Long-term results of laparoscopic cytoreductive surgery and HIPEC for the curative treatment of low-grade pseudomyxoma peritonei and multicystic mesothelioma.

BACKGROUND: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) provide long-term survival for low-grade pseudomyxoma peritonei (PMP) and multicystic peritoneal mesothelioma (MM). Feasibility of laparoscopic CRS-HIPEC has been reported for selected patients but data regarding long-term outcomes are missing to assess the oncological interest. This study aimed to report long-term outcomes for low-grade PMP and MM treated by laparoscopic approach. METHODS: From a prospectively maintained CRS-HIPEC database, all patients who underwent laparoscopic CRS-HIPEC with curative intent were analyzed. Selection criteria for laparoscopic approach were low-grade PMP or MM, with pathological confirmation prior to CRS-HIPEC, ASA 2, age < 75 years, no extrap-eritoneal disease, Peritoneal Cancer Index (PCI) < 10, and a limited history of abdominal surgery. RESULTS: Between March 2009 and June 2017, 43 patients were scheduled for laparoscopic CRS and HIPEC. Laparoscopic CRS and HIPEC was completed (LSC) in 32 patients and 11 patients were converted to open surgery (CONV). Median age was 44.5 years (17.13-71.4) in the LSC group and 54.9 years (22.5-70.5) in the CONV group (p = 0.086). Median BMI was not different between groups, 21.2 and 23.9 for LSC and CONV groups, respectively (p = 0.267). There were 21 and 11 patients in the LSC group, and 8 and 3 in the CONV group, with PMP and MM, respectively (p = 0.794). Median PCI was 2.5 (0-9) and 7 (1-15) in the LSC and CONV groups, respectively (p = 0.004). There was no difference in the completeness of cytoreduction score (p = 0.256). After a median follow-up of 31.6 months (95% CI 19.3-36.4), 2 patients in the LSC group and 2 patients in the CONV group presented with peritoneal recurrence. CONCLUSION: For selected patients with low aggressive peritoneal disease, laparoscopic CRS-HIPEC provides interesting long-term outcomes.

[Benign multicystic peritoneal mesothelioma (BMPM) - a surprising differential diagnosis in case of an expected intraabdominal abscess formation]. / Das benigne multizystische peritoneale Mesotheliom (BMPM) ­ Eine überraschende Differenzialdiagnose bei Verdacht auf einen intraabdominellen Abszess.

The benign multicystic peritoneal mesothelioma is a rare disease. Most frequently, young women in reproductive age are affected by this disease. Nevertheless, there are known cases of multicystic peritoneal mesothelioma in male patients. The pathogenesis remains uncertain. Whereas asbestos fibers can cause the development of malignant mesothelioma, the exposure to asbestos particles cannot induce this type of mesothelioma. An inflammatory genesis is discussed as well as the idea of a neoplastic development. Since a high rate of recurrence after surgery is observed, an aggressive surgical treatment is recommended. The complete resection of affected tissue is recently considered to be the therapy of choice. The combination of complete surgical tumor reduction with an intraperitoneal hyperthermic chemotherapy (HIPEC) seems to be promising. Although malignant transformation is detected very rarely a close follow up in centers with high surgical and oncological expertise is recommended.

Peritoneal cystic mesothelioma: are surgery and HIPEC optimal first-line treatments?

BACKGROUND: Peritoneal cystic mesothelioma (PCM) is an uncommon clinical pathology. Its high rate of recurrence following partial or total resection as well as its spontaneous onset of malignancy have been well documented in a series of case studies. The medical community has yet to define standardized treatment guidelines for PCM. CASE REPORTS: This study reviews the case of 2 patients admitted and treated for PCM. Recent studies have reported improved recurrence and survival rates achieved by means of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC), especially when used as first-line treatments. However, whether or not the use of CRS and HIPEC is more effective than a surgical regimen of multiple debulking procedures, is still the subject of debate. CONCLUSION: CRS and HIPEC as first-line treatments have lower morbidity and mortality rates than regimens of multiple back-to-back surgical procedures, and as such, the CRS/HIPEC method appears to be the more successful approach.

Multi-institutional experience of diffuse intra-abdominal multicystic peritoneal mesothelioma.

