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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Malignant epithelioid mesothelioma - high mag.jpg | |||
| Width = | |||
| Caption = Malignant mesothelioma. [[H&E stain]]. | |||
| Micro = infiltrative atypical cells (epithelioid, spindled or both) | |||
| Subtypes = biphasic mesothelioma, epithelioid mesothelioma, desmoplastic mesothelioma, sarcomatoid mesothelioma. | |||
| LMDDx = mesothelial hyperplasia, [[fibrosing pleuritis]], [[adenocarcinoma]] - esp. [[lung adenocarcinoma|lung]], [[serous carcinoma]] | |||
| Stains = | |||
| IHC = calretinin +ve, D2-40 +ve, [[CK5/6]] +ve, WT-1 +ve, [[CK7]] +ve, CEA -ve, [[TTF-1]] -ve | |||
| EM = | |||
| Molecular = +/-p16 deletion | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[lung]], [[peritoneum]], [[omentum]], [[pericardium]] | |||
| Assdx = [[asbestosis]] | |||
| Syndromes = | |||
| Clinicalhx = +/-asbestos exposure | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = rare | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = very poor | |||
| Other = | |||
| ClinDDx = | |||
}} | |||
'''Malignant mesothelioma''', also '''mesothelioma''', is a form of [[cancer]]. It arises from the mesothelium. | |||
It should '''not''' be confused with ''[[benign multicystic mesothelioma]]'' and ''[[benign papillary mesothelioma]]''. | |||
==General== | |||
*Incidence | |||
**Rare tumor accounting for 4-7 cases per million individuals. | |||
**More common in men in 5th and 6th decades of life. | |||
*Poor prognosis<ref>{{Cite journal | last1 = Haber | first1 = SE. | last2 = Haber | first2 = JM. | title = Malignant mesothelioma: a clinical study of 238 cases. | journal = Ind Health | volume = 49 | issue = 2 | pages = 166-72 | month = | year = 2011 | doi = | PMID = 21173534 }}</ref> - median survival <12 months.<ref name=pmid23413596>{{Cite journal | last1 = Mineo | first1 = TC. | last2 = Ambrogi | first2 = V. | title = Malignant pleural mesothelioma: factors influencing the prognosis. | journal = Oncology (Williston Park) | volume = 26 | issue = 12 | pages = 1164-75 | month = Dec | year = 2012 | doi = | PMID = 23413596 }}</ref> | |||
Locations: | |||
*Lung. | |||
*Primary [[peritoneum|peritoneal]]. | |||
*Primary [[pericardium|pericardial]].<ref name=pmid22740748>{{Cite journal | last1 = Sardar | first1 = MR. | last2 = Kuntz | first2 = C. | last3 = Patel | first3 = T. | last4 = Saeed | first4 = W. | last5 = Gnall | first5 = E. | last6 = Imaizumi | first6 = S. | last7 = Lande | first7 = L. | title = Primary pericardial mesothelioma unique case and literature review. | journal = Tex Heart Inst J | volume = 39 | issue = 2 | pages = 261-4 | month = | year = 2012 | doi = | PMID = 22740748 }}</ref> | |||
Epidemiology: | |||
*Strong association with asbestos exposure. | |||
Treatment: | |||
*+/-Surgical debulking (cytoreduction) with heated chemotherapy - for intraperitoneal mesothelioma.<ref name=pmid24158467>{{cite journal |author=Wong J, Koch AL, Deneve JL, Fulp W, Tanvetyanon T, Dessureault S |title=Repeat cytoreductive surgery and heated intraperitoneal chemotherapy may offer survival benefit for intraperitoneal mesothelioma: a single institution experience |journal=Ann. Surg. Oncol. |volume=21 |issue=5 |pages=1480–6 |year=2014 |month=May |pmid=24158467 |doi=10.1245/s10434-013-3341-7 |url=}}</ref> | |||
