Primary Malignant Peritoneal Mesothelioma in an ... - Springer Link

Surg Today (2005) 35:421–424 DOI 10.1007/s00595-004-2920-4

Primary Malignant Peritoneal Mesothelioma in an Incarcerated Groin Hernia: Report of a Case Mario Testini1*, Anna Scattone2, Beatrice Di Venere1, Germana Lissidini1, Giuseppe Piccinni1, Silvia Palmisano1, and Gabriella Serio2* 1

Department of Applications in Surgery of Innovative Technologies, Section of General Surgery and Vascular Surgery and Clinical Oncology, and 2 Department of Pathology and Genetics, Section of Pathology, University of Bari, Cesare 11, 70124 Bari, Italy

Abstract Malignant peritoneal mesothelioma arising from the inguinal hernia sac is rare. We report the case of a 71year-old man examined in our emergency department for a bilateral inguinoscrotal hernia, which was recurrent in the right groin, and primary and incarcerated in the left groin. An emergency exploratory operation revealed a firm mass, 10 cm in diameter, in the left inguinal hernia sac. The remaining peritoneal surface appeared macroscopically normal. Therefore, we resected the mass and performed a Rutkow hernioplasty. The patient was discharged after a short, uneventful recovery, and was referred to the oncology department for adjuvant therapy. He is now well and asymptomatic with no evidence of ascites, 26 months after his operation. A mesothelioma of the hernial sac peritoneum was the final histological diagnosis. Key words Peritoneal mesothelioma · Groin hernia · Emergency surgery

Introduction Malignant mesotheliomas are rare, with an annual incidence of between 0.7 and 2.2 cases per million in the population.1 Malignant peritoneal mesothelioma (MPM) accounts for less than 10%–20% of all malignant mesotheliomas1 and very few cases of MPM appearing to arise from the inguinal hernia sac have been reported.2–7 These tumors are usually found incidentally during surgery and, in contrast to pleural and peritoneal diffused malignant mesothelioma, they are not * Authors who contributed equally to the research. Reprint requests to: M. Testini Received: November 27, 2003 / Accepted: July 13, 2004

asbestos-related. However, histologically, they exhibit similar epithelial, sarcomatous, and biphasic patterns to the more common pleural counterpart. Establishing a preoperative diagnosis is difficult, and consequently often delayed because of the absence of specific clinical features. Therefore, it must be always confirmed by histologic examination. Moreover, no previous cases of incarcerated inguinal hernia resulting from MPM have ever been described and it has rarely been reported as the cause of a surgical emergency.8–10 We report a case of unsuspected localized MPM of the hernia sac in an elderly man who underwent emergency surgery for an incarcerated left inguinal hernia.

Case Report Clinical Features A 71-year-old man, a retired greengrocer with no referred history of asbestos work exposure, was brought to our emergency department for examination of a bilateral inguinoscrotal hernia, recurrent in the right groin and primary and incarcerated in the left groin. Physical examination revealed a large reducible recurrent right inguinal hernia and a voluminous unreducible left groin hernia causing inguinal pain. There was moderate peritoneal effusion, but no abdominal masses. His medical history revealed a chronic hepatitis B virus-related and benign prostate hyperplasia. Laboratory investigations showed leukocytosis and an elevated carbohydrate antigen (CA) 125 (90.49 U/ml, normal ⬍35 U/ml) with normal values of the other tumor markers (carcinoembryonic antigen [CEA], tumor polypeptide antigen, α-fetoprotein, β2-microglobulin, CA 15.3, CA 19.9, prostate-specific antigen). The patient stated that 6 months earlier he had undergone ultrasonography, computed tomographic scan, and nuclear magnetic resonance, none of which showed any signs of portal

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M. Testini et al.: Peritoneal Mesothelioma in a Groin Hernia

Fig. 1. Intraoperative photograph showing the mass in the left inguinal hernia sac

