Pseudomyxoma Peritonei

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Paul H Sugarbaker - One of the best experts on this subject based on the ideXlab platform.

  • management of an inguinal hernia in patients with Pseudomyxoma Peritonei
    Ejso, 2017
    Co-Authors: Paul H Sugarbaker
    Abstract:

    Abstract Background Pseudomyxoma Peritonei is a disease that results from a perforated mucinous neoplasm of the appendix so that mucinous ascites and mucin-producing tumor cells are widely disseminated in a characteristic pattern throughout the abdomen and pelvis. The intraabdominal mucus can accumulate in the inguinal canal and by physical examination be indistinguishable from the usual inguinal hernia. Methods A database of patients with Pseudomyxoma Peritonei was used to identify patients who had an inguinal hernia prior to or at the time of cytoreductive surgery (CRS) and perioperative hyperthermic chemotherapy (HIPEC). At the time of CRS, care was taken in all patients to remove the peritoneal lining of the inguinal canal. Patients who had the inguinal hernia repaired prior to definitive treatment with CRS and HIPEC had all tissue and mesh associated with prior herniorrhaphy resected. Results In 178 Pseudomyxoma Peritonei patients, 17 had a new onset or previously repaired inguinal hernia that required extraction of mucus and mucinous tumor from the hernia site. No repair of the open inguinal canal was attempted at the time of CRS. No recurrent inguinal hernias were recorded and no patients required an inguinal incision at a later time to resect progressive disease within the inguinal canal. Conclusions Inguinal hernias caused by mucinous ascites and tumor were definitively treated by cytoreductive surgery plus HIPEC. Extraction of tumor and peritoneum from the inguinal canal facilitates fibrous closure of the hernia defect so that hernia recurrence was not observed.

  • cytoreductive surgery plus hyperthermic intraperitoneal chemotherapy for Pseudomyxoma Peritonei arising from urachus
    Annals of Surgical Oncology, 2015
    Co-Authors: Haruaki Ishibashi, Paul H Sugarbaker, Masamitsu Hirano, Kazuyoshi Takeshita, Akiyoshi Mizumoto, Masumi Ichinose, Eisei Nishino, Ippei Kashu, Yoshihiro Yamamoto, Yutaka Yonemura
    Abstract:

    Background Pseudomyxoma Peritonei (PMP) is a rare locoregional disease characterized by disseminated intraperitoneal mucinous tumors. However, little is known about PMP from urachal neoplasm as a result of its rarity.

  • mesenteric cyst report of a case resulting in Pseudomyxoma Peritonei
    Tumori, 2010
    Co-Authors: Luis Zappa, Paul H Sugarbaker
    Abstract:

    A mesenteric cyst may have an embryonic, traumatic, neoplastic or infectious origin. In rare cases mesenteric cysts may contain neoplastic epithelium. A mesenteric cyst has not previously been recorded as the cause of Pseudomyxoma Peritonei.A patient who developed widespread mucinous intraperitoneal tumor as a result of a ruptured mesenteric cyst is reported. A literature review of the clinical features, diagnosis, treatment, and prognosis of the mesenteric cyst is presented.This patient was treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy. Her recovery was uneventful and she remains well two years after treatment.Mesenteric cyst is a rare cause of Pseudomyxoma Peritonei. The definite treatment of a ruptured neoplastic mesenteric cyst can, by analogy, be compared to the treatment for Pseudomyxoma Peritonei of appendiceal origin. The results of cytoreductive surgery with perioperative intraperitoneal chemotherapy are expected to be good.

  • a systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for Pseudomyxoma Peritonei
    Annals of Surgical Oncology, 2007
    Co-Authors: Tristan D Yan, Deborah Black, Renaldo Savady, Paul H Sugarbaker
    Abstract:

    Background The efficacy of cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) for patients with Pseudomyxoma Peritonei (PMP) remains to be established.

