Pseudomyxoma peritonei

Pseudomyxoma peritonei is a clinical entity characterized by a gelatinous ascite associated with mucinous tumor deposits spreading on peritoneal surface and potentially invading abdominal organs. It is considered as a tumor process linked, in most of cases, to a mucinous appendiceal neoplasm. Pseudomyxoma peritonei may benefit from a therapeutic strategy combining cytoreductive surgery and intra-peritoneal chemotherapy, which has led to a major prognosis improvement. Different classifications are available and the last one corresponds to the WHO 2010 version, which individualizes pseudomyxoma peritonei in two classes: low grade and high grade mucinous carcinoma. The very low frequency of this entity and its specific therapeutic strategy need specific health care centres, as well as physicians and pathologists collaborating through dedicated networks. The aim of this article is to summarize the pathology, causes, mechanisms and therapeutic approaches of pseudomyxoma peritonei, as well as their interfaces with dedicated networks.

Pseudomyxoma peritonei or gelatinous ascites is a rare clinical entity, and its pathogenesis remains obscure. It most often follows a mucinous tumor of the appendix. An ovarian origin in woman has been suggested but remains controversial. Its onset is often insidious: an increase in the abdominal perimeter may be the first sign noted. Preoperative diagnosis is facilitated by modern imaging techniques. Ultrasonography and computed tomography provide complementary signs: septa and scalloping of the liver margins, respectively. Effusion in the lesser peritoneal cavity suggests this diagnosis. Magnetic resonance imaging, by showing the gelatinous ascites, their septa and the scalloping of liver and spleen, can strengthen the probability of the diagnosis. Only laparotomy can confirm it, however. Appendectomy is required in all cases. Recurrence is more frequent in the forms associated with malignant or bipolar tumors. Cytoreductive surgery combined with hyperthermic intraperitoneal chemo...

This report focuses on the borderline category of ovarian mucinous tumors and summarizes the points of general agreement and persistent controversies identified by experts in the field who participated in the Borderline Ovarian Tumor Workshop held in Bethesda, MD, in August 2003. Points of agreement and persistent controversies regarding nomenclature, diagnostic criteria, and behavior are addressed for the following ovarian mucinous tumor categories: mucinous borderline ovarian tumor (M-BOT; synonymously referred to as atypical proliferative mucinous tumor of ovary or mucinous ovarian tumor of low malignant potential), M-BOT with intraepithelial carcinoma, and M-BOT with microinvasion. The morphologic spectrum of M-BOTs with regard to distinction from mucinous cystadenoma and the confluent glandular/expansile type of invasive mucinous carcinoma is also addressed. Non-ovarian mucinous tumors, including the secondary ovarian mucinous tumors associated with pseudomyxoma peritonei and m...

We present an unusual case of peritoneal mesothelioma with ultrasound (US) and computed tomographic (CT) features of scalloping of liver margins and ascitic septations mimicking pseudomyxoma peritonei. A brief review of the literature is also presented.

Pseudomyxoma peritonei is a rare disease characterized by a large amount of mucinous ascites with peritoneal and omental implants. The etiology of the disease remains unclear. Different histological categories have been described: the benign, malignant, and the intermediate forms. It is commonly diagnosed incidentally at laparotomy. Most investigators agree that radical surgical debulking and appendectomy are the cornerstone of treatment, but the optimal management of the disease remains controversial. The role of intraoperative and intraperitoneal chemotherapy has been evaluated by a number of authors. The clinical outcomes vary widely between the different histological types and treatment modalities.

Pseudomyxoma peritonei (PMP) is characterized by mucinous ascites, predominantly arising form a perforated tumour of the appendix. This study aimed to assess Health-Related Quality of Life (HRQL) in patients following cytoreductive surgery and intraperitoneal chemotherapy for PMP.Over a one year period, 49 consecutive patients (13 male, 36 females) with a median age of 55 (range 37–81 years) were enrolled. Patients were asked to complete the European Organization for Research and Treatment of Cancer (EORTC) QLQ-C30 questionnaire prior to surgery and at one, three, six and twelve months post-operatively.26 patients (53%) underwent complete cytoreduction and 20 (42%) patients had major tumour debulking. One patient died from progressive disease three months from surgery and two patients withdrew from the study within 6 months of surgery. Baseline questionnaire compliance was 100 per cent and remained high (overall 98% of eligible patients) during follow up. Grade III/IV morbidity occurred in 4 patients (9%). Patients undergoing both complete cytoreduction and major tumour debulking reported a clinically significant improvement in emotional well-being, appetite and global HRQL at 1 year following surgery.Despite the high morbidity associated with cytoreductive surgery and intraperitoneal chemotherapy, an improvement in quality of life at 1 year following the procedure was seen following both complete cytoreduction and major tumour debulking. Longer term assessment is required to demonstrate the durability of this enhancement.

