Pseudomyxoma peritonei (PMP) and its therapy – 20 years’ experience of a single surgical department

2020 ◽  
Vol 99 (4) ◽  
pp. 159-166
Keyword(s):  
Median Survival ◽  
Intraperitoneal Chemotherapy ◽  
Pseudomyxoma Peritonei ◽  
High Efficiency ◽  
Entire Group ◽  
Low Grade ◽  
Imaging Methods ◽  
Surgical Department ◽  
Mucinous Tumours ◽  
The Mean

Introduction: Pseudomyxoma peritonei (PMP) is a rare malignant disease with various grades of malignancy, producing mucinous and gelatinous masses. The origin of PMP is usually connected with the rupture of appendiceal mucinous tumours, other mucinous tumours of the gastrointestinal tract or of the ovary. The staging of this disease is determined by the PCI score (peritoneal cancer index), and the efficiency of surgical procedure by the CC score. Clinical presentation is very variable and depends on the stage of the disease. Many patients are asymptomatic with a minimal clinical finding, presented only with abdominal discomfort. A typical finding of the “jelly belly“ syndrome expands with progression of the disease. The diagnosis consists in preoperative determination of the tumour characteristics and PCI based on imaging methods, especially CT imaging. Methods: The Sugarbaker technique of complete tumour removal or the so-called cytoreductive surgery (CRS) was used, including hyperthermic intraperitoneal chemotherapy (HIPEC) or alternatively early postoperative intraperitoneal chemotherapy (EPIC). We performed retrospective evaluation of 73 patients with pseudomyxoma peritonei undergoing surgery, 39 males and 34 females, mean age 50.6 and 56.4 years, respectively. Surgical revision was performed in 18 patients, 14 males and 4 females. The mean age of this group was 48.8 for the males and 47 for the females. The surgical procedures were performed between 1999 and 2018. Survival rates, median survival, complications based on Clavien-Dindo classification, lethality rates, and PCI and CC scores were assessed in the patient group. Results: 96 surgeries were performed in 73 patients with pseudomyxoma peritonei at our surgical department between 1999−2018. The surgery had to be repeated in 18 patients (24.6%). High grade (HG) pseudomyxoma was diagnosed in 29 patients (39.7%), and low grade (LG) pseudomyxoma in 44 patients (60.3%). Overall morbidity was 27.3%, and the mortality rate was 5.4%. The mean overall survival (OS) was 139.5 months in the LG pseudomyxoma group and 71.5 months in the HG pseudomyxoma group. Median survival was 86 months in the entire group and 72 in the HG pseudomyxoma group; the median was not reached in the LG pseudomyxoma group. Conclusions: Results in the literature and our results are comparable, confirming the high efficiency of this method both in the world and in the Czech republic. The results indicate a highly statistically significant improvement of the OS with acceptable mortality and morbidity. These results confirm this method as a gold standard therapy for selected patients.

EP.FRI.999 Case report of a Low Grade appendiceal mucinous neoplasm (LAMN) and review of literature

2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Kimberly Da Costa ◽  
Sivakumaran Sabanathan
Keyword(s):  
Intraperitoneal Chemotherapy ◽  
Pseudomyxoma Peritonei ◽  
Right Hemicolectomy ◽  
Low Grade ◽  
Mucinous Neoplasm ◽  
Mucinous Neoplasms ◽  
Mucocele Of The Appendix ◽  
Preoperative Ct ◽  
Inflammatory Processes ◽  
Progressive Accumulation

