Peritoneal Carcinomatosis
What is Peritoneal Carcinomatosis?
Management of Peritoneal Carcinomatosis from Appendiceal Neoplasms
Aggressive Management of Peritoneal Carcinomatosis from Mucinous Appendiceal Neoplasms
Frances Austin MD, Arun Mavanur MD, Magesh Sathaiah, Jennifer Steel PhD, Diana Lenzner MS, Lekshmi Ramalingam MD, Matthew Holtzman MD, Steven Ahrendt MD, James Pingpank MD, Herbert J. Zeh MD, David L. Bartlett MD, Haroon A. Choudry MDRegional Cancer Therapies, February 2012
Abstract
Background
Peritoneal carcinomatosis (PC) in the setting of mucinous appendiceal neoplasms is characterized by the intraperitoneal accumulation of mucinous ascites and mucin-secreting epithelial cells that leads to progressive compression of intra-abdominal organs, morbidity, and eventual death. We assessed postoperative and oncologic outcomes after aggressive surgical management by experienced surgeons.
Methods
We analyzed clinicopathologic, perioperative, and oncologic outcome data in 282 patients with PC from appendiceal adenocarcinomas between 2001 and 2010 from a prospective database. Kaplan–Meier survival curves and multivariate Cox-regression models were used to identify prognostic factors affecting oncologic outcomes.
Results
Adequate cytoreduction was achieved in 82% of patients (completeness of cytoreduction score (CC)-0: 49%; CC-1: 33%). Median simplified peritoneal cancer index (SPCI), operative time, and estimated blood loss were 14 (range, 0–21), 483.5 min (range, 46–1,402), and 800 ml (range, 0–14,000), respectively. Pathology assessment demonstrated high-grade tumors in 36% of patients and lymph node involvement in 23% of patients. Major postoperative morbidity occurred in 70 (25%) patients. Median overall survival was 6.72 years (95% confidence interval (CI), 4.17 years not reached), with 5 year overall survival probability of 52.7% (95% CI, 42.4, 62%). In a multivariate Cox-regression model, tumor grade, age, preoperative SPCI and chemo-naïve status at surgery were joint significant predictors of overall survival. Tumor grade, postoperative CC-score, prior chemotherapy, and preoperative SPCI were joint significant predictors of time to progression.
Conclusions
Aggressive management of PC from mucinous appendiceal neoplasms, by experienced surgeons, to achieve complete cytoreduction provides long-term survival with low major morbidity.
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Peritoneal Carcinomatosis Description and Diagnosis
Peritoneal Carcinomatosis is broad description in which multiple tumors develop in, and line the peritoneal abdominal cavity and linings. These tumors may be difficult to completely remove surgically and may recur despite conventional systemic chemotherapy.HIPEC may be an appropriate treatment for this condition.
The description of Peritoneal Carcinomatosis includes a variety of tumors that present with extensive metastasis throughout the peritoneal cavity.
This description is used in conjunction with cancers and conditions of appendix, colon, gall bladder, ovaries, mesothelioma, pancreas, Pseudomyxoma Peritonei, rectal, sarcomas, small bowel, and stomach. When tumor develops from the peritoneum, it is referred to as Primary Peritoneal Surface Malignancy.
Diagnosis
The diagnosis of Peritoneal Mucinous Carcinomatosis indicates the presence of mucin accompanied by abundant malignant epithelium, high-grade atypia (Ronnett et al, 1995). Mucinous Carcinomatosis indicates a more challenging prognosis relative to the more common pattern of Disseminated Peritoneal Adenomucinosis aka DPAM.
Diagnosis may be suspected based on physical symptoms, CT scans, and tumor marker tests. However, the actual diagnosis is confirmed by pathologists after examination of tissue and/or mucin or fluid samples.
Symptoms
Symptoms of Peritoneal Carcinomatosis may initially be detected by appearing on as a diffused thickening of the peritoneum on a CT scan. The appearance of ascites refers to the accumulation of fluid within the peritoneal cavity and may occur for a variety of conditions including post operative inflammation or to cancer.