  • new standard of care for appendiceal epithelial neoplasms and Pseudomyxoma Peritonei syndrome
    Lancet Oncology, 2006
    Co-Authors: Paul H Sugarbaker
    Abstract:

    Summary Appendiceal mucinous neoplasms sometimes present with peritoneal dissemination, which was previously a lethal condition with a median survival of about 3 years. Traditionally, surgical treatment consisted of debulking that was repeated until no further benefit could be achieved; systemic chemotherapy was sometimes used as a palliative option. Now, visible disease tends to be removed through visceral resections and peritonectomy. To avoid entrapment of tumour cells at operative sites and to destroy small residual mucinous tumour nodules, cytoreductive surgery is combined with intraperitoneal chemotherapy with mitomycin at 42°C. Fluorouracil is then given postoperatively for 5 days. If the mucinous neoplasm is minimally invasive and cytoreduction complete, these treatments result in a 20-year survival of 70%. In the absence of a phase III study, this new combined treatment should be regarded as the standard of care for epithelial appendiceal neoplasms and Pseudomyxoma Peritonei syndrome.

David L Morris - One of the best experts on this subject based on the ideXlab platform.

  • mucinous appendiceal neoplasms with or without Pseudomyxoma Peritonei a review
    Anz Journal of Surgery, 2020
    Co-Authors: Joshua Lansom, Mikael L Soucisse, Mohammad S Alshahrani, David L Morris
    Abstract:

    Mucinous appendiceal neoplasms (MANs) are rare tumours and the primary cause of Pseudomyxoma Peritonei. These tumours have a much more benign course than typical colorectal cancers, generally growing for many years before giving any clinical signs. The spectrum of presentations, tumour stages and the underlying cytology is very wide, warranting from the simplest operation like an appendicectomy to the most complicated operation like a complete cytoreduction surgery and hyperthermic intraperitoneal chemotherapy. Fortunately, most patients can be offered a curative treatment, but limiting operative morbidity without compromising oncologic outcomes is the biggest challenge in managing these patients. Histopathology is the cornerstone of decision making for MANs, but is also subject to ongoing debate because of a lack of terminology consensus amongst pathologists. Combined with the rarity of this disease, the multiple histopathologic classification updates of MANs explain the ongoing confusion amongst clinicians in regard to individual optimal treatment. This review will cover the most recent histological classification of MANs and attempt to clarify optimal management of patients with different clinical presentation and histologic combinations.

  • early postoperative intraperitoneal chemotherapy for low grade appendiceal mucinous neoplasms with Pseudomyxoma Peritonei is it beneficial
    Annals of Surgical Oncology, 2017
    Co-Authors: Yeqian Huang, Winston Liauw, Nayef A Alzahrani, Thamer Bin Traiki, David L Morris
    Abstract:

    Background There is little evidence for the use of early postoperative intraperitoneal chemotherapy (EPIC) in patients with low-grade appendiceal mucinous neoplasms (LAMNs) with Pseudomyxoma Peritonei (PMP). This study aims to assess the outcomes regarding the use of EPIC in a large cohort of patients with LAMNs with PMP uniformly treated by cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC), all of whom received hyperthermic intraperitoneal chemotherapy (HIPEC), and most of whom also received EPIC.

  • upfront compared to delayed cytoreductive surgery and perioperative intraperitoneal chemotherapy for Pseudomyxoma Peritonei is associated with considerably lower perioperative morbidity and recurrence rate
    Annals of Surgery, 2011
    Co-Authors: Terence C Chua, Jing Zhao, Winston Liauw, David L Morris
    Abstract:

    Background: Cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) is a recognized management strategy for Pseudomyxoma Peritonei. We seek to evaluate the outcomes of patients treated upfront with CRS PIC compared to patients undergoing delayed CRS PIC as salvage or treatment for recurrences after initial debulking surgery. Methods: Retrospective analysis of patients with low-grade Pseudomyxoma Peritonei treated within our institution were stratified according to upfront versus delayed CRS PIC after intial debulking surgery. Survival was analyzed using the Kaplan-Meier method and compared using the log-rank test. Results: Of 83 patients treated, 35 patients (42%) underwent upfront and 48 patients (58%) underwent delayed CRS PIC. The peritoneal cancer index (P = 0.048), amount of blood transfusion intraoperatively (P = 0.003) and duration of operation (P = 0.007) was lesser in the upfront compared to delayed group. Upfront treatment confers 5-year recurrence-free survival benefit (77% vs 37%; P = 0.011) and 10-year overall survival benefit (67% vs 35%; P = 0.054) over delayed treatment. Conclusion: Upfront CRS PIC seems to confer beneficial perioperative outcomes and lower recurrence rates over delayed CRS PIC. Early referral to centralized treatment centers would seem to be a reasonable strategy to improve outcomes.

  • long term survival in patients with Pseudomyxoma Peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy 10 years of experience from a single institution
    Annals of Surgical Oncology, 2009
    Co-Authors: Terence C Chua, Tristan D Yan, Jing Zhao, Michelle E Smigielski, Katherine J Zhu, David L Morris
    Abstract:

    Background Cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC) has been recognized as a treatment option for Pseudomyxoma Peritonei. This study reports the survival outcomes, clinicopathological prognostic factors, and a learning curve from a single institution’s experience.

  • long term survival in patients with Pseudomyxoma Peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy 10 years of experience from a single institution
    Annals of Surgical Oncology, 2009
    Co-Authors: Terence C Chua, Tristan D Yan, Jing Zhao, Michelle E Smigielski, Katherine J Zhu, David L Morris
    Abstract:

    Cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC) has been recognized as a treatment option for Pseudomyxoma Peritonei. This study reports the survival outcomes, clinicopathological prognostic factors, and a learning curve from a single institution’s experience. Patients with Pseudomyxoma Peritonei underwent CRS and PIC, which was comprised of hyperthermic intraperitoneal chemotherapy (HIPEC) and/or early postoperative intraperitoneal chemotherapy (EPIC), according to a standardized treatment protocol in our institution. Clinicopathological factors were analyzed to determine their prognostic value for survival using univariate and multivariate analysis. Time period comparison was performed to study the effect of a learning curve. A total of 106 patients (43 men and 63 women) were treated. The mortality rate was 3% and severe morbidity rate was 49%. The median follow-up was 23 (range, 0–140) months. The overall median survival was 104 months with a 5-year survival rate of 75%. The progression-free survival was 40 months with a 1-year progression-free survival rate of 71%. Factors influencing survival include histopathological type of tumor, use of both HIPEC and EPIC, peritoneal cancer index, completeness of cytoreduction, and severe morbidity. The results demonstrate a learning curve where patients with a higher peritoneal cancer index (PCI) were treated, reduced amount of blood products required, more patients undergoing HIPEC and the combined HIPEC and EPIC, more redo-procedures performed, and a longer progression-free survival. This report demonstrates long-term survival outcomes, acceptable perioperative outcomes, and a learning curve associated with the treatment of patients with Pseudomyxoma Peritonei.

Marcello Deraco - One of the best experts on this subject based on the ideXlab platform.

Tristan D Yan - One of the best experts on this subject based on the ideXlab platform.

  • long term survival in patients with Pseudomyxoma Peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy 10 years of experience from a single institution
    Annals of Surgical Oncology, 2009
    Co-Authors: Terence C Chua, Tristan D Yan, Jing Zhao, Michelle E Smigielski, Katherine J Zhu, David L Morris
    Abstract:

    Background Cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC) has been recognized as a treatment option for Pseudomyxoma Peritonei. This study reports the survival outcomes, clinicopathological prognostic factors, and a learning curve from a single institution’s experience.