Abstract A mucocele refers to an appendix that has dilated due to progressive accumulation of mucus within its lumen. Appendiceal mucocele is a rare cause of an acute abdomen. They represent 0.2-0.7% of all appendix specimens. LAMN account for less than 0.3% of appendicectomy specimens.  We present a 38 year old man with an acute RIF’s pain who went on to have CT scan which revealed a mucocele of appendix but did not show any features of perforation or pseudomyxoma peritonei. He had a laparoscopic converted to open appendicectomy. The proximal 2 cm of appendix was oedematous but normal calibre. Histology revealed a low grade appendiceal mucinous neoplasm (LAMN) that was completely excised.  The mucocele of the appendix was first described by Rokitansky in 1842. Appendix mucocele may come as a consequence of obstructive or inflammatory processes, cystadenomas or cystadenocarcinomas. Appendiceal mucinous neoplasms commonly presents in the sixth decade of life and our patient was much younger in comparison. Several literatures suggest the value of preoperative CT imaging in obtaining diagnosis and also in planning further treatment. Appendicectomy or a right hemicolectomy is treatment of choice based on presence or absence of following factors 1. Perforated mucocele 2. Involvement of the base of the appendix. 3. Positive lymph nodes of mesoappendix and ileocolic. Patients with malignancy or pseudomyxoma peritonei are likely to require cytoreductive surgery, heated intraoperative intraperitoneal chemotherapy, early postoperative intraperitoneal chemotherapy.

Supratentorial low-grade glioma in adults: an analysis of prognostic factors and timing of radiation.

1997 ◽  
Vol 15 (4) ◽  
pp. 1294-1301 ◽  
Author(s):  
C Leighton ◽  
B Fisher ◽  
G Bauman ◽  
S Depiero ◽  
L Stitt ◽  
...  
Keyword(s):  
Median Survival ◽  
Karnofsky Performance Status ◽  
Univariate Analysis ◽  
Residual Tumor ◽  
Low Grade Glioma ◽  
Cancer Center ◽  
Entire Group ◽  
Low Grade ◽  
Mixed Glioma ◽  
Modern Imaging

PURPOSE To review the outcomes of patients with low-grade glioma diagnosed by modern imaging and treated at a center where postponing radiotherapy was common practice. METHODS We reviewed the records of patients (age > or = 18 years) with pathologically confirmed supratentorial low-grade fibrillary astrocytoma, oligodendroglioma, and mixed glioma treated at a regional cancer center in Canada between 1979 and 1995. RESULTS Median survival for the entire group (N = 167; mean age 40.6 years) was 10.5 years with 5- and 10-year survival rates of 72% and 50%, respectively. Median progression-free survival was 4.9 years with 5- and 10-year progression-free rates of 50% and 12%, respectively. Overall and progression-free survivals were longer for patients with an oligodendroglioma or mixed glioma than with astrocytoma (median 13 v 7.5 years, P = .003; progression-free 5.6 v 4.4 years, p = .054). Age at diagnosis < or = 40 years, seizures at presentation, minimal residual tumor after surgery, Karnofsky performance status > or = 70, and oligodendroglioma or mixed glioma pathology were associated with significantly longer median survival on univariate and multivariate analyses. Radiotherapy deferred until tumor progression (v immediate radiotherapy) was associated with longer survival on univariate analysis, but an imbalance in other variables accounted for this advantage such that timing of radiotherapy was not an independent (favorable or adverse) prognostic factor on multivariate analysis. CONCLUSION Patients with low-grade glioma diagnosed by modern imaging can be expected to live a long time; timing of radiotherapy may be a less important determinant of survival than nontreatment variables and residual tumor bulk.

scholarly journals Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei of Appendiceal Origin: A Single Center Experience

2021 ◽  
Vol 8 ◽  
Author(s):  
Francesco Santullo ◽  
Fabio Pacelli ◽  
Carlo Abatini ◽  
Miriam Attalla El Halabieh ◽  
Giusy Fortunato ◽  
...  
Keyword(s):  
Survival Rate ◽  
Intraperitoneal Chemotherapy ◽  
Cytoreductive Surgery ◽  
Pseudomyxoma Peritonei ◽  
Hyperthermic Intraperitoneal Chemotherapy ◽  
Progression Free Survival ◽  
Free Survival ◽  
Crs And Hipec ◽  
The Mean ◽  
Incomplete Cytoreduction

