A stiff hip: Multi-modality imaging of tumoral calcinosis

Clinical Cases 19.07.2022
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 40 years, male
Authors: Andrea Bellizzi1, Carlene Bellizzi1, Dr Gabriel Galea2, Dr Jonathan L Portelli3
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Details
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AI Report

Clinical History

A 40-year-old male with end-stage kidney disease (ESKD) presented following a history of a few months of increasing painless swelling of the left hip.

Imaging Findings

An initial plain radiograph (Figure 1) showed significant heterotopic calcification overlying the left hip joint.  A non-enhanced CT of the left hip (Figure 2) confirmed a large, multilobulated, periarticular calcific mass containing fluid-fluid levels.  A sedimentation sign was also present (Figure 3).  There was no soft tissue erosion. 

An MRI of the pelvis (Figure 4) was performed, demonstrating encapsulated and well-circumscribed soft tissue masses around both hip joints together with a small amount of surrounding fluid.  There was no additional soft tissue component.  The masses were of homogeneous low signal intensity on T1-weighted imaging and of mixed-signal intensity on T2 weighted imaging with internal fluid-fluid levels mirroring the CT findings of sedimentation sign.  Intravenous gadolinium could not be administered due to the impaired renal function.

18F-Fludeoxyglucose positron emission tomography (FDG-PET)/CT imaging was eventually performed (Figure 5), showing significant tracer uptake by the calcified masses in both hip joints.

Discussion

Background

Tumoral calcinosis (TC) is a rare clinical and histopathologic syndrome characterized by calcium salt deposition in different periarticular soft tissue regions [1]. The term TC should strictly be used to refer to a disease caused by metabolic dysfunction of phosphate regulation associated with massive periarticular calcinosis [2]. 

Clinical Perspective

The clinical presentation depends on multiple factors including family history, renal function and the size of the masses [3]. TC typically presents as painless, firm, tumour-like masses around the joints [4].  Such mass is periarticular, is mainly located at the extensor surface of the joint, and in the anatomic distribution of a bursa.  Commonly affected areas include the hip, elbow, shoulder, foot, and wrist.  

Imaging Perspective

Radiographs in TC have fairly typical appearances showing multilobulated and often large foci of heterotopic calcification. 

CT imaging shows calcific masses of various sizes. Fluid levels and the typical sedimentation sign are usually clearly identifiable on CT [3].  The added value of CT is the definitive exclusion of bony destruction which should not be seen in TC. 

MR imaging of TC usually shows two distinct patterns.  The first pattern is where the TC masses manifest as diffuse low signal intensity masses.  The second pattern occasionally referred to as the bright nodular pattern, is more heterogeneous with alternating areas of high signal intensity and signal voids, sometimes seen as fluid levels. Post-contrast imaging usually shows avid enhancement of the internal septae and capsule due to a mixture of connective tissue and fibrosis [5].

Imaging of TC using FDG-PET/CT has been rarely described in the literature with only a handful of case reports existing. Similar to this case, TC masses usually show mild to moderate uptake depending on the stage of the TC mass. Our patient’s TC mass continued to increase in size over a 1-year follow-up suggesting that they were metabolically active which would fit with the increased avidity seen on FDG-PET/CT.

Bone marrow involvement is also possible and can be demonstrated as periosteal reaction on radiographs and increased radionuclide uptake at bone scintigraphy [3].

Outcome

When TC masses are asymptomatic, observation or conservative measures like physiotherapy are often used [5]. However, when the lesion is actively growing or where there is severe limitation of the joint function, surgical excision can be performed [4].

Take-Home Message / Teaching Points

Tumoral calcinosis is a benign condition occurring due to metabolic phosphate dysfunction.  Correlation of the imaging findings with the clinical history of the patient is essential to rule out other differentials and correctly identify TC.  FDG avidity of periarticular tumoral masses is possible and should not necessarily be attributed to a malignant process.

Differential Diagnosis List

Tumoral calcinosis
Tophaceous gout
Paraosteal osteosarcoma
Calcinosis universalis
Synovial osteochondromatosis
Calcinosis Circumscripta
Calcific Tendonitis
Myositis Ossificans

Final Diagnosis

Tumoral calcinosis

Figures

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Frontal radiograph of the left hip showing large foci of heterotopic calcification related to the left hip joint and proximal

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An axial image in bone window from an unenhanced CT of the left hip shows a large, multilobulated, periarticular calcific mas

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Axial image from unenhanced CT at a slightly lower level than image 2. This shows ‘shading’ within the lobules that form

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Axial fat-suppressed T2-weighted (above) and T1-weighted images (below) at the same level show bilateral well-circumscribed m
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Axial fat-suppressed T2-weighted (above) and T1-weighted images (below) at the same level show bilateral well-circumscribed m

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Unenhanced axial CT (5A, top left), axial 18F-Fludeoxyglucose positron emission tomography (FDG-PET) (5B, top right), fused c
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Unenhanced axial CT (5A, top left), axial 18F-Fludeoxyglucose positron emission tomography (FDG-PET) (5B, top right), fused c
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Unenhanced axial CT (5A, top left), axial 18F-Fludeoxyglucose positron emission tomography (FDG-PET) (5B, top right), fused c
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Unenhanced axial CT (5A, top left), axial 18F-Fludeoxyglucose positron emission tomography (FDG-PET) (5B, top right), fused c