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222 MALIGNANT PERITONEAL MESOTHELIOMA MALIGNANT I i 1 I * i MALIGNANT PERITONEAL MESOTHELIOMA Case report and review of the literature G. Watteeuw, A. D'Hondt, F. Tannouri, P. Recloux, C. Hubert* u t, i1t. !' i SUMMARY . Wc describe a peritoneal mesothelioma. There arc many aspeeific symptoms. Professtonarcxposure is found in only fifty percent of cases. The histological diagnosis is often difficult. Tlie survival period is short because of the absence of curative treatment. Acio Clinica Belgica, 1995: 50-4; 222-6 INTRODUCTION ' We report (lie case of a 59-year-old man who was admitted to the hospital with recurrent ves-' pcral fever, anorexia, weight loss and abdominal distension. The complementary examination showed a malignant peritoneal mesothelioma. Hisiological diagnosis is difficult and needs complementary stains and further hisiochemica! studies. Other exposure than professional expo sure must be researched, The prognosis is poor despite of aggressive combination of therapies. CASK REPORT A 59-year-old man was admited to hospital wit!) recurrent vespera] fever, profuse sweating, anorexia, weight loss, abdominal distension and signs of bowel obslruciion. Tire symptoms had begun four days previously. Occupational expo sure to asbestos was found twenty years ago, during seven years (building construction), and also during hobby work at home (during more C.HXUs Ambrose PartS, Service do Mdccinc lmcnve., fioufevard Kennedy 2.7000 Mon$ Reprims: Dr, G. Wuecuw. Service dcs Urgcnccs AduUcs, Hfcpitat Univcrsilaire Si Pierre, wc Haute 322, fOOO Bnjxeffes Acta Clinica Belgica 50-4,1995 than twenty years). The patient was not taking any medication. On physical examination, (he patient appea red emaciated. He was afebrile. Pulmonary aus cultation was negative. We found one left cervi cal lymphadenopathy. The abdomen was distended and there was mild diffuse tenderness. Bowel sounds were absent. Biological results in the emergency room showed: inflammatory blood chemical findings, thrombocytosis, negative bacteriology, negative tumoral markers. Plain abdominal radiographs suggested small bowel obstruction. Further tests were undertaken. The urogenital check-up was negative. Pulmonary examinations were as fol lows: bronchoalvcolar'lavage revealed tire pre sence of asbestos fibers. Multiple sputum cul tures were negative. Chest X-rays were normal. Tile complementary digestive examination was comributive. The abdominal computed tomo graphy suggested a thickening of both epiploon and mescntcriurn, with a pseudolumoral appea rance. The colonoscopy suggested diverticulitis. At laparoscopy, we found diffuse peritonitis in association with spreading of nodular lesions; biopsies were undertaken (Fig.l), Histological examination included two slages. First, hematoxylin stain (original magni fication x4Q) showed epithelial structure with nuclear abnormalities, suggesting the diagnosis of adenocarcinoma or carcinomatous lymphan gitis (Fig,2). Subsequently, complementary stains and further irrtmunohistochemical studies (Periodic Acid-Schiff coloration. Blue Alcian /I i; t 4T: I !l ! 4> Ij Fig.l- Lapan ji nodular tesio the 4;!* uhii coloration, " nic-Antigen ij and 18 anb Ufr gnant tumo> r In spite ( !' phamid-adi i montlis late ij ii.i DiSCU: i; o Malign; R witli an im i* iv', million pc sotheiiom: 4 * sothcliom K pluripoter I: the pleurr If vaginalis Asbesi primary 11 mesothcl V the durat ( ( vious asi I 83%ofc I asbestos clear (4) gcsting< exposur * exposur 1 OTHEUOMA j MALIGNANT PERITONEAL MESOTHELIOMA t %* A 223 t was not taking ie patient appeaPulmonary ausld one left cerviabdomen was ffuse tenderness. mergency room emical findings, iotogy, negative r ' -adiograpbs rtlier tests .xrk-up was ons were as fol;vcaicd the pre pie sputum culys were normal, xarnination was omputed tomo>f both epiploon >tu moral appea:d diverticulitis, se peritonitis