Background: Pseudomyxoma peritonei (PMP) originating from appendiceal mucinous neoplasm is a rare peritoneal malignancy characterized by the progressive intraperitoneal accumulation of mucus leading to death if left untreated. In recent years, cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) offered increased survival rates. This study aims to identify the clinical, pathological, and surgical features influencing safety and survival outcomes of patients undergoing CRS and HIPEC for PMP of appendiceal origin.Methods: A retrospective analysis of all patients undergoing CRS and HIPEC for PMP of appendiceal origin from January 2015 to May 2019 was conducted at our institution.Results: Study population included 50 patients (74% female, 26% male). The median age at CRS was 60 (38–84). The median peritoneal cancer index (PCI) was 17. Complete cytoreductive surgery (CC 0–1) was achieved in 47 patients (94%). HIPEC chemotherapeutic regimen was based on oxaliplatin for 13 (28%) patients and mitomycin for 34 (72%) patients. We experienced a total of 19 (38%) postoperative complications, of which 14 (74%) of grade I-II and 5 (26%) of grade III-IV, according to the Clavien-Dindo classification. The median follow-up period was 27 months (12–107) from the date of cytoreductive surgery. The mean survival rate was 100 months, with a 5-year OS of 91%. The mean progression-free survival rate was 77 months (0–107), with a 5-year PFS of 63%. Multivariate analysis identified adenocarcinoma histotype and incomplete cytoreduction to significantly worsen progression-free survival, while incomplete cytoreduction was the only independent predictor of poorer overall survival.Conclusion: Complete cytoreduction and appendiceal neoplasm histotype play a crucial role in the survival of patients affected by PMP of appendiceal origin. The rates of morbidity associated with CRS and HIPEC for PMP are acceptable.

scholarly journals Two-step cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei with high peritoneal carcinomatosis index

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Bertrand Trilling ◽  
Alexandre Brind’Amour ◽  
Raphael Hamad ◽  
Jean-Francois Tremblay ◽  
Pierre Dubé ◽  
...  
Keyword(s):  
Peritoneal Carcinomatosis ◽  
Intraperitoneal Chemotherapy ◽  
Cytoreductive Surgery ◽  
Pseudomyxoma Peritonei ◽  
Hyperthermic Intraperitoneal Chemotherapy ◽  
Postoperative Morbidity ◽  
Low Grade ◽  
Peritoneal Carcinomatosis Index ◽  
Second Surgery ◽  
Very High

Abstract Background Complete cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the only curative treatment for pseudomyxoma peritonei (PMP) arising from the appendix. High peritoneal carcinomatosis index (PCI) is associated with an increased risk of surgical complications. The objective of this study was to present the results of a planned two-step surgical strategy to decrease postoperative morbidity and improve resectability of patients with very high PCI. Methods All consecutive patients who underwent a planned two-step surgical approach for PMP between January 2012 and March 2020 were retrospectively included. This approach was offered for patients with low-grade PMP with PCI > 28 for which feasibility of a complete CRS in one operation was uncertain. The first surgery included a complete CRS of the inframesocolic compartment and omentectomy. HIPEC was delivered at the second surgery, after complete CRS of the supramesocolic compartment. Postoperative morbidity was assessed using the Clavien-Dindo classification and survival results were also collected. Results Eight patients underwent the two-step approach. The median PCI was 33 (29–39) and the median time between the two procedures was 111 days (90–212 days). One patient was deemed unresectable at the second surgery. The rate of major morbidity was 0% for the first step and 25% for the second step, with no mortality. Median follow-up was 53.8 months (3–73 months). Conclusion A two-step surgical management for low-grade PMP patients with very high PCI is safe and feasible, with acceptable postoperative morbidity and no compromise on oncological outcomes.

