Solitary fibrous tumor

Changed by Henry Knipe, 23 Mar 2015

Updates to Article Attributes

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solitarySolitary fibrous tumour tumours (SFT)  is aare a rare neoplasm of mesenchymal origin that comprises less thancomprise < 2% of  allall soft tissue tumours tumours. The majority are benign The majority are benign, although up to 20% may be malignant.7

Epidemiology

Intrathoracic SFT usually presents in the  6th to 7th decades. Extrathoracic SFT occurs in men and women of all ages equally.7.

Clinical presentation

Although typically asymptomatic, it  may  present with symptoms and signs related to extrinsic compression of  adjacent organs, or occasionally hypoglycemia4-7.

Pathology

Location

The majority of solitary fibrous tumours occur in an intrathoraciclocation but up to one third may occur in extrathoracic locations, and as such they may be encountered essentially anywhere, including 7:

Clinical presentation

Although typically asymptomatic, it  may  present with  symptoms  and signs related to extrinsic compression of  adjacent organs, or occasionally hypoglycemia.4-7

Pathology

Histology

SFT most likely arises from submesothelial fibroblasts and is similar to a haemangiopericytoma.  

At histology, a collagenous matrix with  arrays of spindle cells is usually seen. Areas of necrosis, cystic or myxoid change, calcification, hemorrhage, increased vascularity, atypia, or malignanry may also be seen.7-8.

RaliographicRadiographic features

CT

CT  reveals a  wellwell-circumscribed,  smoothsmooth, lobulated soft tissue mass that may contain scattered calcifications. Smaller tumors tend to enhance homogeneously, whereas larger lesions may have central tubular or rounded low-attenuation areas due to cystic or necrotic change.

MRI

On MRI, benign  solitary fibrous tumours usually has relatively homogeneous low  to  intermediate-to-intermediate signal intensity  relativerelative to skeletal muscle on  bothboth  T1-weighted imaging and T2-weighted imaging because of fibrous tissue, as well as intense enhancement. 

In addition, there may be areas of subacute haemorrhage that have high T1-weighted signal intensity, as well  as nonenhancingas non-enhancing cystic or necrotic foci that are more heterogeneous and higher in T2-weighted signal intensity relative to  thethe remainder of the tumour.

However, when  a central focus of heterogeneity and variable contrast enhancement is identified in an SFT at CT or MRI, malignant degeneration should be considered.7.