For Appendix Cancer and Psuedomyxoma Peritonei patients, ascites may appear as a rapid increase in fluid within in abdomen. This condition requires immediate medical attention.
The description of Peritoneal Carcinomatosis includes a variety of tumors that present with extensive metastasis throughout the peritoneal cavity.
This description is used in conjunction with cancers and conditions of appendix, colon, gall bladder, ovaries, mesothelioma, pancreas, Pseudomyxoma Peritonei, rectal, sarcomas, small bowel, and stomach. When tumor develops from the peritoneum, it is referred to as Primary Peritoneal Surface Malignancy.
Diagnosis
The diagnosis of Peritoneal Mucinous Carcinomatosis indicates the presence of mucin accompanied by abundant malignant epithelium, high-grade atypia (Ronnett et al, 1995). Mucinous Carcinomatosis indicates a more challenging prognosis relative to the more common pattern of Disseminated Peritoneal Adenomucinosis aka DPAM.
Diagnosis may be suspected based on physical symptoms, CT scans, and tumor marker tests. However, the actual diagnosis is confirmed by pathologists after examination of tissue and/or mucin or fluid samples.
Symptoms
Symptoms of Peritoneal Carcinomatosis may initially be detected by appearing on as a diffused thickening of the peritoneum on a CT scan. The appearance of ascites refers to the accumulation of fluid within the peritoneal cavity and may occur for a variety of conditions including post operative inflammation or to cancer.
For Appendix Cancer and Psuedomyxoma Peritonei patients, ascites may appear as a rapid increase in fluid within in abdomen. This condition requires immediate medical attention.
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Peritoneal Carcinomatosis Treatment with CRS and HIPEC
Dr Pompliliu Piso
Patient Selection, Complications, Quality of Life
Peritoneal carcinomatosis: patients selection, perioperative complications and quality of life related to cytoreductive surgery and hyperthermic intraperitoneal chemotherapy
Abstract below:
Source: Gabriel Glockzin, Hans J Schlitt and Pompiliu Piso, WSJO
Background :Peritoneal tumor dissemination arising from colorectal cancer, appendiceal cancer, gastric cancer, gynecologic malignancies or peritoneal mesothelioma is a common sign of advanced tumor stage or disease recurrence and mostly associated with poor prognosis.
Methods and results:In the present review article preoperative workup, surgical technique, postoperative morbidity and mortality rates, oncological outcome and quality of life after CRS and HIPEC are reported regarding the different tumor entities.
Conclusion: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) provide a promising combined treatment strategy for selected patients with peritoneal carcinomatosis that can improve patient survival and quality of life. The extent of intraperitoneal tumor dissemination and the completeness of cytoreduction are the leading predictors of postoperative patient outcome. Thus, consistent preoperative diagnostics and patient selection are crucial to obtain a complete macroscopic cytoreduction (CCR-0/1).
Peritoneal Carcinomatosis Treatment
Photo courtesy of Dr Marcello Deraco
Peritoneal Carcinomatosis Treatment generally includes specific surgery, with or without HIPEC or systemic chemotherapy. Your specialist can help you determine the best course of treatment for Peritoneal Carcinomatosis.
HIPEC or Hyperthermic Intraperitoneal Chemotherapy, for the Peritoneal Carcinomatosis treatment, may be administered if complete tumor debulking is accomplished through cytoreductive surgery. HIPEC includes the infusion of the abdominal cavity with a specific chemotherapy, heated to approximately 40 degrees Celsius for a period of approximately 90 minutes. The purpose of the HIPEC is to attempt to kill any tumor cells which were not removed during the actual surgery process.
Learn more about HIPEC
Questions to ask your specialist:
How is HIPEC administered?
Is HIPEC effective for the treatment of Pseudomyxoma Peritonei, Appendix Cancer, and Peritoneal Carcinomatosis,etc?
Tumor Grade
“Tumor grade” describes how much the tumor appears like normal tissue when examined under a microscope.
