Figure 1
Plain X-ray of the pelvis
Radiographic findings of KL grade 3 osteoarthritis of hip joints associated with multiple, scattered, juxta-articular and metaphyseal, ovoid and longitudinal radiopacities.[8]
Osteopoikilosis
SectionMusculoskeletal system
Case TypeClinical Cases
AuthorsZeljko L Stepanovic1, Ana Rankovic2
Connected authors
67 years, male
Clinical History
A 67-year-old man was referred to the orthopaedic department for further evaluation of osteoblastic bone lesions incidentally found on pelvis radiograph. Recent examinations revealed benign prostate hyperplasia. 99mTc bone scan (Fig. 6) showed positive radionuclide uptake, indicating possible osteoblastic metastases. His only complaint was moderate pain in the right knee.
Imaging Findings
Plane radiography of the pelvis (Fig. 1) revealed the 3rd. grade osteoarthritis of hip joints according to Kellgren-Lowrence (KL) criteria associated with multiple, scattered, juxta-articular and metaphyseal, ovoid and longitudinal radiopacities.
Knee radiographs (Fig. 2) revealed KL grade 2 and KL grade 3 osteoarthritis of the left and the right knee. Ovoid sclerotic bone islands adjacent to inner cortices were detected in metaphyseal regions of femoral bones.
Shoulder joint radiographs (Fig. 3) detected the same sclerotic bone lesions in peri-articular and metaphyseal region of proximal humeral bones, clavicles and scapulae.
Control MDCT (Fig. 4) and MRI (Fig. 5) of the pelvis were performed to differentiate these lesions between osteoblastic metastases and benign bone dysplasias and to rule out prostate proliferation. The same ovoid and longitudinal symmetric sclerotic bone formations were detected in the pelvis and the proximal femurs.
Classic imaging findings of osteopoikilosis were associated with normal clinical, laboratory and histology results of benign prostate hyperplasia, excluding osteoblastic bone metastases.
Knee radiographs (Fig. 2) revealed KL grade 2 and KL grade 3 osteoarthritis of the left and the right knee. Ovoid sclerotic bone islands adjacent to inner cortices were detected in metaphyseal regions of femoral bones.
Shoulder joint radiographs (Fig. 3) detected the same sclerotic bone lesions in peri-articular and metaphyseal region of proximal humeral bones, clavicles and scapulae.
Control MDCT (Fig. 4) and MRI (Fig. 5) of the pelvis were performed to differentiate these lesions between osteoblastic metastases and benign bone dysplasias and to rule out prostate proliferation. The same ovoid and longitudinal symmetric sclerotic bone formations were detected in the pelvis and the proximal femurs.
Classic imaging findings of osteopoikilosis were associated with normal clinical, laboratory and histology results of benign prostate hyperplasia, excluding osteoblastic bone metastases.
Discussion
Osteopoikilosis represents an autosomal dominant bone dysplasia that mimics osteoblastic bone metastases, and may lead to misdiagnosis and unnecessary treatment. Its incidence is 1 in 50, 000; it is seen in up to 6 per 100, 000 radiographs. [1] A mutation in gene LEMD3 at position 12q13 is responsible for this benign bone condition. [2]
Patients are usually asymptomatic or may complain of nonspecific articular pain in 15-20% of cases. Mostly, these lesions are discovered incidentally on radiographic imaging. [3] Radiographic findings of osteopoikilosis include the appearance of numerous symmetric, ovoid sclerotic foci, ranging from 2 to 10 mm, clustered in periarticular regions of tubular bones. The metaphyseal lesions may be longitudinal and located eccentrically, abutting the endosteal cortical surface. The main distribution of lesions in the pelvis is found about the acetabulum and mostly related to the orientation of the trabecular bone. The skull, mandible, clavicles and thoracic cage are spared. [4] Macroscopically, foci of compact bone within the cancellous bone are found. Histologically, the sclerotic nodular or star-like foci correspond to old and inactive condensation of cancellous bone with peripheral scant osteocytes. Osteoblasts and osteoclasts are in the central core of irregular trabeculae. [5]
Osteopoikilosis could be associated with skeletal and pea-sized fibrous skin changes as seen in Buschke-Ollendorf syndrome. It has been associated with scleroderma, syndactyly, dwarfism, mental retardation, and tuberous sclerosis. [1, 2] Mixed sclerosing bone dysplasia represents another condition, whereas triads of benign bone disorders: osteopoikilosis, osteopathia striata, and melorheostosis occur concomitantly.
