JBUON 2015; 20 (Suppl.
1): S47-S55
ISSN: 1107-0625, online ISSN: 2241-6293 • www.jbuon.com
E-mail: editorial_office@jbuon.com
REVIEW ARTICLE
Management of pseudomyxoma peritonei
Simon A. Fallis, Brendan J. Moran
Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital, Aldermaston Road, Basingstoke, United Kingdom
Summary
Pseudomyxoma peritonei (PMP) is an uncommon clin-
ical condition that typically originates from a perforated
epithelial neoplasm of the appendix. The clinical presenta-
tion is variable, often with non-specific symptoms and is
associated with abdominal distension in advanced cases.
Whilst traditionally considered benign, it is apparent that
PMP represents a spectrum of disease and, at best, should
be considered a “border-line” malignancy.
The condition is characterised by the development of
mucinous ascites. Tumour cells and mucin accumulate at
characteristic sites within the peritoneal cavity according to
the redistribution phenomenon, usually sparing the mobile
small bowel. In advanced cases, high volume disease and
mucinous ascites lead to compression of the gastrointes-
tinal tract, bowel obstruction, and ultimately, starvation.
Controversy still exists over the pathological classification
of PMP and its prognostic value. Computed tomography
Introduction
Pseudomyxoma peritonei (PMP) is an un-
common clinical condition characterized by mu-
cinous ascites and predominantly originates from
a perforated epithelial neoplasm of the appendix
[1,2]. The clinical presentation is variable, often
with non-specific symptoms and is associated
with abdominal distension in advanced cases
[1,2]. Whilst traditionally considered benign, it is
apparent that there is a spectrum varying from
slowly progressive to aggressively malignant dis-
ease such that pseudomyxoma peritonei, at best,
should be considered a “border-line” malignancy
[2]. Similar clinical, radiological and pathological
features may originate from any abdominal muci-
nous tumour, in particular the ovary in females or
Correspondence to: Brendan J. Moran, MCh FRCS FRCSI. Peritoneal Malignancy Institute, Basingstoke and North Hampshire Hospital,
Aldermaston Road, Basingstoke, RG24 9NA, United Kingdom. E-mail: brendan.moran@hhft.nhs.uk
Received: 12/02/2015; Accepted: 02/03/2015
remains the optimal preoperative staging investigation. El-
evation of serum tumour markers correlates with a worse
prognosis.
Optimal treatment involves cytoreductive surgery with
hyperthermic intraperitoneal chemotherapy (HIPEC). With
complete cytoreduction and HIPEC an 80% 5 year survival
can be achieved in patients with low grade disease. Max-
imal tumour debulking can produce good palliation and
long term survival in a small number of patients.
Initial high morbidity and mortality is seen to decrease
with increasing experience and this is likely to represent
improvement in patient selection and postoperative man-
agement as well as surgical expertise.
Key words: appendiceal mucinous tumour, cytoreductive
surgery, HIPEC, jelly belly, peritoneal malignancy, pseu-
domyxoma peritonei
colorectal pathology in males or females. PMP of
non-appendiceal origin tends to be at the adverse
end of the spectrum. The primary tumour is more
likely to be a mucinous adenocarcinoma with a
worse prognosis than that in classical PMP of ap-
pendiceal origin.
The incidence of PMP is unknown as there is
no substantial information on the true incidence of
either appendiceal mucinous tumours or of PMP.
Estimates of an incidence of PMP of 1 per million
per year had been proposed [3], though this was
based on a figure with no scientific evidence. An
epidemiological analysis by Smeenk et al in 2008
of a population based study in the Netherlands [4]
reported an incidence of mucinous epithelial ne-
S48 Management of pseudomyxoma peritonei

oplasm of the appendix of 0.3% and progression

to PMP in 20%. Extrapolations from this paper
estimates the incidence of PMP as 2 per million
per year. However, experience in a high-volume
centralized treatment centre has suggested that
the incidence may be higher with 3 to 4 operable
cases per million per year [5].

