Localised malignant mesothelioma
Definition
A rare localised tumour arising from a serosal surface, usually pleura, otherwise resembling diffuse malignant mesothelioma.
Epidemiology
The median age of patients is early sixties and the tumour is exceedingly rare before the age of forty. 60-70% of cases occur in men. Some patients have a history of asbestos exposure1,3,11.
Clinical features
Tumours present incidentally or with vague chest pain, dyspnea, malaise, fever or night sweats.
Radiology
There is a circumscribed pleura-based mass.
Macroscopic appearances
Most cases are pleural, a few peritoneal6. It may arise from parietal or visceral pleura. A few cases are largely intrapulmonary and may mimic a bronchogenic carcinoma3,4,14. Two cases have occurred within the liver7,12. The mass is localised and solitary and may be pedunculated or sessile10. There is no gross infiltration of the chest wall , lung or peritoneal organs.
Histopathology
The tumours are circumscribed. They may be epithelioid3,15, sarcomatous15 or mixed3 and show the wide range of appearances seen in diffuse malignant mesotheliomas. Microcystic4, deciduoid14 and vacuolated liposarcoma-like8 variants have been reported. One case included rhabdoid cells12.
Immunohistochemistry
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Pankeratin
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19/19(Included epithelioid, mixed and sarcomatoid cases)1, 2/22, 6/63, 1/15, 1/16, 1/18, 1/111, 1/115
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|
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Cam5.2
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1/11, 1/14, 1/15, 1/17
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AE1/AE3
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1/11, 1/14, 1/17, 1/19, 1/110,
1/1(AE1)12,
1/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13
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HMW cytokeratin
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1/16
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LMW cytokeratin
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1/16
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Calretinin
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10/111, 1/14, 1/15, 0/16, 1/17, 1/18, 0/110,
0/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13,
2/2(diffusely positive)14
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HBME-1
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4/51, 1/17
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Cytokeratin 5/6
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3/41, 1/16
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Thrombomodulin
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1/31, 0/14, 1/15, 1/16,
0/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13
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CD15
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0/71, 0/22,
1/6(focal membrane staining in one case)3, 0/14, 0/15, 0/17, 0/18, 0/19, 0/111
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B72.3
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0/91
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CEA
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1/17(focal, probably artefactual, in one case)1, 0/22, 0/63, 0/14, 0/15, 0/17, 0/18, 0/19, 0/111,
0/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13, 0/214, 0/115
|
|
BerEP4
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1/7(faint positivity in one case)1, 0/15,
1/1(faint)6, 0/110,
0/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13
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EMA
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7/8(focal in 2 cases)1, 0/22,
5/6(one of the five positive cases showed only focal staining)3, 0/14, 1/15, 1/16,
1/1(focal)8, 1/19, 1/110, 1/112,
1/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13, 0/115
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TTF-1
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0/41, 0/16, 0/214
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Surfactant
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0/214
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CK7
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2/21, 1/16, 1/114
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CK20
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0/11
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Vimentin
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2/3(only focal in one case)1, 1/53, 1/14,
1/1(focal)5, 1/18,
1/1(faint)9, 1/110, 1/111, 1/112,
1/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13, 1/214, 1/115
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Ca19.9
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0/11
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Ca125
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1/11
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AFP
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0/11, 0/19, 0/112
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CD8
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0/11
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CD31
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0/11
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CD34
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0/15, 0/17, 0/110,
0/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13
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CD56
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0/11
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CD68
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0/11
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S-100
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0/21, 0/14, 0/18, 0/110, 0/112,
0/1(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13, 0/115
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HMB45
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0/11
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Factor VIIIRA
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0/19, 0/115
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Synaptophysin
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0/21
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Chromogranin
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0/11
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SMA
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0/14, 0/214
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Desmin
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1/1(focal)10, 0/112,
1/1(focal: there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass)13
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Myoglobin
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0/112
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LCA
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0/11
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PSA
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0/11
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Alpha-1-antitrypsin
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0/115
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Ultrastructure
Features are as for diffuse malignant mesothelioma with long slender microvilli, bundles of intermediate filaments and well developed desmosomes3,8.