Experience with Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in a pioneer hospital resulted in a treatment protocol that has become the standard in the Netherlands. Outcome of CRS and HIPEC was reviewed to assure differences between the pioneer phase and the period wherein the Dutch HIPEC protocol was clinically implemented. The first consecutive 100 CRS and HIPEC procedures performed in the Netherlands were included as pioneer cohort (1995-1999). Two-hundred and seventy-two procedures that were performed in three participating HIPEC centres after the implementation of the Dutch HIPEC protocol were included as the implementation cohort (2005-2012). Another 100 recent patients of the first centre were included as a control group (2009-2011). Indications for the CRS and HIPEC treatment were peritoneal carcinomatosis (PC) from colorectal carcinoma and pseudomyxoma peritonei (PMP). Of the 472 included procedures, 327 (69 %) procedures were performed ...

Pseudomyxoma peritonei (PMP) is a rare clinical condition characterized by disseminating gelatinous ascites within the peritoneal cavity with mucinous implants on peritoneal surfaces. We present the case of a patient incidentally diagnosed after laparoscopy: definitive diagnosis after the histological examination was PMP. A 37-year-old female patient with a medical history of infertility and mild pelvic pain was found to have several collections in the pelvis and an amount of free fluid into the Douglas pouch at ultrasound examination. The patient underwent laparoscopic surgical exploration. Peritoneal biopsies and appendectomy were performed. Histological examination was about a low-grade appendiceal mucinous tumor limited to the mucosa without submucosal infiltration with perforation of the wall and deposit of periappendicular acellular mucin. The patient was discharged in good health and referred to an oncological peritoneal center where cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) were performed. In conclusion, PMP is an uncommon disease within the abdomen, characterized by a mucinous tumor that produces progressive mucinous ascites. It is characterized by various non-specific symptoms and signs and difficult imaging diagnoses. Histological diagnosis is a determinant to establish the therapy that can differ significantly, depending on the stage of the disease.

Purpose Pseudomyxoma peritonei (PMP) originating from an appendiceal mucinous neoplasm remains a biologically heterogeneous disease. The purpose of our study was to evaluate outcome and long-term survival after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) consolidated through an international registry study. Patients and Methods A retrospective multi-institutional registry was established through collaborative efforts of participating units affiliated with the Peritoneal Surface Oncology Group International. Results Two thousand two hundred ninety-eight patients from 16 specialized units underwent CRS for PMP. Treatment-related mortality was 2% and major operative complications occurred in 24% of patients. The median survival rate was 196 months (16.3 years) and the median progression-free survival rate was 98 months (8.2 years), with 10- and 15-year survival rates of 63% and 59%, respectively. Multivariate analysis identified prior chemotherapy ...

Background This randomized phase III study was to evaluate the efficacy and safety of cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) for the treatment of peritoneal carcinomatosis (PC) from gastric cancer. Methods Sixty-eight gastric PC patients were randomized into CRS alone (n = 34) or CRS + HIPEC (n = 34) receiving cisplatin 120 mg and mitomycin C 30 mg each in 6000 ml of normal saline at 43 ± 0.5°C for 60–90 min. The primary end point was overall survival, and the secondary end points were safety profiles. Results Major clinicopathological characteristics were balanced between the 2 groups. The PC index was 2–36 (median 15) in the CRS + HIPEC and 3–23 (median 15) in the CRS groups (P = 0.489). The completeness of CRS score (CC 0–1) was 58.8% (20 of 34) in the CRS and 58.8% (20 of 34) in the CRS + HIPEC groups (P = 1.000). At a median follow-up of 32 months (7.5–83.5 months), death occurred in 33 of 34 (97.1%) cases in the CRS group and 29 of 34 (85.3%) cases of the CRS + HIPEC group. The median survival was 6.5 months (95% confidence interval 4.8–8.2 months) in CRS and 11.0 months (95% confidence interval 10.0–11.9 months) in the CRS + HIPEC groups (P = 0.046). Four patients (11.7%) in the CRS group and 5 (14.7%) patients in the CRS + HIPEC group developed serious adverse events (P = 0.839). Multivariate analysis found CRS + HIPEC, synchronous PC, CC 0–1, systemic chemotherapy ≥ 6 cycles, and no serious adverse events were independent predictors for better survival. Conclusions For synchronous gastric PC, CRS + HIPEC with mitomycin C 30 mg and cisplatin 120 mg may improve survival with acceptable morbidity.