Pathophysiology of PMP and the Con-

cept of the “Redistribution Phenome-
non”
PMP arises from mucin secreting peritoneal
and omental implants secondary to a perforated
mucinous neoplasm, typically originating in the Figure 1. Omental cake and mucinous ascites with
appendix. The initiating appendiceal neoplasm relative sparing of the small bowel.
progresses and gradually occludes the lumen,
causing the appendix to become distended with
sterile mucin, eventually perforating, and spilling
mucous and mucinous tumour cells into the peri-
toneal cavity. Following the initial rupture there
is often a continued slow leak of mucus.
Although some cases present as acute appen-
dicitis, many occur without symptoms. It is hy-
pothesized that the gradual occlusion of the lu-
men prevents significant bacterial contamination
of the mucin distended distal appendix.
Once free within the peritoneal cavity, epithe-
lial cells continue to proliferate and can produce
significant volumes of mucus. This culminates
in the characteristic accumulation of gelatinous
mucus in the peritoneal cavity, also commonly re-
ferred to as “jelly belly” [6]. Whilst the classical Figure 2. Disease on the under surface of the right
PMP appearances originate form an appendiceal diaphragm.
tumour, the clinical, radiological and indeed im-
age guided biopsy appearances of “jelly belly”
may also originate from a true adenocarcinoma
of the appendix, of the colon or rectum, primary gravity. The physiology of the peritoneal cavity
peritoneal or ovarian malignancies, and there are involves production, circulation and absorption of
indeed case reports and small series of PMP cas- peritoneal fluid. The main sites of fluid reabsorp-
es originating from most intra-abdominal organs, tion are the greater and lesser omentum (account-
including the stomach, pancreas, liver, gallblad- ing for the classical “omental cake”, Figure 1) and
der, urinary bladder and urachus [2,7]. the undersurface of the diaphragm, particularly
The distribution of mucinous tumour im- the right side, resulting in tumour accumulation
plants within the peritoneal cavity is determined in the subdiaphragmatic and suprahepatic regions
by what has been termed “the redistribution phe- (Figure 2).
nomenon” [2,8]. Rupture of the primary tumour The second main mechanism is by gravity
results in release of free floating cells and mucin with cell accumulation in dependent sites, such
which disseminate throughout the abdominal as the recto-vesical pouch, the right retro-hepatic
cavity. The epithelial tumour cells have either space and the paracolic gutters [2,8].
none, or low, adhesion properties and consequent- Mobile organs such as the small bowel and
ly distribute within the peritoneal fluid [8]. Char- its mesentery are usually spared, particularly ear-
acteristically cellular deposits accumulate, and ly on in the course of the disease. In contrast the
proliferate, in predetermined sites by two main less mobile, partially retroperitoneal, ascending
mechanisms, absorption of peritoneal fluid and and sigmoid colon, as well as the fixed points of

JBUON 2015; 20 (Suppl. 1): S48

 Management of pseudomyxoma peritonei S49

the stomach in its distal portion and the duode-

no- jejunal flexure at the ligament of Treitz can
be heavily involved by disease and may warrant
bowel resections such as colectomy and distal
gastrectomy to remove troublesome deposits
(Figure 3 and 4).
The relative sparing of the motile small bow-
el and its mesentery allows complete removal of
tumour in most patients without the need for sub-
stantial small bowel resection.
Extensive small bowel involvement can occur
at an early stage in more aggressive tumours and
even in less invasive tumours when the disease
Figure 3. Extensive disease encasing the stomach.
is at an advanced stage. Prior attempts at tumour
removal, particularly where extensive abdominal
surgery has been performed, can lead to tumour
proliferation in scar tissue and may involve the
small bowel at the sites of adhesions. Extensive
small bowel involvement, particularly if the dis-
ease involves the serosa or infiltrates at the junc-
tion of the small bowel with its mesentery, may
prevent a complete tumour removal.
In advanced cases, high volume disease and
mucinous ascites lead to compression of the gas-
trointestinal tract, bowel obstruction, and ulti-
mately, starvation. More aggressive tumours can
lead to the involvement of the bowel at an earlier
stage.

