Management of pseudomyxoma peritonei

Rockson WeiQueen Mary Hospital

Joint Hospital Surgical Grand Round

25th July, 2009

Pseudomyxoma Peritonei

Definition Low grade malignant disease within

the peritoneal cavity

Characterized by 1. Production and accumulation of mucous

2. Mucinous implants

Epidemiology

Incidence ~ 1 per million a year Over 80% from appendix or ovary Other sites: pancreas, bile duct, colon,

gall bladder and urachus

R.M. Smeenket alAppendiceal neoplasms and pseudomyxoma

peritonei: A population based studyEuro J Surg Oncol 2008; 34, 196-201

Clinical presentation

Abdominal pain and distension Symptoms from the primary tumor

mimicking appendicitis inguinal hernia ovarian mass

“Jelly belly” Abundant intraperitoneal mucous

Qu Z, Liu LManagement of pseudomyxoma peritonei

World J Gastroenterology 12 (38): 6124–7

Natural history

Peritoneal seedlings lead to fistula and adhesion

Excessive mucous accumulation compresses intestine

Compromise gastrointestinal function Intestinal obstruction Ends in mortality unless radically treated

Histopathology

Low grade malignancy

Originate from tumours of appendix / ovary Mucinous (cyst)adenoma Mucoceles Mucinous (cyst)adenocarcinoma

Management

Traditional strategy Repeated surgical debulking procedures Intraperitoneal or systemic chemotherapy

LeucovorinFloxuridine

10 year survival only 20%

Culliford AT, Paty PBSurgical debulking and intrapertioneal chemotherapy for

established peritoneal metastases from colon and appendix cancerAnn Surg Oncol 8 (10): 787

Management

Combined treatment strategy1. Peritonectomy with electrosurgery

Maximum radical oncological cytoreductive surgery

2. Intra-operative hyperthermic intraperitoneal chemotherapyEliminates microscopic or minimal residual diseaseHyperthermia increases drug effectiveness

3. Early post-operative intraperitoneal chemotherapy

SugarbakerNew standard of care for appendiceal epithelial

neoplasms and pseudomyxoma peritonei syndromeLancet Oncol 7 (1): 69–76

Indications for combined treatment

1. Large volume of noninvasive peritoneal carcinomatosis

2. Low volume peritoneal seeding of invasive cancer

3. Perforated gastrointestinal cancers

4. Gastrointestinal cancer with ovarian involvement

SugarbakerManagement of peritoneal surface malignancy using

intraperitoneal chemotherapy and cytoreductive surgeryManual for physicians and nurses 1998

Peritonectomy

Removal of all tumour tissues from the parietal and visceral peritoneum

Large tumour nodules must be resected and all visible tumors removed

Small cancer deposits on the visceral peritoneum are also individually electroevaporated

Hyperthermic intraperitoneal chemotherapy (HIPEC)

Aim: to eradicate microscopic residual disease for curative intent

Performed after completion of peritonectomy Catheters are inserted to dependant positions Thermocouples continuously monitor the

inflow,

outflow, and intraperitoneal cavity temperatures Temporary abdominal skin closure Intraperitoneal temperature maintained 42.5℃

HIPEC setup diagram

HIPEC agents

Depends on the tumor histological characteristics. Pseudomyxoma peritonei - Appendix, colon and

stomach Cisplatin (CDDP; 25 mg/m2 per liter) Mitomycin C (MMC; 3.3 mg/m2 per liter)

Ovary, mesothelioma and others Cisplatin (CDDP 43 mg/m2 per liter) Doxorubicin (15.25 mg/m2 per liter)

HIPEC

Advantages: Hyperthermic conditions increase cytotoxicity Heat has anti-tumour effects Prolonged retention improve drug penetration Manual intra-op chemotherapy allows uniform

distribution of drug Eliminates platelets, neutrophils & monocytes

Diminishes promotion of tumour growth associated with wound healing process

HIPEC

Disadvantages Removal of white cells due to chemotherapy

and heat leaves the patient vulnerable to intra-

abdominal infection Strict aseptic technique is required during

administration of chemotherapy

Early postoperative intraperitoneal chemotherapy

5-Florouracil is utilized Commenced immediately after operation

Infusion via Tenckhoff catheter Chemotherapy agent dwell in the abdomen

for 23 hours and drain for 1 hour Repeat 5 times

Effectiveness of combined treatmentSeries Patient

Number5 year survival

10 year survival

Follow up (months)

3 year disease free survival (%)

Traditional treatment

Miner et al 97 80 21 57 12

Gough et al 56 53 32 144 3

Combined treatment

Sugarbaker et al 385 86 80 38 62

Smeenk et al 103 60 50 51 56

Moran et al 100 72 30 70

Elias et al 36 66 60 48 55

Deraco et al 33 97 29 74

Guner et al 28 80 51

Loungnarath et al 27 52 23

Extrapolation of combined treatment

Disease state Number of patients

3 year survival

Primary and recurrent carcinoma of colon / rectum with carcinomatosis

45 41

Stage IV gastric cancer 13 31

Recurrent abdominopelvic sarcoma 50 43

Peritoneal surface malignancy 48 27

Multiple peritoneal metastases Not readily reproducible Controversial

Sugarbaker 1990, 1998a, 1998b, 2003

Summary

Combined treatment (Sugerbaker) Peritonectomy Intra-operative hyperthermic

intraperitoneal chemotherapy Early post-operative chemotherapy

Effective for pseudomyxoma peritonei