cystic nephroma
Pronunciation: SIS-tik neh-FROH-mah
A benign, purely cystic renal neoplasm of early childhood associated with DICER1 mutations, characterised by multilocular cysts lined by epithelium without solid or blastemal components.
Full Definition
Cystic nephroma in children is a non-malignant, well-circumscribed multilocular cystic tumour of the kidney, now recognised as part of the DICER1 tumour predisposition spectrum. It must be carefully distinguished from cystic partially differentiated nephroblastoma (CPDN), which contains nephrogenic elements within its septa, as the two diagnoses carry different clinical implications. In adults, cystic nephroma is an unrelated lesion with distinct molecular features. Editors must be alert to this age-related distinction: paediatric cystic nephroma ≠ adult cystic nephroma. The tumour is rendered as two words, not hyphenated.
Usage
Usage note: Always clarify patient age context; paediatric and adult cystic nephroma are molecularly and clinically distinct entities.
In Context
- "The resected specimen was classified as cystic nephroma after absence of blastemal elements was confirmed on thorough histological sampling." — Paediatric renal pathology report
- "Authors must specify whether 'cystic nephroma' refers to the paediatric DICER1-associated lesion or the unrelated adult form." — Manuscript editing comment