Clinical presentation
Figure 4. Following complete cytoreduction and par-
In the early stages of PMP, many patients tial gastrectomy prior to gastro-duodenal anastomosis.
have no symptoms. Even when the disease burden
is marked, abdominal symptoms can be vague.
Some patients may have been investigated with
luminal endoscopy and labelled with irritable
bowel syndrome.
The initial appendiceal tumour is commonly
asymptomatic, even when perforated, but suspect-
ed appendicitis is a common mode of presenta-
tion. Management of an unexpected appendiceal
neoplasm has been summarised elsewhere [9].
In 2000, Esquivel and Sugarbaker [10] looked
at 410 patients with appendiceal tumours. Overall
the most common presentations were suspected
appendicitis (27%), increasing abdominal dis-
tention (23%) and a new onset hernia (14%). In
women, the diagnosis was most commonly diag-
nosed after gynaecological investigation revealed
an ovarian mass. Computed tomography (CT) now
plays an increasingly important role in diagnosis.
We examined the mode of presentation of 222
patients undergoing surgery for PMP in Basing-
stoke in 2012 and 2013 [11]. Overall 36.5% of pa- Figure 5. CT image showing scalloping of the liver
tients were diagnosed by preoperative CT alone from mucinous deposits..

JBUON 2015; 20 (Suppl. 1): S49

S50 Management of pseudomyxoma peritonei
and 14.4% by an abnormal CT that led to opera-
tive confirmation. 20.7% were diagnosed at lap-
aroscopy or laparotomy with acute symptoms or
on histology after appendicectomy. An incidental
finding at surgery for a new onset hernia account-
ed for 5%.
Many reports suggest an increased incidence
in women but his may result from a lower thresh-
old for abdominal imaging in women on suspi-
cion of ovarian pathology resulting in both more
frequent and earlier diagnosis. In addition pro-
gressive ovarian involvement may lead to earlier
onset of symptoms.
In advanced cases, physical examination may
detect important clinical signs. Shifting dullness
of ascites suggest serous rather than mucinous
ascites. Mucinous ascites may be too dense to re-
distribute when the patient is repositioned. Large
ovarian masses and an omental cake are some-
times palpable. Disease in the rectovesical pouch
may be felt on digital rectal examination.
Investigations
A CT of the chest, abdomen and pelvis with
intra venous and oral contrast is the investigation
of choice [12]. Mucinous disease is typically rep-
resented by areas of low attenuation with islands
of high attenuation due to solid material within
the mucinous ascites. Tumour deposits on the vis-
ceral surfaces of the liver and spleen lead to the
classical appearances of “scalloping” on CT, dis-
tinguishing it from fluid ascites (Figure 5).
A striking feature of PMP is the relative spar-
ing of the small bowel and its mesentery. In more
advanced cases this may lead to compartmentali-
sation of the small bowel in the central abdomen,
surrounded by a massive omental cake and muci-
nous ascites. Sparing of the small bowel and mes-
entery are essential for complete tumour removal.
Contrast enhanced CT can help predict the likeli-
hood of successful cytoreductive surgery. Adverse
radiological features associated with small bowel
involvement include segmental obstruction and
tumour masses greater than 5cm on the small
bowel and its mesentery. When both features are
present there is an 88% probability of incomplete
resection compared with a 92% probability of
complete resection when both are absent [12].
Magnetic resonance imaging (MRI) in the
assessment of PMP has been proposed [13,14]
and appears promising but requires further eval-
uation. Positron emission tomography (PET) and
PET-CT have a limited role in the investigation of
low grade mucinous disease but may be of value
JBUON 2015; 20 (Suppl. 1): S50
in detecting extra-abdominal disease or liver me-
tastases in patients with adenocarcinoma [15].
Serum tumour markers can provide useful
prognostic information in patients undergoing
surgery for PMP. CEA, Ca 125 and Ca 19.9 have
been found to have both diagnostic and predictive
value in some patients [16-18]. In a study of 519
patients who underwent complete tumour remov-
al in Basingstoke, patients with normal tumour
markers had significantly higher disease free and
overall survival compared with patients with ele-
