Table 1 Reported cases of the urinary bladder PEComas in the English-Language Literature and their clinicopathologic features

1

Pan 2003 [11]

33/F

Symptomless

Left lateral inferior /4.0

W

U

0

No

U

HMB45 (+), SMA (+), vimentin (−), desmin (−),S-100 protein (−), Melan-A (−), pan-cytokeratin (KL-1) (−), ER (−), PR (−)

Partial cystectomy

ANEDx72

2

Kalyanasundaram 2005 [12]

19/F

Hematuria

Left lateral /3.0

I

High

Few

U

U

HMB45 (+), cytokeratin (−), S-100 (−), synaptophysin (−), vimentin (−), desmin (−), chromogranin A (−)

TURBT

U

3

Parfitt 2006 [13]

48/M

Lower abdominal pain and dysuria

Posterior mid-wall /3.0

I

High

0

Inconspicuous

U

HMB45 (+), Melan-A (+), SMA (+), desmin (+), c-kit (+), S-100 protein (−), pan-cytokeratin (AE1/AE3) (−), vimentin (−), MSA (−), myoglobin (−), CD31 (−), CD34 (−), WT-1 (−)

Partial cystectomy and partial small bowel resection with adjuvant INF-a immunotherapy

ANEDx48

4

Weinreb 2007 [14]

39/M

U

Dome/urachal remnant cyst /5.0

I, focally

High, focal

Isolated

Yes, focal

No

HMB45 (+), SMA (+), MSA (+), desmin (+), Melan-A (+), S-100 protein (+), MiTF (+), cyclin D1 (+)

Partial cystectomy

ANEDx8

5

Pianezza 2008 [15]

24/F

Chronic pelvic pain

Posterior/3.4

I, focally

Low to moderate

1

No

U

HMB45 (+), S-100 protein (+), H-caldesmon (+), actin (+), Melan-A (−), CD34 (−), A103 (−), pankeratin (−), calretinin (−), CD99 (−), ALK-1 (−), c-kit (−), desmin (−)

Partial cystectomy

ANEDx3

6

Sukov 2009 [16]

36/M

Hematuria

Anterior /4.8

I

Low to moderate

0

Yes, focal

U

HMB45 (+), SMA (+), tyrosinase (+), desmin (−), Melan-A (−)

Partial cystectomy

ANEDx10

7

37/M

Hematuria

Dome /U

W

Low to moderate

0

No

U

HMB45 (+), pan-cytokeratin (−), vimentin (−), CD56 (−), chromogranin A (−), Melan-A (−), S-100 protein (−), synaptophysin (−), SMA (−), high-molecular-weight cytokeratin (−), inhibin (−)

TURBT

ANEDx21

8

26/F

U

Anterior /5.0

W

Low

0

Yes (related to embolization)

U

HMB45 (+), SMA (+), vimentin (+), Melan-A (−), tyrosinase (+), S-100 protein (−), c-kit (−), MSA (−), cytokeratin (−)

Embolization and partial cystectomy

U

9

Huang 2011 [17]

23/M

Frequent micturition and odynuria

Left lateral /9.2

W

Low

0

U

U

HMB45 (+), vimentin (+), SMA (+), S-100 protein (−), Melan-A (−), synaptophysin (−), c-kit (−), CD34 (−), chromogranin A (−), cytokeratin 8 (−)

Tumorectomy and partial cystectomy

U

10

Chan 2011 [18]

42/M

Vague urethral pain

Right lateral /6.0

W

Low to moderate?

<1

No

U

HMB45 (+), S-100 protein (+), MiTF (+), SMA (+), calponin (+), vimentin (+), desmin (−), myogenin (−),TFE3 (−), pan-cytokeratin (AE1/AE3) (−), CD34 (−), ALK-1 (−), synaptophysin (−), c-kit (−), Ki67 (+, 1%)

TURBT and then partial cystectomy

U

11

Kyrou 2012 [19]

44/F

Dysmenorrhea

Left posterior /2.7

U

U

0

No

U

HMB45(+), S-100 protein (+), EMA (−), cytokeratin (−), desmin(−)

Partial colpectomy and cystectomy/ right ovariectomy/ pelvic lymphadenectomy

ANEDx30

12

Shringarpure 2012 [20]

39/M

Painless hematuria

Left vesicoureteric junction/3

U

U

U

U

U

HMB45 (+), Melan-A (+), SMA (+), vimentin (+), MSA (+), S-100 protein (+), cytokeratin (−), desmin(−)

TURBT

ANEDx3

13

Present case

16/F

Abdominal discomfort and micturition

Posterior/2.5

W

Low

0

No

No

HMB45 (+), SMA (+), MSA (+), desmin (−), H-caldesmon (+), Melan-A (−), ALK-1 (−), myogenin (−), EMA (−), pan-cytokeratin (AE1/AE3) (−), S-100 protein (−), Ki-67 (+, 2%)

TURBT

ANEDx13