Table 1 Patient demographic details, imaging features, histopathological features and follow-up details

Age (years)

28

45

7

Sex

F

M

M

Symptoms

Right flank pain

Left flank pain

Left abdominal mass

Stage

T2bN0Mx

T3bN1M1

T2bN0

Imaging

9.4 * 8 * 11 cm well-defined, exophytic, heterogeneous, multiloculated, cystic lesion from upper pole of left kidney with enhancing septation and calcifications

9.6 * 8.9 * 13.9 cm heterogeneously enhancing lesion completely involving left renal parenchyma, perinephric fat stranding and nodularity, calculi in calyces, tumoral thrombus extending into infrahepatic IVC, no contrast excretion, enlarged L.N in preaortic and para aortic, largest 2.1 * 1.4 cm, liver and lung lesions

Multiple discrete variably sized heterogeneous hypodense cystic lesions in both kidneys, showing heterogeneous enhancement of solid component within lesion and septa, largest lesion in lower pole of right kidney measures 3.6 * 3.8 cm, largest lesion in mid pole of left kidney measures 4.7 * 3.6 cm

Surgery

Left lap radical nephrectomy

Left open radical nephrectomy with renal vein thrombectomy

Left open radical nephrectomy

Biopsy

CK20 = positive, + 3, SDHB = positive, + 3

CK20 = positive, + 2, SDHB = positive, + 3

RCC = positive, + 2, PAN-CK = positive, + 2, PAX 8 = positive, + 2, EMA = positive, + 2, Vimentin = positive, + 3, CK20 = negative, CK7 = negative

Follow-up

CECT at 5 months normal

Was on pazopanib, CECT at 4 months retroperitoneal lymphadenopathy, liver lesions, bone lesion in ischiopubic rami. Expired at 12 months after surgery

Patient's parents were advised for right partial nephrectomy, but not willing for the same, taking alternative therapy