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Other tumors

Osteofibrous dysplasia



Last author update: 17 May 2022
Last staff update: 17 May 2022

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PubMed Search: Osteofibrous dysplasia

Muhammad Usman Tariq, M.B.B.S.
Nasir Ud Din, M.B.B.S.
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Cite this page: Tariq MU, Ud Din N. Osteofibrous dysplasia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/boneosteofibrousdysplasia.html. Accessed December 7th, 2022.
Definition / general
  • Osteofibrous dysplasia (OFD) is a benign fibro-osseous tumor of the pediatric age group with strong predilection for the anterior tibial diaphysis
Essential features
  • Usually occurs in first 2 decades of life, without gender predilection and most commonly involves anterior diaphysis of tibia
  • Radiologically, tumor appears intracortical, multiloculated and radiolucent with well defined sclerotic margins
  • Microscopically, woven bone trabeculae with osteoblastic rimming are seen against bland fibroblastic stroma with storiform pattern
  • Maturation of bone trabeculae towards periphery (zonal architecture) and scattered keratin positive cells in the stroma are useful diagnostic features
  • Has the potential to recur; tumors may regress after skeletal maturity
Terminology
ICD coding
  • ICD-O: 9261/0 - osteofibrous dysplasia
  • ICD-11: 2E83.5 & XH6M86 - benign osteogenic tumors of bone or articular cartilage of limbs and ossifying fibroma
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
Diagnosis
Radiology description
  • Xray: typically intracortical, eccentric, multiloculated radiolucent / osteolytic lesion with internal septa and sharply defined sclerotic borders
  • Some lesions may show ground glass appearance, marked sclerosis and ill defined borders
  • Larger lesions cause cortical expansion, bowing deformity and fracture (Clin Radiol 2014;69:200, Surg Oncol 2021;38:101626)
  • On MRI scan, T1 weighted images show intermediate intensity signals while T2 weighted images show intermediate to hyperintense signals; with contrast, enhancement of homogenous or heterogenous patterns is observed (Korean J Radiol 2014;15:114)
  • MRI scan is useful for assessing medullary involvement and surgical staging but not useful in discrimination from the differentials (Clin Radiol 2014;69:200)
  • Involvement of the medullary cavity is uncommon; however, it has been reported in up to 40% of cases (Clin Radiol 2014;69:200)
  • In a single study, partial or complete medullary involvement was observed on MRI scan in all 24 cases (Korean J Radiol 2014;15:114)
  • CT scan is useful for assessing matrix mineralization, pathological fracture, lack of periosteal reaction and transitional zone; not superior to MRI scan (Clin Radiol 2014;69:200, Radiol Case Rep 2015;6:546)
Radiology images

Contributed by Nasir Ud Din, M.B.B.S., Mark R. Wick, M.D. and AFIP images
Multiloculated radiolucent lesion in tibia

Multiloculated radiolucent lesion in tibia

Multiloculation and sclerotic borders

Multiloculation and sclerotic borders

Thickened sclerotic areas

Thickened sclerotic areas

Marked bowing deformity Marked bowing deformity

Marked bowing deformity


Synchronous tumors in tibia and fibula

Synchronous tumors in tibia and fibula

Contrast enhancing lesion on CT scan

Contrast enhancing lesion on CT scan

Contrast enhancing lesion on MRI scan

Contrast enhancing lesion on MRI scan

Various images

Radiolucent and sclerotic areas

Ill defined lucencies and bowing deformity

Ill defined lucencies and bowing deformity



Images hosted on other servers:
Soft tissue and medullary cavity involvement

Soft tissue and medullary cavity involvement

Soap bubble appearance on CT scan

Soap bubble appearance on CT scan

Cortical lesion with sclerotic rim

Cortical lesion with sclerotic rim

Prognostic factors
Case reports
Treatment
  • Surgical excision (with or without grafting) or curettage for larger tumors and cases with tibial bowing
  • Some tumors are only observed, since the tumors regress after puberty (Surg Oncol 2021;38:101626)
  • Some authors recommend extraperiosteal excision for all cases due to the low recurrence rate (J Bone Joint Surg Br 2006;88:658)
Gross description
Gross images

Contributed by Mark R. Wick, M.D. and AFIP images
Solid tumor involving cortex and medulla

Solid tumor involving cortex and medulla

Focally eroded cortex

Focally eroded cortex

Microscopic (histologic) description
  • Tumor has 2 basic components: fibrous stroma and bone trabeculae
  • Zonation pattern is a characteristic feature
  • In the center, the lesion is more fibrous and the newly formed woven bone trabeculae are thin
  • Bone trabeculae become more numerous, thicker and mature (lamellar) and merge with the outer and inner cortices at the periphery
  • Bone trabeculae are rimmed by epithelioid / active osteoblasts
  • Fibrous stromal component is composed of spindle to stellate cells arranged in short fascicles and vague storiform pattern; background is myxoid
  • Rare epithelial cells in the stroma may be highlighted on cytokeratin
  • Scattered multinucleated giant cells, hemosiderin laden macrophages, hemosiderin and foamy macrophages may also be seen (Hum Pathol 1993;24:1339, Surg Oncol 2021;38:101626)
Microscopic (histologic) images