vated tumour markers [19]. The number of elevat-
ed markers (0 –all three) correlated with a worse
outcome.
Tumour markers appear to provide prognos-
tic information independent of histopathological
grading and may have a role in determining con-
sideration of post-operative systemic chemother-
apy and timing and frequency of follow-up.
When cross sectional imaging is equivocal or
tissue is required for histological confirmation,
laparoscopy and biopsy can be useful. Wherever
possible, laparoscopic ports should be positioned
in the midline, such that these sites can be excised
by a midline laparotomy wound to reduce the risk
of tumour seeding. With advanced or recurrent
disease, laparoscopic access and visualisation of
the peritoneal cavity can be difficult and danger-
ous and for PMP adds little in most cases.
Treatment
The optimal management of pseudomyxoma
peritonei is complete cytoreductive surgery (CRS)
in combination with hyperthermic intraperitoneal
chemotherapy (HIPEC). The aim of this strategy
is to remove all visible disease within the perito-
neal cavity. The intraoperative HIPEC then targets
any residual microscopic disease, or small volume
macroscopic tumour nodules (<2.5mm) [20-22].
Completeness of cytoreduction has been shown to
be the most important prognostic factor.
With an average operating time of 9 hours
[23], CRS and HIPEC is a major surgical interven-
tion. Specialised anaesthetic and perioperative
management is required. Positioning of the pa-
tient on the operating table requires experience
in order to allow full access to the abdomen and
perineum whilst minimising the risk of neurolog-
ical compression and compartment syndrome.
The operation starts with a midline incision
from xiphisternum to symphysis pubis, excising
the umbilicus and any previous midline scar.
Once the abdomen is open a full assessment of
the disease can be made. We usually commence
 Management of pseudomyxoma peritonei
with a right parietal peritonectomy and mobili-
sation of the right colon identifying the right
ureter and gonadal vessels. Providing there is
widespread disease, peritonectomy is continued
to perform a right diaphragmatic peritonectomy
with full mobilisation of the liver. If required, a
liver capsulectomy is performed with the help
of a ball tipped diathermy at maximal setting. A
high power smoke extraction system is essential
to remove the resulting smoke. The same proce-
dure is repeated on the left side with a left parietal
peritonectomy, identification of the left ureter and
gonadal vessels and left diaphragmatic peritonec-
tomy if required.
A radical greater omentectomy is performed
inside the gastro-epiploic arcade and the spleen is
assessed. If the spleen is involved with disease a
splenectomy is performed after full mobilisation.
Care is taken not to damage the tail of the pancre-
as. Dissection into the pelvis starts with mobilisa-
tion of the rectum in the mesorectal plane poste-
riorly. Anteriorly the peritoneum is dissected off
the bladder. The rectum and sigmoid colon can
usually be spared but in advanced disease, and
following prior pelvic surgery, an anterior resec-
tion may be required. In the female, the ovaries
are routinely removed. A hysterectomy may also
be necessary. An appendicectomy may be all that
is required to remove the primary tumour. If there
is extensive disease around the caecum or termi-
nal ileum or if there is a likelihood of adenocar-
cinoma then a right hemicolectomy is performed.
In the upper abdomen, the lesser omentum
is removed from the lesser curve of the stomach
preserving the left gastric vessels. The dissection
continues to the porta hepatis, taking care to take
only the peritoneum and preserve the common
bile duct, hepatic artery and portal vein. Identi-
fication of the portal anatomy is aided by retro-
grade cholecystectomy. After mobilisation of the
liver, disease in the aorto-caval grove is removed.
The peritoneum between the caudate lobe, right
crus of the diaphragm and inferior vena cava is
removed. When there is a high volume of upper
abdominal disease a distal gastrectomy may be
necessary. In the series from Basingstoke this was
necessary in almost 10% of patients with PMP
who had a complete cytoreduction [22].
Once all visible tumour has been removed,
HIPEC is administered by a continuous infusion
of Mitomycin C (10mg/m2, with dose adjustments
