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Laparoscopy and Primary Diffuse Malignant Peritoneal Mesothelioma a Diagnostic Challenge by shade1314

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									Acta chir belg, 2004, 104, 114-117

Laparoscopy and Primary Diffuse Malignant Peritoneal Mesothelioma :
a Diagnostic Challenge
P. Van de Walle, Y. Blomme, L. Van Outryve
Department of General, Vascular and Thoracic Surgery, AZ. Volkskliniek Gent, Belgium.




Key words. Laparoscopy ; malignant peritoneal mesothelioma ; hand-assisted laparoscopic surgery.

Abstract. Primary diffuse malignant peritoneal mesothelioma is a rare malignancy with an estimated incidence of 200
to 400 new cases annually in the USA. We describe a case of diffuse malignant peritoneal mesothelioma arising in a 65-
year old man who presented ascites of unknown origin. The importance of laparoscopy with subsequent histology of
biopsy specimens in the diagnosis of this disease is emphasized. Because of his poor general condition, the patient had
no further treatment. Update of treatment is briefly described with particular attention to multimodality approach.



Introduction                                                  cake). Enlarged lymph nodes were located in the right
                                                              lower quadrant and in the pelvis. Cytological examina-
Malignant peritoneal mesothelioma is a rare neoplasm          tion after percutaneous ultrasonographical-guided
of the peritoneal cavity representing only 10-30% of all      needle aspiration revealed adenocarcinoma.
malignant mesothelioma cases (1, 6). Very few cases              Gastroscopy showed a duodenal ulcer, and a barium
have coexisted with pleural mesothelioma, while pleur-        enema evidenced diverticular disease of the colon. We
al mesothelioma often spreads to the peritoneum. The          decided to perform an exploratory laparoscopy because
actual incidence of disease is poorly documented becau-       a primary gastrointestinal tumour could not be found by
se most reports include pleural and peritoneal mesothe-       means of conventional diagnostic techniques.
lioma together as a single disease. An estimation can            After insertion of the camera system through a
only be made of 200-400 new cases annually in the             10 mm trocar placed at the umbilical level, bloodstained
USA (4). In 75% of the cases the patients are between         ascites was found in the right subdiaphragmatic space
50 and 60 years with a predominance in the male popu-         and right paracolic gutter (Fig. 1). A sample was sent for
lation (sex ratio of 1/10) (5). Although not uniformly        cytological examination. Peritoneal implants were disse-
accepted, asbestos is believed to be the main aetiologi-      minated throughout the whole abdominal cavity. By in-
cal factor. T. van GELDER et al found that in 19 cases of     spection and exploration no tumour could be visualized.
malignant peritoneal mesothelioma, 74% had a profes-
sional exposure to asbestos (2). Several case reports
have linked other agents and this kind of malignancy
including radiation, thorium dioxide (Thorotrast), beryl-
lium, mica exposure, recurrent peritonitis and Simian
virus 40 (3). There was no history of asbestos exposure
in our case. The disease is invariably fatal with a median
survival of less than 1 year from diagnosis.

Case report

A 65-year old male patient was transferred to our depart-
ment after examination for diffuse abdominal pain,
weight loss and anorexia. Biochemical analysis showed
raised inflammatory parameters. On computed tomogra-
phy scan of the abdomen, ascites was visualized in the
perihepatic and perisplenic space, paracolic gutters and                                  Fig. 1
in the pelvis. Peritoneal carcinomatosis was suspected,       Bloodstained ascites and peritoneal implants on the right hemi-
essentially at level of the greater omentum (omental          diaphragm.
Laparoscopy and Primary Diffuse Malignant Péritoneal Mesothelioma                                                         115

