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Leiomyosarcoma of the colon. A very uncommon condition with poor prognosis
Leiomiosarcoma de colon. Una entidad muy infrecuente con mal pronóstico
Mireia Merichal Resinaa,
Corresponding author
mireiamerichal@gmail.com

Corresponding author.
, Carlos Cerdan Santacruzb, Enrique Sierra Grañónb, Jordi Antoni Tarragona Foradadac, Jorge Juan Olsina Kisslera
a Servicio de Cirugía General y del Aparato Digestivo, Hospital Universitario Arnau de Vilanova, Lleida, Spain
b Unidad de Coloproctología, Hospital Universitario Arnau de Vilanova, Lleida, Spain
c Servicio de Anatomía Patológica, Hospital Universitario Arnau de Vilanova, Lleida, Spain
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COPD&#44; dyslipidaemia and appendectomy&#46; For the purpose of testing due to constipation with a one year period of progression&#44; he underwent a complete colonoscopy which revealed a polypoid lesion in the transverse colon&#44; 80<span class="elsevierStyleHsp" style=""></span>cm from the anal margin&#46; Pathology diagnosed the lesion as a leiomyosarcoma&#46; Testing was extended to include a CT scan of the chest&#44; abdomen and pelvis&#59; the results were negative for distant lesions&#46; A small locoregional lymphadenopathy measuring 11<span class="elsevierStyleHsp" style=""></span>mm was discovered&#46; Given these findings&#44; a laparoscopic sub-total colectomy with ileo-sigmoid anastomosis was performed&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">The definitive pathology report described a polypoid and ulcerated lesion with a stenosing lumen measuring 4&#46;5<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>cm&#44; with no necrosis and a mitotic rate of 12 per 10 high-power fields&#44; corresponding to a pT1N0M0 tumour with a histological grade of G2&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Leiomyosarcomas of the colon are aggressive tumours with a high rate of local recurrence and fundamentally haematogenous spread that are associated with a poor prognosis&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Unlike GISTs&#44; leiomyosarcomas preferentially occur in the small bowel &#40;45&#37;&#41; or the large bowel &#40;38&#37;&#41;&#59; they are rarely found in the stomach or oesophagus&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> They may also present in the rectum&#46; They most commonly affect males in their fifties and sixties&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">4&#44;5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Morphologically&#44; they may be indistinguishable from GISTs&#59; immunohistochemistry is required for their diagnosis&#46; This explains why many mesenchymal tumours in the pre-immunohistochemistry era were diagnosed as leiomyosarcomas&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">They usually present as sub-mucosal polyps or intramural nodules arising from the <span class="elsevierStyleItalic">muscularis mucosae</span> or the muscularis propria&#59; ulceration and necrosis are common&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Characteristically&#44; they test positive for smooth muscle markers such as desmin and actin and often calponin and h-Caldesmon&#44; and negative for GIST markers &#40;c-kit&#44; CD34 and Dog-1&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> A proper differential diagnosis between the two diseases is essential&#44; as they have radically different prognoses&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Treatment is surgery with complete removal and suitable margins&#59; even with this treatment&#44; recurrence is seen in many cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;5</span></a> The role of lymphadenectomy is debated&#59; however&#44; the guidelines recommend performing conventional oncology procedures with complete excision of the mesocolon as in intestinal adenocarcinoma in this location&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;5</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">As for adjuvant treatment&#44; these tumours are usually insensitive to chemotherapy and do not respond to imatinib&#46; sunitinib has very limited efficacy&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Adjuvant therapy with anthracyclines is considered the first-line choice in patients with large tumours and high-grade tumours&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">4&#44;5</span></a> The efficacy of radiotherapy is also a matter of debate&#44; although it has been suggested that&#44; as an adjuvant therapy&#44; it may reduce local recurrence in sarcomas of the rectum&#46; Given this low sensitivity to adjuvant therapy&#44; in cases of local recurrence and&#47;or metastasis&#44; the recommended treatment is surgical resection&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">With regard to follow-up&#44; for tumours with an intermediate to high histological grade&#44; it is recommended that a CT scan of the chest and abdomen be performed every 3&#8211;4 months in the first 2 years&#44; then 2 times per year up to 5 years and then once per year after the first 5 years have elapsed&#46; For low-grade tumours&#44; it is recommended that a CT scan of the chest and abdomen be performed every 4&#8211;6 months in the first 5 years&#44; then once per year&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The most common sites of metastasis are the liver and the peritoneum&#44; followed by the lungs&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> Given that spread to the lymph nodes is rare&#44; the most important prognostic indicator is a size &#8805;5<span class="elsevierStyleHsp" style=""></span>cm&#59; mitotic rate has no prognostic value&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Overall five-year survival following radical surgery is 51&#46;6&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;4</span></a> The primary cause of death is secondary to liver and lung metastases&#46; Age over 40 years&#44; tumour necrosis&#44; distant disease and large tumour size have been identified as poor prognostic factors&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Funding</span><p id="par0065" class="elsevierStylePara elsevierViewall">No support in the form of grants was received for the preparation of this article&#46;</p></span></span>"
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