in toduiar lesions; ). included two original magnii structure with ig the diagnosis alous lymphanomplementary hcmical studies n, Blue Alcian [I Tig-L Laparoscopy: visualisation of nodular lesions on the serous side of !' the large bowel. < Tig- !. Uematoxylin-eosin stain (magnification X 40): characteristic epithelial structure. jj coloration. Monoclonal anti-Carcino-Embryo- pathology of malignant mesothelioma appears J: nic-Aniigen (CEA) Antibody, anti-Keralin 8 to be similar in men and women, and in cases ;! and 18 antibodies) suggested epithelial mali associated and unassociated with asbestos (8). gnant tumour compatible with mesothelioma, Other associated exposure or disease include j' In spite of rapid chemotherapies (cyclophosj! phamid-adriamycin twice), death occurred four months later. talc, mica, thorotrast. X-ray, recurrent peritoni tis due to familial Mediterranean fever, and dif fuse lymphocytic lymphoma (2). j: jj DISCUSSION *' Malignant mesothelioma is a rare disease w ith an incidence in the United States of 2.2 per | million per year (1,4,5). Primary'peritoneal me ; sothelioma accounts for 10% to 20% of all nvc** sothcliomas (1-5). Mesotheliomas arise front f pluripotcntial progenitor cells and may occur in 1' the pleura, pericardium, peritoneum and tunica The mean age varies from 49 to 59 years. The median interval between first exposure to asbes tos and diagnosis varies from 27 to 52 years.The mean duration of exposure also varies from 11 to 28 years (1-6). Symptoms arc aspccific: abdominal pain, dysphagia, weight loss, malaise, anorexia, fever, weakness, signs of bowel (sub)obstruction, and abdominal enlargement. More specifically, as vaginalis testis. |: Asbestos exposure seems to constitute the i; primary cause of both pleural and peritoneal |! mesothelioma (PM) in humans. Risk relates to V theduration and intensity of exposure (12). Pre- cites is found in 33% to 90% of cases (3,4). An abdominal mass may be palpated in 13% to 29% of cases (1,4). Laboratory findingsarcnothclpful in making the diagnosis. Some reports describe polyclonal i vious asbestos exposure is found in 0% (1) to j 83% of cases. In some reports, the link between hypcrimmunoglobulinemia (5). thrombocytosis (by persistent secretion of interIeukine-6) (2), asbestos and peritoneal mesothelioma is not |! clcar(4) especially forpediatric population,sug- ectopic hormonal production (insuline-Iikesub stance, growth hormone, antidiuretic hor- I; gesting other eliologic factors than occupational mone)(l.5). Radiographically, CT-scan images jl exposure, such as environmental and familial arc not specific. PM may have multiple manifes *|t exposure (5). In 1993, Dawson slated that the tations on CT-scan; abdominal mass, scattered Acta Citnica Belgica 50^4, 1995 224 MALIGNANT PERITONEA L MESOTHELIOMA MAUGNA3 <* intraabdominal nodules, thickening of mesenterium. The CT-scart's value lies in its ability to direct sites of biopsy and to measure response to therapy (9). The diagnosis of mesothelioma is usually made at the time of laparoscopy or lapa rotomy, Cytological examination of ascitic fluid fails to give diagnosis; nevertheless, van Gelder reports positive cytologies of ascitic fluid in 26% of cases (3). The histological examination permits the differentiation of three lesions, by order of frequency: epithelial (37 to 75%), mixed (19 to 42%), fibrous (2,5 to 21%) (1,4,6,9,12). Vogelzang describes in his study a classically biphasic histology of mesothelioma, with both epithelial and sarcomatous areas pre sent (7). Other more benign forms of PM exist, such as well differentiated papillary mesothelio ma of epithelial subtype and cystic mesothelio ma (CM). CM of (he peritoneum is a well recognized but rare tumor, the mcsothclial origin of which has been suggested by recent ultrastruclural stu dies (16). CM seems to occupy a borderline position between benign adenomatoid tumor and malignant