Localized Malignant Mesothelioma of Peritoneum Arising in the

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					대한병리학회지: 제 37 권 제 1 호 2003
The Korean Journal of Pathology. 2003; 37: 74-7

            Localized Malignant Mesothelioma of Peritoneum Arising in the
                                   Liver Capsule
                                                           - A Case Report -

Hae Joung Sul∙Dae Young Kang                   Mesothelioma originates in several sites including the pleura, peritoneum, pericardium, and
                                               tunica vaginalis. The pleura is the most common site of origin, but cases originating in the per-
Department of Pathology, Chungnam              itoneum is relatively rare. Mesothelial lesions of the peritoneum may pose significant diagnos-
University College of Medicine,                tic problems. Yet, the accurate identification of this lesion is important because of its distinctive
Daejeon, Korea                                 behavior and treatment modality. We herein report a case of malignant mesothelioma of the
                                               peritoneum arising in the capsule of the liver. The accuracy of our diagnosis has been confirmed
Received : October18, 2002                     by the immunohistochemical study and electron microscopic examination.
Accepted : January 22, 2002

Corresponding Author
Hae Joung Sul, M.D.
Department of Pathology, Chungnam University
College of Medicine, 640 Daesadong, Jung-gu,
Daejeon 301-131, Korea
Tel: 042-220-7196
Fax: 042-220-7189
E-mail:                   Key Words : Mesothelioma-Peritoneum

   Peritoneal mesothelioma is a rare neoplasm and accounts for                mesothelioma was made, and the mass was removed. Grossly, the
about 10% of all mesotheliomas.1 Malignant mesothelioma of                    tumor was adherent to the liver and consisted of variably sized
the peritoneum must be distinguished from the more common                     nodules (Fig. 1). Histologically, the tumor consisted of two cell
primary peritoneal serous epithelial neoplasms. This report doc-              types: solid and papillary epithelial growth. In the papillary area,
uments a malignant tumor of the peritoneum that showed ultra-                 the papillary fragments consisted of two-cell thick layers of me-
structural and immunohistochemical features consistent with                   sothelial cells surrounding thin fibrovascular cores (Fig. 2A). In
malignant mesothelioma.                                                       the solid area, the tumor cells were arranged in cohesive groups
                                                                              or sheets. The tumor cells had round or ovoid nuclei in variable
                                                                              size with prominent nucleoli (Fig. 2B). Mitotic figures were less
                             CASE REPORT                                      than 1/10 HPF. The tumor cells were diffusely positive for cal-
                                                                              retinin (1:50, Zymed, San Francisco, U.S.A.) (Fig. 3A) and cytok-
   A 55-year-old female patient presented with malaise, abdom-                eratin (1:50, DAKO, Glostrup, Denmark), while negative for
inal pain, fever, and ascites. She also lost approximately 6 kg dur-          carcinoembryonic antigen (CEA) (1:50, DAKO, Glostrup, Den-
ing the previous three months. She had no history of any other ill-           mark) (Fig. 3B) and factor VIII related antigen (1:100, Zymed,
nesses preceding the onset of these symptoms. The patient had                 San Francisco, U.S.A.). Scanning and transmission electron mi-
no history of asbestos exposure. An ultrasonography and a com-                croscopic examinations using paraffin blocks of the tumor tis-
puted tomography confirmed ascites as well as an ill-defined                  sue showed numerous long microvilli of the tumor cell surfaces
exophytic mass of the liver capsule. The cytologic examination                (Fig. 4). The morphological findings were consistent with local-
of the ascites showed mostly red blood cells with only a few de-              ized malignant mesothelioma. After the tumor excision, the pa-
generated mesothelial cells and inflammatory cells. The fine nee-             tient was treated with Taxol by intraperitoneal injection. The
dle aspiration of the mass revealed mesenchymal elements and                  patient has been free of abnormal symptoms of the tumor for
spindle-shaped epithelial-like cells. A presumptive diagnosis of              three months.

Localized Malignant Mesothelioma                                                                                                                 2

                          DISCUSSION                                        occur in individuals past 40 years of age, but the tumor has been
                                                                            described in young adults,2 children,3 and neonates.4 The obser-
   Mesotheliomas are located superficially in the body cavities.            vation that patients with mesotheliomas are prone to develop
The pleural malignant mesothelioma is by far more common                    pleural effusion or ascites leads to the suggestion that cytologic
(about ten- to thirty-fold) than the peritoneal variants.1 Not in-          examinations of effusion specimens would be useful in establish-
frequently, mesothelioms involve both the pleura and the peri-              ing the diagnosis.5 However, not all patients with mesotheliomas
toneum as the result of direct extension through the diaphragm.             have developed pleural effusion or ascites at the time of clinical
They very rarely occur in other locations (e.g., the pericardium            presentation, and there are also cases of mesothelioma with neg-
and the tunica vaginalis). Most cases of peritoneal mesothelioma            ative results in effusion cytology. Occasionally, the disease may
                                                                            first manifestat itself in a hernial sac or in the umbilicus.6 In other
                                                                            instances ing- uinal or cervical lymphadenopathy resulting from
                                                                            a metastatic disease was the first sign of the tumor.7
                                                                               Immunocytochemically, malignant mesothelioma cells are stro-
                                                                            ngly positive for keratin, epithelial membrane antigen, basement
                                                                            membrane-related proteins (type 4 collagen, laminin, and laminin
                                                                            receptors), and calretinin; and they are generally negative for CEA,
                                                                            B72.3, and Leu-M1.8 The tumor cells may also exhibit positiv-
                                                                            ity for vimentin and, occasionally also, for actin and desmin.9,10
                                                                            Some malignant mesotheliomas also express bcl-2 protein. A
                                                                            recent study demonstrated that Leu-M1 was detected in 94% of
                                                                            adenocarcinomas of the lung and in 58% of adenocarcinomas of
                                                                            other organs but in none of 28 mesotheliomas.11 Thus, anti-Leu-
                                                                            M1 may be of value in differentiating between adenocarcinoma
                                                                            and mesothlioma of the peritoneum.
                                                                               The differential diagnosis of a variety of mesothelial lesions of
                                                                            the peritoneum includes mesothelial hyperplasia, multilocular
Fig. 1. The exophytic tumor is adherent to the liver surface (arrow).       peritoneal inclusion cysts (benign cystic mesothelioma), well-dif-

