Table 1 |
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| The clinicopathologic and molecular genetic characteristics of pulmonary blastoma, pulmonary carcinosarcoma, and the present case | ||||||||||||
| Diagnosis | Age | Sex | Incidence | Smoking | Location | Prognosis | Morphology | Immunohistochemical staining | Cytgenetic aberrations | Gene mutations | ||
| Epithelial elements | Mesenchymal elements | Epithelial elements | Mesenchymal elements | |||||||||
| Biphasic pulmonary blastoma [1,2,5-9] | 35-52 | M:F = 2:1 | 0.25-0.5% of pulmonary neoplasms | Yes | Upper lobe, central/endo-bronchial or peripheral | Poor (5-year survival rate 16%) | Low-grade adenocarcinoma of fetal lung type/well-differentiated fetal adenocarcinoma | Undifferentiated blastema, striated or smooth muscle, cartilage, bone, yolk sac-like areas, melanocytic differentiation, morules | EMA, pan-CK, CEA, TTF-1, CK7, nuclear/cytoplasmatic beta-catenin | Vimentin, desmin, SMA, myoglobin, S-100; morules: synaptophysin, chromogranin A, CD10 | Trisomies 2 and 8, allelic imbalances at 14q24q32 and 17p11p13 | TP53, MDM2, beta-catenin (no EGFR or KRAS) |
| Carcinosarcoma [1,2,4,5] | >50 | M:F = 7:1 | 0.2-0.4% of pulmonary neoplasms | Yes | Upper lobe, central/endo-bronchial or peripheral | Poor (5-year survival rate 20-50%) | Squamous cell carcinoma, adenocarcinoma, adenosquamous carcinoma,large cell carcinoma | Spindle cells, fibrosarcoma, rhabdomyosarcoma, chondrosarcoma, osteosarcoma, blastema-like stroma | EMA, pan-CK, CK7, CAM5.2, CK5/6, p63, napsin, synaptophysin, chromogranin, CD56, membranous beta-catenin (TTF-1 negative) | Desmin, myogenin, myoD1, S-100 | Gains: 1q, 3q, 5p, 8q, 12p; Losses: 3q, 5q, 17p | TP53 (no KRAS or beta-catenin) |
| Present case: blastomatoid variant of carcinosarcoma | 58 | M | – | Yes | Right upper lobe | Alive, no relapse (22 months) | High-grade adenocarcinoma of fetal lung type | Spindle cells, rhabdomyosarcoma | EMA, pan-CK, CK7, CEA, MDM2, CDK4, focal p63, membranous beta-catenin (TTF-1 negative) | Vimentin, desmin, EGFR, CD56, myogenin, MDM2, CDK4 | Gains: 1q, 6p, 6q24qter, 8q, 11q12q14, 11q23qter, 12q12q21, 12q24qter, 17q, 20q; Losses: 5q14q23, 9p13pter, 13q21q21; Amplifications: 12q14q21, 15q24qter, 20q11q12 | TP53, MDM2, CDK4 (no EGFR, KRAS or beta-catenin) |
EMA: epithelial membrane antigen.
CK: cytokeratin.
CEA: carcinoembryonic antigen.
TTF-1: thyroid transcription factor 1.
CDK4: cyclin-dependent kinase 4.
Schaefer et al. BMC Cancer 2012 12:424 doi:10.1186/1471-2407-12-424