Note: | |||
*No association with [[smoking]].{{fact}}<ref name=pmid24351898>{{Cite journal | last1 = Offermans | first1 = NS. | last2 = Vermeulen | first2 = R. | last3 = Burdorf | first3 = A. | last4 = Goldbohm | first4 = RA. | last5 = Kauppinen | first5 = T. | last6 = Kromhout | first6 = H. | last7 = van den Brandt | first7 = PA. | title = Occupational asbestos exposure and risk of pleural mesothelioma, lung cancer, and laryngeal cancer in the prospective Netherlands cohort study. | journal = J Occup Environ Med | volume = 56 | issue = 1 | pages = 6-19 | month = Jan | year = 2014 | doi = 10.1097/JOM.0000000000000060 | PMID = 24351898 }}</ref> | |||
===Asbestos=== | |||
Conditions associated with asbestos exposure (mnemonic ''PALM''):<ref name=Ref_PCPBoD8_375>{{Ref PCPBoD8|375}}</ref> | |||
*[[Pleural plaques]]. | |||
*[[Asbestosis]]. | |||
*[[Lung carcinoma]]. | |||
*Malignant mesothelioma. | |||
Possible association with asbestos exposure: | |||
*[[Gestational trophoblastic disease]].<ref name=pmid19900938>{{Cite journal | last1 = Reid | first1 = A. | last2 = Heyworth | first2 = J. | last3 = de Klerk | first3 = N. | last4 = Musk | first4 = AW. | title = Asbestos exposure and gestational trophoblastic disease: a hypothesis. | journal = Cancer Epidemiol Biomarkers Prev | volume = 18 | issue = 11 | pages = 2895-8 | month = Nov | year = 2009 | doi = 10.1158/1055-9965.EPI-09-0731 | PMID = 19900938 }}</ref> | |||
==Microscopic== | |||
Features:<ref name=Ref_WMSP156>{{Ref WMSP|156}}</ref> | |||
*Infiltrative atypical cells - '''key feature'''. | |||
**Infiltration into fat - ''diagnostic''. | |||
**+/-Epithelioid cells - may be cytologically bland, i.e. benign appearing. | |||
***Variable architecture: sheets, microglandular, tubulopapillary. | |||
***+/-[[Psammoma bodies]]. | |||
**+/-Spindle cells. | |||
*+/-''[[Ferruginous body]]'' - '''strongly supportive'''.<ref>URL: [http://medical-dictionary.thefreedictionary.com/asbestos+body http://medical-dictionary.thefreedictionary.com/asbestos+body]. Accessed on: 4 November 2011.</ref> | |||
** Looks like a (twirling) baton - segemented appearance, brown colour. | |||
** Thin (asbestos) fiber in the core. | |||
Notes: | |||
*''Asbestos body'' is not strictly speaking a synonym for ''ferruginous body''. | |||
*Don't diagnose ''mesothelioma in situ''.{{fact}} | |||
DDx:<ref name=pmid15559051>{{Cite journal | last1 = Corson | first1 = JM. | title = Pathology of mesothelioma. | journal = Thorac Surg Clin | volume = 14 | issue = 4 | pages = 447-60 | month = Nov | year = 2004 | doi = 10.1016/j.thorsurg.2004.06.007 | PMID = 15559051 }} | |||
</ref> | |||
<ref name="pmid15725802">{{Cite journal | last1 = Bégueret | first1 = H. | last2 = Galateau-Salle | first2 = F. | last3 = Guillou | first3 = L. | last4 = Chetaille | first4 = B. | last5 = Brambilla | first5 = E. | last6 = Vignaud | first6 = JM. | last7 = Terrier | first7 = P. | last8 = Groussard | first8 = O. | last9 = Coindre | first9 = JM. | title = Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18)-positive cases from the French Sarcoma Group and the Mesopath Group. | journal = Am J Surg Pathol | volume = 29 | issue = 3 | pages = 339-46 | month = Mar | year = 2005 | doi = | PMID = 15725802 }}</ref> | |||
