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 tumortumour 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 simplymerely reactive masses.
On imaging these lesions are commonly demonstrated as localised, solitary, subcutaneous soft tissue nodules, with low T1 and T2 signal and moderate enhancement.
Epidemiology
Typically, they present in 3rd-5thdecades and have a slight female predilection with aan M:F ratio of 1.5-2.1:1 4.
Clinical presentation
Clinically these masses typicallygenerally present in the hand (although they are found elsewhere also) with localised swelling with or without pain. They are slow growing.
Pathology
GCTTS can cause pressure erosion onof adjacent bone, or rarely can invade the bone mimicking an intraosseous lesion 8.
Macroscopic appearance
GCTTS has 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.
Histology
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
Plain radiographs
As these masses arise from tendons, commonly of the hand, they may cause pressure erosions on the underlying bone in 10-20% of cases. More commonly these masses arise from the palmar tendons. The mass itself is of soft tissue density. Periosteal reaction and calcification isare uncommon 4,5.
Ultrasound
Ultrasound is useful as it allows not only the 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. Typically they appear as:
- 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
-
T1
- low signal
- variable enhancement
- T2: low signal
- T1 C+ (Gd): often show moderate enhancement 6
- GE: low and may demonstrate blooming
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. Metastases can occur, most commonly to lymph nodes and lung 4.
Differential diagnosis
General imaging differential considerations include:
-
ganglion cyst
- cystic component
- attached to underlying joint capsule or tendon sheath 7
- if previously ruptured may appear similar 1
-
pigmented 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>, 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 localised, 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>GCTTS can cause pressure erosion on adjacent bone, or rarely can invade the bone mimicking an intraosseous lesion <sup>8</sup>. </p><h5>Macroscopic appearance</h5><p>GCTTS has been divided macroscopically into <strong>localised</strong> or <strong>diffuse</strong> 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><h5>Histology</h5><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>Plain radiographs</h5><p>As these masses arise from tendons, commonly of the hand, they may cause pressure erosions on the underlying bone in 10-20% of cases. More commonly these masses arise from the palmar tendons. The mass itself is of soft tissue density. Periosteal reaction and calcification is uncommon <sup>4,5</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. Typically they appear as:</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 tumour 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 merely reactive masses.</p><p>On imaging these lesions are commonly demonstrated as localised, 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 an M:F ratio of 1.5-2.1:1 <sup>4</sup>.</p><h4>Clinical presentation </h4><p>Clinically these masses generally 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>GCTTS can cause pressure erosion of adjacent bone, or rarely can invade the bone mimicking an intraosseous lesion <sup>8</sup>. </p><h5>Macroscopic appearance</h5><p>GCTTS has been divided macroscopically into <strong>localised</strong> or <strong>diffuse</strong> 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><h5>Histology</h5><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>Plain radiographs</h5><p>As these masses arise from tendons, commonly of the hand, they may cause pressure erosions on the underlying bone in 10-20% of cases. More commonly these masses arise from the palmar tendons. The mass itself is of soft tissue density. Periosteal reaction and calcification are uncommon <sup>4,5</sup>. </p><h5>Ultrasound</h5><p>Ultrasound is useful as it allows not only the 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. Typically they appear as:</p><ul>