Tumours and tumour mimics in the olecranon

Tumours and tumour mimics in the olecranon

Clinical Radiology xxx (2015) e1ee14 Contents lists available at ScienceDirect Clinical Radiology journal homepage: www.clinicalradiologyonline.net ...

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Clinical Radiology xxx (2015) e1ee14

Contents lists available at ScienceDirect

Clinical Radiology journal homepage: www.clinicalradiologyonline.net

Pictorial Review

Tumours and tumour mimics in the olecranon U. Kularatne a, *, S.L.J. James b, N. Evans b, P.N.M. Tyrrell a, J. Singh a a b

Robert Jones and Agnes Hunt Orthopaedic Hospital, Oswestry, Shropshire SY10 7AG, UK Royal Orthopaedic Hospital, Bristol Road South, Birmingham B31 2AP, UK

art icl e i nformat ion Article history: Received 2 September 2014 Received in revised form 8 February 2015 Accepted 26 February 2015

Lesions in the olecranon are rare and may be identified during the investigation of a clinically suspected abnormality or as an incidental finding. This review describes the spectrum of tumours and tumour-like lesions that can involve the olecranon and illustrates the radiographic, CT, and MRI appearances that may facilitate diagnosis. A variety of pathological processes affecting the olecranon are presented and discussed including the epidemiology and imaging features. Ó 2015 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

Introduction

Case series

Tumours in the olecranon are rare. Primary bone tumours around the elbow represent <1% of all skeletal tumours. The commonest malignant tumour is lymphoma and the commonest benign tumour is osteoid osteoma.1 In a published series of 9973 cases of primary tumours of bone, 102 (1%) were in the ulna.2 Although the exact number of these occurring in the olecranon is not provided, it can be approximated by considering ratio of lesions in the patella (n ¼ 14) to those in the tibia (n ¼ 1153). Thus, tumours in the olecranon are likely to be rarer than even those in the patella. In this review we present radiographic appearances of a variety of both benign and malignant tumours and tumour mimics seen across two specialist bone tumour centres, with cross-sectional imaging correlation. A comprehensive literature review has been carried out. To the authors’ knowledge, this is the first case series describing a spectrum of tumours and tumour-like lesions manifesting in the olecranon.

The databases of two specialist bone tumour centres in the UK were reviewed for lesions occurring in the olecranon. The search revealed 19 cases spanning 5 years. All of the cases apart from three geodes underwent biopsy with histological confirmation. Institutional review board approval was not required for the retrospective data collection of this study. There were nine benign neoplasms [aneurysmal bone cyst (ABC; n ¼ 4), chondroblastoma (n ¼ 1), osteoid osteoma (n ¼ 2) and fibrous dysplasia (n ¼ 2)]; three malignant neoplasms [chondrosarcoma (n ¼ 1), plasmacytoma (n ¼ 1) and metastatic mucinous adenocarcinoma (n ¼ 1)]; and seven non-neoplastic conditions [Paget’s disease (n ¼ 1), tuberculosis (n ¼ 1), haemophilic pseudotumour (n ¼ 2) and geode (n ¼ 3)]. Two patients had pathological fractures (chondroblastoma and chondrosarcoma). There were 12 male and seven female patients with ages ranging between 11 and 82 (Table 1).

* Guarantor and correspondent: U. Kularatne, Royal Orthopaedic Hospital, Bristol Road South, Birmingham B31 2AP, UK. Tel.: þ44 07809 499 834; fax: þ44 01691 404057. E-mail address: [email protected] (U. Kularatne).

Benign neoplastic lesions ABC ABC is an expansile lesion of bone containing thin-walled blood-filled cystic cavities (Fig 1). It is benign but locally

http://dx.doi.org/10.1016/j.crad.2015.02.019 0009-9260/Ó 2015 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.

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Osteoid osteoma

Table 1 Summary of tumours and tumour mimics in the olecranon. Diagnosis

Number Median M F Incidencea age (years)

Benign neoplastic Aneurysmal bone cyst 4 Chondroblastoma 1 Osteoid osteoma 2 Fibrous dysplasia 2 Malignant Chondrosarcoma 1 Plasmacytoma 1 Metastasis 1 Non-neoplastic Paget’s disease 1 Tuberculosis 1 Haemophilic pseudotumour 2 Geode 3 Total 19