*[[Fibrosing pleuritis]] - should ''not'' have nodules, more cellular on the aspect adjacent to the effusion. | |||
*Reactive mesothelial cells - may be atypical. | |||
*Mesothelial hyperplasia. | |||
*[[Adenocarcinoma]] - esp. [[lung adenocarcinoma]]. | |||
*[[Serous carcinoma]]. | |||
*[[Synovial sarcoma]]. | |||
===Images=== | |||
<gallery> | |||
Image:Ferruginous_body.jpg | Ferruginous body. (WC) | |||
Image: Malignant epithelioid mesothelioma - low mag.jpg | MM - low mag. | |||
Image: Malignant epithelioid mesothelioma - intermed mag.jpg | MM - intermed. mag. | |||
Image: Malignant epithelioid mesothelioma - high mag.jpg | MM - high mag. | |||
Image: Malignant epithelioid mesothelioma - very high mag.jpg | MM - very high mag. | |||
Image: Malignant epithelioid mesothelioma - calretinin - intermed mag.jpg | MM - calretinin - intermed. mag. | |||
Image: Malignant epithelioid mesothelioma - calretinin - high mag.jpg | MM - calretinin - high mag. | |||
</gallery> | |||
====www==== | |||
*[http://www.rosaicollection.org/searchresults.cfm/ Mesothelioma (rosaicollection.org/index.cfm)]. | |||
===Subtypes=== | |||
List of subtypes - mnemonic ''BEDS'':<ref name=pmid15559051/><ref name=Ref_WMSP156>{{Ref WMSP|156}}</ref> | |||
*Biphasic mesothelioma. | |||
**10%+ of epithelioid & 10%+ sarcomatoid. | |||
*Epithelioid mesothelioma. | |||
*Desmoplastic mesothelioma. | |||
**Should be 50%+ dense tissue with storiform pattern & atypical cells. | |||
*Sarcomatoid mesothelioma. | |||
Other: | |||
*Small cell mesothelioma.<ref name=pmid1310669>{{Cite journal | last1 = Mayall | first1 = FG. | last2 = Gibbs | first2 = AR. | title = The histology and immunohistochemistry of small cell mesothelioma. | journal = Histopathology | volume = 20 | issue = 1 | pages = 47-51 | month = Jan | year = 1992 | doi = | PMID = 1310669 }}</ref> | |||
==Stains== | |||
*PASD -ve. | |||
*Mucicarmine -ve. | |||
**Typically +ve in adenocarcinoma. | |||
==IHC== | |||
===Mesothelioma versus mesothelial hyperplasia=== | |||
Features:<ref name=pmid20209622>{{Cite journal | last1 = Hasteh | first1 = F. | last2 = Lin | first2 = GY. | last3 = Weidner | first3 = N. | last4 = Michael | first4 = CW. | title = The use of immunohistochemistry to distinguish reactive mesothelial cells from malignant mesothelioma in cytologic effusions. | journal = Cancer Cytopathol | volume = 118 | issue = 2 | pages = 90-6 | month = Apr | year = 2010 | doi = 10.1002/cncy.20071 | PMID = 20209622 }}</ref> | |||
*EMA +ve ~100% (vs. ~10%). | |||
*Desmin -ve ~5% (vs. ~85%). | |||
*GLUT1 +ve ~50% (vs. ~10%) | |||
*p53 +ve ~50% (vs. ~2%). | |||
Note: | |||
*The above are ''not'' very useful in individual cases. | |||
*A simple pankeratin is useful for seening where epithelial cells are. | |||
===Mesothelioma versus adenocarcinoma=== | |||
*Several panel exists - ''no agreed upon best panel''.<ref name=pmid18318582>{{cite journal |author=Marchevsky AM |title=Application of immunohistochemistry to the diagnosis of malignant mesothelioma |journal=Arch. Pathol. Lab. Med. |volume=132 |issue=3 |pages=397-401 |year=2008 |month=March |pmid=18318582 |doi= |url=http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=132&page=397}}</ref> | |||
