Giant cell tumor of soft tissue
Updates to Article Attributes
Giant cell tumours of soft tissue are fibrohistiocytic neoplasms of uncertain behaviour that show morphological similarities to giant cell tumour of bone but are not related genetically.
Terminology
Giant cell tumour of low malignant potential is a term that should not be used any longer1.
Epidemiology
Giant cell tumours of soft tissue are very rare and seen in a wide range of age with a peak incidence in the 5th decade. There is no predilection for gender or ethnicity 1,2.
Clinical features
The tumours often present as indolent slowly-growing mass 1.
Pathology
Giant cell tumours of soft tissue are multi nodularmultinodular soft tissue neoplasms characterised by mononuclear histiocytoid cells and osteoclast-like giant cells frequently with metaplastic bone formation 1-3.
Aetiology
The aetiology of giant cell tumour of soft tissue is unknown 1.
Location
About 70% of the tumours affect the subcutaneous fatty tissue and the skin, approximately 30% involve the deep soft tissues underneath the superficial fascia. Most giant cell tumours of soft tissue are located in the upper and lower limbs and a lesser number is situated in the trunk or thehead and neck area 1.
Macroscopic appearance
Macroscopically giant cell tumours of soft tissue are solid well-demarcated nodular lesions with a brown-reddish to a grey colour. Many tumours feature peripheral gritty regions of mineralised bone 1-3.
Microscopic appearance
The microscopic spectrum of giant cell tumours of soft tissue includes the following features 1-3:
- multinodular architecture with cellular nodules separated by sclerotic septa with sideritic components
-
mixture ofhistiocytoid mononuclear cellsandwith interposed multinucleated osteoclast-like giant cells - stroma with abundant vascularity
- no nuclear pleomorphism
- rare necrosis
- osseous metaplasia in about half of the tumours
- commonly stromal haemorrhage and regressive change
Immunohistochemistry
Immunohistochemistry stains might express vimentin, CD68, smooth muscle actin or alkaline phosphatase. However, they do not seem to be particularly helpful for the diagnosis 1.
Genetics
Different to giant cell tumour of bone mutations of the H3-3A (H3F3A) gene are typically absent 1,2.
Radiographic features
There are only a few reports where imaging features of giant cell tumours of soft tissue have been described, they include the following 3,4:
- cystic lesions with internal nodules or debris
- possible daughter cysts
- adjacent nondescript soft tissue changes
Ultrasound
Ultrasound appearances have been described as cystic with an internal hyperechoic solid nodule possibly associated with daughter cysts and internal debris 3,4.
CT
CT appearance has been described as non-specific soft tissue mass with avid enhancement 4.
MRI
Cystic lesions with fluid-fluid levels and differences in signal intensity as well as internal or adjacent solid portions or nodules have been reported as imaging features. Cyst wall and solid portions of the tumours have been described with the following signal characteristics 3,4:
- T1: low to intermediate signal intensity
- T2: low to intermediate signal intensity
- T2FS/STIR: high signal
- T1 C+ (Gd): avid enhancement of cyst wall and solid portions
Radiology report
The radiological report should include a description of the following featurespoints:
- form, location and size
- tumour margins
- relation to the muscular fascia
- relationship to bones, tendons and joints
- relationship to local nerves and vessels
Treatment and prognosis
The common management is surgical excision. Local recurrence is uncommon and occurs in about 12%. Metastases are very rare 1.
History and etymology
Giant cell tumour of soft tissue was apparently first reported in 1972 by R Salm and HA Sissons 3,5.
