Acta Chirurgica Belgica

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

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 I 15

An 10c1 sion was made on the ri ght side and the of peritoneal mesothel ioma, laparoscopy should be pre-
HandponTM System was in stalled. The left hand was fe red, The major role of laparoscopy is to provide biop-
brought in side and the gastrointestinal tract was palpa- sy material directly from peritoneal tumour nodules suf-
ted. No primary cancer was found. Ti ssue sa mpling was fici ent for histo logy. E. P1cc1GALLOet al. reported in their
don e at the greater omentum and sent for histopatholo- paper a correct diagnosis using laparoscopy and histolo-
gica l examination . The diagnos is was made of a diffu se gica l exam ination of biopsy spec imens (9). C. M. C1-1 u et
mali gnant mesothe lioma. A CT scan of the thorax al. in their study of 129 patients with ascites of unknown
showed an important pl eural effusion on both sides. origin fo und that laparoscopy with biopsy establi shed
Because of the massive invo lvement of the peritonea l the diagnosis in 86% of patients ( I 0).
and serosa l surfaces no surgica l debulking was perfor- Upon entry into the abdomen, peritoneal mesothelio-
med. No chemothera peutic treatment was performed ma typically appears as multiple plaques scattered over
because of the poor genera l co ndition. The pati ent died the peritonea l surfaces, thi ck intraperitoneal adhesions,
2 months after initi al di agnos is. nodu larity and infiltration of the omentum (8) . With di s-
ease progression , mass ive accu mul ations of the tumour
Discussion are often seen in the omentum , in the lower abdomen
and pelvis, and beneath the ri ght hemidiaphragm. In our
There are no specific symptoms of mali gnant peritonea l case, the initi al di agnosis of peritoneal carcinomatosis
mcso theli oma and c lini ca l di agnos is is diffi cult. was made on the basis of CT and cyto logical examin a-
Mali gnant peri tonea l mesothelioma usuall y presents as tion of the ascites. Unsuccessfully, the patient was furt-
an advanced intraabdo minal tumour with sy mptoms her investi gated for a primary gastrointestinal tumour.
such as abdomin al di sco mfon , pain , di stenti on and With laparoscopy, bloodstai ned asci tes was see n
weight loss. Less frequ entl y pat ients presen t with dysp- together with mass ive tumour involvement of both
hag ia. feve r of unknown origin , abdomin al mass or pari etal and vi sceral peritoneum , omentum and mesen-
bowel obstructi on. The onset of sy mptoms is usually tery. Exp loration for a primary tumour fai led and we
gradual. but may be acute. with the picture of an ac ute dec ided to sw ith to a hand-assisted procedure. The
abdomen (7). who le GI-tract was palpated but no tumour was found.
Asc it es that result in abdominal distension appear in Ti ssue sa mpling was performed. Frozen section exami-
70-90% of the patients ( I. 3, 7). Asci ti c fluid ana lys is nation revealed the di agnos is of a poorly differentiated
usuall y revea ls an cx udative process. bu t cytology is se l- epitheli al tumour.
do m contri butory. ha ving a sensiti vity of 25 %. The fluid Three hi stologica l subtypes of diffu se malignant
varies from thi n. straw co lored to thi ck, mucin ous or mesothel ioma have been described : epithelial , sarcoma-
blood tinged in charac ter. In our case, cytology revea led toYd (fibrou s) and mixed (b iphas ic) (Table I) ( I I). The
adcnocarcinoma. majority are epitheli al types. Mesotheliomas invade
Biochemi stry is not helpful in making the diagnos is parenchyma superfi ciall y, rather than deeply. At advan-
of peritoneal mesotheli oma. No tumour marker ca n ced stages of th e di sease, tumour infiltrates the capsule
re li ab ly be used fo r diagnostic purpose. Thrombo- of the liver with furth er ex tension into the parenchyma
cy tos is, clottin g abnormali ti es and polyc lonal hyperim - and retroperitoneal ti ssues. Most commonly, mesothe-
munog lobulin ae mi a have been assoc iated (7). lioma invades the wall of the gastrointest inal tract. The
Rad iolog ica l exa minati on is important in eva lu ati on undersurface of the diaphragm is nearly always involved
of the patients. Contrast studi es of the gastrointestinal bu t full -t hi ckness invasion is re lati vely unco mmon.
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- Table I
nea l mcsothelioma (8). Class ic findin gs at CT are intra- Classifica1 ion of peritoneal mesotheliomas
peritoneal masse s. vari able invo lvement of omentum,
mescnteric thi cke ning. peritoneal studding, signs of hae- Be11ig11
Adenomatoid me so1helioma
morrhage with in the tumour masses, and ascites. CT is Locali zed fibrou s mesotheli oma
also very helpfu l in the detection of pl eural effusion and Borderline
pleural plaques th at ca n be revealed in peritoneal prima- Multicysti c meso1helio111a
ri es in 50-60S7<• of patients. Ncvenheless differentati on Well -d ifferenti ated papi ll ary 111eso1helio111a of peritoneum
from carc inomatosis. gastroi ntestinal malignancies. ova- Malig11a111
Epithelial me sothel ioma: 50-75%
rian carc in omas and lymphomas is ofte n imposs ibl e. Fibrosarco111a1ous mesothelioma : 5-20%
Because of the nonspec ifi c clini cal features and the Mixed type 111cso1hclio111a (biphasic): 15---+0'lc
unreli ab ilit y of conve nti onal tec hniques in the diagnos is
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 Although promising, further clinical studi es have to
the pleural cavity. Metastases can occur in lymph nodes, be performed to determine whether a mu lti moda lity tre-
viscera, liver, lung and adrenals (7, l l ). atment of cytored ucti ve surgery. CHPPC, and possib ly
The diffe rential diagnosis with reactive mesothelial postoperative sys temi c combin ation chemotherapy ca n
hyperplasia, metastatic adenocarcinoma or ovarian car- improve quality of li ve and surviva l.
cinoma with peritoneal involvement can present a dia-
gnostic challenge. Other possibilities are tuberculous Conclusion
peritonitis and a primary tumour of the mese ntery (5).
Definitive diagnos is of mal ignant mesothelioma can Primary diffu se malignant peritoneal mesotheli oma is a
on ly be established by histo logy and sometimes immu- di sease rare ly obse rved and difficult to diagnose.
nohi stochemistry is required fo r differential diagnosis. Exploratory laparoscopy offers the quickest. safest and
After histology with immunohi stochemistry, the diagno- least invas ive way to confirm the diagnosis of peritoneal
sis of an epithelial type of diffuse malignant mesothelio- malignant mesothelioma. A spec ifi c therapeutic prot ocol
ma was made in our patient. The tumour was negative does not ex ist, but cytoreductive surgery in assoc iati on
for CEA, positive for CAM 5.2 and intermediary for with CHPPC is to be cons idered the best alternati ve.
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
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