1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA) 2.Department of Pathology, Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
Kidney lesion with similar genetic and histopathological (i.e., cytologic) characteristics to clear cell renal cell carcinoma (CCRCC), but completely different architectural and clinical outcome, including a favorable to benign prognosis without recurrence or progression.
<1% of renal tumors, middle-aged adults.
Unilateral solitary lesion, asymptomatic and frequently detected incidentally.
The tumors contain fluid-filled cysts separated by thin fibrous septae lined by renal epithelial cells. These single layers of cells have clear cytoplasm similar to that seen in CCRCC and small nuclei typically no more than ~10microns in diameter. Rare small, microscopic invasive tumor nests may be seen in septae stroma; however, there is an absence of solid nodular growth (ie, mural nodules), necrosis, and sarcomatoid differentiation 1. If the latter is present, a diagnosis of CCRCC with cystic features should be made.
Deletion of chromosome 3p/monosomy 3 by FISH analysis has been demonstrated, with comparable frequency to clear cell renal carcinoma 1,2.
VHL mutations have also been detected 3, but not KRAS mutations 4.
Six novel genetic alterations GIGYF2, FGFR3, SETD2, BCR, KMT2C and TSC2 could be potential candidate genes for differentiating multilocular cystic renal neoplasm of low malignant potential from clear cell renal cell carcinoma with cystic change 5.
Favorable prognosis, do not recur or metastasize
Paola Dal Cin ; Michelle S. Hirsch
Multilocular cystic renal neoplasm of low malignant potential
Atlas Genet Cytogenet Oncol Haematol. 2021-10-29
Online version: http://atlasgeneticsoncology.org/solid-tumor/208919/multilocular-cystic-renal-neoplasm-of-low-malignant-potential