惡性子宮平滑肌肉瘤的新發展

Management of Uterine Leiomyosarcoma: An Update

惡性子宮平滑肌肉瘤(uterine leiomyosarcoma)是少見而且惡化速度極快的腫瘤。通常腫瘤體積較大，且經由血液轉移，即使腫瘤只局限在子宮體，手術後仍具有高度的復發率。病患症狀通常類似於良性子宮肌瘤，大部分病患都是在手術後才診斷出來。疑似轉移的惡性子宮平滑肌肉瘤建議接受淋巴結摘除手術，而確診為轉移的惡性子宮平滑肌肉瘤則建議接受完整的減積手術。目前的文獻對於早期的惡性子宮平滑肌肉瘤術後是否需要輔助化學治療或放射線治療仍未有定論。對於晚期惡性子宮平滑肌肉瘤，則需要輔助性化學治療。針對復發性的惡性子宮平滑肌肉瘤則往往需要各種第二線的治療。現階段治療方法對於惡性子宮平滑肌肉瘤的預後並不好，目前研究的方向包括：早期的影像學診斷、整合治療、預後的推測、分子生物的致病機轉、甚至標靶治療，希望藉由這些研究能改善病患的預後，以及對於這個疾病有更進一步的認識。

Uterine leiomyosarcomas (LMSs) are rare aggressive tumors. Patients present with symptoms similar to those of patients with leiomyomas. Most patients are diagnosed with LMS postoperatively. Even when LMSs are confined to the uterine corpus at the time of diagnosis, the recurrence rate is high . These tumors are large myometrial masses which typically spread hematogenously. In the presence of metastatic disease, complete surgical cytoreduction should be attempted when feasible. Lymphadenectomy should be performed only in patients with nodes suspected of harboring metastatic disease. The role of adjuvant chemotherapy or radiation therapy for early-stage disease is still controversial. However, chemotherapy with gemcitabine/docetaxel followed by doxorubicin holds promise in the treatment of LMS. Patients with advanced-stage disease should receive gemcitabine and docetaxel adjuvant chemotherapy. Patients with recurrent disease are candidates for a wide variety of second-line treatment or target therapy (Pazopanib, multikinase inhibitor). Given the rarity of these tumors and the lack of clinical trials to guide management, patients with uterine sarcomas should be encouraged to enroll in clinical trials.