BACKGROUND: This study was undertaken to measure survival of patients with multicystic peritoneal mesothelioma treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy through a multi-institutional collaboration. METHODS: A multi-institutional data registry, established by the Peritoneal Surface Oncology Group, was used to identify patients with peritoneal mesothelioma and the subgroup with multicystic tumours, treated by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. Outcomes for this subgroup are reported. The primary endpoint was overall survival. A secondary endpoint was the incidence of treatment-related complications. RESULTS: Of 405 patients with peritoneal mesothelioma, 26 (6·4 per cent) had multicystic tumours. There were 20 women and six men with a mean(s.d.) age of 42(12) years. The median peritoneal carcinomatosis index (PCI) was 14 (range 6-39). There was no perioperative mortality. Six patients developed grade III or IV complications. After a median follow-up of 54 (range 5-129) months, all 26 patients were still alive. CONCLUSION: Multicystic peritoneal mesothelioma appears to be a distinct subtype of peritoneal mesothelioma, where long-term survival may be achieved through cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

Multicystic peritoneal mesothelioma treated by surgical cytoreduction and hyperthermic intra-peritoneal chemotherapy (HIPEC).

BACKGROUND: Multicystic peritoneal mesothelioma (MPM) is an extremely uncommon lesion with uncertain malignant potential. Multiple recurrences after surgical interventions and transition to aggressive malignancies have been reported. Here, we review our experience with cytoreduction and hyperthermic intraperitoneal chemotherapy (HIPEC) in the management of MPM. PATIENTS AND METHODS: Five women with MPM underwent 6 procedures of cytoreduction and close-abdomen HIPEC with cisplatin and doxorubicin. Three patients had recurrent disease after 1, 2 and 4 previous debulkings, respectively. RESULTS: Optimal cytoreduction (residual tumor nodules < or =2.5 mm) was performed in all the procedures. One grade 4 postoperative complication (NCI/CTCAE v.3.0) and no operative mortality occurred. Median follow-up was 31 months (range 3-102). MPM recurred in two patients: one is presently disease-free after a second cytoreduction with HIPEC and the other is alive with minimal stable disease. CONCLUSION: Definitive eradication by means of cytoreduction and HIPEC seems a safe and effective therapeutic option for MPM.

Multicystic mesothelioma: Operative and long-term outcomes with cytoreductive surgery and hyperthermic intra peritoneal chemotherapy.

BACKGROUND: Multicystic peritoneal mesothelioma (MCPM) is an extremely rare disease with 40-50% rate of recurrence after surgical debulking. Due to the recurrent nature of the disease, the option of cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) was offered for this condition. In the present study we aimed to describe the outcomes of this strategy in a single center cohort. METHODS: We retrospectively reviewed a prospectively collected database of all patients with MCPM that underwent the combined procedure in our center. Clinical presentation, operative procedures and outcomes were reviewed. RESULTS: Between August 1997 and October 2017, 19 patients with MCPM underwent 20 cytoreduction and HIPEC procedures in our center. The majority of the patients were females (n = 17, 89%), and mean age was 42, as reported in other series. Disease extent, as measured by mean peritoneal carcinomatosis index (PCI) was 15.5 ± 9.9 and total number of procedures performed 6.7 ± 2.6. Major complications occurred in 3 (15%) patients, with no perioperative mortality. After a median of follow-up of 69 months (range 4-220) all patients were alive and 4 patients had recurrence (21%). Patients with high PCI (defined by median PCI) had shorter recurrence free survival (RFS) than patient with low PCI (mean RFS = 106.4 ± 6.6 for high PCI vs. 125.6 ± 34.1 for low PCI, p = 0.03). CONCLUSION: Cytoreduction and HIPEC offer low recurrence rate and prolonged mean RFS for patients with MCPM. The combined procedure can be offered with acceptable morbidity in specialized centers.

Multicystic peritoneal mesothelioma: A short review.

Multicystic peritoneal mesothelioma (MCPM) is a rare neoplasm, predominantly affecting female patients during their reproductive years. The lesion is usually distributed diffusely in the abdomen and pelvis, but the peritoneum of the pelvic organs is the most common site. MCPM is composed of fluid-filled translucent cysts, connected by varying amounts of fibrous tissue, and lined by a layer of mesothelial cells. Because of the rarity of this disease, the pathogenesis and natural history of MCPM remain poorly understood and continuously debated. Some authors consider it to be a reactive process for its association with prior surgery or abdominal inflammation. But its high rate of local-regional recurrence, as well as its malignant potential, suggests a neoplastic etiology. Preoperative diagnosis is often very difficult. Imaging methods, such as ultrasound, computed tomography, and magnetic resonance imaging, are of little value for an accurate diagnosis of MCPM. The definitive diagnosis relies on histologic examination of target lesions combined with immunohistochemical stains. There is no consensus on the clinical management of MCPM, although surgical removal remains the first-line treatment of choice. But no standards have been reached concerning which surgical options-traditional debulking surgery or more aggressive one-should be chosen. Alternative therapeutic approaches include hand-off treatment, hormonal supplementation, laser vaporization, and sclerotherapy, and they all come with uncertain results. Moreover, the lesions show no response to adjuvant chemotherapy and radiotherapy. This article aimed to focus on those controversial problems in pathogenesis, natural history, diagnosis, and treatment strategies to help medical workers to better understand this rare disease.