Background Cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC) can improve survival in selected patients with primary or secondary peritoneal malignancies. With the opportunity for long-term survival, questions about the impact of those procedures in fertility in women of childbearing age can be raised. Materials and Methods An international survey was performed among all teams participating in the International Peritoneal Surface Malignancy Group in order to collect data about pregnancies and their outcome in women having undergone previous CRS with adjuvant HIPEC. Results There were 7 pregnancies reported after CRS and HIPEC in women treated for peritoneal malignancies. All these women conceived spontaneously, most of them within 2 years after the procedure. They delivered most often by vaginal way after an uneventful pregnancy. Their newborns were healthy, except 1 case of congenital diaphragmatic hernia requiring emergent surgery. There were 2 additional uneventful pregnancies reported after the diagnosis of pseudomyxoma peritonei and before CRS and HIPEC, with the support of the medical team. Another woman having undergone oocytes retrieval and embryo cryopreservation prior to the surgery was mother of twins after the procedure via a surrogate mother. Conclusion Childbearing after cytoreductive surgery and heated intraperitoneal chemotherapy is possible in women conserving their genital organs after the procedure. The question of fertility should be considered and discussed in women in reproductive age prior to cytoreductive surgery and heated intraperitoneal chemotherapy. Different options could be offered in this setting. Multidisciplinary decision making involving surgical oncologists and fertility specialists is important.

L'organisation de la prise en charge des patients atteints de cancers rares du péritoine s'appuie sur un réseau national (RENAPE) dont l'objectif est de garantir à chaque patient l'accès à l'expertise médicale quel que soit son lieu de traitement, à la fois par la mise en place d'une relecture de ses prélèvements tumoraux par des experts pathologistes pour un diagnostic certain, et par la discussion systématique de son dossier médical par des experts cliniciens pour guider la prise en charge thérapeutique. Le réseau RENAPE participe à l'actualisation de recommandations de bonnes pratiques cliniques et contribue à promouvoir la recherche sur ces cancers rares à travers des études fondamentales, translationnelles et cliniques qui peuvent s'appuyer sur une base de don-nées nationale et une tumorothèque virtuelle associée. Abstract The French health care organization for rare peritoneal malignancies has been structured with a national network (RENAPE). The RENAPE network is responsible for arranging double reading of tumour specimens, structur-ing referral multidisciplinary consultative meetings, sponsoring multicentre studies in basic, translational and clinical research on these rare cancers, drafting and updating recommendations for good practice, establishing a national database to contribute to the epidemiological observation of these cancers, organizing training for all health professionals involved, working in collaboration with the patient association (AMARAPE), and providing relevant information to general public.

Pseudomyxoma peritonei (PMP) is primarily the result of a ruptured mucinous appendix neoplasm (MAN). Often MAN is lumped with but biologically distinct from intestinal appendiceal adenocarcinoma. Nodal and systemic dissemination are rare with the peritoneal cavity being the primary site of recurrence. Routine performance of right hemicolectomy (RHC) for PMP/MAN has been extensively debated without consensus. Our objective was to ascertain whether RHC has a survival advantage over appendectomy. We hypothesize if RHC is mandatory, then increased tumor recurrence and mortality should be observed in appendectomy only. Retrospective chart review was carried out in patients with tumors that met the Ronnett classification for PMP/MAN. Demographics, tumor grade, extent, recurrence, and progression were recorded. We report the rate of nodal involvement/recurrence in patients treated with RHC versus appendectomy as well as the rate of systemic and peritoneal recurrence and survival. Multivariate logistic regression was done to identify factors that impact survival. Of 120 patients, 48 had appendectomy and 72 had RHC. Seven per cent of patients undergoing RHC had positive lymph nodes and no nodal failures (0%) in patients undergoing appendectomy. Appendectomy versus RHC recurrence rates (21 vs. 28%, P = 0.12) and death resulting from disease (8 vs. 22%, P = 0.27) were similar. Logistic regression revealed that the type of surgery had no impact on recurrence and mortality, only optimal resection score and performance status. There was no difference in tumor recurrence or survival based on treatment by appendectomy or RHC. Performance status and complete cytoreduction are the only factors associated with survival. Lymph node involvement is rare and selective RHC is safe in PMP/MAN.

Pseudomyxoma peritonei (PMP) is a rare clinical condition, with an incidence of 1-2 cases per million, characterized by the dissemination of mucinous implants on the peritoneal surface and progressive gelatinous ascites. Although it usually presents an indolent behavior, its non-specific clinical presentation contributes to many cases remaining undiagnosed until a laparotomy is performed. With late diagnosis, performed after a long period of clinical deterioration and disease progression, it is common to find complications such as the formation of intestinal fistulas and obstruction. Review of the medical record and search for references in the Medline, Lilacs, SciELO and MD Consult databases. There are rare case reports found in the literature demonstrating atypical PMP presentations. Our report is that of a 17-year-old adolescent with a sporadic tumor diagnosed in a primary site in the transverse colon, contrary to data commonly found in the literature that mention a more frequent...

A 51-year old male presented with a one-month history of abdominal pain and diarrhea (2-3 episodes daily, with blood (red coloured) in the feces). He also had hemorrhoids, and so he thought that these were the reason for the blood. On CT scanning of the whole abdomen, an inflammatory mass with cystic and solid characteristics was seen, arising from the