Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, USA.
Clin Transplant. 2018 Mar;32(3):e13186. doi: 10.1111/ctr.13186. Epub 2018 Jan 18.
Intra-abdominal fibromatosis often involves the mesentery root which is non-resectable by conventional surgery. Multivisceral transplant (MVT), as a potential cure to non-resectable fibromatosis, has rarely been reported and the prognosis is unknown.
Six patients who underwent MVT for intra-abdominal fibromatosis were reviewed. Clinicopathological features, immunohistochemistry for β-catenin, p53, and Ki67, and outcomes were evaluated. Appropriate data for comparative analysis were obtained from a cohort of 24 patients who underwent conventional resection for intra-abdominal fibromatosis.
Among six MVT patients, four had familial adenomatous polyposis (FAP). Two patients had an initial intestinal transplantation, three had multiple prior surgeries, and two had adjuvant therapy. One patient died of hemorrhagic stroke shortly after MVT, and five patients (83%) survived with a median follow-up of 64 months. The 1-year and 5-year survival rates were 67% for all five patients. Two patients had recurrences after MVT and one of them had FAP. In comparison, six of 24 patients who underwent conventional surgery had FAP; six (25%) had recurrences and three had FAP. For FAP patients; the mean recurrence time was 13 months for MVT versus 6 months for conventional surgery. Ki67 proliferative index, β-catenin, and p53 expression did not significantly correlate to recurrence.
Multivisceral transplant (MVT) is a viable option for patients who have non-resectable intra-abdominal fibromatosis with promising surviving rates, although recurrence still occurs. Surgical margin, Ki67 proliferative index, β-catenin, and p53 expression are not predicative for recurrence of fibromatosis.
腹腔内纤维瘤病常累及肠系膜根部，常规手术无法切除。多脏器移植（MVT）作为一种治疗不可切除纤维瘤病的潜在方法，鲜有报道，其预后尚不清楚。
回顾性分析 6 例行 MVT 治疗腹腔内纤维瘤病的患者。评估其临床病理特征、β-连环蛋白、p53 和 Ki67 的免疫组化结果及转归。从 24 例行常规手术治疗的腹腔内纤维瘤病患者中获得了适当的对比分析数据。
在 6 例 MVT 患者中，4 例有家族性腺瘤性息肉病（FAP）。2 例患者有初始肠移植史，3 例患者有多次既往手术史，2 例患者有辅助治疗。1 例患者在 MVT 后因脑出血死亡，5 例患者（83%）存活，中位随访时间为 64 个月。5 例患者的 1 年和 5 年生存率均为 67%。2 例患者在 MVT 后复发，其中 1 例有 FAP。相比之下，24 例行常规手术的患者中有 6 例患有 FAP；6 例（25%）复发，其中 3 例有 FAP。对于 FAP 患者；MVT 的平均复发时间为 13 个月，而常规手术为 6 个月。Ki67 增殖指数、β-连环蛋白和 p53 表达与复发无显著相关性。
对于不可切除的腹腔内纤维瘤病患者，MVT 是一种可行的选择，其生存率较高，但仍有复发。手术切缘、Ki67 增殖指数、β-连环蛋白和 p53 表达不能预测纤维瘤病的复发。
