Multilocular cystic renal neoplasm of low malignant potential

2021-10-29   Paola Dal Cin , Michelle S. Hirsch 

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)

Classification

Definition

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.

Clinics and Pathology

Epidemiology

<1% of renal tumors, middle-aged adults.

Clinical features

Unilateral solitary lesion, asymptomatic and frequently detected incidentally.

Histopathology

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.

Genetics

Fish analysis

Deletion of chromosome 3p/monosomy 3  by FISH analysis has been demonstrated, with comparable frequency to clear cell renal carcinoma 1,2.

Mutations

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.

Cytogenetics

Prognosis and treatment

Favorable prognosis, do not recur or metastasize

Bibliography

Reference NumberPubmed IDLast YearTitleAuthors
1203488772010Multilocular cystic renal cell carcinoma is a subtype of clear cell renal cell carcinoma.Halat S et al
2297708532018Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum.Tretiakova M et al
3211510992011VHL mutations and dysregulation of pVHL- and PTEN-controlled pathways in multilocular cystic renal cell carcinoma.von Teichman A et al
4264383002015Unlike in clear cell renal cell carcinoma, KRAS is not mutated in multilocular cystic clear cell renal cell neoplasm of low potential.Raspollini MR et al
5310984212019SETD2, GIGYF2, FGFR3, BCR, KMT2C, and TSC2 as candidate genes for differentiating multilocular cystic renal neoplasm of low malignant potential from clear cell renal cell carcinoma with cystic change.Kim SH et al

External Links

Citation

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