The differential diagnosis includes conditions with radiographic appearance similar to those of
osteopoikilosis such as osteoblastic metastases, melorheostosis, osteopathia striata, mastocytosis, tuberous sclerosis and Paget disease. [4, 5] However, these bone conditions have additional distinctive physical findings.
Bone scans are mostly normal in osteopoikilosis, which has been used to differentiate it from metastatic bone disease. [6] There are a few previous reports of abnormal scintigraphy findings in osteopoikilosis, mimicking osteoblastic bone metastases. [7] Bone scan of a patient with bone metastastases usually reveals an increased uptake, with asymmetrical distribution when multiple sites throughout the skeleton are involved. The main problem is its low specificity of osteoblastic activity. Abnormal bone scan does not exclude the diagnosis of osteopoikilosis if the radiographic findings are characteristic of that entity.
Take Home Message:
Benign bone dysplasia that mimics osteoblastic bone metastases.
Usually asymptomatic.
Radiographic findings are characteristic and diagnostic.
Bone strength is normal.
Abnormal bone scan does not exclude the diagnosis of osteopoikilosis.
Patients are usually asymptomatic or may complain of nonspecific articular pain in 15-20% of cases. Mostly, these lesions are discovered incidentally on radiographic imaging. [3] Radiographic findings of osteopoikilosis include the appearance of numerous symmetric, ovoid sclerotic foci, ranging from 2 to 10 mm, clustered in periarticular regions of tubular bones. The metaphyseal lesions may be longitudinal and located eccentrically, abutting the endosteal cortical surface. The main distribution of lesions in the pelvis is found about the acetabulum and mostly related to the orientation of the trabecular bone. The skull, mandible, clavicles and thoracic cage are spared. [4] Macroscopically, foci of compact bone within the cancellous bone are found. Histologically, the sclerotic nodular or star-like foci correspond to old and inactive condensation of cancellous bone with peripheral scant osteocytes. Osteoblasts and osteoclasts are in the central core of irregular trabeculae. [5]
Osteopoikilosis could be associated with skeletal and pea-sized fibrous skin changes as seen in Buschke-Ollendorf syndrome. It has been associated with scleroderma, syndactyly, dwarfism, mental retardation, and tuberous sclerosis. [1, 2] Mixed sclerosing bone dysplasia represents another condition, whereas triads of benign bone disorders: osteopoikilosis, osteopathia striata, and melorheostosis occur concomitantly.
The differential diagnosis includes conditions with radiographic appearance similar to those of
osteopoikilosis such as osteoblastic metastases, melorheostosis, osteopathia striata, mastocytosis, tuberous sclerosis and Paget disease. [4, 5] However, these bone conditions have additional distinctive physical findings.
Bone scans are mostly normal in osteopoikilosis, which has been used to differentiate it from metastatic bone disease. [6] There are a few previous reports of abnormal scintigraphy findings in osteopoikilosis, mimicking osteoblastic bone metastases. [7] Bone scan of a patient with bone metastastases usually reveals an increased uptake, with asymmetrical distribution when multiple sites throughout the skeleton are involved. The main problem is its low specificity of osteoblastic activity. Abnormal bone scan does not exclude the diagnosis of osteopoikilosis if the radiographic findings are characteristic of that entity.
Take Home Message:
Benign bone dysplasia that mimics osteoblastic bone metastases.
Usually asymptomatic.
Radiographic findings are characteristic and diagnostic.
Bone strength is normal.
Abnormal bone scan does not exclude the diagnosis of osteopoikilosis.
Differential Diagnosis List
Osteopoikilosis (osteopathy condensans disseminata)
Osteoblastic bone metastases
Melorheostosis
Osteopathia striata
Tuberous sclerosis
Mastocytosis
Final Diagnosis
Osteopoikilosis (osteopathy condensans disseminata)
References
[1] Korkmaz MF, Elli M, Özkan MB, Bilgici MC, Dağdemir A, Korkmaz M, Tosun FC (2015) Osteopoikilosis: report of a familial case and review of the literature. Rheumatol Int 35(5):921-4. doi: 10.1007/s00296-014-3160-6.
