Transcript Updates on Pseudomyxoma Peritonei

Joint Hospital Surgical Grand Round 2/2/2013
Princess Margaret Hospital
HM Li
Pseudomyxoma peritonei
 Low grade malignant disease within the peritoneal
cavity, with characteristic mucus production and
accumulation
 Wide spectrum of diseases of intraperitoneal
extravasations of mucus secretion of various causes
 Disseminated peritoneal adenomucinosis (DPAM)
 Peritoneal mucinous carcinomatosis (PMCA)
 Peak incidence: 5th decade, female > male
 Incidence: 1-2 per million per year
Appendiceal neoplasms and Psuedomyxoma peritonei: a population based study
Smeenk et.al
Eur J Surg Oncol 2008; 34:196-201
Presenting symptoms
 Increase intra-abdominal pressure:
bloating, reflux, hernia (new-onset),
uterovaginal prolapse
 Mimicking acute appendicitis – at
laparotomy / laparoscopy
 Palpable abdominal lump: ovarian
mass, omental cake
 Non-specific abdominal pain
 Constitutional symptoms: anorexia,
weight loss, ascites
Disease progression
 Peritoneal seeding
 Adhesion
 Intestinal compression  obstruction
 Death
 Features of aggressive diseases
 Short time interval from first to subsequent
presentations
 Poor general condition (cachexia, muscle wasting)
 P/E: multiple solid lumps
Investigation
 Blood: ↓Hb, ↑CRP, ↑ESR, ↑CEA/CA125/CA19.9
 Imaging:
 Xray: usually not helpful in diagnosis; except when
complications arise (e.g. IO)
 USG:
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
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Non-mobile echogenic ascites
Homogenous tumour deposits
Scalloping of liver and spleen
Small mucinous vesicles
Guidance for less viscous areas for diagnostic paracentesis
 Contrast CT:
Ebrahim et.al
SA Journal of radiology Sep2011: 79-81
 MRI:
 Similar morpholocial features shown by CT
 T2 weighted images enable differentiation between
mucinous and fluid ascites
Management
 Surgery
 Complete cytoreduction
 Debulking
 Chemotherapy
 Intraperitoneal chemotherapy
 Systemic chemotherapy
Debulking + Chemotherapy
 Traditional approach
 Removal of mucin and tumour bulk
 +/- limited resectional procedures
 Right hemicolectomy
 Partial omentectomy
 Hysterectomy and bilateral oophorectomy’
 Repeated laparotomies for symptomatic recurrences
 Ineffective and dangerous for risk of bowel injury, fistula
formation, or death
Combined treatment Approach
 Complete cytoreductive surgery (CRS)
 Intra-operative hyperthermic intraperitoneal
chemotherapy (HIPEC)
 Early post-operative intraperitoneal chemotherapy
(EPIC)
New standard of care for appendiceal epithelial
neoplasms and pseudomyxoma peritonei syndrome
Sugarbaker
Lancet Oncol 2006; 7:69-76
Complete cytoreductive surgery
(CRS)
 Aim: remove macroscopic tumour (no residual tumour
deposit >2.5mm in diameter)
 Peritonectomy
 Right and left parietal peritonectomy
 Bilateral subdiaphragmatic peritonectomy
 Pelvic peritonectomy
 Greater and lesser omentectomy
 Visceral resection of involved organs
 Right hemicolectomy, AR, HBSO, splenectomy,
cholecystectomy, liver capsulectomy, gastrectomy, distal
pancreatectomy, small bowel resection
Intraperitoneal chemotherapy
 Aim: microscopic clearance after effective surgical
cytoreduction
 Eradicates cells and small-volume peritoneal disease
by a local effect
 Chemotherapeutic agents enter tumour cells by
diffusion and penetrate tumour nodules up to 2-3mm
 Mitomycin C – intraoperative IPC
  5-Fluorouracil for 4-5 days postoperative IPC
 Cisplatin, paclitaxel – under evaluation
Hyperthermic intraperitoneal
chemotherapy (HIPEC)
 Coliseum technique
 Chemotherapeutic agents (Mitomycin) are heated to
42C within the abdomen perfusion of intraperitoneal organs
 Heat
 Anti-neoplastic effects
 Synergize cytotoxicity of the chemotherapeutic agents
 Augment the penetration of drug into tissues
Value of laparoscopy before CRS and HIPEC for
Peritoneal carcinomatosis
Iversen et.al; British Journal of Surgery 2013; 100: 285–
292
Peritonectomy
Sugarbaker
Surg Oncol Clin N Am 2003; 12: 703-727
Hyperthermic intraperitoneal
chemotherapy (HIPEC)
 Coliseum technique
 Chemotherapeutic agents (Mitomycin) are heated to
42C within the abdomen perfusion of intraperitoneal organs
 Heat
 Anti-neoplastic effects
 Synergize cytotoxicity of the chemotherapeutic agents
 Augment the penetration of drug into tissues
Early postoperative intraperitoneal
chemotherapy (EPIC)
 5-Florouracil for 5 days, on POD0
 Infusion via the tenchkoff catheter
 Chemotherapeutic agent stay within the peritoneum
for 23 hours than drain for an hour for 5 times
Complications of combined approach
