Pseudomyxoma peritonei, also known as "jelly belly", is a condition characterized by mucus accumulation and disseminated tumor cells in the peritoneal cavity. It typically arises from a primary appendiceal or colon tumor that ruptures, releasing mucus and cells. Treatment involves surgical debulking to remove all visible tumor, followed by hyperthermic intraperitoneal chemotherapy to address remaining microscopic disease. Complete cytoreduction and low disease burden based on the peritoneal cancer index are associated with improved outcomes.

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PSEUDOMYXOMA PERITONEI
DR. PRIYADARSHAN KONAR (PGT)
DEPARTMENT OF SURGERY
IMS &SUM HOSPITAL, BHUBANESWAR

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INTRODUCTION
 Also known as ‘JELLY BELLY’.
 The condition was first described by the pathologist Carl
Von Rokitansky in 1842.
 Incidence is 1-2 per million/year in western population.
 Median age of disease onset is 55 years.
 Female to male ratio is 7:3

3.  Approximately 10% epithelial appendiceal neoplasm
develop PMP
 Increased to 20% in mucinous appendiceal
neoplasm
 THEORY 1 (Theory of ‘Secretion’)
• Intra-peritoneal extravasation of mucus secretion
of whatever cause.
 THEORY 2 (Theory of ‘collection’)
• Consider as a clinical syndrome
• Intra-peritoneal collection of gelatinous material
& mucinous tumor cells.
 Sound definition is not available.

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CLASSIFICATION
Ronnet et al suggested a common classification system
with 3 subtypes based on different pathological
characteristics & prognoses. They are:
① Disseminated Peritoneal Adenomucinosis (DPAM)
• Low grade lesion
• Abundant extracellular mucin
• Lack of cytological atypia or mytotic activity
• Usually from mucinous neoplasms of appendix
• Good prognosis

5. ② Peritoneal Mucinous Adenocarcinoma (PMCA)
• High grade metastatic adenocarcinoma
• Abundant mucinous epithelium with architectural
& cytological features of carcinoma
• Derived from appendix & colon
③ Intermediate type PMP (PMCA-I)
• Predominant features of DPAM
• Also focal areas of PMCA
• Prognosis between the DPAM & PCMA

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PATHOPHYSIOLOGY
ORIGIN:
 Appendix, generally mucinous adenoma.
 Colon, Stomach, Pancreas, Ovary and Urachus.
 The primary tumour consists
1) Mucinous cystadenoma
2) Cystadenocarcinoma with low malignant
potential.
 Controversy over origin at appendiceal/ovarian sites
– Immunoreactivity test
• Ovarian neoplasms +ve for CK7
• PMP +ve for CK18 & CK20

7. Appendiceal mucinous neoplasm
Mucus production by tumour cell in the appendiceal lumen
Obstruction of the appendiceal base by tumour or fecal material
Intraluminal mucus accumulation
Appendiceal mucocele
Rising intraluminal pressure
Small perforation of the appendiceal wall or blow out of the mucocele

8. Slow leak or sudden release of mucus
Free mucinous epithelial tumour cells in the peritoneal cavity
Tumour cells continue to proliferate
Mucinous ascites production
Free mucus at different abdominal sites, containing no
or few epithelial tumour cells.
This stage is called Pseudomyxoma Peritonei.

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A. Pseudomyxoma Peritonei (Jelly Belly)
B. Mucocele Appendix

10. Redistribution phenomenon
• Gravity accumulates deposits in the pelvis
• Irregular surface of ovaries & fimbrie act as a
stepping stone PMP tumor cells

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CLINICAL PRESENTATION
1. Abdominal distension (30%-50%) – “Jelly Belly”.
2. Intestinal obstruction associated with compressing
tumour and ascites.
3. Local symptoms, like Appendicitis (50%-80%) –
reflecting the location of the primary and metastatic
tumour.
4. Incidentally detected in female patient (20%-30%)
during evaluation of lower abdominal mass, pelvic
mass, menstual problem or infertility.