Fig. 2. Epithelial pattern of the mesothelioma showing aggregates of polygonal epithelioid cells with abundant eosinophilic cytoplasm set in a myxoid stroma (H&E, ⫻250)

hypertension, but confirmed the presence of intestinal loops in both inguinal hernia sacs. Emergency exploratory surgery revealed a firm mass, 10 cm in diameter, in the left inguinal hernia sac (Fig. 1). The spermatic cord and testis appeared normal but the peritoneal cavity contained a small quantity of clear, straw-colored fluid. The remaining peritoneal surface appeared macroscopically normal. The patient underwent resection of the mass and a Rutkow hernioplasty. His recovery was uneventful and he was discharged after a short time. During follow-up, he received adjuvant chemotherapy, consisting of six courses of CAP (cyclophosphamide, adriamicin, cisplatin) at doses of 900 mg cyclophosphamide, 60 mg adriamycin, and 80 mg cisplatin. At his 6-month follow-up, the CA-125 value remained moderately high (52.13 U/ml, normal ⬍35 U/ml). However, 26 months after the operation the patient is still completely without symptoms or any further evidence of ascites, and with unremarkable laboratory data including tumor markers. Pathological Findings Cytologic examination of the ascitic fluid was negative for neoplastic cells. The specimen consisted of a hernial sac, 120 ⫻ 80 mm, and gross examination revealed an irregular, firm, white mass 60 ⫻ 50 mm in size, with multiple nodules ranging from 3 to 20 mm on its adjacent surface. These nodules were pale gray and firm. Hematoxylin–eosin-stained sections revealed that the tumor extensively consisted of sheets of polygonal epithelioid cells with vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm set in a myxoid stroma (Fig. 2). Mitoses were conspicuous. There was invasive tubulopapillary or a nodular architectural pat-

Fig. 3. Immunohistochemistry for calretinin. There was strong nuclear and cytoplasmic positivity (⫻250)

tern in the adjacent adipose tissue. An admixture of sarcomatous spindle-cell pattern was evident. Finally, the hyperplastic mesothelial cell lining of the hernial sac appeared to be in continuity with the neoplastic cells. Immunohistochemical assay, performed by the avidin-biotin-complex (ABC) technique after pretreatment with a pressure cooker (Hsu), using antibodies for cytokeratins (5/6; Dako, Glostrup, Denmark), epithelial membrane antigen (EMA) (Dako), HBME-1 (Dako), calretinin (DBA, Milan, Italy), CEA (Dako), vimentin (Dako), Ki67/MIB-1 (Dako), and Leu-M1(CD15) (Becton Dickinson, San José, CA, USA) revealed positivity for cytokeratins, vimentin, calretinin (Fig. 3), HBME-1, and EMA. Conversely, there was no immunoreactivity for the markers CEA and CD15. The cell


Fig. 4. Immunohistochemistry for MIB-1. Only a few nuclei were positive (⫻400)

proliferation index, quantitatively evaluated with antibody Ki67/MIB-1 and expressed as the percentage of tumoral cells with positive nuclear immunostaining per 500 cells observed at ⫻400, was 5% (Fig. 4). Thus, the final diagnosis was a mesothelioma of the peritoneum of the hernial sac, with a biphasic pattern.

Discussion Most mesotheliomas develop in the pleural or peritoneal cavity, and a mesothelioma arising from the cells lining the hernial sacs is extremely rare.11 In fact, only about 7% of patients with a peritoneal mesothelioma have associated inguinal or umbilical hernias resulting from increased intra-abdominal pressure caused by ascites.8 Our recent updating review of the literature revealed only six cases of primary mesothelioma of the hernia sac.2–7 The patients, who were all men, ranged in age from 33 to 87 years and because of the lack of characteristic clinical symptoms, the preoperative diagnosis was never established or even firmly suspected. Most of these tumors were found intraoperatively. Clinically, rapid growth of the hernia with associated abdominal pain and ascites should alert the surgeon to the possibility of this tumor. Preoperative ultrasonography can provide further information by showing inhomogeneous effusion or an exophytic tumor. Cytoanalysis of the fluid can also be diagnostic and is therefore recommended. A neoplastic diagnosis is often suspected intraoperatively and always needs histological confirmation. The differential diagnoses of primary mesothelioma of the hernial sac include diffuse peritoneal malignant mesothelioma and metastatic carcinomas. Diagnostic

