Azzaza Mohamed, Ammar Houssem, Abdessayed Nihed, Gupta Rahul, Nakhli Mohamed Said, Chhaider Amine, Abdennaceur Nafis, Ali Ali Ben
Department of Digestive Surgery, Hospital Sahloul, Sousse, Tunisia.
Department of Pathology, Farhat Hached Hospital, Sousse, Tunisia.
Int J Surg Case Rep. 2017;30:97-100. doi: 10.1016/j.ijscr.2016.11.046. Epub 2016 Dec 2.
Gastrointestinal stromal tumors (GIST) are tumors of mesenchymal origin commonly detected in stomach and small bowel. GIST arising primarily from the anal canal is extremely rare. Due to the malignant potential, these tumors are treated with radical surgery like abdominoperineal resection. But with the advent of imatinib therapy and a better understanding of the tumor biology, some cases have been successfully treated with wide local excision.
We describe a case of a 70-year-old lady presenting with a 2cm mass in the anal canal. Endoanal ultrasound revealed a well-circumscribed solid nodule in the intersphincteric space. The patient was successfully treated by wide local excision and adjuvant therapy with imatinib mesylate.
Only 14 confirmed cases of primary anal GIST have been reported in the literature. It appears as a well circumscribed hypoechoic mass arising from the intersphincteric space encroaching into the lumen on endorectal ultrasound. Lymphadenopathy is absent. Anal sphincters get involved as the lesion increases in size. Treatment is often planned based on the extent of the disease, the mitotic rate, patient's general condition and willingness for a permanent colostomy.
Small lesions (<2cm) with low mitotic rate may be successfully managed by local excision. Radical surgery should be reserved for large, aggressive tumors.
胃肠道间质瘤(GIST)是一种间叶源性肿瘤,常见于胃和小肠。主要起源于肛管的GIST极为罕见。鉴于其恶性潜能,这些肿瘤采用根治性手术治疗,如腹会阴联合切除术。但随着伊马替尼治疗的出现以及对肿瘤生物学的深入了解,一些病例已通过广泛局部切除成功治疗。
我们描述了一名70岁女性患者,其肛管内有一个2厘米的肿块。肛管超声显示括约肌间间隙有一个边界清晰的实性结节。该患者通过广泛局部切除及甲磺酸伊马替尼辅助治疗成功治愈。
文献中仅报道了14例确诊的原发性肛管GIST病例。在直肠内超声检查中,它表现为起源于括约肌间间隙、边界清晰的低回声肿块,侵入管腔。无淋巴结病。随着病变增大,肛门括约肌会受累。治疗方案通常根据疾病范围、有丝分裂率、患者一般状况以及接受永久性结肠造口术的意愿来制定。
有丝分裂率低的小病变(<2厘米)可通过局部切除成功治疗。根治性手术应保留用于治疗大的侵袭性肿瘤。