Congenital mesoblastic nephroma 50 years after its recognition: A narrative review

Abstract

Congenital mesoblastic nephroma (CMN) is a rare pediatric renal tumor with low malignant potential that most commonly occurs early in infancy. Treatment strategies are based on the few published CMN series, while a significant number of CMN patients have been described in case reports. The aim of this narrative review was to create an up-to-date overview of the literature. Complete surgical removal is curative in most cases. The risk of treatment-related mortality (both surgery- and chemotherapy-related) is relatively high in the first weeks of life, indicating that these young patients deserve special attention with respect to timing and type of treatment.

Dramatic bone remodeling following larotrectinib administration for bone metastasis in a patient with TRK fusion congenital mesoblastic nephroma

“…Recurrences are rare and have been described in approximately 4% of the cases . Relapses more commonly occur locally, but distant metastasis in the lung and liver have been reported; bone metastases are extremely rare and account for 5.5% of reported cases with metastasis …”

“…Subsets of patients who are at high‐risk for recurrence include those who are older than 3 months of age at diagnosis, those with a cellular type that contains an NTRK gene‐fusion, and those with an incomplete surgical resection of the mass . Gooskens et al.…”

“…Gooskens et al. estimated that recurrence with cellular CMN was as high as 10% with a mortality of 57% in the cohort reviewed …”

ETV6–NTRK3 in congenital mesoblastic nephroma: A report of the SIOP/GPOH nephroblastoma study

“…Prognosis of CMN is generally excellent with an event‐free survival and overall survival (OS) of 94% and 95%, respectively . Nevertheless, several studies described local recurrences and distant metastases in the lung and liver .…”

“…Nevertheless, several studies described local recurrences and distant metastases in the lung and liver . Reported possible risk factors are incomplete resection, patient age, and cellular type of MN . These data are generally based on smaller studies with often limited further characterization.…”