평활근 육종(LMS)은 점막근층 또는 고유근층에서 발생하는 소장의 희귀한 종양이다. 소장에서 LMS가 발생하는 가장 흔한 부위는 공장이고 회장과 십이지장이 그 뒤를 잇는다. 일반적인 징후로는 복부 질량, 복부 통증, 현성 위장관 출혈 등이 있다. LMS는 남성에게 조금 더 많이 나타나며 주로 60대에 발생한다. 특히 양성종양과 악성종양을 구별하는 점에서 소장종양의 수술전 진단은 어렵다. 최근의 문헌 검토 결과, 컴퓨터 단층 촬영(CT)과 자기 공명(MR) 장운동기록법, 고위관장법이 소장의 LMS를 평가하는 좋은 방법임이 밝혀졌다. CT 및 MRI에서 모두 놓칠 수 있는 표재성 병변은 워터 캡슐 내시경 검사로 탐지할 수 있으며, 검출률은 약 80%이다. 조직학적으로 LMS는 위장관기질종양과 유사하지만 면역조직화학적 검사 상 CD117과 CD34에는 음성이고 평활근 액틴(actin)과 데스민(desmin)에는 양성이다. LMS의 크기가 5cm 이상일 때는 보통 간(65%), 기타 위장기관(15%) 및 폐(4%)로 혈행성 전이된다. 또한 림프계(13%)나 복막의 경로를 통해 전이될 수 있다. 소장의 LMS를 효과적으로 치료하는 유일한 방법은 수술이다. 원발성 종양은 장간막을 광범위하게 절제하면서 근치적으로 제거되어야 한다. 화학요법에 대한 LMS의 반응은 알려져 있지 않으며, 방사선요법은 치료에 영향을 미치지 않는다. 따라서 가능하면 전이 절제술을 고려해야 한다. 도세탁셀(docetaxel)과 젬시타빈(gemcitabine)의 병용과 관련한 대규모 2상 및 3상 실험에서 LMS(대부분 자궁에서 기원한)에 대한 인상적인 반응률이 보고되었다. 그러나 일부 연구에서는 이 복합요법의 효능을 확인할 수 없었다. 최근 트라벡테딘(trabectedin)이 LMS에 대해 최대 56%의 반응률을 보였으며, 안트라사이클린(anthracycline)과 이포스파미드(ifosfamide)의 병용요법이 실패한 후, 중증 진행 전이성 LMS에 대해 특히 유용한 것으로 나타났다.
Leiomyosarcoma are 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 jejunum followed by ileum and then duodenum. The common presentations include abdominal mass, abdominal pain or overt gastrointestinal bleeding. They are mainly seen in 6th decade of life with slight male preponderance. Preoperative diagnosis of small intestinal tumors is difficult, especially differentiating between benign and malignant tumors. For LMS in small intestine, recent review of literature revealed that CT and MRI-enterography and enteroclysis are good options.Cases of superficial lesion which can be missed by both CT and MRI, can however be detected by water capsule endoscopy with a detection rate of around 80%. Histologically LMS resembles like GIST, however they are CD117 and CD34 negative by immunohistochemistry and positive for smooth muscle antigen (SMA) and desmin. When these tumors are more than 5 cm they commonly spread hematogenously to liver (65%), other GI organs (15%), lung(4%). It also has the capability to spread via lymphatics (13%) or via peritoneal route (18%). The only effective treatment for small intestine LMS is surgery. The primary tumor should be excised radically, including a wide resection of the mesentry. Response to chemotherapy is doubtful, and there is no role for radiotherapy. Therefore, metastasectomy, if possible, should be considered. Large phase II and III studies combining docetaxel and gemcitabine yielded impressive response rates in 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% in 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 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.
