Department of Surgery, University of California San Diego, La Jolla, CA, USA.
Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.
Ann Surg Oncol. 2021 Aug;28(8):4685-4694. doi: 10.1245/s10434-020-09499-y. Epub 2021 Jan 7.
Peritoneal dissemination of low-grade appendiceal mucinous neoplasms (LAMNs), sometimes referred to as pseudomyxoma peritonei, can result in significant morbidity and mortality. Little is known about the natural history of localized (non-disseminated) LAMNs.
The goal of this study was to evaluate the risk of peritoneal recurrence in patients with localized LAMNs.
We performed a multi-institutional retrospective review of patients with pathologically confirmed localized LAMNs. Baseline characteristics, pathology, and follow-up data were collected. The primary endpoint was the rate of peritoneal recurrence.
We identified 217 patients with localized LAMNs. Median age was 59 years (11-95) and 131 (60%) patients were female. Surgical management included appendectomy for 124 (57.1%) patients, appendectomy with partial cecectomy for 26 (12.0%) patients, and colectomy for 67 (30.9%) patients. Pathology revealed perforation in 46 patients (37.7% of 122 patients with perforation status mentioned in the report), extra-appendiceal acellular mucin (EAM) in 49 (22.6%) patients, and extra-appendiceal neoplastic cells (EAC) in 13 (6.0%) patients. Median follow-up was 51.1 months (0-271). Seven (3.2%) patients developed a peritoneal recurrence, with a median time to recurrence of 14.4 months (2.5-47.0). Seven (15.2%) patients with histologic evidence of perforation had recurrence, versus no patients (0%) without perforation (p < 0.001); five (10.2%) patients with EAM versus two (1.2%) patients without EAM (p = 0.007), and one (7.7%) patient with EAC versus six (2.9%) patients without EAC (p = 0.355) had recurrence.
This multi-institutional study represents the largest reported series of patients with localized LAMNs. In the absence of perforation or extra-appendiceal mucin or cells, recurrence was extremely rare; however, patients with any of these pathologic findings require careful follow-up.
低级别阑尾黏液性肿瘤（LAMN）的腹膜播散，有时也称为假性黏液瘤腹膜病，可导致显著的发病率和死亡率。对于局限性（非播散性）LAMN 的自然病史知之甚少。
本研究旨在评估局限性 LAMN 患者腹膜复发的风险。
我们对经病理证实为局限性 LAMN 的患者进行了多机构回顾性研究。收集了基线特征、病理和随访数据。主要终点是腹膜复发率。
我们确定了 217 例局限性 LAMN 患者。中位年龄为 59 岁（11-95），131 例（60%）为女性。手术治疗包括阑尾切除术 124 例（57.1%），阑尾切除术加部分盲肠切除术 26 例（12.0%），结肠切除术 67 例（30.9%）。病理检查显示 46 例（提及穿孔状态的 122 例患者中的 37.7%）有穿孔，49 例（22.6%）有阑尾外无细胞黏液（EAM），13 例（6.0%）有阑尾外肿瘤细胞（EAC）。中位随访时间为 51.1 个月（0-271）。7 例（3.2%）患者发生腹膜复发，中位复发时间为 14.4 个月（2.5-47.0）。有组织学穿孔证据的 7 例（15.2%）患者复发，而无穿孔的 0 例（0%）患者（p<0.001）；5 例（10.2%）有 EAM 的患者与 2 例（1.2%）无 EAM 的患者（p=0.007），1 例（7.7%）有 EAC 的患者与 6 例（2.9%）无 EAC 的患者（p=0.355）复发。
本多机构研究代表了迄今为止报道的最大系列局限性 LAMN 患者。在没有穿孔、阑尾外黏液或细胞的情况下，复发极为罕见；然而，有任何这些病理发现的患者需要密切随访。