2 1 2 2

82 44 56

1 0 0.19b 0 1 0.19b,d 0 1 <0.01e

64 38 28 52

0 0 2 2 12

2 0 0 0

1.60b 0.70b 2.30b <0.01c

12 39 17 44

1 1 0 1 7

<0.01f 1.45e3.6337 <0.01g <0.01h

a

Incidence in olecranon as a percentage of all skeletal locations for a given lesion. b Incidence in proximal ulna in a large study.2 c One reported case of occurrence in olecranon.11 d Incidence of myeloma. e Three reported cases of occurrence in olecranon.28 f Two reported cases of occurrence in olecranon.32,33 g One reported case of occurrence in olecranon.42 h Few reported cases of occurrence in the olecranon.45e49

destructive. Two cases have been reported in the proximal ulna in the English literature, one in a rare metachronous presentation.3,4 ABCs account for approximately 2.5% of benign primary bone tumours and may develop as a primary lesion or secondary to another lesion or trauma.4 Most patients are less than 20 years of age at presentation. In the present series, the median age of presentation was 12 years. Typically in radiographs a lytic, expansile lesion is seen with a thin sclerotic margin in the metaphysis of a long bone. At MRI, cysts of different signal intensity are seen representing different stages of blood products, often with fluidefluid levels.

Chondroblastoma Chondroblastoma is a benign cartilaginous tumour (Fig 2). It accounts for less than 1% of primary bone tumours and arises most commonly in the epiphysis of long bones. Commonly presenting in the second decade, it has a male preponderance of 2:1.5 Most frequent locations are about the knee and proximal humerus.6 Occurrence in the proximal ulna has not been reported previously in the English literature to the authors’ knowledge. The most common radiographic appearance is of an expansile lytic lesion with a sclerotic border within the epiphysis of a long bone (Fig 2aeb). At MRI, low to intermediate signal on T1weighted images and a heterogeneous intermediate signal on T2-weighted images are seen. A feature of chondroblastoma at MRI is extensive peritumoural bone marrow oedema (Fig 2f).6

Osteoid osteoma is a benign osteoblastic tumour (Fig 3). In a large series, osteoid osteomas accounted for 3% of all bone tumours.2 The elbow location is considered to be rare, accounting for 3% of all osteoid osteomas.7 Radiographically, a radiolucent or variably mineralized “nidus” is seen within an area of dense reactive osteosclerosis (Fig 3aeb). At CT, the nidus is seen as a well-defined lesion of decreased attenuation with surrounding sclerosis (Fig 3ced). At MRI, intermediate signal intensity on T1-weighted images and intermediate to high signal on T2-weighted images with surrounding marrow and soft-tissue oedema is seen (Fig 3eef).8

Fibrous dysplasia Fibrous dysplasia is a developmental anomaly in which normal bone marrow is replaced by fibro-osseous tissue and may be encountered in any decade of life. Approximately 70e80% cases are monostotic.9 Radiographically a radiolucent lesion with well-defined or sclerotic margins is seen (Fig 4). Diffusely increased internal density can give “ground-glass” appearance. T1-weighted images reveal fibrous dysplasia as hypointense and T2-weighted images reveal fibrous dysplasia as either hyperintense or hypointense.10 Occurrence in the elbow region is rare. The literature search revealed one case of occurrence in the olecranon as part of polyostotic disease.11 Monostotic occurrence in the olecranon has not been reported in the English literature.

Malignant lesions Chondrosarcoma Chondrosarcoma (Fig 5) is the third most common malignant bone tumour. The most frequent histological type (>90%) is conventional or central chondrosarcoma. Rare histological varieties exist.12 In a large series, primary chondrosarcoma accounted for 14% of all malignant bone tumours. Of those, less than 0.3% occurred in the ulna.2 The literature search revealed a report of one case in the olecranon13 and another in the proximal ulna.14 Patients most commonly present in the 4th to 5th decades with a 1.5e2:1 male predilection.15 Radiographic appearances can be potentially confusing with low-grade chondrosarcoma mimicking benign lesions.16 Typically a mixed lytic and sclerotic lesion with ringearc pattern of mineralized chondroid matrix is revealed. Deep endosteal scalloping is a sensitive discriminator between central chondrosarcoma and enchondroma.17 On T1-weighted MRI images low to intermediate signal intensity is seen, possibly with punctate areas of signal void due to matrix mineralization. On T2weighted images, the non-mineralized components have high signal intensity reflecting the high water content of hyaline cartilage.15 CT demonstrates the mineralized component to better advantage.

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Figure 1 Aneurysmal bone cyst in a 12-year-old female patient. Anteroposterior (a) and lateral (b) radiographs show an expansile lucent lesion within the olecranon with multiple septa within it. Axial T1 (c), fat-saturated T2 (d), sagittal T1 (e), STIR (f) MRI images demonstrate multiple cystic cavities. Cysts of different signal intensity represent different stages of blood products.

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Figure 2 Chondroblastoma in a 39-year-old man. Anteroposterior (a) and lateral (b) radiographs and axial (c) and sagittal (d) CT images show a well-defined expansile lytic lesion with a scalloped cortical border in the olecranon. There is an associated pathological fracture. The lesion demonstrates intermediate signal intensity on the axial T1-weighted MRI image (e). It is hyperintense on the sagittal STIR image (f) with extensive surrounding soft-tissue oedema.

