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Start free with EleplanMultilocular cystic renal neoplasm of low malignant potential
ORPHA:319287Histopathological subtype
Also called MCRCC · Multilocular clear cell adenocarcinoma · Multilocular clear cell carcinoma · Multilocular clear cell renal cell adenocarcinoma · Multilocular clear cell renal cell carcinoma · Multilocular cystic renal cell adenocarcinoma · Multilocular cystic renal cell carcinoma
What it is
Multilocular cystic renal neoplasm of low malignant potential is a rare subtype of clear cell renal cell carcinoma with distinct pathological features of cysts lined by occasionally flattened cuboidal clear cells and septa containing aggregates of epithelial cells with clear cytoplasm, and excellent prognosis. The tumor usually presents as an asymptomatic, unilateral, solitary lesion, macroscopically consisting of numerous, fluid-filled, septated cysts of variable size. Rarely, the symptoms typically associated with renal tumors (flank pain, hematuria, palpable mass) may be present.
Key facts
- Classified as
- Histopathological subtype
Recorded for the broader condition
- Prevalence
- 1-9 / 100 000 (annual incidence, Europe)Clear cell renal carcinoma
- Age of onset
- Adult, ElderlyClear cell renal carcinoma
- Inheritance
- Not applicableClear cell renal carcinoma
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
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
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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