Uterine leiomyosarcoma

Changed by Jeremy Jones, 16 Sep 2014

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Uterine leiomyosarcomas is a are malignant uterine tumour thattumours that arises from the myometrium. They can account for up to one third of uterine sarcomas but only 1.3 % of all uterine cancers 7. The uterus is the commonest location for a leiomyosarcoma.

Epidemiology

Most leiomyosarcomas are thought to arise de novo 5. The incidence of sarcomatous transformation in benign uterine leiomyomas is reported to be 0.1 - 0-0.8% 2. Typically present in women in the 5th decade.

Pathology

Leiomyosarcomas may arise either de novo from uterine musculature or the connective tissue of uterine blood vessels, or in a preexisting leiomyoma.

The pattern of tumour spread is to the myometrium, pelvic blood vessels and lymphatics, contiguous pelvic structures, abdomen, and then distantly, most often to the lungs.

Histology can be similar to leiyomyosarcomas at other sites. A leiomyosarcoma is differentiated histologically from a leiyomyoma by noting the presence of infiltrative margins, nuclear atypia and increased mitotic figures.

Radiographic features

General

The uterus is often massively enlarged.

CT
  • may show irregular central zones of low attenuation, suggesting extensive necrosis 8 and haemorrhage
  • foci of calcification may be present but rare.
MRI

Although it has been suggested that an irregular margin of a uterine leiomyoma on MRI is suggestive of sarcomatous transformation, this is not considered that specific.

Treatment and prognosis

They generally carry a poor prognosis 6.

Differential diagnosis

Consider other uterine masses such as:

  • -<p>A <strong>uterine leiomyosarcoma</strong> is a malignant uterine tumour that arises from the myometrium. They can account for up to one third of <a href="/articles/uterine-sarcoma" title="Uterine sarcoma">uterine sarcomas</a> but only 1.3 % of all uterine cancers <sup>7</sup>. The uterus is the commonest location for a <a href="/articles/leiomyosarcoma" title="Leiomyosarcoma">leiomyosarcoma</a>.</p><h4>Epidemiology</h4><p>Most leiomyosarcomas are thought to arise de novo <sup>5</sup>. The incidence of sarcomatous transformation in benign uterine leiomyomas is reported to be 0.1 - 0.8% <sup>2</sup>. Typically present in women in the 5<sup>th</sup> decade.</p><h4>Pathology</h4><p>Leiomyosarcomas may arise either de novo from uterine musculature or the connective tissue of uterine blood vessels, or in a preexisting leiomyoma. </p><p>The pattern of tumour spread is to the myometrium, pelvic blood vessels and lymphatics, contiguous pelvic structures, abdomen, and then distantly, most often to the lungs.</p><p>Histology can be similar to leiyomyosarcomas at other sites. A leiomyosarcoma is differentiated histologically from a leiyomyoma by noting the presence of infiltrative margins, nuclear atypia and increased mitotic figures.</p><h4>Radiographic features</h4><h5>General</h5><p>The uterus is often massively enlarged. </p><h5>CT</h5><ul>
  • -<li>may show irregular central zones of low attenuation, suggesting extensive necrosis <sup>8</sup> and haemorrhage</li>
  • -<li>foci of calcification may be present but rare. </li>
  • -</ul><h5>MRI</h5><p>Although it has been suggested that an irregular margin of a uterine leiomyoma on MRI is suggestive of sarcomatous transformation, this is not considered that specific.</p><h4>Treatment and prognosis</h4><p>They generally carry a poor prognosis <sup>6</sup>.</p><h4>Differential diagnosis</h4><p>Consider other uterine masses such as </p><ul>
  • -<li>other <a href="/articles/uterine-sarcoma" title="Uterine sarcoma">uterine sarcomas</a><ul>
  • -<li>
  • -<a href="/articles/malignant-mixed-mullerian-tumour-of-the-uterus" title="malignant mixed Mullerian tumour of the uterus">malignant mixed Mullerian tumour of the uterus</a> (MMMT)</li>
  • -<li>
  • -<a href="/articles/endometrial-stromal-sarcoma" title="Endometrial stromal sarcoma (ESS)">endometrial stromal sarcoma</a> (ESS)</li>
  • +<p><strong>Uterine leiomyosarcomas</strong> are malignant uterine tumours that arises from the myometrium. They can account for up to one third of <a href="/articles/uterine-sarcoma">uterine sarcomas</a> but only 1.3 % of all uterine cancers <sup>7</sup>. The uterus is the commonest location for a <a href="/articles/leiomyosarcoma">leiomyosarcoma</a>.</p><h4>Epidemiology</h4><p>Most leiomyosarcomas are thought to arise de novo <sup>5</sup>. The incidence of sarcomatous transformation in benign uterine leiomyomas is reported to be 0.1-0.8% <sup>2</sup>. Typically present in women in the 5<sup>th</sup> decade.</p><h4>Pathology</h4><p>Leiomyosarcomas may arise either de novo from uterine musculature or the connective tissue of uterine blood vessels, or in a preexisting leiomyoma.</p><p>The pattern of tumour spread is to the myometrium, pelvic blood vessels and lymphatics, contiguous pelvic structures, abdomen, and then distantly, most often to the lungs.</p><p>Histology can be similar to leiyomyosarcomas at other sites. A leiomyosarcoma is differentiated histologically from a leiyomyoma by noting the presence of infiltrative margins, nuclear atypia and increased mitotic figures.</p><h4>Radiographic features</h4><h5>General</h5><p>The uterus is often massively enlarged.</p><h5>CT</h5><ul>
  • +<li>may show irregular central zones of low attenuation, suggesting extensive necrosis <sup>8</sup> and haemorrhage</li>
  • +<li>foci of calcification may be present but rare</li>
  • +</ul><h5>MRI</h5><p>Although it has been suggested that an irregular margin of a uterine leiomyoma on MRI is suggestive of sarcomatous transformation, this is not considered that specific.</p><h4>Treatment and prognosis</h4><p>They generally carry a poor prognosis <sup>6</sup>.</p><h4>Differential diagnosis</h4><p>Consider other uterine masses such as:</p><ul>
  • +<li>other <a href="/articles/uterine-sarcoma">uterine sarcomas</a><ul>
  • +<li>
  • +<a href="/articles/malignant-mixed-mullerian-tumour-of-the-uterus">malignant mixed Mullerian tumour of the uterus</a> (MMMT)</li>
  • +<li>
  • +<a href="/articles/endometrial-stromal-sarcoma">endometrial stromal sarcoma</a> (ESS)</li>
  • -</li>
  • -<li><a href="/articles/endometrial-carcinoma" title="endometrial carcinoma ">endometrial carcinoma</a></li>
  • -<li>large <a href="/articles/uterine-leiomyoma" title="Uterine leiomyoma">uterine leiomyoma </a>(fibroid) : especially those with <a href="/articles/myxoid-degeneration-of-a-leiomyoma" title="Myxoid degeneration of leiomyoma">myxoid degeneration</a> or <a href="/articles/myxoid-uterine-leiomyoma" title="Myxoid uterine leiomyoma">myxoid sub type</a> <sup>9</sup>.</li>
  • +</li>
  • +<li><a href="/articles/endometrial-carcinoma">endometrial carcinoma</a></li>
  • +<li>large <a href="/articles/uterine-leiomyoma">uterine leiomyoma </a>(fibroid) : especially those with <a href="/articles/myxoid-degeneration-of-a-leiomyoma">myxoid degeneration</a> or <a href="/articles/myxoid-uterine-leiomyoma">myxoid sub type</a> <sup>9</sup>
  • +</li>
  • -<a href="/articles/uterine-smooth-muscle-tumours-of-uncertain-malignant-potential" title="uterine smooth muscle tumours of uncertain malignant potential">uterine smooth muscle tumours of uncertain malignant potential</a> - rare</li>
  • +<a href="/articles/uterine-smooth-muscle-tumours-of-uncertain-malignant-potential">uterine smooth muscle tumours of uncertain malignant potential</a>: rare</li>

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