 Death rate 1-2%
 Sepsis (associated with intestinal fistulae)
 Cardiovascular and respiratory complications
 Serious morbidity 30-40%
 Sepsis (chest, intra-abdominal, wound, urinary)
 Anastomotic leakage
 Fistula formation
 Reoperation for bleeding
 Thromboembolism
 Survival benefit noted
with complete CRS
 Complete CRS had a
survival of 70% at 20 years
New standard of care for appendiceal epithelial neoplasms and
pseudomyxoma peritonei syndrome
Sugarbaker; Lancet Oncol 2006; 7:69-76
 Debulking procedures
 5YSR ~40%
 ~90%: disease recurrence within 2 year and require
multiple debulking procedures
 Combined cytoreductive surgery and IPEC
 5YSR ~75%
 5YDFS: 38-74%
 Mortality 1-2%
 Significant morbidity ~40%
Improved survival of patients with PMP receiving intraperitoneal
chemotherapy with cytoreductive surgery - a systemic review and
meta-analysis; McBride et.al; Journal of surgical research 2013: 1-7
Conclusion
 PMP is a rare disease, yet it can cause significant
morbidity and mortality if left untreated
 Combined approach of complete cytoreductive surgery
and IPC had been replacing the use of debulking
surgery
 Improved survival
 Significant morbidity
References
 Peritoneal pseudomyxoma: results of a systematic policy of complete cytoreductive surgery and
hyperthermic intraperitoneal chemotherapy; Elias et.al; British Journal of Surgery 2008; 95: 1164-1171
 Pseudomyxoma peritonei: a French multicenter study of 301 patients treated with cytoreductive
surgery and intraperitoneal chemotherapy; Elias et.al: EJSO 2010; 36:456-462
 Tumour Review: Pseudomyxoma peritonei; Smeenk et.al; Cancer Treatment Reviews 2007; 33: 138145
 Appendiceal neoplasms and pseudomyxoma peritonei: a population based study; Smeenk et.al;
EJSO 2008; 34: 196-201
 Pseudomyxoma peritonei: review of the controversy; Bradley et.al; Current Diagnostic Pathology
2007;13: 410-416
 Operative findings, early complications, and long-term survival in 456 patients with
pseudomyxoma peritonei syndrome of appendiceal origin; Youssef et.al; Diseases of the colon and
rectum 2011;54(3): 293-299
 Overview of pseudomyxoma peritonei; Harshen et.al; Clinical Oncology 2003; 15: 73-77
 Critical Analysis of Treatment failure after complete cytoreductive surgery and perioperative
intraperitoneal chemotherapy for peritoneal dissemination from appendiceal mucinous neoplasms;
Yan et.al; Annals of surgical oncology 2007; 14(8): 2289-2299
 Evaluation of complete cytoreductive surgery and two intraperitoneal chemotherapy techniques in
pseudomyxoma peritonei; Sorensen et.al; EJSO 2012; 38: 969-976
 The importance of the learning curve and surveillance of surgical performance in peritoneal surface
malignancy programs; Kusamura et.al; Surg Oncol Clin N Am 2012; 21: 559-576
 Management of peritoneal surface malignancy; Sugarbaker et.al; Surg Oncol Clin N Am 2003: 12:
xxi-xxiv
References
 New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome;
Sugarbaker; Lancet Oncol 2006; 7: 69-76
 Peritonectomy procedures; Sugarbaker et.al; Surg Oncol Clin N Am 2003; 12: 703-727
 NICE guideline 2004
 Outcome differences between debulking surgery and cytoreductive surgery in patients with
pseudomyxoma peritonei; Andreasson et.al; ESJO 2012; 38: 962-968
 Pseudomyxoma peritonei; Mukherjee et.al; Surgery (Oxford) 2004; 22: 238-241
 Pseudomyxoma peritonei; Curr Probl Surg 2008; 45: 527-575
 Pseudomyxoma peritonei – a rare entity (CT); Ebrahim et.al; SA Journal of Radiology 2011: 79-81
 Secondary cytoreduction and perioperative intraperitoneal chemotherapy after initial debulking of
pseudomyxoma peritonei: a study of timing and the impact of malignant dedifferentiation; Chua
et.al; J Am Coll Surg 2010; 211: 526-535
 The etiology, clinical presentation, and management of pseudomyxoma peritonei; Moran et.al; Surg
Oncol Clin N Am 2003; 12: 585-603
 Improved survival of patients with PMP receiving intraperitoneal chemotherapy with cytoreductive
surgery - a systemic review and meta-analysis; McBride et.al; Journal of surgical research 2013: 1-7
 Value of laparoscopy before cytoreductive surgery and hyperthermic intraperitoneal chemotherapy
for peritoneal carcinomatosis; Iversen et.al; British Journal of Surgery 2013; 100: 285-292
 Disseminated Peritoneal Adenomucinosis and peritoneal mucinous carcinomatosis - A
clinicopathologic Analysis of 109 cases with emphasis on distinguishing pathologic features, site of
origin, prognosis, and relationship to pseudomyxoma peritonei; Ronnett et.al; The American
Journal of Surgical Pathology 1995; 19(12): 1390-1408
Thank You