Radiological predictors of complete cytoreduction in 59 patients with peritoneal mesothelioma treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy at a UK referral centre.

OBJECTIVE: To assess the imaging features of peritoneal mesothelioma and identify key anatomical sites that aid patient selection for complete cytoreduction. METHODS: Pre-operative imaging of 59 (32 males, 27 females) patients who underwent cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) for histologically proven peritoneal mesothelioma [36 malignant peritoneal mesothelioma, 23 cystic mesothelioma were reviewed. Imaging findings were correlated with surgical outcome. Best imaging predictors of complete cytoreduction, n = 22 and major tumour debulking, n = 12 were assessed. RESULTS: Most patients (88.9%) had diffuse peritoneal disease with mean radiological peritoneal cancer index of 18 ± 12 (range 2-39). Disease in the lesser omentum (n = 10), porta hepatis (n = 8), perigastric area (n = 5), mesentery (n = 25), small bowel (n = 17), hydronephrosis (n = 1), concurrent pleural disease (n = 2), lymph nodes (n = 1) and abdominal wall disease (n = 4) was considered unfavourable. While 78.9% of patients who underwent complete cytoreduction had no disease at unfavourable sites, 75% of those who underwent MTD did have disease at these sites. There was significant difference in the radiological peritoneal cancer index, severity of upper abdominal disease, small bowel and mesenteric involvement between patients who underwent complete cytoreduction and MTD for malignant peritoneal mesothelioma. Complete cytoreduction was not achieved in the presence of a rind of soft tissue around the small bowel (p = 0.016) and was unlikely in the presence of large volume upper abdominal disease (p = 0.06). CONCLUSION: Involvement of key anatomical sites such as small bowel serosa and large volume upper abdominal disease reduced the likelihood of achieving complete cytoreduction in patients with malignant peritoneal mesothelioma. Advances in knowledge: Demonstration of small bowel disease and large volume upper abdominal disease on imaging in patients with malignant peritoneal mesothelioma can be used to identify patients who may not benefit from cytoreductive surgery.

Successful catheter drainage of recurrent benign multicystic mesothelioma of the peritoneum.

The case history of a patient with pulmonary embolism 4 months following a kidney transplantation is presented. Ultrasonography of the abdomen suggested a large ovarian tumour for which she had a laparotomy. Histological examination after resection of the tumour revealed a benign multicystic mesothelioma of the peritoneum. During the course of 1 year the tumour recurred 5 times and different attempts to aspirate and obliterate the cyst were ineffective. Eventually, cure was achieved by permanent transvaginal catheter drainage, which resulted in infection and obliteration of the cyst.

Peritoneal cystic mesothelioma: a case series.

BACKGROUND: Cystic peritoneal mesothelioma is a rare disease associated with a favorable short-term prognosis. Longer follow-up documenting a persistence of symptoms and a high rate of recurrence after debulking surgery along with an uncertain natural history prompt a re-evaluation of prior treatment recommendations. No prior long-term clinical study of these patients is available. METHODS: The experience with five cases of cystic peritoneal mesothelioma, four females and one male, are reviewed. All of these patients were treated with cytoreductive surgery with peritonectomy procedures and heated intraoperative intraperitoneal chemotherapy. CT, pathology and current status were investigated in order to learn more about the natural history of this disease. RESULTS: All patients were symptomatic from abdominal distention and three of the four complained of severe pain. Female patients complained of long periods of recurrent abdominal and pelvic pain poorly managed by oral analgesics. In one patient prolonged conservative management over ten years resulted in transition to an invasive process with extensive lymph nodal metastases. Her prognosis for long-term survival is guarded because of mesothelioma extension into the chest. Disease control of both ascites and pain in the abdomen and pelvis was achieved in all five patients treated with cytoreductive surgery plus intraperitoneal chemotherapy. CONCLUSIONS: Cystic peritoneal mesothelioma should no longer be referred to as "benign" cystic mesothelioma. An aggressive approach with complete disease eradication is the correct goal of treatment. From our experience, cytoreductive surgery to remove all visible tumor and intraperitoneal chemotherapy to control microscopic residual disease will help patients with peritoneal cystic mesothelioma to remain symptom- and disease-free over an extended time period with a single surgical intervention. Disease eradication may prevent the transition to an aggressive and fatal disease process.

Multicystic peritoneal inclusion cysts: the use of CT guided drainage for symptom control.

