Rapamycin Treatment for Benign Multicystic Peritoneal Mesothelioma: A Rare Disease with a Difficult Management

Abstract

Patient: Male, 65Final Diagnosis: Multicistic peritoneal mesotheliomaSymptoms: Strong slimmingMedication: —Clinical Procedure: —Specialty: NephrologyObjective:Unusual or unexpected effect of treatmentBackground:Benign multicystic peritoneal mesothelioma (BMPM) is a rare intra-abdominal tumor. Although considered by many to be benign, this tumor has a high local recurrence rate. Because of its rarity, preoperative diagnosis is difficult and its origin and pathogenesis are uncertain. There are no evidence-based treatment strategies for BMPM. It is agreed that the best treatment strategy for BMPM is the combination of surgical cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC). An increasing body of evidence supports a pivotal role of the cytoplasmic serine/threonine kinase mTOR in the development and progression of several neoplastic diseases and specific mTOR inhibitors, including rapamycin, have been suggested as potential therapeutic options for different cancers.Case Report:A 65-year-old male with end-stage renal disease on hemodialysis for seven years presented with BMPM. He underwent surgery to remove multiple peritoneal cysts, but four months later he experienced a recurrence of the disease. Immunohistochemistry of the cysts demonstrated a high level of phosphorylation of p70S6 kinase, a downstream mTOR target, and since a target therapy that blocks PI3K/Akt/mTOR pathway has been shown to have a scientific and logical rationale to treat this rare intra-abdominal neoplasia, we started the patient on low dose rapamycin therapy, an mTOR inhibitor. Long-term mTOR inhibition resulted in a complete and stable remission of BMPM.Conclusions:The current case is the first report of BMPM successfully treated with rapamycin, which resulted in a long-lasting response to mTOR inhibition.

Benign Multicystic Peritoneal Mesothelioma Presenting as a Colonic Mass

“…The pathogenesis of BMPM is undetermined but may result from serosal inflammation, among other proposed etiologies [8]. Treatment relies primarily on surgical resection but may also include tamoxifen [9], gonadotropin-releasing hormone (GnRH) agonists [10], rapamycin [11], and hyperthermic intraperitoneal chemotherapy [12]. Although the recurrence rate is nearly 50% in women and 33% in men [3], long-term survival is favorable [1].…”

Multicystic and diffuse malignant peritoneal mesothelioma in children

“…Although the resection was complete, the tumor relapsed 17 months later (case 14). Other treatments have been reported, but their success has yet to be established by large-scale studies: rapamycin, 47 hormonal therapy, 48 and sclerotherapy of peritoneal inclusion cysts. 49 This study has limitations associated with retrospective and multicentric studies.…”

Benign Multicystic Peritoneal Mesothelioma (BMPM) as a rare cause of abdominal pain in a young male: case report and review of the literature

“…Chemotherapy and radiotherapy did not show effects in this neoplasm [1]. An interesting case report of 2017 described the use of Rapamycin after primary surgery since the high level of phosphorylation of p70S6 kinase, a downstream mTOR target, found in BMPM [8]. No time lapse of follow up is known, with recurrence of this pathology reported as far as 36 years after surgery.…”

“…Due to its rarity, preoperative diagnosis is still a challenging clinical problem [6]. No evidence-based guidelines for treatment are actually available, although a surgical resection is recommended: some authors suggest a combination of cytoreductive surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) [7], and new strategies based upon immunohistochemistry knowledge of the neoplasm may lead to others pharmaceutical strategies [8]. Benign Multicystic Peritoneal Mesothelioma (BMPM) is a rare benign cystic neoplasm that arise from the peritoneum, typically found in young females (83%), with a high incidence of local recurrence after resection (almost 50% of cases).…”