Differential diagnosis
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Diffuse malignant mesothelioma with a dominant mass: any microscopic foci of disseminated tumour excludes localised mesothelioma.
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Solitary fibrous tumour
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Synovial sarcoma
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Epithelioid angiosarcoma
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Adenomatoid tumour
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Vacuolated variant: liposarcoma8
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If intra-pulmonary, non-small cell carcinoma
Management
Prognosis
Biological behaviour is difficult to predict3. Some patients show longterm survival; others develop local recurrence3,9 , metachronous tumours15 or metastases10,14 but, with rare exceptions11,
13(there is a suspicion that this case was a diffuse malignant mesothelioma, initially with a dominant mass), not diffuse serosal dissemination1. Tumour size and histological subtype do not correlate with behaviour1.
References
1 Allen TC, Cagle PT, Churg AM, et al. Localized malignant mesothelioma. Am J Surg Pathol 2005; 29:866-73
2 Okike N, Bernatz PE,Woolner LB. Localized mesothelioma of the pleura: benign and malignant variants. J Thorac Cardiovasc Surg 1978; 75:363-72
3 Crotty TB, Myers JL, Katzenstein AL, et al. Localized malignant mesothelioma. A clinicopathologic and flow cytometric study. Am J Surg Pathol 1994; 18:357-63
4 Umezu H, Kuwata K, Ebe Y, et al. Microcystic variant of localized malignant mesothelioma accompanying an adenomatoid tumor-like lesion. Pathol Int 2002; 52:416-22
5 Gomez-Roman JJ, Mons-Lera R, Olmedo IS, et al. Flow cytometric analysis of a localized malignant mesothelioma. Ann Thorac Surg 2002; 73:1292-4
6 Val-Bernal JF, Figols J,Gomez-Roman JJ. Incidental localized (solitary) epithelial mesothelioma of the pericardium: case report and literature review. Cardiovasc Pathol 2002; 11:181-5
7 Imura J, Ichikawa K, Takeda J, et al. Localized malignant mesothelioma of the epithelial type occurring as a primary hepatic neoplasm: a case report with review of the literature. Apmis 2002; 110:789-94
8 Shimazaki H, Aida S, Iizuka Y, et al. Vacuolated cell mesothelioma of the pericardium resembling liposarcoma: a case report. Hum Pathol 2000; 31:767-70
9 Erkilic S, Sari I,Tuncozgur B. Localized pleural malignant mesothelioma. Pathol Int 2001; 51:812-5
10 Okamura H, Kamei T, Mitsuno A, et al. Localized malignant mesothelioma of the pleura. Pathol Int 2001; 51:654-60
11 Ojeda HF, Mech K, Jr.,Hicken WJ. Localized malignant mesothelioma: a case report. Am Surg 1998; 64:881-5
12 Matsukuma S, Aida S, Hata Y, et al. Localized malignant peritoneal mesothelioma containing rhabdoid cells. Pathol Int 1996; 46:389-91
13 Gotfried MH, Quan SF,Sobonya RE. Diffuse epithelial pleural mesothelioma presenting as a solitary lung mass. Chest 1983; 84:99-101
14 Asioli S, Dal Piaz G,Damiani S Localised pleural malignant mesothelioma. Report of two cases simulating pulmonary carcinoma and review of the literature. Virchows Arch 2004; 445:206-9
15 Obers VJ, Leiman G, Girdwood RW, et al. Primary malignant pleural tumors (mesotheliomas) presenting as localized masses. Fine needle aspiration cytologic findings, clinical and radiologic features and review of the literature. Acta Cytol 1988; 32:567-75
This page last revised 4.9.2005.
©SMUHT/PW Bishop