Differential diagnosis
The differential diagnosis of giant cell tumours of soft tissue are the following 3,4:
-<p><strong>Giant cell tumours of soft tissue </strong>are fibrohistiocytic neoplasms of uncertain behaviour that show morphological similarities to giant cell tumour of bone but are not related genetically.</p><p>Terminology</p><p><strong>Giant cell tumour of low malignant potential</strong> is a term that should not be used any longer.</p><p>Epidemiology</p><p>Giant cell tumours of soft tissue are very rare and seen in a wide range of age with a peak incidence in the 5th decade. There is no predilection for gender or ethnicity 1,2.</p><p>Clinical features</p><p>The tumours often present as indolent slowly-growing mass 1.</p><p>Pathology</p><p>Giant cell tumours of soft tissue are multi nodular soft tissue neoplasms characterised by mononuclear histiocytoid cells and osteoclast-like giant cells frequently with metaplastic bone formation 1-3.</p><p>Aetiology</p><p>The aetiology of giant cell tumour of soft tissue is unknown 1.</p><p>Location</p><p>About 70% of the tumours affect the subcutaneous fatty tissue and the skin, approximately 30% involve the deep soft tissues underneath the superficial fascia. Most giant cell tumours of soft tissue are located in the upper and lower limbs and a lesser number is situated in the trunk or the head and neck area 1. </p><p>Macroscopic appearance</p><p>Macroscopically giant cell tumours of soft tissue are solid well-demarcated nodular lesions with a brown-reddish to grey colour. Many tumours feature peripheral gritty regions of mineralised bone 1-3. </p><p>Microscopic appearance</p><p>The microscopic spectrum of giant cell tumours of soft tissue includes the following features 1-3:</p><ul>- +<p><strong>Giant cell tumours of soft tissue </strong>are fibrohistiocytic neoplasms of uncertain behaviour that show morphological similarities to <a title="Giant cell tumour of bone" href="/articles/giant-cell-tumour-of-bone">giant cell tumour of bone</a> but are not related genetically.</p><h4>Terminology</h4><p><strong>Giant cell tumour of low malignant potential</strong> is a term that should not be used any longer <sup>1</sup>.</p><h4>Epidemiology</h4><p>Giant cell tumours of soft tissue are very rare and seen in a wide range of age with a peak incidence in the 5th decade. There is no predilection for gender or ethnicity <sup>1,2</sup>.</p><h4>Clinical features</h4><p>The tumours often present as indolent slowly-growing mass <sup>1</sup>.</p><h4>Pathology</h4><p>Giant cell tumours of soft tissue are multinodular soft tissue neoplasms characterised by mononuclear histiocytoid cells and osteoclast-like giant cells frequently with metaplastic bone formation <sup>1-3</sup>.</p><h5>Aetiology</h5><p>The aetiology of giant cell tumour of soft tissue is unknown <sup>1</sup>.</p><h5>Location</h5><p>About 70% of the tumours affect the subcutaneous fatty tissue and the skin, approximately 30% involve the deep soft tissues underneath the superficial fascia. Most giant cell tumours of soft tissue are located in the upper and lower limbs and a lesser number is situated in the trunk or the head and neck area <sup>1</sup>. </p><h5>Macroscopic appearance</h5><p>Macroscopically giant cell tumours of soft tissue are solid well-demarcated nodular lesions with a brown-reddish to a grey colour. Many tumours feature peripheral gritty regions of mineralised bone <sup>1-3</sup>. </p><h5>Microscopic appearance</h5><p>The microscopic spectrum of giant cell tumours of soft tissue includes the following features <sup>1-3</sup>:</p><ul>
-<li>mixture of histiocytoid mononuclear cells and multinucleated osteoclast-like giant cells </li>- +<li>histiocytoid mononuclear cells with interposed multinucleated osteoclast-like giant cells </li>
-</ul><p>Immunohistochemistry</p><p>Immunohistochemistry stains might express vimentin, CD68, smooth muscle actin or alkaline phosphatase. However they do not seem to be particularly helpful for the diagnosis 1.</p><p>Genetics</p><p>Different to giant cell tumour of bone mutations of the <em>H3-3A</em> (<em>H3F3A</em>) gene are typically absent 1,2.</p><p>Radiographic features</p><p>There are only few reports where imaging features of giant cell tumours of soft tissue have been described, they include the following 3,4:</p><ul>- +</ul><h5>Immunohistochemistry</h5><p>Immunohistochemistry stains might express vimentin, CD68, smooth muscle actin or alkaline phosphatase. However, they do not seem to be particularly helpful <sup>1</sup>.</p><h5>Genetics</h5><p>Different to giant cell tumour of bone mutations of the <em>H3-3A</em> (<em>H3F3A</em>) gene are typically absent <sup>1,2</sup>.</p><h4>Radiographic features</h4><p>There are only a few reports where imaging features of giant cell tumours of soft tissue have been described, they include the following <sup>3,4</sup>:</p><ul>