  • long term survival in patients with Pseudomyxoma Peritonei treated with cytoreductive surgery and perioperative intraperitoneal chemotherapy 10 years of experience from a single institution
    Annals of Surgical Oncology, 2009
    Co-Authors: Terence C Chua, Tristan D Yan, Jing Zhao, Michelle E Smigielski, Katherine J Zhu, David L Morris
    Abstract:

    Cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy (PIC) has been recognized as a treatment option for Pseudomyxoma Peritonei. This study reports the survival outcomes, clinicopathological prognostic factors, and a learning curve from a single institution’s experience. Patients with Pseudomyxoma Peritonei underwent CRS and PIC, which was comprised of hyperthermic intraperitoneal chemotherapy (HIPEC) and/or early postoperative intraperitoneal chemotherapy (EPIC), according to a standardized treatment protocol in our institution. Clinicopathological factors were analyzed to determine their prognostic value for survival using univariate and multivariate analysis. Time period comparison was performed to study the effect of a learning curve. A total of 106 patients (43 men and 63 women) were treated. The mortality rate was 3% and severe morbidity rate was 49%. The median follow-up was 23 (range, 0–140) months. The overall median survival was 104 months with a 5-year survival rate of 75%. The progression-free survival was 40 months with a 1-year progression-free survival rate of 71%. Factors influencing survival include histopathological type of tumor, use of both HIPEC and EPIC, peritoneal cancer index, completeness of cytoreduction, and severe morbidity. The results demonstrate a learning curve where patients with a higher peritoneal cancer index (PCI) were treated, reduced amount of blood products required, more patients undergoing HIPEC and the combined HIPEC and EPIC, more redo-procedures performed, and a longer progression-free survival. This report demonstrates long-term survival outcomes, acceptable perioperative outcomes, and a learning curve associated with the treatment of patients with Pseudomyxoma Peritonei.

  • a systematic review on the efficacy of cytoreductive surgery and perioperative intraperitoneal chemotherapy for Pseudomyxoma Peritonei
    Annals of Surgical Oncology, 2007
    Co-Authors: Tristan D Yan, Deborah Black, Renaldo Savady, Paul H Sugarbaker
    Abstract:

    Background The efficacy of cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) for patients with Pseudomyxoma Peritonei (PMP) remains to be established.

  • cytoreductive surgery and perioperative intraperitoneal chemotherapy for Pseudomyxoma Peritonei from appendiceal mucinous neoplasms
    British Journal of Surgery, 2006
    Co-Authors: Tristan D Yan, Matthew Links, Peter Kam, Derek Glenn, David L Morris
    Abstract:

    Background: Cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) has been used to treat Pseudomyxoma Peritonei. The aim of this prospective study was to evaluate survival outcome and treatment-related prognostic markers in patients who underwent CRS and PIC for Pseudomyxoma Peritonei from appendiceal mucinous neoplasms. Methods: Survival data and 12 clinicopathological and treatment-related prognostic variables for survival were obtained prospectively in 50 consecutive patients (23 men). Univariate analysis was used to determine their prognostic significance for overall survival, determined from the time of CRS. Results: The mean(s.d.) age was 52(12) years. Eighteen patients had moderate complications, and six patients had severe complications that required operation or intensive care support. Two patients died after surgery. The actuarial 5-year survival rate was 69 per cent. Univariate analysis demonstrated that the extent of previous surgery (P = 0·045) and Ronnett's histopathological classification (P < 0·001) were significantly related to overall survival. Conclusion: CRS combined with PIC was associated with improved survival in patients with less extensive previous surgery and diffuse peritoneal adenomucinosis histopathological type. Copyright © 2006 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.

F A N Zoetmulder - One of the best experts on this subject based on the ideXlab platform.