lubulopapillary mesothelioma (16). It occurs mostly in the female population (89%). and mean age is 38 years. The most frequent complaint is an abdominal mass (16,17). CT-scan more accurately demonstrates its location and extent. Diagnosis is made by laparotomy; this tumor has a clear predilection for the peritoneum and the omentum of the pel vic viscera. In all cases, surgical resection is uncomplicated. No fatal cases have been repor ted. CM is not chcrno- or radiosensitive. Recur rence occurs in 26% of cases (16,17). Differen tial diagnosis must be done with lymphangioma, ovarian cystadenoma or cystadenocarcinoma, teratoma, cystic smooth-inuscle tumor and endomclriosis. Malignant mesothelioma is difficult to dia gnose. Classic Hcmatoxylin-eosin stain fails to give diagnosis. Alciari blue stainingfor hyaluro nic acid secreted by mcsothclial cells is a good alternative. Exclusion of intracellular mucin Acta Clinica Belgica 50-4, 1995 with a Periodic Acid-Schiff stain usually rules out carcinoma (2). Immunohislochemical stu- ** dies currently have a role in the routine diagnosis of malignant mesothelioma (MM). Numerous monoclonal and polyclonal antibodies reactive - with mucosubstances, lectins, intermediate fila ments (keratins), cytoplasmic or membranous oligosaccharide, are now available and may be applied to routinely fixed and paraffm-embedded tissues (11). Shcibani et al. describe in his' report a series of antibodies, permitting the dif ferential diagnosis between MM and adenocar cinoma (10): Leu-Ml Antigen, Monoclonal Antibody B72-3, Human Milk Fat Globules , (HMFG-2), Carcinoembryonic Antigen, Anti Keratins, Vimentin, Monoclonal Antibody BerEP4, Monoclonal Antibody 44-3A6, Lectins, Antimcsotlielioma Monoclonal Antibodies. On the other side, electron microscopy makes the < diagnosis easier by revealing the characteristic ultrastruclural elements: basement membrane, desmosomes, microvilli (4). hi a large autopsies' series, it is determined that local invasion (gastrointestinal tract, abdo minal wall, liver, pancreas, diaphragm and retroperitoneum) and metastases (lymph nodes, liver, lung, pleura, pericardium, heart, adrenal glands) are common (1-3,5,6). In two studies (12,13) wc found a relationship between histological classificaiion and lung asbestos fiber content; PM seems to be associated with higer doses (12). In the second study, the authors conclude that a dose-response relation appears to exist, but thaL threshold must be determined by the life span of the person exposed (13), The mean survival fluctuates around 12 months in most of series, because of the absence of curative therapy. The review of Fraire ct al. who analyse mesothelio ma in childhood suggest a negative prognosis with a survival rate of 30% after one year (14). '1 ,, '* '* For the trealmem, some studies marginally describe an improvement of the survival with aggressive combination of therapies: * - the asst py-intracav of radic per - combin cin-CispIati -tiieassc driamycin() In conch of its rarity profess iona short perio: form with b rapies. Exp< there may ! familial (hr exposures, must be kc X-ray, recu dilerranean cause of the Electron n studies am content are I prognosis it nation of (hi approaches RESUM Nous dCci symptflmes c sition profes: des cas. Lc d cite. La sarv (hdrapcutiqui SAMEN We besetn symptomcn ? nclc expositir gevonden. L inoeilijk.Dc; er geen curai: - MESOTHELIOMA MALIGNANT PERITONEAL MESOTHELIOMA 225 f slain usually rules ohistochemical stuhe routine diagnosis i (MM). Numerous antibodies reactive s, intermediate filalic or membranous ailable and may be nd paraffin-embed- t al. describe in his , permitting the difMM and adenocartigen. Monoclonal Vlilk Fat Globules nic Antigen, Antianal Antibody Bcr' 44-3 A6, Lectins, nal Antibodies. On roscopy makes the ' die characteristic lennent membrane, * ^ ' - "s detenu in ed i tract, abdoipnragm and rctroymph nodes, liver, irl, adrenal