Tumor pathology
Congenital Mesoblastic nephroma is a malignant tumorous growth of the kidneys mesenchyme (i.e. connective tissue cells). Histologic examination of these tumors provides critical information on their prognoses. This examination divides congenital mesoblastic nephroma into three types:

1) The classic type occurs in ~39% of patients. Its tissues show interlaced spindle-shaped smooth muscle cells evidencing low mitotic activity with no evidence of tumor encapsulation; and infiltration into and entrapment of normal kidney tissue. 2) The cellular type occurs in ~42% of patients. Its tissues show densely packed fibrosarcoma-like cells evidencing high rates of mitosis, less infiltration of normal kidney tissue, and multiple areas of hemorrhage and cysts. 3) The mixed type occurs in ~19% of patients. It shows a mixture of the classic and cellular types in different areas of the neoplasm. Genetics
A study conducted in 1998 found that congenital mesoblastic nephroma tissues taken from some patients contained an acquired mutation, the ETV6-NTRK3 fusion gene. This gene results from a translocation of genetic material from the ETV6 gene located on the short arm (designated p) of chromosome 12 at position p13.2 (i.e. 12p13.2) to the NTRK3 gene located on the long arm (designated q) of chromosome 15 at position q25.3 (i.e. 15q25.3). This ETV6-NTRK3 gene fusion is notated as t(12;15)(p13;q25) and consists of the 5 end of ETV6 fused to the 3 end  of NTRK3. In consequence, the chimeric protein product of this gene lacks ETV6 proteins transcription factor activity while having NTRK3 proteins tyrosine kinase in an unregulated and continuously active form.
Unlike mesoblastic nephroma, clear cell sarcoma of the kidney presents with metastasis, particularly to bone, in 5-6% of cases; it histology is diverse and has been mistaken for mesoblastic nephroma. One chromosomal translocations t,(10;17)(q22;p13), has been repeatedly reported to be associated with clear cell sarcoma of the kidney. Infantile myofibromatosis is a fibrous tumor of infancy and childhood most commonly presenting during the first 2 years of life as a single subcutaneous nodule of the head and neck region or less commonly as multiple lesions of skin, muscle, bone, and in ~33% of these latter cases, visceral organs. All of these lesions have an excellent prognosis and can regress spontaneously except for those in which there is visceral involvement where the prognosis is poor. While infantile myofibromatosis and classic mesoblastic nephroma have been suggested to be the same diseases because of their very similar histology, studies on the distribution of cell-type markers (i.e. cyclin D1 and Beta-catenin) indicate that they have different cellular origins. Treatment
Based on a survey of >800, surgical removal of the entire involved kidney plus the peri-renal fat appeared curative for the majority of all types of mesoblastic nephroma; the patient overall survival rate was 94%. Of the 4% of non-survivors, half were due to surgical or chemotherapeutic treatments. Another 4% of these patients suffered relapses, primarily in the local area of surgery rare cases of relapse due to lung or bone metastasis.. About 60% of these recurrent cases had a complete remission following further treatment.