Acta Chirurgica Belgica

ISSN: 0001-5458 (Print) (Online) Journal homepage: http://www.tandfonline.com/loi/tacb20

Laparoscopy and Primary Diffuse Malignant

Peritoneal Mesothelioma: a Diagnostic Challenge

P. Van de Walle, Y. Blomme & L. Van Outryve

To cite this article: P. Van de Walle, Y. Blomme & L. Van Outryve (2004) Laparoscopy and
Primary Diffuse Malignant Peritoneal Mesothelioma: a Diagnostic Challenge, Acta Chirurgica
Belgica, 104:1, 114-117, DOI: 10.1080/00015458.2003.11978408

To link to this article: https://doi.org/10.1080/00015458.2003.11978408

Published online: 01 Feb 2018.

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Acta chir belg, 2004, 104, l 14-11 7

Laparoscopy and Primary Diffuse Malignant Peritoneal Mesothelioma

a Diagnostic Challenge
P Van de Walle, Y Blomme, L. Van Outryve
Department of General, Vascular and Thorac ic Surgery, AZ. Volkskl iniek Gent, Belgium .

Key words. Laparoscopy ; malignant peritoneal mesothelioma ; hand-ass isted laparoscopi c surgery.

Abstract. Primary diffuse mal ignant peritoneal mesothelioma is a rare malignancy with an estim ated incidence of 200
to 400 new cases annually in the USA. We describe a case of diffuse mali gnant peritoneal mesotheli oma ari sin g in a 65-
year old man who presented ascites of unknow n ori gin. The importance of laparoscopy with subsequent hi stol ogy of
biopsy specimens in the diagnosi s of thi s di sease is emphasized. Because of his poor ge nera l conditi on, the pati ent had
no further treatment. Update of treatment is briefly described with parti cular attention to mu ltimodality approach.

Introduction
Malignant peritoneal mesothelioma is a rare neopl asm
of the peritoneal cavity representing only I0- 30% of all
malignant mesothelioma cases (I , 6). Very few cases
have coexisted with pleural mesothel ioma, while pl eur-
al mesothelioma often spreads to the peritoneum . The
actual incidence of di sease is poorly documented becau-
se most reports include pleural and peritonea l mesothe-
lioma together as a single disease. An estimation can
only be made of 200-400 new cases annuall y in the
USA (4). In 75 % of the cases the pati ents are betwee n
50 and 60 years with a predominance in the male popu -
lation (sex ratio of 1/10) (5). Although not uni fo rml y
accepted , asbestos is believed to be the main aetiolog i-
cal fac tor. T. van GELDER et al found that in l 9 cases of
malignant peritoneal mesothelioma, 74% had a profes-
sional exposure to asbestos (2). Severa] case reports
have linked other agents and this kind of mali gnancy
including radiation, thorium diox ide (Thorotrast). bery l-
lium, mica exposure, recurrent peritoniti s and Simian
virus 40 (3 ). There was no hi story of as bestos ex posure
in our case. The disease is invariabl y fa tal with a medi an
surv ival of less than I year from di agnosis.
cake) . Enl arged lymph nodes were located in the ri ght
lower quadrant and in the pelvis. Cytolog ica l exa min a-
ti on after percut aneo us ultraso nog raphi ca l-g uid ed
needl e aspi rati on revea led adenoca rcinoma.
Gastroscopy showed a duodenal ul ce r, and a barium
enema ev idenced di ve rti cular di sease of the co lon. We
dec ided to perfo rm an ex pl oratory laparoscopy because
a primary gastro intestin al tumour could not be fo und by
means of conventi onal di ag nosti c tec hniques.
After in serti on of the camera system throu gh a
IO mm trocar pl aced at the umbili ca l leve l. bl oodstained
asc ites was fo und in the ri ght subcli aphragmati c space
and right paraco li c gutter (Fi g. I ). A sa mple was se nt for
cytolog ical exa min ati on. Peritonea l impl ant s we re di sse-
min ated thro ughout the whole abdominal cav ity. By in -
spection and ex pl orati on no tum our co uld be visualized.

