Localized tenosynovial giant cell tumor
Updates to Article Attributes
Giant cell tumours of the tendon sheath (GCTTS), also known as pigmented villonodular tumour of the tendon sheath (PVNTS) or extra-articular pigmented villonodular tumor of the tendon sheath, are uncommon and usually benign lesions that arise from the tendon sheath. It is unclear whether these lesions represent neoplasms or simply reactive masses2
It is also knownOn imaging these lesions are commonly demonstrated aspigmented villonodular tumour of the tendon sheath (PVNTS) or extra-articular pigmented villonodular tumor of the tendon sheath4
Epidemiology
Typically, they present in 3rd-5thdecades and have a slight female predilection with a M:F ratio of 1.5-2.1:1 4.
Clinical presentation
Clinically these masses typically present in the hand (although they are found elsewhere also) with localised swelling with or without pain. They are slow growing.
Pathology
They have been divided macroscopically into localised or diffuse forms, and appear as rubbery multinodular masses that are well circumscribed. They have an enveloping fibrous capsule, and the cut surface is variably coloured depending on the relative proportions of fibrous tissue, haemosiderin and pigmented foam cells 2.
The tumour is histologically identical to pigmented villonodular synovitis (PVNS) and is composed of fibroblasts and multinucleated giant cells, foamy histiocytes and inflammatory cells on a background fibrous matrix 1-2.
Radiographic features
Radiography
As these masses arise from tendons, commonly of the hand, they may cause pressure erosions on the underlying bone. This is only seen in 10-20% of cases. More commonly these masses arise from the palmar tendons. The mass itself is of soft tissue density. Calcification is uncommon 5.
Ultrasound
Ultrasound is useful as it allows not only characterisation of the lesion but also is able to demonstrate the relationship with the adjacent tendon. On dynamic scan, there is free movement of the tendon within the lesion (case 5).
- typically associated with the volar surface of the digits
- does not move with flexion or extension of adjacent tendons
- usually homogeneously hypoechoic, although some heterogeneity may be seen in echotexture in a minority of cases 1
- most will have some internal vascularity.
MRI
Not surprisingly, given the histological similarity to PVNS, giant cell tumours of the tendon sheaths also share the same finding on MRI, mainly on account of hemosiderin accumulation.
Signal characteristics include:
-
T1
- low signal
- variable enhancement
- T2: low signal
- T1 C+ (Gd): often show moderate enhancement 6
Treatment and prognosis
Local surgical excision usually suffices, with local recurrence (seen in 10-20% of cases) requiring more extensive surgery with or without radiotherapy being uncommon 1.
Differential diagnosis
General imaging differential considerations include:
-
ganglion cyst
- cystic component
- if previously ruptured may appear similar 1
-
pignented villonodular synovitis (PVNS)
- histologically identical
- involves larger joints
- desmoid tumour
- fibroma/fibrosarcoma
If in the hand consider:
- glomangioma: has high T2 signal on MRI
-<p><strong>Giant cell tumours of the tendon sheath (GCTTS)</strong> are uncommon and usually benign lesions that arise from the <a href="/articles/tendon-sheath">tendon sheath</a>. It is unclear whether these lesions represent neoplasms or simply reactive masses <sup>2</sup>.</p><p>It is also known as <strong>pigmented villonodular tumour of the tendon sheath (PVNTS) </strong>or<strong> extra-articular pigmented villonodular tumor of the tendon sheath</strong> <sup>4</sup>. </p><h4>Epidemiology </h4><p>Typically, they present in 3<sup>rd</sup>-5<sup>th</sup>decades and have a slight female predilection with a M:F ratio of 1.5-2.1:1 <sup>4</sup>.