Treatment and prognosis

  • a benign SFT less than 10cm< 10 cm in size typically has a favorable outcome with surgical resection alone.
  • an SFT greater than> 10 cm that also harbors malignant foci, and has positive surgical margins tends to  have a poor prognosis, despite surgical treatment;
    • optimal adjuvant  therapytherapy for the latterthis group is unknown, and close-interval follow-up is advised because there is an increased incidence of local recurrence.
  • -<p>A <strong>solitary fibrous tumour (SFT) </strong> is a rare neoplasm of mesenchymal origin that comprises less than 2% of  all soft tissue tumours. The majority are benign, although up to 20% may be malignant.<sup>7</sup></p><h4>Epidemiology</h4><p>Intrathoracic SFT usually presents in the  6<sup>th</sup> to 7<sup>th</sup> decades. Extrathoracic SFT occurs in men and women of all ages equally.<sup>7</sup></p><h5>Location</h5><p>The majority of solitary fibrous tumours occur in an intrathoracic<strong> </strong>location but up to one third may occur in extrathoracic locations, and as such they may be encountered essentially anywhere, including <sup>7</sup>:</p><ul>
  • +<p><strong>Solitary fibrous tumours (SFT) </strong>are a rare neoplasm of mesenchymal origin that comprise &lt; 2% of all soft tissue tumours. The majority are benign, although up to 20% may be malignant.</p><h4>Epidemiology</h4><p>Intrathoracic SFT usually presents in the  6<sup>th</sup> to 7<sup>th</sup> decades. Extrathoracic SFT occurs in men and women of all ages equally <sup>7</sup>.</p><h4>Clinical presentation</h4><p>Although typically asymptomatic, it  may  present with symptoms and signs related to extrinsic compression of  adjacent organs, or occasionally <a href="/articles/hypoglycemia">hypoglycemia</a> <sup>4-7</sup>.</p><h4>Pathology</h4><h5>Location</h5><p>The majority of solitary fibrous tumours occur in an intrathoracic<strong> </strong>location but up to one third may occur in extrathoracic locations, and as such they may be encountered essentially anywhere, including <sup>7</sup>:</p><ul>
  • -<li>dura (see <a title="solitary fibrous tumour of the dura" href="/articles/solitary-fibrous-tumour-of-the-dura">solitary fibrous tumour of the dura</a>)</li>
  • +<li>dura (see <a href="/articles/solitary-fibrous-tumour-of-the-dura">solitary fibrous tumour of the dura</a>)</li>
  • -<li>abdominal parenchymal organs,  </li>
  • -<li>retroperitoneum</li>
  • -<li>peritoneum  </li>
  • +<li>abdominal parenchymal organs</li>
  • +<li><a href="/articles/retroperitoneum">retroperitoneum</a></li>
  • +<li><a href="/articles/peritoneum">peritoneum</a></li>
  • -</ul><h4>Clinical presentation</h4><p>Although typically asymptomatic, it  may  present with  symptoms  and signs related to extrinsic compression of  adjacent organs, or occasionally <a href="/articles/hypoglycemia">hypoglycemia</a>.<sup>4-7</sup></p><h4>Pathology</h4><h5>Histology</h5><p>SFT most likely arises from submesothelial fibroblasts and is similar to a <a href="/articles/haemangiopericytoma-1">haemangiopericytoma</a>.  </p><p>At histology, a collagenous matrix with  arrays of spindle cells is usually seen.  Areas of necrosis, cystic or myxoid change, calcification, hemorrhage, increased vascularity, atypia, or malignanry may also be seen.<sup>7-8</sup></p><h4>Raliographic features</h4><h5><strong>CT</strong></h5><p>CT  reveals a  well-circumscribed,  smooth,  lobulated soft tissue mass that may contain scattered calcifications. Smaller tumors tend to enhance homogeneously, whereas larger lesions may  have central tubular  or  rounded low-attenuation areas due to cystic or necrotic change.</p><h5><strong>MRI</strong></h5><p>On MRI, benign  solitary fibrous tumours usually has relatively homogeneous low  to  intermediate signal intensity  relative to skeletal muscle on  both  T1-weighted imaging and T2-weighted imaging because of fibrous tissue,  as well as intense enhancement. </p><p>In addition, there may be areas of subacute haemorrhage that have high T1-weighted signal intensity, as well  as nonenhancing cystic or necrotic foci that are more heterogeneous and higher in T2-weighted signal intensity relative to  the remainder of the tumour.</p><p>However, when  a central focus of heterogeneity and variable contrast enhancement is identified in an SFT at CT or MRI, malignant degeneration should be considered.<sup>7</sup></p><h4>Treatment and prognosis</h4><ul>
  • -<li>a benign SFT less than 10cm in size typically has a favorable outcome with surgical resection alone.</li>
  • -<li>an SFT greater than 10 cm that also harbors malignant foci  and has positive surgical margins tends to  have a poor prognosis, despite surgical treatment;  <br>optimal adjuvant  therapy for the latter group is unknown, and close-interval follow-up is advised because there is an increased incidence of local recurrence.</li>
  • +</ul><h5>Histology</h5><p>SFT most likely arises from submesothelial fibroblasts and is similar to a <a href="/articles/haemangiopericytoma-1">haemangiopericytoma</a>.  </p><p>At histology, a collagenous matrix with  arrays of spindle cells is usually seen. Areas of necrosis, cystic or myxoid change, calcification, hemorrhage, increased vascularity, atypia, or malignanry may also be seen <sup>7-8</sup>.</p><h4>Radiographic features</h4><h5><strong>CT</strong></h5><p>CT  reveals a well-circumscribed, smooth, lobulated soft tissue mass that may contain scattered calcifications. Smaller tumors tend to enhance homogeneously, whereas larger lesions may have central tubular or rounded low-attenuation areas due to cystic or necrotic change.</p><h5><strong>MRI</strong></h5><p>On MRI, benign  solitary fibrous tumours usually has relatively homogeneous low-to-intermediate signal intensity relative to skeletal muscle on both  T1-weighted imaging and T2-weighted imaging because of fibrous tissue, as well as intense enhancement. </p><p>In addition, there may be areas of subacute haemorrhage that have high T1-weighted signal intensity, as well as non-enhancing cystic or necrotic foci that are more heterogeneous and higher in T2-weighted signal intensity relative to the remainder of the tumour.</p><p>However, when  a central focus of heterogeneity and variable contrast enhancement is identified in an SFT at CT or MRI, malignant degeneration should be considered <sup>7</sup>.</p><h4>Treatment and prognosis</h4><ul>
  • +<li>benign SFT &lt; 10 cm in size typically has a favorable outcome with surgical resection alone</li>
  • +<li>SFT &gt; 10 cm that also harbors malignant foci, and has positive surgical margins tends to  have a poor prognosis, despite surgical treatment;<ul><li>optimal adjuvant therapy for this group is unknown, and close-interval follow-up is advised because there is an increased incidence of local recurrence</li></ul>
  • +</li>
Images Changes:

Image 1 CT (C+ CTPA) ( update )

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Case 13: pleural SFT of pleura

Image 2 Annotated image ( update )

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