Our case study is that of a teenage male presenting with multilocular peritoneal inclusion cystic disease that is now managed symptomatically with a minimally invasive, repeatable technique. Between admissions he leads a relatively normal life. Symptomatic control in MPIC is possible using repeated CT guided aspirations.

[Retroperitoneal cystic mesothelioma and lymphangioma]. / Mesotelioma y linfangioma quísticos retroperitoneales.

Retroperitoneal cysts are uncommon entities of difficult diagnosis because of their insidious symptomatology. Urinary apparatus involvement is quite often the mode of presentation and the reason for calling on the urologist. The origin of many of these retroperitoneal cysts remains practically unknown. Surgery with exeresis is the choice management method. Follow-up is necessary for cystic mesothelioma because of the highly frequent relapses. The outlook of hormonal conservative therapy for relapses appears as a future alternative to treatment.

Peritoneal inclusion cysts: a review.

OBJECTIVE: To provide a comprehensive review of peritoneal inclusion cysts in the female patient population. To define the optimal diagnostic modalities and review the medical and surgical options for management, enabling the gynecologist to individualize treatment for patients. DATA SOURCES: We searched the MEDLINE database for articles with keywords "peritoneal inclusion cyst" and "benign (multicystic) mesothelioma." Our search was limited to the English language. All reports included a tissue-confirmed diagnosis, except 1. Case reports and case series with adolescent and adult patients were reviewed. METHOD OF STUDY: We evaluated all studies meeting our criteria for clinical features, histologic criteria for diagnosis, imaging and laboratory studies, and treatment modalities. TABULATION, INTEGRATION, AND RESULTS: Fifty-two descriptive studies and 1 prospective cohort study meet criteria for review. Eleven articles focused on imaging modalities. Nineteen articles depicted histopathology. Eight addressed treatment modalities. CONCLUSION: This is a comprehensive review of peritoneal inclusion cysts. We specifically focus on the method of diagnosis and management. There is no standard algorithm by which the patients are evaluated, treated, or followed up. Peritoneal inclusion cysts have minimal mortality but high morbidity. Diagnosis is made by clinical history, ultrasound imaging, and CA-125 correlation. Magnetic resonance imaging is useful if ultrasound is unclear. Tissue sample is necessary for definitive diagnosis. Prior studies have suggested that cure is only accomplished with surgical resection; however, patients have a 50% risk of recurrence. We suggest that the goal for such a chronic disease should not be cure, but symptomatic relief through individualization of treatment. TARGET AUDIENCE: Obstetricians & Gynecologists, Family Physicians. LEARNING OBJECTIVES: After completion of this article, the reader should be able to summarize imaging characteristics of peritoneal inclusion cysts, explain the epidemiology and risk factors for the development of peritoneal inclusion cysts, and describe possible treatment options for peritoneal inclusion cysts.

Benign multicystic peritoneal mesothelioma: cases reports in the family with diverticulosis and literature review.

We report on benign multicystic peritoneal mesothelioma in two siblings whose family had a history of multiple familial diseases including diverticulosis. After a genetic evaluation and a chromosomal analysis, we were not able to identify a specific genetic cause of the family's pattern of disease. We assumed that previous surgical procedures and the chronic inflammatory process from diverticulitis were the underlying etiology. Both patients had multiple recurrences with indolent courses similar to those reported in other cases. After the recurrences, one patient was treated with cystic aspiration and the other with hormones. The cysts in both cases regressed partially but the patients were relieved of their clinical symptoms, for 2 years after cystic drainage in one case and for 5 years after hormonal treatment in the other.

Mesotelioma quístico del peritoneo: informe de un paciente / Cystic mesothelioma of peritoneum: a case report

Objetivo: Informar el caso de una paciente en quien se hizo el diagnóstico clínico por imagenología e histológico de mesotelioma quístico de peritoneo. Sede: Servicio de cirugía general del Hospital Central Militar. Informe de caso: Se trató de una mujer de 58 años de edad con antecedente de cirugía abdominal múltiple, con cuadro clínico de 6 meses de evolución caracterizado por pérdida de peso, aumento de perímetro abdominal, dolor abdominal y masa palpable en la fosa iliaca izquierda. Los estudios por imagen de ultrasonografía y tomografía computada revelaron múltiples colecciones líquidas que rodeaban los órganos y una masa quística que ocupaba más del 60 por ciento de la cavidad abdominal. La laparotomía exploradora reveló una enorme masa quística que contenía 6 litros de líquido claro y múltiples quistes adosados al diafragma, colon, páncreas e hígado. El diagnóstico histológico fue: Mesotelioma peritoneal monofásico de tipo fibroso. La pacientes se encuentra actualmente viva y asintomática