An incision was made on the right side and the               of peritoneal mesothelioma, laparoscopy should be pre-
Handport™ System was installed. The left hand was            fered. The major role of laparoscopy is to provide biop-
brought inside and the gastrointestinal tract was palpa-     sy material directly from peritoneal tumour nodules suf-
ted. No primary cancer was found. Tissue sampling was        ficient for histology. E. PICCIGALLO et al. reported in their
done at the greater omentum and sent for histopatholo-       paper a correct diagnosis using laparoscopy and histolo-
gical examination. The diagnosis was made of a diffuse       gical examination of biopsy specimens (9). C. M. CHU et
malignant mesothelioma. A CT scan of the thorax              al. in their study of 129 patients with ascites of unknown
showed an important pleural effusion on both sides.          origin found that laparoscopy with biopsy established
Because of the massive involvement of the peritoneal         the diagnosis in 86% of patients (10).
and serosal surfaces no surgical debulking was perfor-          Upon entry into the abdomen, peritoneal mesothelio-
med. No chemotherapeutic treatment was performed             ma typically appears as multiple plaques scattered over
because of the poor general condition. The patient died      the peritoneal surfaces, thick intraperitoneal adhesions,
2 months after initial diagnosis.                            nodularity and infiltration of the omentum (8). With dis-
                                                             ease progression, massive accumulations of the tumour
Discussion                                                   are often seen in the omentum, in the lower abdomen
                                                             and pelvis, and beneath the right hemidiaphragm. In our
There are no specific symptoms of malignant peritoneal       case, the initial diagnosis of peritoneal carcinomatosis
mesothelioma and clinical diagnosis is difficult.            was made on the basis of CT and cytological examina-
Malignant peritoneal mesothelioma usually presents as        tion of the ascites. Unsuccessfully, the patient was furt-
an advanced intraabdominal tumour with symptoms              her investigated for a primary gastrointestinal tumour.
such as abdominal discomfort, pain, distention and           With laparoscopy, bloodstained ascites was seen
weight loss. Less frequently patients present with dysp-     together with massive tumour involvement of both
hagia, fever of unknown origin, abdominal mass or            parietal and visceral peritoneum, omentum and mesen-
bowel obstruction. The onset of symptoms is usually          tery. Exploration for a primary tumour failed and we
gradual, but may be acute, with the picture of an acute      decided to swith to a hand-assisted procedure. The
abdomen (7).                                                 whole GI-tract was palpated but no tumour was found.
   Ascites that result in abdominal distension appear in     Tissue sampling was performed. Frozen section exami-
70-90% of the patients (1, 3, 7). Ascitic fluid analysis     nation revealed the diagnosis of a poorly differentiated
usually reveals an exudative process, but cytology is sel-   epithelial tumour.
dom contributory, having a sensitivity of 25%. The fluid        Three histological subtypes of diffuse malignant
varies from thin, straw colored to thick, mucinous or        mesothelioma have been described : epithelial, sarcoma-
blood tinged in character. In our case, cytology revealed    toïd (fibrous) and mixed (biphasic) (Table I) (11). The
adenocarcinoma.                                              majority are epithelial types. Mesotheliomas invade
   Biochemistry is not helpful in making the diagnosis       parenchyma superficially, rather than deeply. At advan-
of peritoneal mesothelioma. No tumour marker can             ced stages of the disease, tumour infiltrates the capsule
reliably be used for diagnostic purpose. Thrombo-            of the liver with further extension into the parenchyma
cytosis, clotting abnormalities and polyclonal hyperim-      and retroperitoneal tissues. Most commonly, mesothe-
munoglobulinaemia have been associated (7).                  lioma invades the wall of the gastrointestinal tract. The
   Radiological examination is important in evaluation       undersurface of the diaphragm is nearly always involved
of the patients. Contrast studies of the gastrointestinal    but full-thickness invasion is relatively uncommon.
tract can reveal compression and dislocation of bowel
loops by extrinsic masses, segmental stenosis, signs of
intestinal obstruction. J. C. STAMAT et al. found CT cle-
arly superior to ultrasonography in diagnosis of perito-                                 Table I
neal mesothelioma (8). Classic findings at CT are intra-              Classification of peritoneal mesotheliomas
peritoneal masses, variable involvement of omentum,
mesenteric thickening, peritoneal studding, signs of hae-    Benign
morrhage within the tumour masses, and ascites. CT is               Adenomatoid mesothelioma
                                                                    Localized fibrous mesothelioma
also very helpful in the detection of pleural effusion and   Borderline
pleural plaques that can be revealed in peritoneal prima-           Multicystic mesothelioma
ries in 50-60% of patients. Nevertheless differentation             Well-differentiated papillary mesothelioma of peritoneum
from carcinomatosis, gastrointestinal malignancies, ova-     Malignant
rian carcinomas and lymphomas is often impossible.                  Epithelial mesothelioma : 50-75%
                                                                    Fibrosarcomatous mesothelioma : 5-20%
   Because of the nonspecific clinical features and the             Mixed type mesothelioma (biphasic) : 15-40%
unreliability of conventional techniques in the diagnosis
116                                                                                                                   P. Van de Walle et al.

