Malignant Mesothelioma



Malignant Mesothelioma











Extrapulmonary pneumonectomy consisting of both visceral and parietal pleuras, lung, and portions of diaphragm and pericardium is shown. Note the marked pleural thickening.






Sarcomatoid malignant mesothelioma shows a spindle cell proliferation closely mimicking the features of a mesenchymal neoplasm.


TERMINOLOGY


Synonyms



  • Epithelioma



    • Term used in older literature


Definitions



  • Malignant neoplasm arising in serosal surfaces


ETIOLOGY/PATHOGENESIS


Environmental Exposure



  • Mesotheliomas have been linked closely to asbestos exposure


Other Possible Etiologies



  • Viral infection SV40


  • De novo


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare tumor that accounts for approximately 4-7 cases per 1,000,000 individuals


  • Age



    • More common in 5th and 6th decades of life


  • Gender



    • More common in men


Presentation



  • Weight loss


  • Chest pain


  • Cough


  • Shortness of breath


  • Pleural effusion


Treatment



  • Surgical approaches



    • Extrapulmonary pneumonectomy


    • Decortication


    • Pleural peeling


  • Adjuvant therapy



    • Chemotherapy


Prognosis



  • Poor



    • 12-18 month average survival


MACROSCOPIC FEATURES


General Features



  • Diffuse thickening of pleura


  • In rare cases, tumor will present as localized pleural-based tumor


MICROSCOPIC PATHOLOGY


Histologic Features



  • Homogeneous cellular proliferation without much mitotic activity or cellular pleomorphism


Predominant Pattern/Injury Type



  • Tubulo-papillary


  • Sarcomatoid


  • Biphasic


Predominant Cell/Compartment Type



  • Epithelial


  • Spindle


Other Growth Patterns



  • Clear cell



    • This pattern is characterized by presence of cellular proliferation in which cells show clear cytoplasm


    • Low mitotic count


    • Most of these tumors do not show extensive areas of necrosis


  • Deciduoid




    • This pattern is composed of medium-sized cells with excentric nuclei and inconspicuous nucleoli


    • Low mitotic count


    • Necrosis and prominent nuclear atypia are not common


  • Adenomatoid



    • Cords of neoplastic cells forming cystic spaces


    • Signet ring cell-like


    • Low mitotic count


    • Areas of necrosis or hemorrhage are not common


  • Glandular



    • Prominent glandular component


    • Glands of different sizes


    • Most closely mimics adenocarcinoma


  • Mucohyaline



    • Extensive pools of mucoid material


    • Neoplastic cells embedded or floating in pools of mucoid material


  • Cartilaginous and osseous metaplasia



    • Very unusual growth pattern


    • Tumor shows extensive areas of cartilaginous or osseous differentiation


    • Numerous osteoclast giant cells


  • Desmoplastic



    • Low cellularity


    • Easily mistaken for a benign tumor


    • Neoplastic cells, either spindle or oval, dissecting fibrocollagen


    • Areas of necrosis are not readily apparent


  • Lymphohistiocytic



    • Mixture of epithelioid cells, lymphocytes, and histiocytes


    • May mimic thymoma or lymphoma


ANCILLARY TESTS


Electron Microscopy



  • Transmission



    • Elongated microvilli


    • Well-developed cell junctions


DIFFERENTIAL DIAGNOSIS


Metastatic Adenocarcinoma



  • Shows positive staining for carcinomatous epitopes, including CEA, MOC31, and BER-EP4


  • History of primary tumor elsewhere is important


Sarcoma



  • Sarcomas encasing lung parenchyma are rather unusual


  • It would be unusual for mesothelioma to stain with mesenchymal markers other than vimentin


  • Clinical history is important to rule out a metastasis


Synovial Sarcoma



  • Either mono- or biphasic tumors may mimic mesothelioma


  • Monophasic tumors will show positive staining for Bcl-2, which would be unusual in mesothelioma


  • Synovial sarcoma shows characteristic X;18 translocation


Thymoma



  • In cases of lymphohistiocytic mesothelioma



    • Use of immunohistochemical studies show strong p63(+) in thymoma


  • Although thymomas may occur as pleural tumors, they are more common in anterior mediastinum


Metastatic Clear Cell Carcinoma



  • In cases of clear cell mesothelioma



    • Use of immunohistochemical studies will lead to correct interpretation


Metastatic Osteogenic Sarcoma



  • In cases of mesothelioma with osseous and cartilaginous components



    • Clinical history of previous osteosarcoma will be necessary for correct diagnosis



DIAGNOSTIC CHECKLIST


Clinically Relevant Pathologic Features



  • Invasive pattern



    • Penetration of tumor cells into fat



SELECTED REFERENCES

1. Arrossi AV et al: Histologic assessment and prognostic factors of malignant pleural mesothelioma treated with extrapleural pneumonectomy. Am J Clin Pathol. 130(5):754-64, 2008

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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Malignant Mesothelioma

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