Department of Urology, Keio University School of Medicine, Tokyo, 160-8582, Japan.
Department of Nephrology, Toho University Faculty of Medicine, 7-5-23 Omorinishi Ota-ku, Tokyo, 143-0015, Japan.
BMC Nephrol. 2019 Apr 3;20(1):120. doi: 10.1186/s12882-019-1310-5.
Gastrointestinal stromal tumors (GISTs) in transplant recipients are very rare and only a handful of cases have been reported to date. Here we present the first known case of a huge GIST in a kidney transplant recipient with perforation of small intestine.
A 64-year-old male presented at our hospital with right colic pain; he had received an ABO incompatible kidney transplant 6 years earlier and was treated with cyclosporine, mycophenolate mofetil, and methylprednisolone. Radiological evaluation revealed a huge (11 cm in diameter) solitary tumor at the small intestine without distant metastasis. The small intestinal wall at the tumor location was perforated one week after diagnosis and the patient underwent emergency surgery. The pathological findings were compatible with GIST and the tumor consisted of spindle cells with positive staining for KIT, CD34, and DOG1 and negative or weak staining for desmin and S-100 protein. A mutation in exon 11 of the c-kit gene was also detected. Cyclosporine was withdrawn and imatinib mesylate (400 mg daily) was introduced. However, thereafter, we needed to decrease the dose at 300 mg daily due to severe hyponatremia. Reduced imatinib treatment was well tolerated and recurrence was not observed for 18 months after surgery.
The occurrence of GISTs in transplant patients is rare, and huge GISTs should be resected immediately after diagnosis because gastrointestinal tract at the tumor site could be perforated. Imatinib treatment is feasible in transplant recipients under immunosuppression, although immunosuppressive drugs metabolized by CYP3A4 should be used at a reduced dosage or withdrawn.
胃肠间质瘤(GIST)在移植受者中非常罕见,迄今为止仅报道了少数几例。本文报告首例移植受者发生巨大 GIST 并导致小肠穿孔。
一名 64 岁男性因右结肠疼痛就诊,他在 6 年前接受了 ABO 不相容的肾移植,目前接受环孢素、霉酚酸酯和甲基强的松龙治疗。影像学评估显示小肠有一个巨大(直径 11cm)的孤立性肿瘤,无远处转移。诊断后 1 周,小肠壁在肿瘤部位穿孔,患者接受了紧急手术。病理检查结果符合 GIST,肿瘤由梭形细胞组成,KIT、CD34 和 DOG1 阳性染色,结蛋白和 S-100 蛋白阴性或弱阳性染色。还检测到 c-kit 基因外显子 11 的突变。停用环孢素,引入甲磺酸伊马替尼(400mg 每日)。然而,此后由于严重低钠血症,需要将剂量减至 300mg 每日。减少剂量后伊马替尼治疗可耐受,术后 18 个月未观察到复发。
移植受者 GIST 罕见,巨大 GIST 应在诊断后立即切除,因为肿瘤部位的胃肠道可能穿孔。免疫抑制的移植受者可进行伊马替尼治疗,但应减少或停用经 CYP3A4 代谢的免疫抑制剂。