Adult mesoblastic nephroma: expansion of the morphologic spectrum and review of literature.
Review
Overview
abstract
Mesoblastic nephroma (MN) is a distinctive tumor that is seen mostly in early infancy and that consists of classic and cellular (atypical) variants. Mesoblastic nephroma rarely occurs in adulthood, but MN in this age group still is poorly characterized because there are only 17 reported cases. We describe five additional cases of adult MN, including one case of the cellular variant, characterize the immunohistochemical profiles in detail, and critically review the previously reported cases. The collective data obtained from these 22 cases of adult MN showed that the patients predominantly were women (20 cases), ranging in age from 19 to 78 years, who were asymptomatic (5 cases) or had nonspecific signs and symptoms referable to a renal mass. Twenty tumors were classified as classic and 2 as cellular. The tumors were 2-24 cm, well circumscribed, and partially encapsulated and displayed a solid/ cystic cut surface, with a predominantly solid component in most tumors. One tumor, however, was almost purely cystic. Most tumors extended to the renal sinus. and some appeared entirely intrapelvic on imaging studies; however, gross and microscopic evaluation did not show destructive invasion of the pelvic wall. Extension of the tumor beyond the renal capsule has not been described. Each tumor was composed of epithelial and stromal components both. The epithelial component, which displayed no difference between the classic and cellular variants, was composed of isolated or clustered tubules and cysts lined by a benign epithelium with a wide range of cytologic differentiation. The stromal cells were composed of fibroblasts, myofibroblasts, and smooth muscle cells in various combinations. Stromal cellularity was low for the classic variant but high for the cellular variant. Hemorrhage, necrosis, and high mitotic index were noted in the stroma of the cellular, but not in the classic variant. Immunohistochemical study applied to the five current cases and seven normal control kidneys confirmed the presence of fibroblasts, myofibroblasts, smooth muscle cells, and prominent vessels in the stroma of each tumor. Most cysts and tubules within the tumors had a distinctive immunohistochemical profile, similar to that of collecting duct but different from those of other portions of the nephron in the normal control kidneys. After total or partial nephrectomy, without adjuvant chemotherapy or radiotherapy, 19 patients, including the 2 with cellular MN, were alive and well at 8-months to 48-years follow-up. Follow-up was not available in two patients. The remaining patient had recurrence at the surgical site 24 years after nephrectomy. Adult MN displays a distinctive morphologic spectrum that parallels that of its pediatric congener. It probably is a benign tumor that can be treated successfully by complete excision. The collecting duct differentiation expressed by most tubules and cysts of adult MN implies ureteric bud, which is the exclusive embryologic origin of collecting duct, as an important element in the histogenesis of this rare but fascinating type of tumor.