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distinction of primary hernial sac mesothelioma from diffuse peritoneal mesothelioma may not be possible in small biopsies, because of the histologically and immunophenotypically identical profiles of these tumors; only an accurate radiological and surgical description of the tumor site and its distribution can define which type of malignancy it is. Distinction from metastatic hernial sac tumors can also be made microscopically by mucin stains and immunohistochemical study. Diffuse malignant mesothelioma is well known to be associated with asbestos exposure and various asbestoscontaining materials.9 Nevertheless, there are several apparently non-asbestos-related mesotheliomas, which suggests that other as yet unidentified environmental agents may play a role in the development of mesothelioma. Long-standing chronic irritation and mesothelial hyperplasia have also been considered as predisposing factors.10,12,13 Chahinian et al.10 and Gentiloni et al.12 reported two cases of peritoneal mesothelioma arising in patients with a long history of familial Mediterranean fever and recurrent peritonitis. Riddell et al.13 also reported a case of peritoneal mesothelioma in a patient with a history of severe persistent and recurrent diverticulitis. The evidence provided by these case reports suggests an association between persistent chronic inflammation and primary hernial sac mesothelioma. All tumors documented in the literature, which presented as localized masses in a hernial sac with minimal dissemination to the abdomen, were associated with a much better prognosis than diffuse pleural and peritoneal mesothelioma. Our patient had no apparent history of exposure to asbestos and his clinical history was similar to that described in past reports. Its early symptoms were nonspecific, and laboratory and radiological findings were not helpful in establishing the diagnosis. The patient only had an elevated serum level of CA125 preoperatively. This finding is typical in women with epithelial serous tumors, but has also been reported in some cases of peritoneal mesothelioma.14 Therefore, we think that serum CA125 could be used to help diagnose this disease and manage the patients. Malignant peritoneal mesothelioma is a clinically aggressive tumor associated with a poor prognosis and therefore, the selection of therapeutic strategies is important.15 Aggressive cytoreductive surgery, abdominal irradiation, and systemic chemotherapy are all generally ineffective against peritoneal mesothelioma and related to performance status, age, and histology.1,16 In our patient, the surgical sparing of the spermatic cord and testis was justified by the emergency approach without a preoperative diagnosis of malignancy, and by the distance of the mass from these anatomical structures. Systemic chemotherapy alone will not improve the patients’ survival,17 but when used in combination with

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tumor debulking it could have a positive impact,18 despite which this combined approach has not become routine. In our patient, the combination of systemic chemotherapy with surgical resection was successful in achieving long-term survival without any further symptoms. Alternatively, chemotherapy can be administered directly into the peritoneum, but it is remarkable that the best responses to intraperitoneal chemotherapy were achieved in patients with small-volume residual disease.19 The literature suggests that localization at the onset of the disease is an important prognostic factor supported by the fact that our patient has survived longer than any others with this disease. We believe that a combination of surgery and chemotherapy could be very effective for patients with localized disease. In conclusion, one of the major difficulties in managing patients with hernial sac mesothelioma is the rarity of this tumor. Moreover, the original presentation in an incarcerated inguinal hernia, the lack of factors indicative of an aggressive form of MPM, and the localization of the disease are atypical peculiarities of this presentation.


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Acknowledgments. We gratefully acknowledge the assistance of Professor Malcolm Clark in the preparation of the English manuscript and Dr. Teresa Cipriani in the preparation of the photographic material.

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