Radcliffe Crocker, in 1889, then described EMPD following an observation of a patient with urinary carcinoma affecting the penis and scrotum, showing symptoms that were almost identical to MPD as described by Paget. Later in 1893, Darier and Coulillaud described the perianal location of EMPD. References
External links
Definition at cancer.org
Either event can drive the malignant growth of cells but in most cases the chimeric proteins tyrosine kinase activity appears responsible for doing so. Based on a limited number of genetic studies (a total of 65 patients), the ETY6-NTRK3 fusion gene appears to occur in most cases of the cellular and some cases of the mixed but no cases of the classical types of congenital mesolastic nephroma. However, a more recent study of 19 patients detected the fused gene in all 8 cases of cellular, 5 of 6 cases of mixed, and 0 of 5 cases of classic mesoblastic nephroma. This suggests that expression of this fused gene may be more common in cellar and mixed mesoblastic nephroma than previously appreciated.Trisomy, i.e. pathological presence of an extra chromosome, also occurs in these neoplasms. Trisomy of chromosome 11 (e.g. trisomy 11) appears to be the most commonly found trisomy in this disease, being detected in 7 of 13 genetically studied cases. Individual case reports have also found trisomy 8 (9 cases), 17 (4 cases), 20 (4 cases), 7 (3 cases), 10 (3 cases), 18 (2 cases), 2 (2 cases), and 9 (2 cases) associated with the disease. The contribution of these trisomies to the development of mesoblastic nephroma is unclear. Diagnosis
Diagnosis of mesoblastic nephroma and its particular type (i.e. classic, mixed, or cellular) is made by histological examination of tissues obtained at surgery.