12. 5. Coincidentally detected (1%-20%) during USG / CT
Scan for some other reason, at laparotomy, or during
hernia repair when mucus is found in the hernial sac.
6. Patient present with suspected bladder tumour or
right femoral neuropathy (1%).
 Interval between the primary (appendiceal) tumor and
the clinical diagnosis of PMP vary significantly
 Approximately 2 years
 Upto 20 years

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LABORATORY TESTS &
IMMUNOHISTOCHEMICAL
MARKERS
 CA 125
• Gynaecological marker to exclude ovarian neoplasm.
• Not widely used as tumor marker.
• Elevated in benign and inflammatory diseases.
• Sensitivity 60%
 Cytokeratin (CK) 20, CDX-2 are +ve in primary tumors
of colorectal and appendiceal origin.
 CK7 +ve in primary tumors of ovarian origin

14.  IL 9 +ve in 96% patients.
 CEA elevation in 56% to 75 % patients.
 CA 19-9 elevation in 58% to 67% patients.

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TUMOUR ASSESSMENT
USG Abdomen:
• Useful in appendiceal mucocele or PMP
• Usually in combination with CT
• Advantages
 Low cost
 Accessibility
 Possibility of of immediate FNA Biopsy
 Disappointing
 Inconclusive
o Sparse cellular density of both low and
high grade lesions.

16. USG Abdomen shows ascites with multiple
septations and echogenic masses in
peritoneal cavity

17. CT Scan with oral, rectal and IV contrast:
• Gold standard diagnostic.
• Mucinous Ascites
• Low attenuation of soft tissue masses
• Rim like calcifications.
• Septae.

18. Peritoneal Cavity with mucinous tumor in the pelvis

19.  MRI:
• Reported signal characteristics of the collections
include
 T1: Typically low signal
 T2: Typically high signal
 T1 C + (Gd): May show enhancement

20. MRI image of Pseudomyxoma peritonei.
Blue arrow showing thickened mesentry.

21. PERITONEAL CANCER INDEX (PCI):

22. SUGARBAKER Peritoneal Cancer Index
 Used in decision making process as abdomen is
explored.
 Size of intraperitoneal nodules must be assessed.
 Number of nodules is not scored.
 Regions 13
 Maximum score 13 x 3 = 39
 Score < 12 is Favourable Prognosis.
Berthet B et al. Eur J Cancer 1999

23.  Non-invasive malignancy:
• Large mass of non-invasive tumor can be
completely cytoreduced.
• PCI of 39 for these tumors can be converted to 0
by cytoreduction.
 Invasive tumor at crucial anatomic site:
• Eg. Invasive tumor not resectable from CBD
cause poor prognosis even with low PCI.

24.  In earliest stage
 Mucus around the appendix and appendiceal
primary neoplasm or mucocele
 Visceral and mesentric sparing – favourable
post-surgery prognosis
 In end-stage disease
 Entanglement of the gastric Antrum, lesser
omentum, left sub-phrenic region, spleen and
rectosigmoid

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TREATMENT
A. SURGICAL MANAGEMENT OF
PSEUDOMYXOMA PERITONEI (CRS)
B. HYPERTHERMIC INTRAPERITONEAL
CHEMOTHERAPY (HIPEC)
C. COMBINED MODALITY TREATMENT
CRS + HIPEC (Introduced by SUGARBAKER in 1990)

26. A. SURGICAL MANAGEMENT OF PSEUDOMYXOMA
PERITONEI:
 Also known as CytoReductive Surgery (CRS)
 Aim is complete removal of all macroscopically visible
tumor cells.
 Magnitude of surgery depends on extent and
distribution of disease.
 Only diseased peritoneum is removed.
 Normal peritoneum may be removed “Peritonectomy
of convenience” to mobilise right and left colon and
gain access to pelvis.

27.  Selection of patient for surgical interventions:
Two main indications –
1) Where complete tumor removal is likely
2) For symptoms amenable to surgical intervention
by palliative tumor debulking

28.  Most common procedures include:
 Lesser & greater omentectomy
 Right & left parietal peritonectomy
 Right & left diaphragmatic & pelvic peritonectomy
 Removal of liver visceral peritoneum by liver
capsulectomy with routine cholecystectomy
 Bilateral salpingo-oophorectomy & hysterectomy in
females

29.  Splenectomy if tumor invaginates spleen
 Partial or total gastrectomy
 Sigmoid and upper rectal resection may be needed,
particularlly in women with h/o previous major pelvic
surgery
 If Anterior resection is needed, many surgeons
defunction the anastomosis by loop ileostomy
 If the primary is in situ, a radical appendectomy may
suffice in low grade tumors, though right
Hemicolectomy is recommended for high grade
tumors where needed.

