reported two cases of pulmonary blastoma
and three cases of well differentiated fetal adenocarcinoma of the lung with squamoid morules which had somatic missense mutations in the [beta]-catenin gene.
. Am J Clin Oncol 2007; 30: 645-648.
Sin embargo, una caracteristica histologica que ayuda a diferenciar estos dos tumores, es la presencia de un estroma embrionario maligno en el blastoma
pulmonar bifasico [1, 3].
(2,21,29) TP53 mutations are also seen in carcinosarcomas and pulmonary blastoma
Gastroblastoma has been compared to other blastomas
, such as pleuropulmonary blastoma
, hepatoblastoma, and nephroblastoma.
There is a continuum of differentiation between pleomorphic or spindle/giant cell carcinomas, carcinosarcomas, and blastomas
. (2,4,7) The mechanisms of carcinogenesis by which these tumors develop have been debated for years.
At imaging, round pneumonias may appear similar to a parenchymal bronchogenic cysts, pleuropulmonary blastoma
or metastatic disease.
It appears as a fetal lung at 10 to 15 weeks of gestation, resembling pulmonary blastoma
, but the adjacent mesenchymal tissue is histologically benign (10).
Nephro means kidney, and a blastoma
is a tumour of embryonic tissue that has not yet fully developed.
Neoplastic lesions included neurofibromas (2), ganglioneuroma (1), lymphoma (1), pulmonary blastoma
SLCTs have been associated sporadically for years with thyroid disease, embryonal rhabdomyosarcoma of the cervix, and pleuropulmonary blastoma
. As each of these is now known to be associated with germline or tumorspecific mutations in DICER1, it is not surprising that most Sertoli-Leydig cell tumors have also been found to be DICER1 related.
(9) There have been no reports of metastases, making the designation "blastoma
" confusing, since this term is usually reserved for malignant tumors.
(20) Pulmonary sarcomas can be generally grouped into 4 categories: (1) spindle cell sarcomas, including SS, malignant peripheral nerve sheath tumor, leiomyosarcoma, inflammatory myofibroblastic tumor, solitary fibrous tumor, and undifferentiated pleomorphic sarcoma; (2) vascular tumors, including angiosarcoma, Kaposi sarcoma, epithelioid hemangioendothelioma, and pulmonary artery intima sarcoma; (3) primitive/embryonal tumors, including primary pleuropulmonary blastoma
, primary NET, and rhabdomyosarcoma; and (4) others, including primary pulmonary myxoid sarcoma, angiomatoid fibrous histiocytoma, and alveolar soft part sarcoma.
(65,66) An additional consideration for the Sertoli cell nodules that have been colonized by IGCNU is gonado blastoma
, especially when there is matrix calcification.
Other than pleuropulmonary blastoma
family tumor and dysplasia syndrome, a rare syndrome related to germline mutation of DICER1 gene, most intraocular medulloepitheliomas occur sporadically and are not associated with congenital malformation or cytogenic abnormality.