for patients with renal impairment, significant ab-
dominal distention, recent chemotherapy and old-
er age) heated to 42 degrees for one hour. We use
an open method to administer the chemotherapy
S51
utilising a “coliseum” technique.
After HIPEC the abdomen is washed out and
any gastrointestinal anastomoses are performed.
If an anterior resection has been required a stapled
colorectal anastomosis is performed and routine-
ly defunctioned with a loop ileostomy. Up to four
abdominal drains are inserted. It is our routine
practice to place chest drains if the diaphragmatic
peritoneum has been removed. Patients are man-
aged post-operatively on an intensive care unit. In
some units early post-operative intra-peritoneal
chemotherapy (EPIC) is used. In Basingstoke se-
lected patients receive 5-fluorouracil at 15mg/kg
for up to 4 days post-operatively via a tenckhoff
catheter.
If complete cytoreduction is not possible,
maximal tumour debulking (MTD) is performed.
MTD usually involves a greater omentectomy and
either an ileocolic anastomosis or a total colecto-
my and ileostomy.
Histopathological classification
The classification of pseudomyxoma peritonei
has been confusing. There have been a number of
different terminologies and classification systems
used for epithelial appendiceal neoplasms. In ad-
dition, the clinical presentation of PMP can also
result from high grade colonic mucinous neo-
plasms, adenocarcinoma of the appendix and mu-
cinous adenocarcinomas originating from other
intra-abdominal organs. PMP of appendiceal ori-
gin is a best a borderline malignant condition but
more accurately represents a spectrum of disease
from low to high grade.
Different pathological classifications of PMP
have led to difficulties in the interpretation of
treatment outcomes. Some series include all cases
including those originating from adenocarcinoma
while some report only those arising from low-
grade appendiceal tumours.
In 1995, Ronnet et al. [24] produced the first
internationally recognised classification system
based on patients who had undergone cytoreduc-
tive surgery in Washington by Sugarbaker’s group.
They divided PMP into three categories: Dissem-
inated peritoneal adenomucinosis (DPAM), peri-
toneal mucinous carcinomatosis (PMCA) and an
intermediate group. Bradley et al. [25] proposed
classification into two distinct categories, muci-
nous carcinoma peritonei low grade and muci-
nous carcinoma peritonei high grade. The former
incorporating DPAM and the intermediate group,
the latter PMCA, including cases that are moder-
ately to poorly differentiated and those with sig-
JBUON 2015; 20 (Suppl. 1): S51
S52 Management of pseudomyxoma peritonei
net ring cell morphology.
A classification by the WHO in 2010 [26] di-
vided PMP of appendiceal origin into low and
high grade. A review of over 270 cases by Carr
et al. [27], correlating histology with clinical find-
ings and survival data found that categorisation
as either low grade or high grade correlated well
with prognosis. The Peritoneal Surface Group In-
ternational is working with the leading patholo-
gists on appendiceal tumours to reach a consen-
sus on this classification.
The pathological classification is important
as it provides an indication of prognosis follow-
ing CRS and HIPEC. Patients with low grade PMP
appear to gain maximal benefit.
Outcomes of CRS and HIPEC
Surgery for pseudomyxoma peritonei tradi-
tionally involved repeated debulking for symp-
tomatic relief with limited expectation of long
term survival and no prospect of cure. The lack
of a successful treatment strategy and the rarity
of PMP meant that historical series were small
and selective. In a series from the Mayo Clinic, be-
tween 1957 and 1983, Gough and colleagues [28]
reported a 32% 10-year survival in 56 patients
who underwent serial debulking and selective in-
traperitoneal radiotherapy or chemotherapy.
The modern management strategy of CRS and
HIPEC was developed and popularised by the work
of Paul Sugarbaker and colleagues at the Wash-
ington Cancer Institute [29]. In 1999 Sugarbaker
et al. [21] published as series of 385 patients, 205
of which received HIPEC. Complete cytoreduction
was associated with 5 year survival of 80% com-
pared to 20% in whom macroscopic tumour re-
moval could not be achieved. What has come to be
known as the “Sugarbaker procedure” is now the