The tumor grade helps physicians predict how quickly the cancer may grow.
G1: well-differentiated tumor cells
G2: moderately differentiated tumor cells
G3: poorly differentiated tumor cells
G4: undifferentiated tumor cells
Peritoneal Carcinomatosis Research, International
Review an extensive series of research articles by the world's most renowned specialists in this field:
Study of 523 Colorectal Peritoneal Carcinomatosis Cases: CRS and IPC
Source: Journal of Clinical Oncology,2010
Management of Peritoneal Carcinomatosis from Gastrointestinal Malignancies:
Research from 3rd International Symposium on Peritioneal Carcinomatosis, June 2008
Source: Prof Pompiliu Piso and ColleaguesToward curative treatment of peritoneal carcinomatosis from nonovarian origin by cytoreductive surgery combined with perioperative intraperitoneal chemotherapy: a multi-institutional study of 1290 patients.Glehen O, Gilly FN, Boutitie F, Bereder JM, Quenet F, Sideris L, Mansvelt B, Lorimier G, Msika S, Elias D; the French Surgical Association.Cancer. 2010 Aug 24. [Epub ahead of print]PMID: 20737573 [PubMed - as supplied by publisher]Related citations
Secondary Cytoreduction and Perioperative Intraperitoneal Chemotherapy after Initial Debulking of Pseudomyxoma Peritonei: A Study of Timing and the Impact of Malignant Dedifferentiation.
Chua TC, Al-Zahrani A, Saxena A, Liauw W, Zhao J, Morris DL.J Am Coll Surg. 2010 Aug 20. [Epub ahead of print]PMID: 20729102 [PubMed - as supplied by publisher]Related citations
[Prospective study of quality of life after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy using oxaliplatin for peritoneal carcinomatosis.]
Lim C, Tordjmann D, Gornet JM, Nemeth J, Valleur P, Pocard M.Bull Cancer. 2010 Aug 6. [Epub ahead of print] French. PMID: 20693116 [PubMed - as supplied by publisher]
Primary peritonectomy/HIPEC for disseminated peritoneal adenomucinosis achieves much lower recurrence rates and better survival than secondary procedures.
Zhu KJ, Morris DL.Surgeon. 2009 Dec;7(6):345-50.PMID: 20681377 [PubMed - indexed for MEDLINE]
Mucinous adenocarcinomas with intra-abdominal dissemination: a review of current therapy.
Winder T, Lenz HJ.Oncologist. 2010;15(8):836-44. Epub 2010 Jul 23.PMID: 20656916 [PubMed - in process]
[Prognostic factors and reproductive outcomes of borderline ovarian tumors: a review of 186 patients]
Tao T, Cao DY, Yang JX, Huang HF, Wu M, Pan LY, Lang JH, Guo LN, Shen K.Zhonghua Yi Xue Za Zhi. 2010 May 18;90(19):1304-8. Chinese. PMID: 20646576 [PubMed - indexed for MEDLINE]Case records of the Massachusetts General Hospital. Case 19-2010. A 35-year-old man with adenocarcinoma of the cecum.
Ryan DP, Engelman JA, Ferrone CR, Sahani DV, Lisovsky M.N Engl J Med. 2010 Jun 24;362(25):2411-9. No abstract available. PMID: 20573930 [PubMed - indexed for MEDLINE]
Mesenteric cyst: report of a case-resulting in pseudomyxoma peritonei.