Treatment of pseudomyxoma peritonei by intraoperative and intraperitoneal chemotherapy

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e16540-e16540
Author(s):  
M. Friedrich ◽  
W. Poleska ◽  
J. Baltzer ◽  
D. Salehin
Keyword(s):  
Side Effects ◽  
Intraperitoneal Chemotherapy ◽  
Pseudomyxoma Peritonei ◽  
Mucinous Cystadenoma ◽  
Ovarian Tumour ◽  
The Other ◽  
Mucinous Cystadenocarcinoma ◽  
Borderline Tumour ◽  
Mucinous Tumours

e16540 Background: Pseudomyxoma peritonei occurs mostly in conjunction with the type of intestinal mucinous borderline tumour and is characterized by building up a lot of mucus pour of cells. The most common tumor is the pseudomyxoma peritoneii with mucinous borderline tumours of the ovaries or with mucinous tumours of the appendix, normally without showing a rupture of the ovarian tumour pre- or intraoperatively. The diagnosis of pseudomyxoma peritonei is mainly difficult and guidelines for the treatment are unknown. Methods: In the period from 1991 to 2008, 52 patients with pseudomyxoma peritonei were treated by tumour debulking and intraoperative and intraperitoneal chemotherapy with Mitoxantron (40 mg in 300 ml of NaCl over 72 hours). During the tumour debulking a CUSA system was used. Results: The median follow-up was 8.2 years. There were the following histologies: mucinous cystadenoma of the ovary n = 29, mucinous cystadenoma of the appendix n = 10, mucinous cystadenocarcinoma n = 13. Reccurrences were seen in 4 patients. These patients were treated for reccurrences by the same way as mention above. All these patients were now without any recurrences. The other 48 patients are still alive with recurrences. Conclusions: The instillation of mitoxantron intraperitoneally and intraoperatively is an effective and safe therapy without any side effects after maximal tumour debulking of pseudomyxoma peritoneii. No significant financial relationships to disclose.

Cytoreductive surgery combined with perioperative intraperitoneal chemotherapy for the management of peritoneal carcinomatosis from digestive or primitive origin: A multi-institutional study of 1,290 patients

2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 4102-4102
Author(s):  
O. Glehen ◽  
D. Elias ◽  
F. N. Gilly ◽  
F. Boutitie ◽  
J. M. Bereder ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Peritoneal Carcinomatosis ◽  
Median Survival ◽  
Intraperitoneal Chemotherapy ◽  
Cytoreductive Surgery ◽  
Pseudomyxoma Peritonei ◽  
Peritoneal Mesothelioma ◽  
Term Survival ◽  
Mortality And Morbidity ◽  
Appendiceal Adenocarcinoma

4102 Background: Peritoneal carcinomatosis (PC) from non-gynecologic malignancies has long been regarded a terminal disease with a short median survival. Over the past decade, new locoregional therapeutic approaches combining cytoreductive surgery with perioperative intraperitoneal chemotherapy (PIC) have evolved and suggest improved survival. Methods: A retrospective multicentric study was performed in French speaking centers to evaluate the toxicity and the principal prognostic factors in order to identify the best indications. All patients had cytoreductive surgery and PIC (hyperthermic intraperitoneal chemotherapy (HIPEC) and or early postoperative intraperitoneal chemotherapy (EPIC)). Results: The study included 1290 patients from 25 institutions between February 1989 and December 2007. The principal etiologies of PC were colorectal adenocarcinoma (N=523), pseudomyxoma peritonei (N=301), gastric adenocarcinoma (N=159), peritoneal mesothelioma (N=88), and appendiceal adenocarcinoma (N=50). Mortality and morbidity rates were 4.1% and 33.8%, respectively. By multivariate analysis, age, extent of the PC, and the institution's experience had a significant influence on toxicity. The overall median survival was 34 months: 30 months for colorectal PC, not reached for pseudomyxoma peritonei, 9 months for gastric PC, 41 months for peritoneal mesothelioma, and 77 months for PC from appendiceal adenocarcinoma. Independent prognostic indicators by multivariate analysis were the institution's experience, etiology, completeness of cytoreductive surgery, extent of carcinomatosis, and lymph node involvement. Conclusions: The therapeutic approach combining cytoreductive surgery with PIC may achieve long-term survival in a selected group of patients with PC from non-gynecologic origin with acceptable mortality and morbidity. This treatment should be reserved to experienced institutions involved in the management of PC. No significant financial relationships to disclose.