Case report

A 65 -year old male patient was transferred to our depart-

ment after examin ati on fo r diffu se abdominal pain.
weight loss and anorexia. Biochemical analysis showed
raised inflammatory parameters. On computed tomogra-
phy scan of the abdomen, asc ites was visuali zed in the
perihepatic and peri splenic space, paracolic gutters and Fig. I
in the pelvis. Peritoneal carcinomatos is was uspected. Bloodstained asc ites and peritoneal implants on the right he mi-
essentially at leve l of the greater omentum (omental di aphragm.
Laparoscopy and Prima,y Diffuse Malignant Peritoneal Mesothelioma
An 10c1 sion was made on the ri ght side and the
HandponTM System was in stalled. The left hand was
brought in side and the gastrointestinal tract was palpa-
ted. No primary cancer was found. Ti ssue sa mpling was
don e at the greater omentum and sent for histopatholo-
gica l examination . The diagnos is was made of a diffu se
mali gnant mesothe lioma. A CT scan of the thorax
showed an important pl eural effusion on both sides.
Because of the massive invo lvement of the peritonea l
and serosa l surfaces no surgica l debulking was perfor-
med. No chemothera peutic treatment was performed
because of the poor genera l co ndition. The pati ent died
2 months after initi al di agnos is.
Discussion
There are no specific symptoms of mali gnant peritonea l
mcso theli oma and c lini ca l di agnos is is diffi cult.
Mali gnant peri tonea l mesothelioma usuall y presents as
an advanced intraabdo minal tumour with sy mptoms
such as abdomin al di sco mfon , pain , di stenti on and
weight loss. Less frequ entl y pat ients presen t with dysp-
hag ia. feve r of unknown origin , abdomin al mass or
bowel obstructi on. The onset of sy mptoms is usually
gradual. but may be acute. with the picture of an ac ute
abdomen (7).
Asc it es that result in abdominal distension appear in
70-90% of the patients ( I. 3, 7). Asci ti c fluid ana lys is
usuall y revea ls an cx udative process. bu t cytology is se l-
do m contri butory. ha ving a sensiti vity of 25 %. The fluid
varies from thi n. straw co lored to thi ck, mucin ous or
blood tinged in charac ter. In our case, cytology revea led
adcnocarcinoma.
Biochemi stry is not helpful in making the diagnos is
of peritoneal mesotheli oma. No tumour marker ca n
re li ab ly be used fo r diagnostic purpose. Thrombo-
cy tos is, clottin g abnormali ti es and polyc lonal hyperim -
munog lobulin ae mi a have been assoc iated (7).
Rad iolog ica l exa minati on is important in eva lu ati on
of the patients. Contrast studi es of the gastrointestinal
trac t can re\'cal co mpress ion and di slocati on of bowel
loops by ex trinsic masses. segmental stenos is, signs of
intestinal obstructi on.· J.C. STAMAT et al. found CT cle-
arl y superi or to ultraso nography in diagnos is of perito-
nea l mcsothelioma (8). Class ic findin gs at CT are intra-
peritoneal masse s. vari able invo lvement of omentum,
mescnteric thi cke ning. peritoneal studding, signs of hae-
morrhage with in the tumour masses, and ascites. CT is
also very helpfu l in the detection of pl eural effusion and
pleural plaques th at ca n be revealed in peritoneal prima-
ri es in 50-60S7<• of patients. Ncvenheless differentati on
from carc inomatosis. gastroi ntestinal malignancies. ova-
rian carc in omas and lymphomas is ofte n imposs ibl e.
Because of the nonspec ifi c clini cal features and the
unreli ab ilit y of conve nti onal tec hniques in the diagnos is
I 15
of peritoneal mesothel ioma, laparoscopy should be pre-
fe red, The major role of laparoscopy is to provide biop-
sy material directly from peritoneal tumour nodules suf-
fici ent for histo logy. E. P1cc1GALLOet al. reported in their
paper a correct diagnosis using laparoscopy and histolo-
gica l exam ination of biopsy spec imens (9). C. M. C1-1 u et
al. in their study of 129 patients with ascites of unknown
origin fo und that laparoscopy with biopsy establi shed
the diagnosis in 86% of patients ( I 0).
Upon entry into the abdomen, peritoneal mesothelio-
ma typically appears as multiple plaques scattered over
the peritonea l surfaces, thi ck intraperitoneal adhesions,
nodu larity and infiltration of the omentum (8) . With di s-
ease progression , mass ive accu mul ations of the tumour
are often seen in the omentum , in the lower abdomen
and pelvis, and beneath the ri ght hemidiaphragm. In our
case, the initi al di agnosis of peritoneal carcinomatosis
was made on the basis of CT and cyto logical examin a-
tion of the ascites. Unsuccessfully, the patient was furt-
her investi gated for a primary gastrointestinal tumour.
With laparoscopy, bloodstai ned asci tes was see n
together with mass ive tumour involvement of both
pari etal and vi sceral peritoneum , omentum and mesen-
tery. Exp loration for a primary tumour fai led and we
dec ided to sw ith to a hand-assisted procedure. The
who le GI-tract was palpated but no tumour was found.
Ti ssue sa mpling was performed. Frozen section exami-
nation revealed the di agnos is of a poorly differentiated
epitheli al tumour.
Three hi stologica l subtypes of diffu se malignant
mesothel ioma have been described : epithelial , sarcoma-
toYd (fibrou s) and mixed (b iphas ic) (Table I) ( I I). The
majority are epitheli al types. Mesotheliomas invade
parenchyma superfi ciall y, rather than deeply. At advan-
ced stages of th e di sease, tumour infiltrates the capsule
of the liver with furth er ex tension into the parenchyma
and retroperitoneal ti ssues. Most commonly, mesothe-
lioma invades the wall of the gastrointest inal tract. The
undersurface of the diaphragm is nearly always involved
bu t full -t hi ckness invasion is re lati vely unco mmon.
Table I
Classifica1 ion of peritoneal mesotheliomas
Be11ig11
Adenomatoid me so1helioma
Locali zed fibrou s mesotheli oma
Borderline
Multicysti c meso1helio111a
Well -d ifferenti ated papi ll ary 111eso1helio111a of peritoneum
Malig11a111
Epithelial me sothel ioma: 50-75%
Fibrosarco111a1ous mesothelioma : 5-20%
Mixed type 111cso1hclio111a (biphasic): 15---+0'lc
116 P Van de Wal le et al.