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Figure 3 Osteoid osteoma in a 17-year-old male patient. Anteroposterior (a) and lateral (b) radiographs show diffuse sclerosis with a focal lucent area within the olecranon. Coronal (c) and sagittal (d) CT images show a well-defined lucent nidus with central mineralization within a background of diffuse sclerosis. Coronal STIR MRI image (e) shows bone marrow and soft-tissue oedema surrounding the nidus, which is isointense to muscle on the T1-weighted fat-saturated image (f).

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Figure 4 Fibrous dysplasia in a 45-year-old man. Coronal (a) and sagittal (b) CT images show a well-defined ground-glass lucency with a sclerotic margin. Coronal T1-weighted (c) and proton density fat-saturated (d), axial T1-weighted (e) and sagittal T2-weighted (f) MRI images show a well-defined homogeneous lesion surrounded by a sclerotic margin. It has intermediate to low signal intensity in T1- and T2-weighted sequences.

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Figure 5 Chondrosarcoma in an 82-year-old man. Lateral (a) and anteroposterior (b) radiographs show a fracture through a lytic lesion with popcorn-like calcification within it and in the adjacent soft tissue. Sagittal T1-weighted MRI image (c) shows an intermediate signal lobular mass. T2-weighted image (d) shows areas of low signal intensity representing matrix mineralization within a high signal intensity lesion.

Solitary plasmacytoma Solitary plasmacytoma of the bone (Fig 6) is a local primary bone tumour consisting of malignant plasma cells without systemic involvement. It is considered to represent the early stage of multiple myeloma.18 It occurs most frequently in patients in their 60s with a male predilection of 2e3:1.19 Occurrence is most frequently in the bones of the axial skeleton such as vertebra and skull.20 In a large series, out of 1057 cases of multiple myeloma, only four involved the ulna.2 The literature search revealed one reported case of plasmacytoma in the olecranon.21 Radiographically, a large expansile lytic lesion is seen. Apparent trabeculation and “soap-bubble” appearance is common (Fig 6aeb). T1-weighted MRI images generally depict a focal plasmacytoma as a hypointense area. On T2-weighted and short tau inversion recovery (STIR) images, focal lesions are hyperintense.22

each of adamantinoma,23 malignant melanoma,24 synovial sarcoma,25 and osteosarcoma.26

Metastatic disease Metastatic disease of the bone occurs more commonly than do primary sarcomas of bone.27 Cancers that most commonly metastasise to bone are lung, breast, renal cell, thyroid, and prostate carcinomas. Metastasis to the olecranon is extremely rare in all cancers. Three cases have been reported in the English literature where the primary cancers were renal cell carcinoma and multiple myeloma.28 The present series includes a case of a mucin-producing adenocarcinoma of the colon metastasizing to the olecranon (Fig 7). Most metastatic lesions are osteolytic but many deposits from carcinoma of the prostate and occasionally others are osteoblastic.2

Non-neoplastic lesions

Other primary malignancies

Paget’s disease

Other primary malignancies occurring in the olecranon revealed by the literature review are one reported case

Paget’s disease (Fig 8) is a chronic skeletal disorder characterized by abnormal and excessive remodelling of

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Figure 6 Plasmacytoma in a 45-year-old woman. Anteroposterior (a) and lateral (b) radiographs show a “bubbly” expansile lesion in the olecranon. Axial T1-weighted MRI image (c) shows intermediate signal areas within the lesion due to post-radiotherapy changes. Sagittal STIR image (d) shows diffuse hyperintensity within the lesion.

bone. It affects approximately 3% of the European population and more than 90% of patients are older than 40 years of age. There is a male predilection of 1.5:1.29 Axial skeleton and proximal long bones, particularly the femur (25e35% of cases) are commonly affected. There is a preference for the lower extremities.30 Monostotic disease accounting for 10e35% of cases predominates in the axial skeleton. Involvement of the ribs, fibula, and small bones in the hands and feet is infrequent.31 The literature review revealed two cases affecting the proximal ulna.32,33 Radiographic features depend on the stage of the disorder. A flame-shaped radiolucency in the osteolytic phase, thickening of the cortex and coarse trabeculation in the mixed phase and diffuse increase of bone density with enlargement and widening of the bone in the sclerotic phase are observed. CT demonstrates classic findings including osteolysis, trabecular coarsening, cortical thickening, and osseous expansion to better advantage. MRI signal intensity characteristics are variable. In long-standing disease, signal intensity similar to that of fat is noted. In the early mixed active phase, low T1 and high T2 signal intensity is seen. Low signal intensity on

both T1 and T2-weighted images is seen in the late blastic inactive phase.31

Tuberculous infections Tuberculous infections (Fig 9) manifest as musculoskeletal disease in 1e3% of cases, primarily involving the axial skeleton.34 Although occurrence within the olecranon has rarely been reported,35,36 in a study of 38 cases of elbow involvement, the most common site of osseous lesion was the olecranon followed by the lateral epicondyle.37 Plain radiography may show soft-tissue swelling, minimal periosteal reaction, osteolysis, periarticular osteoporosis, and erosions. MRI demonstrates marrow changes as low and high signal intensity on T1-weighted and T2-weighted images respectively with contrast enhancement. The nonspecificity of imaging findings renders tuberculosis to be a mimic of other disease processes.34 Involvement of the olecranon by other opportunistic Mycobacteria species has been reported in the immunocompromised.38

