Multicystic and well-differentiated papillary peritoneal mesothelioma treated by surgical cytoreduction and hyperthermic intra-peritoneal chemotherapy (HIPEC).
ABSTRACT Multicystic peritoneal mesothelioma (MPM) and well-differentiated papillary peritoneal mesothelioma (WDPPM) are exceedingly uncommon lesions with uncertain malignant potential and no uniform treatment strategy. The aim of the current study was to review our experience with cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) in these clinical settings.
Four women with MPM and eight with WDPPM underwent 13 procedures of cytoreduction and close-abdomen HIPEC with cis-platin and doxorubicin. Seven patients had recurrent disease after previous debulking (one operation in five patients, two in one, four in one). Potential clinicopathological prognostic factors were assessed.
Optimal cytoreduction (residual tumor nodules <or=2.5 mm) was performed in 12 of 13 procedures. Median follow-up was 27 months (range 6-94). One grade 4 postoperative complication (NCI/CTCAE v.3.0) and no operative mortalities occurred. One patient underwent the procedure twice due to locoregional MPM recurrence. Transition of typical WDPPM to malignant biphasic mesothelioma was documented in one patient who died of disease progression following incomplete cytoreduction and HIPEC. Following multimodality treatment, 5-year overall and progression-free survival were 90.0% (standard error = 9.0) and 79.7% (11.9), respectively. Progression-free survival following previous debulking surgery (median 24 months; range 2-87) was statistically worse (P = .0156). Incomplete cytoreduction and poor performance status correlated to both reduced overall and progression-free survival after cytoreduction and HIPEC.
MPM and WDPPM are borderline tumors capable of transformation into potentially lethal processes. Definitive tumor eradication by means of cytoreduction and HIPEC seems more effective than debulking surgery in preventing disease recurrence or transition to aggressive malignancies.
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ABSTRACT: Highlights ► We present a case of endometrioid ovarian cancer with concomitant well differentiated papillary mesothelioma of the peritoneum. ► Endometrioid ovarian cancer with concomitant well differentiated papillary mesothelioma is an extremely rare surgical finding of uncertain prognostic significance. ► Treatment with carboplatin and gemcitabine was given with no evidence of disease six months after diagnosis.04/2013; 4:53–55. DOI:10.1016/j.gynor.2013.01.003
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ABSTRACT: Malignant peritoneal mesothelioma (PM) is an infrequent disease which has historically been associated with a poor prognosis. Given its long latency period and non-specific symptomatology, a diagnosis of PM can be suggested by occupational exposure history, but ultimately relies heavily on imaging and diagnostic biopsy. Early treatment options including palliative operative debulking, intraperitoneal chemotherapy, and systemic chemotherapy have marginally improved the natural course of the disease with median survival being approximately one year. The advent of cytoreduction (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has dramatically improved survival outcomes with wide median survival estimates between 2.5 to 9 years; these studies however remain largely heterogeneous, with differing study populations, tumor biology, and specific treatment regimens. More recent investigations have explored extent of cytoreduction, repeated operative intervention, and choice of chemotherapy but have been unable to offer definitive conclusions. CRS and HIPEC remain morbid procedures with complication rates ranging between 30% to 46% in larger series. Accordingly, an increasing interest in identifying molecular targets and developing targeted therapies is emerging. Among such novel targets is sphingosine kinase 1 (SphK1) which regulates the production of sphingosine-1-phosphate, a biologically active lipid implicated in various cancers including malignant mesothelioma. The known action of specific SphK inhibitors may warrant further exploration in peritoneal disease.
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ABSTRACT: Background: Due to the increased adoption of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS and HIPEC), patients with malignant peritoneal mesothelioma (MPM) have seen improved outcomes. We aimed to evaluate and synthesize the recent published literature. Methods: The review was conducted according the recommendation of the Meta-Analysis of Observational Studies in Epidemiology (MOOSE) group with prespecified inclusion and exclusion criteria. DEALE method was used to combine mortality rates and imputation techniques were used to calculate standard errors. Meta-regression techniques were used to synthesize data. Publication bias was assessed using funnel plots. Results: Of 6,528 citations collected, 20 articles reporting on 1047 patients were included in the analysis The median age was 51 years (IQR 49-55) with 59 % (54-67) female. The median peritoneal carcinomatosis index (PCI) score was 19 (16-23). Complete cytoreduction (CC-0, 1) was performed in 67% (46-93%) of patients. Pooled estimates of survival yielded a 1, 3 and 5 year survival of 84, 59 and 42% respectively. Patients receiving early postoperative intraperitoneal chemotherapy (EPIC) (44%) and those receiving cisplatin intraperitoneal chemotherapy alone (48%) or in combination (44%) had an improved 5 year survival. Conclusions: While CRS+HIPEC has led to an improved survival for patients with MPM compared to historic data, heterogeneity of studies precludes generalizable inferences. EPIC chemotherapy and Cisplatin chemoperfusion may infer survival benefit.Annals of Surgical Oncology 08/2014; 22(5). DOI:10.1245/s10434-014-3978-x · 3.94 Impact Factor