glands) studies(12,13) we histological dasfiber content; PM igerdoses (12). in s conclude that a s to exist, but that by tire lifespan of te mean survival in most of series, itive therapy. The alyse mcsotheliosgative prognosis ter one year (14). tudies marginally the survival with rapics: < iT ' <t - the association surgery-external radiothera py-intracavitary radioactive 32P or I98Au (risk ofradio peritonitisXI); - combinated chemotherapies like Doxorubi- cin-Cisplatin or Mitomycin-Cisplatin; - tlie association surgery-intracavitary 32P-Adriamycin(18). In conclusion, this case is interesting because of its rarity, the acute presentation, the short professional period of exposure, but also the short period of survival despite of epithelial form with better prognosis and rapid chemothe rapies. Exposure to asbestos must be researched; there may be professional, environmental and familial (hobby activity, family and workers) exposures. Nevertheless, other causes of MM must be kept in mind (mica, talc, thorotrast, X-ray, recurrent peritonitis due to familial Me diterranean fever,...). Diagnosis is difficult be cause of the absence of specificity of symptoms. Electron microscopy, iminunohistochcmical studies and analysis of lung asbestos fiber content arehelpful in the chain ofdiagnosis. The prognosis is poor despite of aggressive combi nation of therapies and therefore innovative new approaches arc needed. RESUME Nous dCcrivons un indsoihdliomc pdriiondal. Les symptomes en sont divers et asp&ifiques. Uneexpo sition profcssionnctlc n'esi rcirouvde que dans 50% dcs cas. Le diagnostic histoiogique cst sou vent diffi cile. La survic moyenne cst courtc. cn I'abscnce de thdrapeuiique curative. SAMENVATTING Wcbcschrijvcnecn pcriioncaal mesothelioma. De symploincn zijn vcrschillcndcn aspccifiek.Professionele expositic is slcchts in 50% van dcgevallcn tcruggevonden. De histologisclic diagnose is dikwijls moeilijk. Degemiddcldcovcrleving is kon aangczicn cr geen curaticvc bchandding is. REFERENCES 1. Brenner J.SordilloPP.MagillGB et al. Malignant peritoneal mesothelioma. Am / Gastroenterol. 1981;75:311-3. 2. Chahinian AP, PajakTF, Holland JFct al. Diffuse malignanl mesothelioma. Ann Intern Med. 1982; 96: 746-55. 3. van Gclder T, Hoogsteden HC, Vcrsnel MA et al. Malignant peritoneal mesothelioma: a scries of 19 cases. Digestion. 1989; 43: 222-7. 4. Plaus WJ. Peritonea] mesothelioma.Arch Surg. 1988; 123:763-6. 5. Ascnsio JA, Goldblatt P, Thomford NR. Primary malignant peritoneal mesothelioma. A rch Surg. 1990; 125: 1477-81. 6. Kanncrstein M. Churg I. Peritoneal mesothelioma. Hum Path. 1977; 8: 83-94. 7; Vogclzang NJ. Malignant mesothelioma: diagnosis and management strategics for 1992..Semin Oncol. 1992; 19 (Suppl): 64-71. 8. Dawson A, Gibbs AR, Poolcy FD ct al. Malignant mesothelioma in women. Thorax. 1993; 48: 269 74. 9. Smith TR. Malignant peritoneal mesothelioma: marked variability of CT findings. Abdom Imag. 1994; 19: 27-9. 10. Shcibani K, Esteban JM, Bailey A ct al. Immunopalhologic and molecular studies as an aid to the diagnosis of malignant mesothelioma. Hum Pa thol. 1992: 23: 107-16. 11. Donna A, Bctta DG. Bcllingcri D cl al. New mar ker for mesothelioma: an immunoperoxidasc stu dy. J Clin Patlwl. 1986: 39: 961-8. 12. Ixrigh J, Rogers Al, Ferguson DA ct al. Lung as bestos fiber content and mesothelioma cell type, site and survival. Cancer. 1991; 68: 135-41. 13. Craighead JE. Mossman BT. The pathogenesis of asbestos-associated diseases. N Engl J Med, 1982; 306: 1446-54. 14. Frairc AE, CoopcrS, Greenberg SD ct al. Meso thelioma ofchildhood. Cancer. 1988; 62: 838-47, 15. Ackerman LV. Mesothclialand related neoplasms in children and adolescents: a clinicopathologic and immunohistochcmical analysis ofeight cases. Pediatr Pathol. 1992; 12: 333-47. 16. Katsubc V, Mukai K.SilveibcrgSG. Cystic meso thelioma of the peritoneum. Cancer. 1982; 50; 1615-22. Acta Clinica Belgica 50-4. 1995