Zappa L, Sugarbaker PH.Tumori. 2010 Mar-Apr;96(2):332-5.PMID: 20572595 [PubMed - indexed for MEDLINE]
[Peritoneal carcinosis can have as good a prognosis as primary colonic cancer, and should be managed according to evidence-based practice]
Graf W, Mahteme H.Lakartidningen. 2010 May 12-25;107(19-20):1320. No abstract available. PMID: 20556987 [PubMed - indexed for MEDLINE]
Clinical Trials
Click here for a listing of research studies and clinical trials
Peritoneal Carcinomatosis Index Score
Peritoneal Carcinomatosis Index (PCI) Score
Source: European Journal of Surgical Oncology, 2003
Peritoneal Carcinomatosis Index Score: Peritoneal Carcinomatosis Prognosis
Source: Dr Paul H Sugarbaker, 2005
Peritoneal Carcinomatosis Gene Coding
MUC 2 Gene Coding for Peritoneal Carcinomatosis
REG 4 Gene Coding for Peritoneal Carcinomatosis
8th International World Congress on Peritoneal Surface Malignancy
October 31-November 2, 2012
Visit our Conferences and Events page for details
Conference Topics of Discussion:The surgical treatment of peritoneal carcinomatosis
• Perioperative and systemic chemotherapy
• Interdisciplinary treatment options
• Current results of treatment of peritoneal dissemination
of colorectal cancer, gastric cancer, ovarian cancer,
peritoneal mesothelioma, and peritoneal sarcomatosis
• Pathology
• Quality of life issues post-treatment
• Basic research including chemotherapy pharmacology
Prognosis
Patients with DPAM have a significantly more favorable prognosis than patients with peritoneal mucinous carcinomatosis
Source: Drs B Ronnett, Paul Sugarbaker, 2001
Source: Drs B Ronnett, Paul Sugarbaker, 2001
Peritoneal Carcinomatosis Prognosis
Morbidity and Mortality: Study of HIPEC and CRS for Treatment of Peritoneal Carcinomatosis
Source: Annals of Surgery, Feb 2009, Dr David Morris, Dr Tristan Yan
CRS and HIPEC offer longterm survival for Peritoneal Carcinomatosis of disseminated Appendiceal tumor origin
Source: Dr Armando Sardi, Mercy Med Center, Journal of American College of Surgeons, Sept 2009
Peritoneal Carcinomatosis Prognosis following treatment with surgery and HIPEC
Source: Prof P Piso, 2009
Prognosis for Peritoneal Carcinomatosis following surgery and IP treatment at Mayo Clinic
Phase I Study for Patients with Peritoneal Carcinomatosis
Advances in Peritoneal Surface Oncology
Source: Dr S. González-Moreno
http://www.springer.com/west/home/medicine/oncology?SGWID=4-10073-22-112580385-0
Peritoneal Carcinomatosis :
“Appendiceal Carcinoma with Peritoneal Dissemination: Outcomes for the Best of the Best.”
Source: Annals of Surgical Oncology,2007, Dr Edward Levine, WFU
Peritoneal Adenomucinosis vs Peritoneal Mucinous Carcinomatosis
Peritoneal Carcinomatosis and Cox II expression
Peritoneal Carcinomatosis (multiple publications
Peritoneal Carcinomatosis: What Can We Do About It?
Source:ASCO 2004
Prognostic indicators for Peritoneal Carcinomatosis originating with gastrointestinal adenocarcinoma
Source: Dr Paul Sugarbaker, 2005
Prognosis for Peritoneal Carcinomatois originating with Appendix Cancer
Source: Dr Paul Sugarbaker, Washington Hospital Center
Peritoneal Carcinomatosis Prognosis following treatment with TPN administered at home
Source: Pub Med, per "Nutrition" 2006
Peritoneal Carcinomatosis Prognosis with HIPEC
New findings by Dr David L Morrris: CRS and Survival of Appendiceal Cancer Patients
Surgical cytoreduction and survival in appendiceal cancer peritoneal carcinomatosis: an evaluation of 46 consecutive patients.
by Chua TC, Al-Alem I, Saxena A, Liauw W, Morris DL.
CONCLUSIONS: Cytoreductive surgery and intraperitoneal chemotherapy may achieve long-term survival in appendiceal malignancies with peritoneal dissemination for which the predictors of outcomes identified through this study may tailor the disease management to commit patients early toward this successful surgical strategy. Source:Ann Surg Oncol. 2011 Jun;18(6):1540-6. Epub 2011 Apr 14.
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