Multicystic peritoneal mesothelioma: outcomes and patho-biological features in a multi-institutional series treated by cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC)

Abstract

Aim 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.Methods 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.Results 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 = .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.Conclusions 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.

Multicystic mesothelioma has malignant potential: its grounds and mechanisms of peritoneal metastasis

“…2,6 Article Some authors considered it to be a mesothelial neoplasm because it may recur locally and in rare cases may show malignant transformation. 4,9 In the present study, Case 1 had diffuse involvement of peritoneal surface by the cystic tumors and pushing invasion into mesentery.…”

“…It grows along the serosa as multiple, translucent, fluid-filled cysts. [1][2][3] Because of the association of previous pelvic operation in young women, and no relation with asbestos exposure, this disease may develop by inflammatory reaction. 1 Additionally, it has shown an indolent biological behavior in the majority of patients, and the postoperative survival has been good.…”

“…[1][2][3] However, the etiology remains unknown. Although the disease has shown an indolent clinical behavior after cytoreductive surgery (CRS) in most cases, early recurrence after complete resection, 2,3 transformation to diffuse peritoneal mesothelioma, 1,[4][5][6] and even death due to compression of bowel have been described. [1][2][3] For the present study, results of CRS for MCPM and pathological findings are reported.…”

Peritoneal Mesothelioma

“…The difference in PFS after 11 debulking operations carried out in seven patients before referral to the Milan NCI was statistically significant in favor of CRS plus HIPEC (p = 0.016). According to these data, definitive tumor eradication by CRS plus HIPEC is recommended as the standard option to prevent disease recurrence or transition into malignant conditions [42].…”

Novel TNS3-MAP3K3 and ZFPM2-ELF5 fusion genes identified by RNA sequencing in multicystic mesothelioma with t(7;17)(p12;q23) and t(8;11)(q23;p13)

“…The median age was 41 years with 1 year as the minimum and 86 years as the maximum (Appendix: Supplementary Table S1). Most publications have described single cases, further testifying to the rarity of the disease, but also a few larger series were reported [4][5][6][7][8]. Only 4 cases of multicystic mesothelioma have been reported so far from Norway [9,10].…”