[2] Baasanjav et al (2010) Osteopoikilosis and multiple exostoses caused by novel mutations in LEMD3 and EXT1 genes respectively - coincidence within one family. BMC Medical Genetics 11:110 (PMID: 20618940)
[3] Borman P, Ozoran K, Aydog S, Coçskun S (2002) Osteopoikilosis: report of a clinical case and review of literature. Joint Bone Spine 69:230-233. (PMID: 12027319)
[4] Resnick D (1988) Diagnosis of Bone and Joint Disorders. Philadelphia: WB Saunders 4084-4088
[5] Lagier R, Mbakop A, Bigler A (1984) Osteopoikilosis: a radiological and pathological study. Skeletal radiology 11(3), 161-168. (PMID: 6719141)
[6] Van Hul W, Vanhoenacker F, Balemans W, Janssens K, De Schepper AM (2001) Molecular and radiological diagnosis of sclerosing bone dysplasias. European journal of radiology 40 (3), 198-207. (PMID: 11731208)
[7] An YS, Yoon JK, Lee MH, Joh CW, Yoon SN (2004) Abnormal bone scan in an adult with osteopoikilosis. Clin Nucl Med 29(12):856-8. (PMID: 15545908)
[8] Kellgren JH, Lawrence JS (1957) Radiological assessment of osteo-arthrosis. Ann Rheum Dis 16:494–502 (PMID: 13498604)
Case information
| URL: | https://eurorad.org/case/13006 |
| DOI: | 10.1594/EURORAD/CASE.13006 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
Plain X-rays of knee joints
Standing plain radiograph showing KL grade 2 and KL grade 3 osteoarthritis of the left and right knee, associated with ovoid sclerotic foci adjacent to the endosteal cortex in the metaphyseal region of both femora. [8]
Figure 3
Plain X-rays of shoulder joints
Shoulder joint radiographs detected elliptic and longitudinal sclerotic bone lesions in epiphyseal and metaphyseal region of proximal humeral bones, distal clavicles and scapulae.
Figure 4
Coronal and axial MDCT images of the pelvis
Coronal and axial MDCT findings of the pelvis showed symmetric, mixed lytic and hyperdense ovoid and irregular sclerotic lesions scattered in peri-acetabular regions and in epiphyseal and metaphyseal parts of the proximal femora.
Figure 5
Coronal and axial MRI images of the pelvic region
Coronal T1w and axial T2w MRI images show multiple symmetric, ovoid, hypointense lesions in all sequences within the cancellous bone and along the inner cortices of proximal femora and acetabuli, without their destruction.
Figure 6
99m Tc MDP bone scan
99m Tc MDP bone scan findings were defined as possible osteoblastic metastases with increased tracer uptake in the pelvic region, hip and shoulder joints. Same findings may also be recognized as degenerative, arthritic lesions.
Plain X-ray of the pelvis
Radiographic findings of KL grade 3 osteoarthritis of hip joints associated with multiple, scattered, juxta-articular and metaphyseal, ovoid and longitudinal radiopacities.[8]
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
Plain X-rays of knee joints
Standing plain radiograph showing KL grade 2 and KL grade 3 osteoarthritis of the left and right knee, associated with ovoid sclerotic foci adjacent to the endosteal cortex in the metaphyseal region of both femora. [8]
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
Plain X-rays of shoulder joints
Shoulder joint radiographs detected elliptic and longitudinal sclerotic bone lesions in epiphyseal and metaphyseal region of proximal humeral bones, distal clavicles and scapulae.
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
Coronal and axial MDCT images of the pelvis
Coronal and axial MDCT findings of the pelvis showed symmetric, mixed lytic and hyperdense ovoid and irregular sclerotic lesions scattered in peri-acetabular regions and in epiphyseal and metaphyseal parts of the proximal femora.
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
Coronal and axial MRI images of the pelvic region
Coronal T1w and axial T2w MRI images show multiple symmetric, ovoid, hypointense lesions in all sequences within the cancellous bone and along the inner cortices of proximal femora and acetabuli, without their destruction.
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
© \"Department of Radiology, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2015\"
99m Tc MDP bone scan
99m Tc MDP bone scan findings were defined as possible osteoblastic metastases with increased tracer uptake in the pelvic region, hip and shoulder joints. Same findings may also be recognized as degenerative, arthritic lesions.
© \"Department of Nuclear Medicine, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2014\"
© \"Department of Nuclear Medicine, Clinical Center Kragujevac, Faculty of Medical Sciences University of Kragujevac/Serbia 2014\"