Leiomyosarkom (LMS), muskularis mukozasından veya muskularis propriadan kaynaklanan, nadir bir ince bağırsak tümörüdür. İnce bağırsakta LMS’nin en sık görüldüğü bölge jejunum olup, bunu ileum ve duodenum takip eder. Yaygın belirtileri arasında karında kitle, karın ağrısı ve açık gastrointestinal kanama bulunur. LMS, çoğunlukla yaşamın 6. on yılında ortaya çıkar ve erkeklerde bir miktar daha baskındır. İnce bağırsak tümörlerinin preoperatif tanısı, özellikle benign ve malign tümörleri ayırt etme açısından zordur. Yakın tarihli bir literatür taraması, bilgisayarlı tomografi (BT) ve manyetik rezonans (MR) enterografi ve enteroklizinin, ince bağırsakta LMS'nin değerlendirilmesi için iyi yöntemler olduğunu ortaya koymuştur. Hem BT hem de MR görüntülemede atlanabilen yüzeysel lezyonlar, kapsül endoskopi ile yaklaşık %80’lik bir saptama oranıyla saptanabilir. Histolojik olarak LMS, gastrointestinal stromal tümöre benzerdir, ancak immünohistokimyada CD117 ve CD34 açısından negatif ve düz kas aktini ve desmini açısından pozitiftir. LMS'nin büyüklüğü 5 cm'nin üzerinde olduğunda, genellikle hematojen olarak karaciğere (%65), diğer gastrointestinal organlara (%15) ve akciğerlere (%4) yayılır. Lenfatik sistem (%13) veya periton yolu (%18) yoluyla da yayılabilir. İnce bağırsakta LMS için tek etkili tedavi cerrahidir. Primer tümör, mezentrinin geniş rezeksiyonu ile radikal bir şekilde eksize edilmelidir. LMS'nin kemoterapiye yanıtı bilinmemektedir ve radyoterapinin de LMS tedavisinde bir rolü bulunmamaktadır. Bu nedenle, mümkünse metastazektomi düşünülmelidir. Dosetaksel ve gemsitabin kombinasyonunu içeren büyük faz II ve III çalışmalarda, LMS'ler için (çoğunlukla uterus kaynaklı) etkileyici yanıt oranları bildirilmiştir. Bununla birlikte, bazı çalışmalarda bu kombinasyonun etkililiği doğrulanamamıştır. Son zamanlarda trabectedin, LMS'ler için %56'ya varan yanıt oranları ortaya koymuş ve antrasiklinler ve ifosfamid kombinasyonunun başarısız olmasını takiben çok ilerlemiş ve metastatik LMS'lere karşı özellikle faydalı olduğu görülmüştür.
Leiomyosarcoma (LMS)1 areis
a rare tumors of small intestine which arise from the muscularis
mucosa or muscularis propria. The most common site of LMS in the small
intestine is the jejunum, followed by the ileum
and then duodenum. The common
presentations include abdominal mass, abdominal pain,
and2 or overt gastrointestinal
bleeding. They are mainly seenobserved3 in 6th decade of life with slight male
preponderance. The Ppreoperative
diagnosis of small intestinal tumors is difficult, especially in terms of differentiating between benign and
malignant tumors. For LMS in the small
intestine, a recent review of literature
revealed that computed tomography (CT) and magnetic
resonance (MR)I- enterography
and enteroclysis are good detection4 options. Cases of superficial lesions, which can be missed by both CT and MRI imaging,
can however be detected by water capsule endoscopy,
with a detection rate of aroundapproximately 80%. Histologically, LMS resembles likegastrointestinal stromal tumorGIST,;
however they areit is negative for5 CD117 and CD34 negative by immunohistochemistry and positive for
smooth muscle antigenactin6 (SMA)
and desmin on immunohistochemistry. When
these tumors are more than 5 cm, they
commonly spread hematogenously to liver (65%), other gastrointestinalGI organs
(15%), and the lungs (4%). It They
also has have the capability to spread
via the lymphatics system (13%)
or via peritoneal route (18%). The only
effective treatment for LMS in the small
intestine LMS 7is surgery. The primary
tumor should be excised radically, including a wide resection of the mesentry.
Response to chemotherapy is doubtfulunknown, and there is no role for8of
radiotherapy. Therefore, metastasectomy, if possible, should be
considered. Large phase II and III studies combining docetaxel and
gemcitabine yielded impressive response rates forin LMSs (mostly of uterine origin). However,
others were not able to confirm the efficacy of this combination. Recently,
trabectedin showed response rates up to 56% forin LMSs, and it appeared to be especially useful
in far-advanced and metastatic LMSs after failure of the combination of
anthracyclines and ifosfamide.