-</ul><p>Ultrasound</p><p>Ultrasound appearances have been described as cystic with an internal hyperechoic solid nodule possibly associated with daughter cysts and internal debris 3,4.</p><p>CT</p><p>CT appearance has been described as non-specific soft tissue mass with avid enhancement 4. </p><p>MRI</p><p>Cystic lesions with fluid-fluid levels and differences in signal intensity as well as internal or adjacent solid portions or nodules have been reported as imaging features. Cyst wall and solid portions of the tumours have been described with the following signal characteristics 3,4:</p><ul>- +</ul><h5>Ultrasound</h5><p>Ultrasound appearances have been described as cystic with an internal hyperechoic solid nodule possibly associated with daughter cysts and internal debris <sup>3,4</sup>.</p><h5>MRI</h5><p>Cystic lesions with fluid-fluid levels and differences in signal intensity as well as internal or adjacent solid portions or nodules have been reported as imaging features. Cyst wall and solid portions of the tumours have been described with the following signal characteristics <sup>3,4</sup>:</p><ul>
-</ul><p>Radiology report</p><p>The radiological report should include a description of the following features:</p><ul>- +</ul><h4>Radiology report</h4><p>The radiological report should include a description of the following points:</p><ul>
-</ul><p>Treatment and prognosis</p><p>The common management is surgical excision. Local recurrence is uncommon and occurs in about 12%. Metastases are very rare 1.</p><p>History and etymology</p><p>Giant cell tumour of soft tissue was first reported in 1972 by R Salm and HA Sissons 3,5.</p><p>Differential diagnosis</p><p>The differential diagnosis of giant cell tumours of soft tissue are the following 3:</p><p> </p><ul>-<li>haematoma</li>- +</ul><h4>Treatment and prognosis</h4><p>The common management is surgical excision. Local recurrence is uncommon and occurs in about 12%. Metastases are very rare <sup>1</sup>.</p><h4>History and etymology</h4><p>Giant cell tumour of soft tissue was apparently first reported in 1972 by R Salm and HA Sissons <sup>3,5</sup>.</p><h4>Differential diagnosis</h4><p>The differential diagnosis of giant cell tumours of soft tissue are the following <sup>3,4</sup>:</p><ul>
- +<li><a href="/articles/haematoma">haematoma</a></li>
-<li>malignant fibrous histiocytoma</li>-<li>giant cell tumour of tendon sheath</li>-<li>plexiform fibrous histiocytoma</li>-<li>undifferentiated pleomorphic sarcoma</li>- +<li><a href="/articles/undifferentiated-pleomorphic-sarcoma-1">malignant fibrous histiocytoma</a></li>
- +<li><a href="/articles/tenosynovial-giant-cell-tumour">tenosynovial giant cell tumour</a></li>
- +<li><a href="/articles/plexiform-fibrohistiocytic-tumour">plexiform fibrohistiocytic tumour</a></li>
- +<li><a href="/articles/undifferentiated-pleomorphic-sarcoma-1">undifferentiated pleomorphic sarcoma</a></li>
References changed:
- 1. Oliveira AM, Lee JC. Giant cell tumour of soft tissue. In: WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Lyon (France): International Agency for Research on Cancer; 2020. (WHO classification of tumours series, 5th ed.; vol. 3). <a href="https://publications.iarc.fr/Book-And-Report-Series/Who-Classification-Of-Tumours/Soft-Tissue-And-Bone-Tumours-2020">https://publications.iarc.fr</a>
- 2. Lee J, Liang C, Fletcher C. Giant Cell Tumor of Soft Tissue is Genetically Distinct from Its Bone Counterpart. Mod Pathol. 2017;30(5):728-33. <a href="https://doi.org/10.1038/modpathol.2016.236">doi:10.1038/modpathol.2016.236</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28084336">Pubmed</a>
- 3. Chopra A, Kinsella M, Edwards S, Smith I, Robinson P. Giant Cell Tumour of Soft Tissue—a Rare Presentation of a Common Pathology. BJR|case Reports. 2020;6(3):20200012. <a href="https://doi.org/10.1259/bjrcr.20200012">doi:10.1259/bjrcr.20200012</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/32922842">Pubmed</a>
- 4. An S, Choi J, Chung J, Oh J, Kang H. Giant Cell Tumor of Soft Tissue: A Case with Atypical US and MRI Findings. Korean J Radiol. 2008;9(5):462. <a href="https://doi.org/10.3348/kjr.2008.9.5.462">doi:10.3348/kjr.2008.9.5.462</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18838857">Pubmed</a>
- 5. Salm R & Sissons H. Giant-Cell Tumours of Soft Tissues. J Pathol. 1972;107(1):27-39. <a href="https://doi.org/10.1002/path.1711070106">doi:10.1002/path.1711070106</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/4262633">Pubmed</a>
Systems changed:
- Musculoskeletal
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