Department of Surgery, Istituto Nazionale per la Cura e lo Studio dei Tumori, Milan, Italy.
Eur J Surg Oncol. 2010 Nov;36(11):1047-53. doi: 10.1016/j.ejso.2010.08.130. Epub 2010 Sep 15.
This retrospective multi-institutional study addresses the role of surgical cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) in the treatment of multicystic peritoneal mesothelioma (MCPM). MCPM is an uncommon tumour with uncertain malignant potential and no current standard therapy. Additionally, poorly defined pathological and biological features of this disease were investigated.
Twelve patients with MCPM underwent 14 procedures of cytoreduction and HIPEC in two Italian referral centres. Nine patients had recurrent disease after previous debulking (one operation in six patients, two in two, four in one). Biological markers related to mesothelioma origin and clinical features were assessed by immunohistochemical studies.
Median follow-up was 64 months (range 5-148). Optimal cytoreduction (residual tumour nodules ≤2.5 mm) was performed in all the procedures. One grade IV postoperative complication (NCI/CTCAE v.3.0) and no operative death occurred. All the patients are presently alive with no evidence of disease, including two patients who underwent the procedure twice, due to locoregional disease recurrence. Five- and ten-year progression-free survival was 90% and 72%, accounting for a. statistically significant difference (P = 0.0001) with progression-free survival following previous debulking surgery (median 11 months; range 2-31). All cases showed low proliferative activity assessed by mitotic rate and Ki-67 expression.
MCPM is a borderline tumour with a high propensity to local-regional recurrence. Definitive tumour eradication by means of cytoreduction and HIPEC seems more effective than debulking surgery in preventing disease relapse. Low mitotic rate and poor Ki-67 expression might be related to the peculiar behaviour of MCMP.
本回顾性多机构研究探讨了外科减瘤术和腹腔内热灌注化疗（HIPEC）在治疗多囊性腹膜间皮瘤（MCPM）中的作用。MCPM 是一种罕见的肿瘤，恶性潜能不确定，目前尚无标准治疗方法。此外，还研究了这种疾病病理和生物学特征的不明确性。
在两个意大利转诊中心，12 名 MCPM 患者接受了 14 次减瘤术和 HIPEC 治疗。9 名患者在先前减瘤术后出现复发（6 名患者中 1 次手术，2 名患者中 2 次手术，4 名患者中 1 次手术）。通过免疫组织化学研究评估与间皮瘤起源和临床特征相关的生物学标志物。
中位随访时间为 64 个月（范围 5-148）。所有手术均实现了完全减瘤（残留肿瘤结节≤2.5 毫米）。仅发生 1 例术后 4 级并发症（NCI/CTCAE v.3.0），无手术死亡。所有患者均存活且无疾病证据，包括 2 名因局部复发而接受 2 次手术的患者。5 年和 10 年无进展生存率分别为 90%和 72%，与先前减瘤术后无进展生存率（中位数 11 个月；范围 2-31）相比有统计学显著差异（P=0.0001）。所有病例的增殖活性均较低，通过有丝分裂率和 Ki-67 表达评估。
MCPM 是一种交界性肿瘤，局部区域复发倾向较高。通过减瘤术和 HIPEC 实现肿瘤的彻底清除似乎比减瘤手术更能有效预防疾病复发。低有丝分裂率和 Ki-67 表达不良可能与 MCMP 的特殊行为有关。