                                                                   Table II
                                 Treatment modalities for primary diffuse malignant peritoneal mesothelioma
        Reference                     Number of patients   Treatment                                                  Median survival
        QUILICHINI et al. (12)                1            6 doxorubicin/cisplatin
                                                           recurrence after 4 months : cisplatin/5FU
        TANI et al. (13)                      2            cisplatin/doxorubicin + cytotoxic T-lymphocytes
        ELTABBAKK et al. (17)                 3            debulking + systemic paclitaxel/cisplatin                  12,5 months
        MONGERO et al. (14)                   3            Stage I : debulking + intraperitoneal
                                                                      doxorubicin and cisplatin
                                                           Stage II : debulking + CHPPC* : continuous
                                                                      hyperthermic peritoneal perfusion
                                                                      chemotherapy
                                                                      (cisplatin/mitomycin)
        DE   BREE et al. (15)                 1            debulking + CHPPC (cisplatin)                              28 months
                                                           postoperative :carboplatin/ paclitaxel/VP-16/
                                                           ifosfamide/ mesna
        PARK et al. (16)                     18            debulking + CHPPC (cisplatin)                              26 months
        SEBBAG et al. (4)                    33            debulking + CHPCC (cisplatin,doxorubicin)                  31 months

         * CHPPC : continuous hyperthermic peritoneal perfusion chemotherapy.



Consequently, peritoneal mesotheliomas rarely spread to                       Although promising, further clinical studies have to
the pleural cavity. Metastases can occur in lymph nodes,                   be performed to determine whether a multimodality tre-
viscera, liver, lung and adrenals (7, 11).                                 atment of cytoreductive surgery, CHPPC, and possibly
   The differential diagnosis with reactive mesothelial                    postoperative systemic combination chemotherapy can
hyperplasia, metastatic adenocarcinoma or ovarian car-                     improve quality of live and survival.
cinoma with peritoneal involvement can present a dia-
gnostic challenge. Other possibilities are tuberculous                     Conclusion
peritonitis and a primary tumour of the mesentery (5).
   Definitive diagnosis of malignant mesothelioma can                      Primary diffuse malignant peritoneal mesothelioma is a
only be established by histology and sometimes immu-                       disease rarely observed and difficult to diagnose.
nohistochemistry is required for differential diagnosis.                   Exploratory laparoscopy offers the quickest, safest and
After histology with immunohistochemistry, the diagno-                     least invasive way to confirm the diagnosis of peritoneal
sis of an epithelial type of diffuse malignant mesothelio-                 malignant mesothelioma. A specific therapeutic protocol
ma was made in our patient. The tumour was negative                        does not exist, but cytoreductive surgery in association
for CEA, positive for CAM 5.2 and intermediary for                         with CHPPC is to be considered the best alternative.
epithelial surface antigen.
   The median survival of untreated patients in most
series is between 4 and 12 months (7, 15, 16). Because
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