30.  Mucinous tumor on parietal peritoneum
• Resection by high grade electrocautery with ball-
tipped handpiece on high voltage pure cut.
• Possible due to non-invasive behaviour of PMP.
Adenomucinosis
• Dissected with finger & gauge if involving
residual visceral peritoneum on stomach , colon
& small bowel.

31.  Concentration of disease in Ileocecal region
• Ileocecal resection or a right hemicolectomy
• Ileum might be anastomosed or brought out as a
ileostomy

32.  Greater omentectomy
• Ligation of the gastric branches in the (right)
gastric arcade
• Contribute blood supply to stomach & left
gastric artery may not be able to sustain
adequate gastric blood flow – resulting in
gastric paresis & postoperative ileus
• Therefore, gastrostomy & high jejunostomy for
gastric emptying & nutrition

33. B. HYPERTHERMIC INTRAPERITONEAL
CHEMOTHERAPY (HIPEC)
 Direct delivery into the peritoneal cavity
 Generally Mytomycin C @ 10mg/sq.m heated to 42 °c
 Permits high concentrations of drugs directly towards the
tumor deposits
 Without systemic side effect
 Hyperthermia enhance penetration of cytostatic drugs
 It can only penetrate tumor nodules up to 2-3 mm in
size.

34.  Should be performed intra-operatively before
anastomoses, to maximize uniform drug distribution
& tumor exposure.
 In h/o prior surgery, adhesiolysis should be
performed to prevent nonuniform drug distribution
 If microscopic tumor residues not eliminated – may
lead to recurrent disease.
 Also apply post-operative intra-peritoneal
chemotherapeutic agent.

35.  Two Techniques
1) OPEN
• Small bowel floats in drug solution
2) CLOSED
• No loss of heat or drug
• Entire peritoneum is exposed
• Allow administration of drug under pressure.

36. • Doxorubicin(15mg/sq.m)
• Melphalan
• Mitomycin-C(12mg/sq.m)
• Cisplatin(50mg/sq.m)
• Gemcitabine
• Mitoxantrone
• Oxaliplatin
• Etoposide
 CHEMOTHERAPEUTIC AGENTS:
• Irinotecan
• Paclitaxel
• Docetaxel
• 5-Fluorouracil
(500mg/sq.m)
• Carboplatin

37.  CARRIERS OF CHEMOTHERAPEUTIC AGENTS:
A. Isotonic salt solutions & Dextrose solutions
• Most commonly used
• Rapid absorption
• Inability to maintain a prolonged high
intraperitoneal fluid volume
B. Hypotonic solutions
• Cisplatin accumulates in tumor cells & enhance
cytotoxicity
• High incidence of unexplained postoperative
peritoneal bleeding with Oxaloplatin

38. C. Hypertonic solutions
• High intra-peritoneal volume achieved for
prolonged duration
D. Isotonic high molecular weight solutions
• Prolonged high intra-peritoneal volume

39.  DURATION FOR PERFUSION:
• 41°c x 90 minutes
• 42°c x 60 minutes
• 43°c x 30-40 minutes

40.  COMPLICATIONS:
• Occurs in 30-45% cases
• Chemotherapy toxicity to kidney, bone marrow,
liver, lungs (2-5%)
• Organ damage secondary to hyperthermia
• Surgical complications – small bowel fistula (25-
30%)
• Mortality (0-5%)

41. HIPEC – machine & method

42. HIPEC – open method

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Summary
 “Jelly Belly” – Incidence is 2 per million.
 Redistribution phenomenon predicts location
 Peritoneal disseminated disease from a primary
appendiceal mucinous epithelial neoplasm unless
proven otherwise.
 It is suspected in c/o intraperitoneal mucus with cellular
content, found during laparotomy or at physical
examination.

44. Combination of surgical debulking with peritonectomy
& hyperthermic intraperitoneal chemotherapy seems to
improve outcome.
Patients with high-grade disease (peritoneal mucinous
carcinomatosis), disease extent more than 5 involved
abdominal regions, and/or small bowel involvement
should receive palliative treatment because they do not
benefit from aggressive treatment approaches.

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