  • Pseudomyxoma Peritonei a review of 62 cases
    Ejso, 2003
    Co-Authors: S Van Ruth, Y I Z Acherman, M J Van De Vijver, A A M Hart, V J Verwaal, F A N Zoetmulder
    Abstract:

    Aim: Pseudomyxoma Peritonei (PMP) is a rare disease characterized by the abundance of mucus in the abdomen without extra-peritoneal growth. Methods: Our patients with PMP have been treated with cytoreduction and hyperthermic intraperitoneal chemotherapy since 1996. The clinical and histopathological features of PMP and the relation of these features with disease-free interval and survival were assessed. Results: Sixty-two patients with PMP (24 M/38 F) were studied. Adenomatous mucosal changes were present in 31 patients. In females, the ovaries were normal in 5 patients and Pseudomyxoma ovarii was present in 20 patients. Patients with minimal atypia and with 1% focal proliferation or less ðn ¼ 38Þ had a better survival ðp ¼ 0:0008Þ than those with more focal proliferation ðn ¼ 14Þ. Conclusion: In most patients with PMP the appendix is affected; in females the ovaries are usually also involved. Focal proliferation appears to be a prognostic factor.

  • prognostic value of baseline and serial carcinoembryonic antigen and carbohydrate antigen 19 9 measurements in patients with Pseudomyxoma Peritonei treated with cytoreduction and hyperthermic intraperitoneal chemotherapy
    Annals of Surgical Oncology, 2002
    Co-Authors: S Van Ruth, A A M Hart, V J Verwaal, J M G Bonfrer, F A N Zoetmulder
    Abstract:

    Tumor markers are useful for diagnosis and follow-up. We studied the prognostic value of baseline and serial carcinembryonic antigen (CEA) and carbohydrate antigen 19.9 (CA 19.9) measurements in patients with Pseudomyxoma Peritonei treated with cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC). Sixty-three patients with Pseudomyxoma Peritonei were treated with cytoreductive surgery and HIPEC. The tumor markers CEA and CA19.9 were collected before therapy and at 3-month intervals during follow-up. Preoperative CEA and CA19.9 levels were increased in, respectively, 75% and 58% of the patients. Baseline tumor marker values were related to the extent of tumor. Immediately after HIPEC, both tumor markers decreased markedly (P<.0001). CA19.9 was shown to be a more useful tumor marker than CEA for follow-up. During follow-up, a high absolute CA19.9 level (P=.0005) was predictive for imminent recurrence. Patients who never attained a normal CA19.9 level showed a higher recurrence rate at 1 year (53%; SE, 15%), in comparison to patients with did so (6%; SE 4%). The median lead time of increased CA19.9 to recurrence was 9 months. The measurement of the tumor marker CA19.9 is useful in evaluating therapy in patients with Pseudomyxoma Peritonei treated with cytoreductive surgery and HIPEC. CA19.9 is a prognostic factor for predicting recurrent disease.

  • extensive surgical cytoreduction and intraoperative hyperthermic intraperitoneal chemotherapy in patients with Pseudomyxoma Peritonei
    British Journal of Surgery, 2001
    Co-Authors: A J Witkamp, E De Bree, M M Kaag, G W Van Slooten, F Van Coevorden, F A N Zoetmulder
    Abstract:

    Background: Pseudomyxoma Peritonei remains a fatal disease. However, extensive surgical cytoreduction combined with intraoperative heated intraperitoneal chemotherapy (HIPEC) has recently emerged as a new treatment modality, which might improve survival. Methods: Patients underwent treatment if the tumour appeared to be technically resectable on preoperative abdominal computed tomography and there were no distant metastases. After aggressive surgical cytoreduction, HIPEC with the administration of mitomycin C was performed for 90 min. Depending on histological grading, patients received adjuvant 5-fluorouracil and leucovorin therapy. Results: Forty-six patients were treated. Optimal surgical cytoreduction was obtained in 40 patients. Postoperative surgical complications occurred in 18 patients. Four patients died as a direct result of the treatment. Bone marrow suppression due to mitomycin C toxicity occurred in 22 patients. There was no other major toxicity related to the HIPEC procedure. After a median follow-up of 12 months, 40 patients are alive, eight of whom have proven recurrence. The actuarial survival rate (Kaplan‐Meier) at 3 years was 81 per cent. Conclusion: These results confirm that extensive surgery combined with HIPEC is feasible in patients with Pseudomyxoma Peritonei and that improved long-term survival might be achieved.