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Figure 7 Metastasis from a mucin-producing adenocarcinoma of the colon in a 56-year-old woman. Anteroposterior (a) and lateral (b) radiographs and CT images obtained in a slightly oblique coronal (c) and sagittal (d) plane show permeative change within the olecranon. Heterogeneous low signal intensity on the sagittal T1-weighted MRI image (e) and high signal intensity on the STIR image (f) represent diffuse infiltration within the olecranon.

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Figure 8 Paget’s disease in a 61-year-old man. Anteroposterior (a) and lateral (b) radiographs show cortical thickening and trabecular coarsening within the olecranon. Axial (c) and coronal (e) T1-weighted MRI images show thickening of the ulnar cortex. Patchy areas of decreased signal intensity within the marrow represent fibrovascular connective tissue. Axial (d) and coronal (f) STIR images show high signal intensity in the corresponding areas.

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Figure 9 Tuberculous osteomyelitis in a 39-year-old woman. Anteroposterior (a) and lateral (b) radiographs show a poorly defined lucent lesion in the olecranon. Pre-contrast axial (c) and post-contrast axial (d), coronal (e), and sagittal (f) T1-weighted fat-saturated MRI images show an illdefined lesion. There is diffuse medullary enhancement post-contrast medium administration.

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Figure 10 Haemophilic pseudotumour in a 26-year-old man. Anteroposterior (a) and lateral (b) radiographs show a multiloculate lytic lesion with endosteal scalloping in the olecranon. Axial (c) and sagittal (d) CT images show an intramedullary cystic expansile lesion. Superimposed degenerative changes are present in the elbow joint.

Haemophilia Haemophilia has bone and joint changes among its most common manifestations.39 Pseudotumours are rare complications as a result of recurrent haemorrhage, occurring in 1e2% of patients with severe cases of haemophilia.40 The bones most commonly involved are the femur, pelvis, tibia, and bones of the hand.41 The literature search revealed one reported case of occurrence in the olecranon42 and one within the proximal ulna.43 Radiographically, they are welldefined, lobulated, expansile, lytic lesions, which may demonstrate endosteal scalloping, cortical thinning, and pathological fractures (Fig 10).44 Trabeculae frequently extend across the osteolytic lesions (Fig 10b). Radiographic findings vary with extent, location, and different stages of haemorrhages, and hence cause confusion with other tumourous or infectious conditions. CT is useful for evaluation of crossing trabeculae, cortical change and periosteal reaction (Fig 10d). The characteristic MRI appearance is an intramedullary cystic lesion with fluid components that have complex signal intensities reflecting the effects of recurrent haemorrhage.40 Haemosiderin deposits can be

identified with gradient-echo sequences, showing markedly low-signal.44

Geodes Geodes (Fig 11) are subarticular cystic lesions, often found during the course of rheumatoid arthritis. The sites most commonly affected are the tibial plateau and femoral neck.45 There are only a few cases describing occurrence in the olecranon in the literature.45e49 Large geodes may be confused with other destructive lesions that develop close to joints.50 Radiographs show a cystic lesion with a sclerotic margin. CT may demonstrate communication with the joint. MRI images show a lesion following fluid signal in all sequences.

Conclusion Lesions in the olecranon are rare. The spectrum of tumours and tumour-like lesion that can involve the olecranon is wide. Hence, the differentiation of benign from malignant lesion must be based on a combination of the clinical, imaging, and

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Figure 11 Geode in a 49-year-old man. Anteroposterior (a) and lateral (b) radiographs show a cystic lesion with a sclerotic margin. There is marked arthropathy within the elbow joint with loose body formation. (c) Axial T1-, (d) axial T2-, (e) coronal T1-, and (f) sagittal T2-weighted MRI images show a lesion following fluid signal in all sequences.

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pathological findings and is best done in a multidisciplinary meeting. Biopsy is not invariably required, and is preferably performed at a specialist tumour unit.

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Please cite this article in press as: Kularatne U, et al., Tumours and tumour mimics in the olecranon, Clinical Radiology (2015), http://dx.doi.org/ 10.1016/j.crad.2015.02.019