                                                                        A                                                                       B

Fig. 2. (A) Papillary structures with thin fibrovascular cores are surrounded by malignant mesothelial cells. (B) Solid sheets of the tumor
cells have eosino philic cytoplasm and vesicular nuclei with prominent nucleoli.
3                                                                                                             Hae Joung Sul∙Dae Young Kang

                                                                      A                                                                    B

Fig. 3. (A) The tumor cells are diffusely immmunostained for calretinin. (B) Immunohistochemical staining for carcinoembryonic antigen is
completely negative in the tumor cells.

                                                                  A                                                                        B

Fig. 4. Scanning (A) and transmission (B) electron micrographic views of the tumor cells show the intercellular spaces with multiple slen-
der microvilli (arrow) and desmosome (empty arrows).

ferentiated papillary mesothelioma, and diffuse malignant meso-           is important for proper treatment. For undifferentiated tumors
thelioma. These lesions are usually different. The presence of many       involving the pleura or peritoneum, the main diagnostic consid-
three-dimensional groupings of neoplastic cells with prominent            erations are malignant mesothelioma and peritoneal serous epithe-
nucleoli, nuclear pleomorphism, and cell clusters in irregular ar-        lial neoplasm (primary or metastatic). Unlike mesotheliomas, se-
rangements as in this case was not consistent with mesothelial            rosal carcinomas do not show a diffuse and/or perinuclear distri-
hyperplasia. The distinction between malignant mesothelioma               bution of tonofilaments.12,13 The long, thin, branching microvil-
and other malignant neoplasms diffusely involving the peritoneum          li of relatively well differentiated mesotheliomas are often lost in
Localized Malignant Mesothelioma                                                                                                                   4

poorly differentiated mesotheliomas.14 In these poorly differen-           5. Triol JH, Coston AS, Chandler SV. Malignant mesothelioma. Cyto-
tiated examples, however, the diffuse cytoplasmic or perinuclear             pathology of 75 cases seen in a New Jersey community hospital. Acta
distribution of tonofilaments often remains a good marker of me-             Cytol 1984; 28: 37-45.
sothelial differentiation. These tumor cells showed characteristi-                                                                       nod-
                                                                           6. Chen KT. Malignant mesothelioma presenting as Sister Joseph’
cally abundant long slender microvilli on the surfaces of the ep-            ule. Am J Dermatopathol 1991; 13: 300-3.
ithelial type of tumor cells. The scanning electron micrscopie             7. Sussman J, Rosai J. Lymph node metastasis as the initial manifesta-
examination using paraffin blocks is a very effective method to              tion of malignant mesothelioma. Report of six cases. Am J Surg Pathol
confirm the diagnosis of mesothelioma, especially when the imm-              1990; 14: 819-28.
unohistochemical results are difficult to interprete. Immunohisto-         8. Gosh AK, Butler EB. Immunohistological staining reactions of anti-
chemically, serous neoplasms are more likely to be reactive for              carcinoembryonic antigen. CA and antihuman milk fat globule mon-
CEA, Leu-M1, B72.3, CA-125, S-100 protein, placental alkaline                oclonal antibodies on benign and malignant exfolated mesothelial
phosphatase, and amylase than are the mesotheliomas15. Of these,             cells. J Clin Pathol 1987; 40: 1424-7.
CEA, Leu-M1, and B72.3 have the most discriminatory value.15               9. Rosai J, Dehner LP. Nodular mesothelial hyperplasia in hernia sacs.
   The prognosis differs slightly according to the type of mesothe-          A benign reactive condition simulating a neoplastic process. Cancer
lioma. It is grave in the diffuse epithelial and biphasic types and          1975; 354: 165-75.
even less favorable in fibrous type; most patients with these tu-         10. Kung ITM, Thallas V, Spenser EJ, Wilson SM. Expression of muscle
mors die of complications caused by the primary neoplasm with-               actin in diffuse mesothelioma. Hum Pathol 1995; 26: 565-70.
in 12-18 months after diagnosis.2 Metastases occur usually at a           11. Sheibani K, Battifore H. Antigenic phenotype of malignant mesothe-
late stage of the disease. There is still no effective mode of ther-         lioma and pulmonary adenocarcinoma: an immunohistological anal-
apy, but multiagent chemotherapy and radiation therapy, in addi-             ysis demonstrating the value of Leu-M1 antigen. Am J Pathol 1986;
tion to surgery, seem to alleviate symptoms and prolong survival.            123: 212-9.
                                                                          12. Warhol MJ, Hickey WF, Corson JM. Malignant mesothelioma: ultra-
                                                                             structual distinction from adenocarcinoma. Am J Surg Pathol 1982;
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