</p><h4>Clinical presentation </h4><p>Clinically these masses typically present in the hand (although they are found elsewhere also) with localised swelling with or without pain. They are slow growing. </p><h4>Pathology</h4><p>They have been divided macroscopically into localised or diffuse forms, and appear as rubbery multinodular masses that are well circumscribed. They have an enveloping fibrous capsule, and the cut surface is variably coloured depending on the relative proportions of fibrous tissue, haemosiderin and pigmented foam cells <sup>2</sup>.</p><p>The tumour is histologically identical to <a href="/articles/pigmented-villonodular-synovitis">pigmented villonodular synovitis (PVNS)</a> and is composed of fibroblasts and multinucleated giant cells, foamy histiocytes and inflammatory cells on a background fibrous matrix <sup>1-2</sup>. </p><h4>Radiographic features</h4><h5>Radiography</h5><p>As these masses arise from tendons, commonly of the hand, they may cause pressure erosions on the underlying bone. This is only seen in 10-20% of cases. More commonly these masses arise from the palmar tendons. The mass itself is of soft tissue density. Calcification is uncommon <sup>5</sup>. </p><h5>Ultrasound</h5><p>Ultrasound is useful as it allows not only characterisation of the lesion but also is able to demonstrate the relationship with the adjacent tendon. On dynamic scan, there is free movement of the tendon within the lesion (case 5).</p><ul>- +<p><strong>Giant cell tumours of the tendon sheath (GCTTS)</strong>, also known as <strong>pigmented villonodular tumour of the tendon sheath (PVNTS) </strong>or<strong> extra-articular pigmented villonodular tumor of the tendon sheath</strong>, are uncommon and usually benign lesions that arise from the <a href="/articles/tendon-sheath">tendon sheath</a>. It is unclear whether these lesions represent neoplasms or simply reactive masses.</p><p>On imaging these lesions are commonly demonstrated as localized, solitary, subcutaneous soft tissue nodules, with low T1 and T2 signal and moderate enhancement. </p><h4>Epidemiology </h4><p>Typically, they present in 3<sup>rd</sup>-5<sup>th</sup>decades and have a slight female predilection with a M:F ratio of 1.5-2.1:1 <sup>4</sup>.</p><h4>Clinical presentation </h4><p>Clinically these masses typically present in the hand (although they are found elsewhere also) with localised swelling with or without pain. They are slow growing. </p><h4>Pathology</h4><p>They have been divided macroscopically into localised or diffuse forms, and appear as rubbery multinodular masses that are well circumscribed. They have an enveloping fibrous capsule, and the cut surface is variably coloured depending on the relative proportions of fibrous tissue, haemosiderin and pigmented foam cells <sup>2</sup>.</p><p>The tumour is histologically identical to <a href="/articles/pigmented-villonodular-synovitis">pigmented villonodular synovitis (PVNS)</a> and is composed of fibroblasts and multinucleated giant cells, foamy histiocytes and inflammatory cells on a background fibrous matrix <sup>1-2</sup>. </p><h4>Radiographic features</h4><h5>Radiography</h5><p>As these masses arise from tendons, commonly of the hand, they may cause pressure erosions on the underlying bone. This is only seen in 10-20% of cases. More commonly these masses arise from the palmar tendons. The mass itself is of soft tissue density. Calcification is uncommon <sup>5</sup>. </p><h5>Ultrasound</h5><p>Ultrasound is useful as it allows not only characterisation of the lesion but also is able to demonstrate the relationship with the adjacent tendon. On dynamic scan, there is free movement of the tendon within the lesion (case 5).</p><ul>
- +<li>
- +<strong>T1 C+ (Gd):</strong> often show moderate enhancement<sup> 6</sup>
- +</li>
References changed:
- 6. Bassetti E, Candreva R, Santucci E. Giant Cell Tumor of the Flexor Tendon of the Wrist: US and MRI Evaluation. Case Report. J Ultrasound. 2011;14(1):37-9. <a href="https://doi.org/10.1016/j.jus.2010.12.001">doi:10.1016/j.jus.2010.12.001</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23396659">Pubmed</a>
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