Definition
A rare localised tumour arising from a serosal surface, usually pleura, otherwise resembling diffuse malignant mesothelioma.
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.
Tumours present incidentally or with vague chest pain, dyspnea, malaise, fever or night sweats.
There is a circumscribed pleura-based mass.
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.
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.
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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1/11, 1/14, 1/15, 1/17 |
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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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1/16 |
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1/16 |
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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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4/51, 1/17 |
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3/41, 1/16 |
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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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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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0/91 |
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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 |
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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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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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0/41, 0/16, 0/214 |
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0/214 |
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2/21, 1/16, 1/114 |
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0/11 |
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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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0/11 |
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1/11 |
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0/11, 0/19, 0/112 |
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0/11 |
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0/11 |
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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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0/11 |
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0/11 |
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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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0/11 |
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0/19, 0/115 |
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0/21 |
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0/11 |
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0/14, 0/214 |
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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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0/112 |
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0/11 |
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0/11 |
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0/115 |
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Features are as for diffuse malignant mesothelioma with long slender microvilli, bundles of intermediate filaments and well developed desmosomes3,8.
Diffuse malignant mesothelioma with a dominant mass: any microscopic foci of disseminated tumour excludes localised mesothelioma.
Solitary fibrous tumour
Synovial sarcoma
Epithelioid angiosarcoma
Adenomatoid tumour
Vacuolated variant: liposarcoma8
If intra-pulmonary, non-small cell carcinoma
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.
9 Erkilic S, Sari I,Tuncozgur B. Localized pleural malignant mesothelioma. Pathol Int 2001; 51:812-5
This page last revised 4.9.2005.
©SMUHT/PW Bishop