Table II
Treatment modalities for primary diffuse mal ignant peri toneal mesotheli oma

Reference Number of patients Treatment Median survival

QUILICHL'II et al. ( l 2) I 6 doxorubicin/ci splatin

recurrence after 4 months : cisplatin/5FU
TANI et al. (l 3) 2 cisplatin/doxorubicin + cytotoxic T-l ymphocytes
ELTABBAKK et al. ( l 7) 3 debulking + systemic paclitaxel/c isplat in 12.5 months

MoNGERO et al. (1 4) 3 Stage I : debulking + intraperitoneal

doxorubicin and cisplatin
Stage II : debulking + CHPPC* : conti nuous
hypertherrnic peritoneal perfusion
chemotherapy
(cisplatin/mitom yc in )

DE BREE et al. ( l 5) l debulking + CHPPC (cispl atin ) 28 months

postoperative :carboplatin/ pac litaxel/VP-16/
ifosfamide/ mes na

PARK er al. ( l 6) 18 debulking + CHPPC (c isplatin) 26 months

SEBBAG er al. (4) 33 debu lking + CHPCC (c isplati n.doxorubicin ) 31 month s

* CHPPC : continuous hypertherrnic peritoneal perfusion chemotherapy.

Consequently, peritoneal mesotheliomas rarely spread to
the pleural cavity. Metastases can occur in lymph nodes,
viscera, liver, lung and adrenals (7, l l ).
The diffe rential diagnosis with reactive mesothelial
hyperplasia, metastatic adenocarcinoma or ovarian car-
cinoma with peritoneal involvement can present a dia-
gnostic challenge. Other possibilities are tuberculous
peritonitis and a primary tumour of the mese ntery (5).
Definitive diagnos is of mal ignant mesothelioma can
on ly be established by histo logy and sometimes immu-
nohi stochemistry is required fo r differential diagnosis.
After histology with immunohi stochemistry, the diagno-
sis of an epithelial type of diffuse malignant mesothelio-
ma was made in our patient. The tumour was negative
for CEA, positive for CAM 5.2 and intermediary for
epithel ial surface ant igen.
The median surviva l of untreated patients in most
series is between 4 and 12 months (7, 15, 16). Because
of the rarity of this disease, large studies exami ning or
comparing therapeutic moda liti es do not exist in the lite-
rature. Currently there is no universall y accepted treat-
ment. Complete surgical resection is rarely feasib le and
has not shown any survival benefit wi thout adjuvant the-
rapy. The role of radiation therapy is unclear and lim ited
because of the diffuse ex tension of this type of tumour.
The results of primary intravenous chemotherapy have
been disappointing with response rates ranging from Oto
40%. Continuous hypertherm ic peritoneal perfusion
chemotherapy (CHPPC) is a treatment modality, combi-
ning cytoreductive surgery wi th intraperitoneal chemo-
therapy and hypertherrnia. Di ffe ren t treatment modali -
ties are summarized in Table II.
Although promising, further clinical studi es have to
be performed to determine whether a mu lti moda lity tre-
atment of cytored ucti ve surgery. CHPPC, and possib ly
postoperative sys temi c combin ation chemotherapy ca n
improve quality of li ve and surviva l.
Conclusion
Primary diffu se malignant peritoneal mesotheli oma is a
di sease rare ly obse rved and difficult to diagnose.
Exploratory laparoscopy offers the quickest. safest and
least invas ive way to confirm the diagnosis of peritoneal
malignant mesothelioma. A spec ifi c therapeutic prot ocol
does not ex ist, but cytoreductive surgery in assoc iati on
with CHPPC is to be cons idered the best alternati ve.
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P. Van de Walle
AZ. Volkskliniek Gent
Ti chelrei I
B -9000 Gen l. Belgiu m