Leiomyosarcoma (LMS)1 areis
a rare tumors of small intestineintestinal
tumor, which arises from the
muscularis mucosa or muscularis propria. The most common site of occurrence of 2LMS in the small intestine is the jejunum,
followed by the ileum and then duodenum. TheIts common presentations manifestations3 include abdominal mass, abdominal pain, and4 or overt gastrointestinal bleeding. They are mainly seenobserved5 in the 6th
decade of life with slight male preponderance. In
general, the The Ppreoperative diagnosis of small intestinal tumors such as LMSs is difficult, especially in terms of differentiating between benign and
malignant tumors. For LMS in the small
intestine, 6According to a recent review of
literature revealed that review computed tomography (CT) and magnetic
resonance (MR)I- enterography
and enteroclysis are good 7detection options. Cases of superficial modalities for the assessment of LMS. Superficial lesions, which
can be missed by both CT and MRI imaging, can however be
detected by water capsule endoscopy, with
a detection rate of aroundapproximately 80%. Histologically, LMS resembles like8gastrointestinal stromal tumorGIST,;
however, on immunohistochemical analysis,
they areit is has been found to be negative for9 CD117 and CD34 negative by immunohistochemistry and positive for
smooth muscle antigenactin (SMA)10
and desmin on immunohistochemistry.
When these tumors arethe size of LMS is 11more than 5 cm, they commonly spread
it can hematogenously spread to the
liver (65%), other gastrointestinalGI organs (15%), and
the lungs (4%). It TheyIt can also has have the capability to spread via the lymphatics
system (13%) or via peritoneal route (18%). The response of LMS to chemotherapy is unknown, and radiotherapy
does not play a role in the treatment. Therefore, surgery is the only
effective treatment for LMS in the small
intestine. LMS
12is surgery. The primary tumor should
be excised radically, including a with wide resection of the mesentry. Response to chemotherapy is doubtfulunknown, and
there is no role forof13 radiotherapy. Therefore, metastasectomy, iIf possible, metastasectomy
should be considered. Large phase II and III studies
combiningtrials involving the
combination of docetaxel and gemcitabine yieldedhave reported impressive response rates forin LMSs
(mostly of uterine origin). However, others weresome studies have14 not been
able to confirm the efficacy of this combination. Recently, trabectedinTrabectedin
has recently showed response rates of up
to 56% forin
LMSs, and it appeared to be especiallyparticularly useful inagainst far-advanced and metastatic LMSs after
failure of the combination of anthracyclines
and ifosfamide, combination therapy.
Leiomyosarcoma (LMS) is a rare small intestinal tumor, which arises from the muscularis mucosa or muscularis propria . The most common site of occurrence of LMS in the small intestine is the jejunum, followed by the ileum and duodenum. Its common manifestations include abdominal mass, abdominal pain, and overt gastrointestinal bleeding.. They are mainly observed in the 6th decade of life, with slight male preponderance. In general, the preoperative diagnosis of small intestinal tumors such as LMSs is difficult, especially in terms of differentiating between benign and malignant tumors. According to a recent literature review computed tomography (CT) and magnetic resonance (MR) enterography and enteroclysis are good modalities for the assessment of LMS. Superficial lesions, which can be missed by both CT and MR imaging, can be detected by water capsule endoscopy, with a detection rate of approximately 80%. Histologically, LMS resembles gastrointestinal stromal tumor; however, on immunohistochemical analysis, it has been found to be negative for CD117 and CD34 and positive for smooth muscle actin and desmin. When the size of LMS is more than 5 cm, it can hematogenously spread to the liver (65%), other gastrointestinal organs (15%), and the lungs (4%). It can also spread via the lymphatic system (13%) or peritoneal route (18%). The response of LMS to chemotherapy is unknown, and radiotherapy does not play a role in the treatment. Therefore, surgery is the only effective treatment for LMS in the small intestine. The primary tumor should be excised radically with wide resection of the mesentery. If possible, metastasectomy should be considered. Large phase II and III trials involving the combination of docetaxel and gemcitabine have reported impressive response rates for LMSs (mostly of uterine origin). However, some studies have not been able to confirm the efficacy of this combination. Trabectedin has recently showed response rates of up to 56% for LMSs, and it appeared to be particularly useful against far-advanced and metastatic LMSs after failure of anthracyclines and ifosfamide combination therapy.