Contributed by Nasir Ud Din, M.B.B.S. and Mark R. Wick, M.D.
Fibro-osseous lesion with zonation pattern Fibro-osseous lesion with zonation pattern

Fibro-osseous lesion with zonation pattern

Thickened trabeculae at the periphery

Thickened trabeculae at the periphery

Bone trabeculae and storiform pattern

Bone trabeculae and storiform pattern

Osteoblastic rimming of bone trabeculae

Osteoblastic rimming of bone trabeculae

Osteoblastic rimming and storiform pattern

Osteoblastic rimming and storiform pattern


Osteoblastic rimming Osteoblastic rimming

Osteoblastic rimming

Multinucleated osteoclast-like giant cells Multinucleated osteoclast-like giant cells

Multinucleated osteoclast-like
giant cells

Keratin immunostain Keratin immunostain

Keratin immunostain

Positive stains
Electron microscopy description
  • Stromal cells show fibroblast-like cells with prominent rough endoplasmic reticulum; epithelial differentiation was not seen
  • Thickened woven bone trabeculae showed increased remodeling with osteoblastic and osteoclastic activity (Mod Pathol 2012;25:56)
Molecular / cytogenetics description
Videos

Osteofibrous dysplasia

Fibrous dysplasia and osteofibrous dysplasia: bone forming tumors,
part 4

Imaging osteofibrous dysplasia

Sample pathology report
  • Tibia, Tru-Cut / incisional biopsy:
    • Fibro-osseous tumor (see comment)
    • Comment: Morphological features are those of osteofibrous dysplasia (OFD); however, the possibility of classic adamantinoma and OFD-like adamantinoma cannot be entirely ruled out because these lesions also contain areas with morphological features of OFD. Correlation with clinical and radiological findings is recommended. A more definitive diagnosis would be possible on excision biopsy specimen.

  • Tibia, curettage / resection specimen:
    • Fibro-osseous tumor, consistent with osteofibrous dysplasia (see comment)
    • Comment: Histological examination showed a fibro-osseous tumor. The osseous component is composed of woven bone trabeculae rimmed by osteoblasts and fibroblastic stroma. CK AE1 / AE3 is positive in scattered stromal cells. No epithelial nests are seen. Correlation with clinical and radiological findings is recommended. OFD has the potential to recur locally; therefore, close follow up is advised.
Differential diagnosis
Board review style question #1

A 10 year old boy complained of pain in his right leg. Xray of right leg showed a intracortical multiloculated, osteolytic lesion with a well defined sclerotic margin involving the diaphysis of tibia. Microscopically, the tumor showed trabeculae of woven bone with osteoblastic rimming. The background showed fibroblastic stroma with storiform pattern. On immunohistochemistry, only rare stromal cells showed positivity for cytokeratin. What is the most likely diagnosis?

  1. Fibrous dysplasia
  2. Nonossifying fibroma
  3. Osteofibrous dysplasia
  4. Osteofibrous dysplasia-like adamantinoma
  5. Osteoid osteoma
Board review style answer #1
C. Osteofibrous dysplasia. Clinical, radiological, histological and immunohistochemical features are characteristic of osteofibrous dysplasia. Fibrous dysplasia is a fibroosseous lesion that usually lacks osteoid rimming of bone trabeculae. Nonossifying fibroma lacks bone trabeculae. Osteofibrous dysplasia-like adamantinoma closely resembles osteofibrous dysplasia. On IHC, small clusters and cords of cytokeratin positive epithelial cells are seen in the stroma rather than scattered epithelial cells seen in osteofibrous dysplasia. Osteoid osteoma shows loose fibrovascular stroma between bone trabeculae.

Comment Here

Reference: Osteofibrous dysplasia
Board review style question #2
Which of the following is the most helpful feature in differentiating osteofibrous dysplasia from osteofibrous dysplasia-like adamantinoma?

  1. Bone trabeculae without osteoblastic rimming
  2. Clusters and small nests of epithelial cells
  3. Patient's age
  4. Radiological findings
  5. Tumor location
Board review style answer #2
B. Clusters and small nests of epithelial cells. Osteofibrous dysplasia-like adamantinoma closely resembles osteofibrous dysplasia and the distinction is not possible clinically or radiologically. The differentiating feature on histology is the presence of cluster, cords and small nests of epithelial cells in the stroma of osteofibrous dysplasia-like adamantinoma, which are further highlighted on cytokeratin immunostains. Histologically, epithelial cells are not seen in the stroma of osteofibrous dysplasia and only scattered cytokeratin positive epithelial cells are highlighted on cytokeratin immunostains. Absence of osteoblastic rimming around bone trabeculae is a feature of fibrous dysplasia.

Comment Here

Reference: Osteofibrous dysplasia
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