Foster Rosemary, Solano Steven, Mahoney Jennifer, Fuller Arlan, Oliva Esther, Seiden Michael V
Division of Hematology/Oncology, Massachusetts General Hospital, Boston, MA 02114, USA.
Gynecol Oncol. 2006 May;101(2):363-6. doi: 10.1016/j.ygyno.2005.12.022. Epub 2006 Jan 24.
Extragastrointestinal stromal tumors (eGISTs) are rare mesenchymal-derived tumors arising outside of the GI tract. eGISTs are often histologically confused with leiomyosarcoma. Distinction between eGIST and leiomyosarcoma is critical because of the unique responsiveness of eGISTs to the molecularly targeted agent imatinib.
A woman presented with a history of tubal spindle cell tumor that was initially diagnosed and treated as a leiomyosarcoma. Because of minimal response to sarcoma directed chemotherapy, the possibility that the tumor was in fact an eGIST was investigated and supported by immunohistochemical and mutational analyses of the c-Kit receptor tyrosine kinase. The patient currently has stable disease control on imatinib for the last 18 months.
The possibility of eGIST should be considered in the differential diagnosis of tumors with a spindle cell morphology in the gynecologic tract especially when involving the ovary, fallopian tube, or uterine serosa.
胃肠道外间质瘤（eGISTs）是起源于胃肠道外的罕见间叶源性肿瘤。eGISTs在组织学上常与平滑肌肉瘤混淆。区分eGIST与平滑肌肉瘤至关重要，因为eGISTs对分子靶向药物伊马替尼有独特的反应性。
一名女性有输卵管梭形细胞瘤病史，最初被诊断为平滑肌肉瘤并接受治疗。由于对肉瘤定向化疗反应极小，对肿瘤实际上是eGIST的可能性进行了调查，并通过c-Kit受体酪氨酸激酶的免疫组化和突变分析得到支持。该患者在过去18个月中使用伊马替尼病情一直稳定。
在鉴别诊断妇科具有梭形细胞形态的肿瘤时，尤其是涉及卵巢、输卵管或子宫浆膜时，应考虑eGIST的可能性。
