Table 2.

List of adult cystic nephroma cases reported in literature

S. no.	Author	Age (y/gender)	History	Size	CT finding/USG	Surgical procedure	IHC performed
1	Cavıldak et al. [15]	48 y/F	Lumbar disc hernia	4.6 × 3.8 × 4.5 cm	Well defined, lobulated, encapsulated cystic lesion with multiple thin septa in lower pole of left kidney	Open partial nephrectomy	NA
2	Sharma et al. [16]	25 y/F	intermittent dull aching left-sided flank pain of 7 days' duration	6 cm × 5 cm	Large well defined lesion of the size 9.9 cm × 8.2 cm × 8.1 cm arising from left lower pole of kidney	Open left partial nephrectomy	NA
3	Ajit et al. [17]	28 y/F	Progressively increasing right abdomen lump with flank pain and haematuria for two years	31 × 19 × 19.6 cm	Multicystic enhancing tumour of 31 × 19 × 19.6 cm size arising from right kidney, was crossing the midline and was pushing the duodenum, head of pancreas and IVC to the left	Radical nephrectomy	Positive for ER, PR, SMA, WT1 and CK; negative for HMB-45 and S-100
4	Dell et al. [18]	66 y/M	Dysuria, frequency and recent episode of UTI H/O of HT, DM II and underwent appendectomy 50 years ago	4.2 × 2.6 cm	Unilocular, well-circumscribed cyst arising upper pole the left kidney Numerous calcifications on the walls and variably thin and thick septa. No solid components seen CT scan showed a poorly enhancing cystic lesion protruding into sinus	Laparoscopic Nephrectomy	Lining epithelial cells keratin and EMA +ve F VIII, CD34, and vimentin −ve
5	Dong et al. [19]	30 y/M	Intermittent right-flank pain and gross hematuria since 2 years	5 × 4.5 × 3.5-cm nephrectomy specimen with a 4.5 × 3 × 2-cm tumoral mass	Well-circumscribed, polycystic and heterogeneous mass with areas of calcifications arising from the upper pole of the right kidney No solid components evident	Laparoscopic Nephron sparing surgery	NA
6	Ozturk et al. [20]	59 y/F	Left flank pain and abdominal pain	22 × 10.9 × 8.2 cm	24.5 × 11.9 × 9.8 cm multilocular cystic renal mass in the left kidney with hypointense appearance in T1-weighted images and hyperintense in T2-weighted images, and a multicystic appearance in ureter projection	Transperitoneal radical nephroureterectomy	NA
7	Qiu et al. [21]	20 y/F	Left renal cyst for 1 year with pain in her left flank one month ago	–	–	Laparoscopic Partial Nephrectomy	NA
8	Abrol et al. [22]	27 y/F	Left loin pain and fever	Size not mentioned	Well-defined fluid attenuating round lesion with multiple daughter cysts in segments five and eight of the liver A similar-looking multi-septated cystic mass was seen in the upper pole of the left kidney There was no solid component or calcification	Partial Nephrectomy with surgery of liver cyst Diagnosis of hydatid cyst of the liver and left kidney was made	NA
9	Mohanty et al. [23]	45 y/F 25 y/F	Intermittent, non-colicky pain in right hypochondrium and lumbar region of 12-months duration Persistent dull aching pain in the right lumbar region for six months with no urinary complaints	6.6 × 6.5 × 4.4 cm 6.5 × 6.0 × 4.2 cm	Cystic mass with fine internal septations and delayed enhancement of the wall, involving the mid-pole of the right kidney compressing the pelvis and upper ureter Non-enhancing multi-locular cystic mass involving upper pole of the right kidney extending into the middle part, with no solid component. No vascular invasion, ascites or lymphadenopathy was seen	Right nephrectomy and cholecystectomy was performed Radical nephrectomy	NA NA
10	Wilkinsen et al. [9] Case series of 6	35/M 39/F 60/F 62/F 53/F 65/F	Loin pain with hematuria and Recurrent urinary tract infection	Avg size 8.1 cm		Laparoscopic radical nephrectomy with case 2 underwent Open nephrectomy
11	Index case	29/F	Left Flank mass and pain	13 × 13 × 4.5 cm	Predominantly cystic, with diffusely distributed, multiple thickened, faint contrast-enhancing internal septations, and loculations with areas of linear calcification anteriorly was identified consistent with Bosnaik IV type cyst and radiological impression of cystic renal cell carcinoma	Open radical nephrectomy	CK +ve in the cyst lining epithelium ER, PR, SMA, desmin, vimentin +ve in the stromal cells and calretinin negative

NA not available, UTI urinary tract infection, H/O history of, HT hypertension, DM diabetes mellitus