Appendiceal Cancer and Peritoneal Carcinomatosis: A Report of 29 Cases

2006 ◽  
Vol 72 (8) ◽  
pp. 714-718
Author(s):  
Jesus Esquivel ◽  
Barry Merriman ◽  
Sallie Davis ◽  
Darrell Manning
Keyword(s):  
Median Survival ◽  
Intraperitoneal Chemotherapy ◽  
Cytoreductive Surgery ◽  
Peritoneal Dissemination ◽  
Hazard Ratio ◽  
Low Grade ◽  
Peritoneal Surface Malignancies ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Peritoneal surface malignancies of appendiceal origin arise from a perforated neoplasm with gradual expansion of the tumor within the abdomen. We report our experience with 29 patients. Between February 2000 and November 2004, 29 patients were classified into one of three groups based on the features of their peritoneal dissemination. Group 1 included those with extracellular mucin with little cellular atypia (disseminated peritoneal adenomucinosis/low-grade mucinous adenocarcinomas). Group 2 included those with peritoneal mucinous carcinomatosis/high-grade mucinous adenocarcinomas, and Group 3 included those with nonmucinous carcinomatosis. There were 17 patients in Group 1, 9 in Group 2, and 3 in Group 3. The majority had cytoreductive surgery and intraperitoneal chemotherapy. There were no operative deaths. Mean follow-up was 21 months. Median survival for Groups 1 and 2 has not been reached. Group 3 patients were more likely to die than Group 1 patients, with a hazard ratio of 48.0 ( P = 0.001), and Group 2 patients with a hazard ratio of 7.8 ( P = 0.029). Median survival for Group 3 was 5 months. These data add to the growing evidence that supports cytoreductive surgery and intraperitoneal chemotherapy in a selected group of patients. Those with mucinous peritoneal dissemination are more likely to benefit from this approach. It appears that in patients with nonmucinous carcinomatosis, the biology of the tumor predicts their outcome.

scholarly journals Two-step cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for pseudomyxoma peritonei with high peritoneal carcinomatosis index

2020 ◽  
Author(s):  
Bertrand Trilling ◽  
Alexandre Brind'Amour ◽  
Raphael Hamad ◽  
Jean-Francois Tremblay ◽  
Pierre Dubé ◽  
...  
Keyword(s):  
Peritoneal Carcinomatosis ◽  
Intraperitoneal Chemotherapy ◽  
Cytoreductive Surgery ◽  
Pseudomyxoma Peritonei ◽  
Hyperthermic Intraperitoneal Chemotherapy ◽  
Postoperative Morbidity ◽  
Low Grade ◽  
Peritoneal Carcinomatosis Index ◽  
Second Surgery ◽  
Very High

Abstract Background Complete cytoreductive surgery (CRS) combined with hyperthermic intraperitoneal chemotherapy (HIPEC) is the only curative treatment for pseudomyxoma peritonei (PMP) arising from the appendix. High peritoneal carcinomatosis index (PCI) is associated with an increased risk of surgical complications. The objective of this study was to present the results of a planned two-step surgical strategy to decrease postoperative morbidity and improve resectability of patients with very high PCI.Methods All patients who underwent a planned two-step surgical approach for PMP between January 2012 and March 2020 were included. Data was retrospectively reviewed and analyzed. The first surgery included a complete CRS of the inframesocolic compartment and omentectomy. HIPEC was delivered at the second surgery, after complete CRS of the supramesocolic compartment. Postoperative morbidity was assessed using the Clavien-Dindo classification and survival results were also collected. Results Eight patients underwent the two-step approach. Seven had a low-grade disease and one had a high-grade disease. The median PCI was 33 (29-39) and the median time between the two procedures was 111 days. One patient was deemed unresectable at the second surgery. The rate of major morbidity was 0% for the first step and 25% for the second step, with no mortality. Median follow-up was 53.8 months. Conclusion A two-step surgical management for low-grade PMP patients with very high PCI is safe and feasible, with acceptable postoperative morbidity and no compromise on oncological outcomes.

Sign in / Sign up

Export Citation Format

Share Document