Congenital mesoblastic nephroma

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Congenital mesoblastic nephroma

ORPHA:2665Disease

What it is

A rare renal tumor characterized by a unilateral, solitary, well demarcated, mesenchymal/myofibroblastic neoplasm occurring in very young children. Histopathologically, three subtypes (classic, cellular, and mixed) can be distinguished. The tumor most commonly involves the renal sinus and is typically discovered as a palpable abdominal mass. Patients may also present with hypertension or hematuria, rarely with hypercalcemia or hyperreninemia. Prenatal presentation, usually with polyhydramnios, is not infrequent. The most important prognostic factor is completeness of surgical resection. Overall, malignant potential is low and clinical outcome favorable.

Key facts

Age of onset
Infancy, Neonatal
Classified as
Disease

Genes

ETV6Part of a fusion gene
NTRK3Part of a fusion gene

ICD-10 codes

D41.0filed under a broader ICD-10 category

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

GARD 1493MEDDRA 10070665MESH D018201MONDO 0017043UMLS C1332965

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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