**Usually two carcinoma markers + two mesothelial markers. | |||
Panel:<ref name=pmid18318582/> | |||
*Mesothelial markers: | |||
**Calretinin. | |||
**WT-1. | |||
**D2-40. | |||
**[[CK5/6]]. | |||
*Carcinoma markers: | |||
**CEA (monoclonal and polyclonal). | |||
**[[TTF-1]]. | |||
**[[Ber-EP4]]. | |||
**MOC-31. | |||
**CD15. | |||
**[[B72.3]]. | |||
===Other carcinoma markers=== | |||
*[[PAX8]] -ve.<ref name=pmid23846294>{{cite journal |author=Lee M, Alexander HR, Burke A |title=Diffuse mesothelioma of the peritoneum: a pathological study of 64 tumours treated with cytoreductive therapy |journal=Pathology |volume=45 |issue=5 |pages=464–73 |year=2013 |month=August |pmid=23846294 |doi=10.1097/PAT.0b013e3283631cce |url=}}</ref> | |||
*Claudin-4. | |||
**Mesothelioma may be focally positive.<ref name=pmid23775021>{{cite journal |author=Ohta Y, Sasaki Y, Saito M, ''et al.'' |title=Claudin-4 as a marker for distinguishing malignant mesothelioma from lung carcinoma and serous adenocarcinoma |journal=Int. J. Surg. Pathol. |volume=21 |issue=5 |pages=493–501 |year=2013 |month=October |pmid=23775021 |doi=10.1177/1066896913491320 |url=}}</ref> | |||
*[[p63]] -ve.<ref name=pmid18064689>{{Cite journal | last1 = Pu | first1 = RT. | last2 = Pang | first2 = Y. | last3 = Michael | first3 = CW. | title = Utility of WT-1, p63, MOC31, mesothelin, and cytokeratin (K903 and CK5/6) immunostains in differentiating adenocarcinoma, squamous cell carcinoma, and malignant mesothelioma in effusions. | journal = Diagn Cytopathol | volume = 36 | issue = 1 | pages = 20-5 | month = Jan | year = 2008 | doi = 10.1002/dc.20747 | PMID = 18064689 }}</ref> | |||
*[[CD138]] +ve. | |||
**Usually -ve in mesothelioma.<ref name=pmid12866374>{{Cite journal | last1 = Chu | first1 = PG. | last2 = Arber | first2 = DA. | last3 = Weiss | first3 = LM. | title = Expression of T/NK-cell and plasma cell antigens in nonhematopoietic epithelioid neoplasms. An immunohistochemical study of 447 cases. | journal = Am J Clin Pathol | volume = 120 | issue = 1 | pages = 64-70 | month = Jul | year = 2003 | doi = 10.1309/48KC-17WA-U69B-TBXQ | PMID = 12866374 }}</ref> | |||
==Molecular== | |||
*p16 deletion by [[FISH]].<ref name=pmid24503757>{{cite journal |author=Hwang H, Tse C, Rodriguez S, Gown A, Churg A |title=p16 FISH deletion in surface epithelial mesothelial proliferations is predictive of underlying invasive mesothelioma |journal=Am. J. Surg. Pathol. |volume=38 |issue=5 |pages=681–8 |year=2014 |month=May |pmid=24503757 |doi=10.1097/PAS.0000000000000176 |url=}}</ref> | |||
Notes: | |||
*p16 IHC does ''not'' give the same result. | |||
*Sensitivity of p16 deletion is low. | |||
==Sign out== | |||
<pre> | |||
Pleural Tissue of Right Lung, Removal: | |||
- MALIGNANT MESOTHELIOMA, epithelioid type. | |||
Comment: | |||
IHC confirms the morphologic impression. | |||
The tumour stains as follows: | |||
POSITIVE: calretinin (very strong), CK5/6. | |||
NEGATIVE: TTF-1. | |||
</pre> | |||
==See also== | |||
*[[Lung tumours]]. | |||
*[[Omentum]]. | |||
*[[Empyema peel]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Pulmonary pathology]] | |||