accepted standard of care with subsequent series
confirming the efficacy of CRS and HIPEC. Disease
or progression free survival of 75%, 56-70% and
67% at 1 year, 5 years and 10 years respectively
and overall survival of 69-75% at 5 years, 57% at
10 years have been reported [22,30-33].
Completeness of cytoreductive surgery is a
major predictor of outcome independent of histo-
logical grade. The completeness of cytoreduction
is assessed after surgery with no visible tumour
is graded as CC-0 and residual disease and with
no nodule greater than 2.5mm as CC-1. Residual
disease nodules between 2.5mm and 2.5cm cor-
respond to CC-2 and greater than 2.5cm CC-3 [34].
Scores of CC-0 and CC-1 are taken to represent
complete cytoreduction with significantly bet-
JBUON 2015; 20 (Suppl. 1): S52
ter reported outcomes than seen in patients with
CC-2 or CC-3 residual disease.
With an incomplete cytoreduction (CC-2 and
CC-3), or major tumour debulking, the 5 year
survival was 24%. This is compared with 85% in
patients with CC-0 and 80% with CC-1 complete
cytoreduction [23]. The ability to achieve a com-
plete cytoreduction may depend upon the extent
of disease and histological grade. As previously
discussed, involvement of the small bowel and
mesentery remain the major limiting factors.
Previous surgery, particularly attempts at partial
debulking, can reduce the chances of complete cy-
toreduction with compromise of the natural peri-
toneal barrier and entrapment of tumour within
scar tissue and adhesions [35].
Although complete cytoreduction is the opti-
mal treatment, where it is not possible, maximal
tumour debulking, usually involving a greater
omentectomy, colectomy and end ileostomy can
produce good palliative results and even long
term survival in a small number of patients. In a
recent series, Dayal et al. [5] reported 748 consec-
utive patients who underwent surgery for PMP in
Basingstoke, 205 of whom received maximal tu-
mour debulking. Overall survival was 47%, 30%
and 22% at 3, 5 and 10 years compared with 90%,
82% and 64% in those who received complete cy-
toreduction.
Morbidity and mortality
CRS and HIPEC is a complex surgical inter-
vention and carries a significant risk of compli-
cations including anastamotic leakage, intra-ab-
dominal abscesses, small bowel and pancreatic
fistulae, respiratory infections and venous throm-
boembolism. Operating times are long, averaging
around 9 hours and can result in significant blood
loss. Neutropenia and associated sepsis are rec-
ognised complications of intraperitoneal chemo-
therapy.
Reoperation rates for post-operative compli-
cations have been reported to range from 11% [36[
to 21% [37] and 30 day mortality from 0% to 14%
[38].
It is now clear that the learning curve for sur-
gical units performing complex procedures like
CRS and HIPEC can have a major impact on out-
comes [39]. Initial high morbidity and mortality is
seen to decrease with increasing experience and
this is likely to represent improvement in patient
selection and postoperative management as well
as surgical expertise.
 Management of pseudomyxoma peritonei
Follow-up
The rational for active follow-up is the abil-
ity to detect and treat recurrent disease. Elective
reoperation for recurrent disease is beneficial for
selected patients. Esquivel and Sugarbaker [40]
reported 5 year survival of 74% in selected pa-
tients with recurrent PMP of appendiceal origin
who underwent repeat CRS and HIPEC. Mohamed
et al. [41] reported 5 year survival of 70% from the
initial operation in selective patients who had 3 or
more attempts at CRS and HIPEC.
Our practice involves CT scanning and serum
tumour markers at one year post-operatively and
annually thereafter for 10 years. Earlier imaging
can be employed if symptoms develop or a high
suspicion of recurrence exists. This strategy has
developed due to the fact that early recurrence af-
ter complete cytoreduction is likely to represent
aggressive, rapidly progressive disease, unlikely
to be amenable to salvage surgery. In early recur-
rent disease and particularly in high grade PMP,
systemic chemotherapy is advocated by some al-
though strong evidence is lacking.
Patients likely to benefit from further CRS
and HIPEC are those with slowly progressive dis-
ease. As such, a policy of watch and wait can be
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