : 1011 - 1023
12. Kremli M. Bone tumors and tumor like conditions. Downloaded from : http://slideplayer.com/slide/10836593/
TERIMA KASIH
&
MOHON ASUPAN