Skip to main content

Table 1 Summary of the reported cases of Dermatofibrosarcoma Protuberans of the vulva with clinical, treatment and follow up information

From: Dermatofibrosarcoma protuberans of the vulva: margins assessment and reconstructive options – a report of two cases

Author

Cases

Age, years

Clinical presentation

Size, cm

Initial treatment

Outcome/follow up

Edelweiss and Malpica [1]

1

36

Right labium major mass

5

Excisional biopsy

LR at 12 months. AWD at 16 months

Edelweiss and Malpica [1]

2

57

Right labium major mass

3

Excisional biopsy

LR at 60 months, then WLE (2x) until negative margin. NED at 216 months.

Edelweiss and Malpica [1]

3

69

Mons pubis mass

3

Excisional biopsy + WLE

Dead of other causes at 144 months

Edelweiss and Malpica [1]

4

48

Left paraclitoral mass

1.2

Excisional biopsy + WLE

LR, then WLE. NED at 144 months.

Edelweiss and Malpica [1]

5

46

Right labium major pigmented lesion

4

Excisional biopsy + WLE (x2)

NED at 84 months.

Edelweiss and Malpica [1]

6

76

Right labium major and crural fold mass

15

Excisional biopsy + WLE

LR at 7 months, then WLE with negative margin. At 14 months, metastasis to chest wall. At 18 months, metastasis to hip and thigh. Death of the disease at 34 months.

Edelweiss and Malpica [1]

7

44

Left labium major mass

4

Excisional biopsy + WLE

NED at 53 months

Edelweiss and Malpica [1]

8

39

Right labium major mass

N/A

WLE

LR at 48 months. Then, WLE with positive margin. At 60 months, local recurrence. Then WLE with positive margin. Used Gleevec. NED at 72 months

Edelweiss and Malpica [1]

9

30

Right labium major and mons pubis mass

N/A

Excisional biopsy + WLE

LR at 11 months. Then, WLE. AWD at 24 months

Edelweiss and Malpica [1]

10

23

Right labium major mass

4

Excisional biopsy + WLE

NED at 2 months

Edelweiss and Malpica [1]

11

30

Left labium major mass

N/A

Excisional biopsy

LR at 60 months followed by WLE with positive margin. Then, partial vulvectomy with negative margin. NED at 96 months

Edelweiss and Malpica [1]

12

44

Right labium major mass

2.5

Excisional biopsy + radical vulvectomy

NED at 36 months

Edelweiss and Malpica [1]

13

58

Paraclitoral mass extending into vagina

4.8

WLE

NED at 3 months

Soltan [10]

14

83

Left labium major/minor nodule

5

Excisional biopsy

N/A

Agress et al. [11]

15

56

Left labium major

N/A

Excisional biopsy

LR, then WLE

Bock et al. [12]

16

52

Right mons pruritus

8

Excisional biopsy

LR, then WLE

Barnhill et al. [13]

17

42

Right vulva lateral to clitoris nodule

1

Excisional biopsy

LR, then WLE and hemivulvectomy

Leake et al. [14]

18

37

Left mons and labium major nodule

6.2

Excisional biopsy

LR, then wide radical excision

Leake et al. [14]

19

59

Right labium major nodule

5

Excisional biopsy

LR, then partial radical vulvectomy

Panidis et al. [15]

20

30

Right labium major nodule

2

WLE

LR, then radical vulvectomy

Aartsen and Albus-Lutter [16]

21

50

N/A

1.2

Radical vulvectomy

N/A

Karlen et al. [17]

22

36

Left labium major irritated lump

5

WLE

N/A

Nirenberg et al. [18]

23

41

Left labium major lump

8

WLE

N/A

Alverez-Canas [19]

24

58

Left labium major mass

3.2

WLE

N/A

Soergel et al. [20]

25

47

Left vulva mass + lung metastasis

3

Partial radical vulvectomy + chemotherapy

LR, then WLE + radiotherapy

Ghorbani et al. [21]

26

47

Left paraclitoral area

N/A

Hemivulvectomy

LR, then wide radical excision

Ghorbani et al. [21]

27

44

Left labium major mass

4

Wide radical excision

N/A

Ghorbani et al. [21]

28

66

Mons mass

1.5

WLE

N/A

Ghorbani et al. [21]

29

36

Right labium mass

5

Excsional biopsy

N/A

Moodley and Moodley [22]

30

39

Left labium major mass

12

WLE

N/A

Vanni et al. [23]

31

39

Inferior vulva/perineum mass

6

WLE

N/A

Kholova et al. [24]

32

31

Left labium major recurrent nodule

1.5

Excisional biopsy

LR, then WLE + radiotherapy

Ohlinger et al. [25]

33

36

Left vulva nodule

2.8

Excisional biopsy

LR, then WLE

Hancox et al. [26]

34

55

Right labium major mass

8

MMS

NED

Hammonds and Hendi [6]

35

59

Right labium major mass

4

MMS

NED

Doufekas et al. [3]

36

39

Left labium major nodule

N/A

Incisional biopsy

MMS with primary closure. NED at 3 years

Zizi-Sermpetzoglou et al. [27]

37

66

Mons pubis

N/A

WLE

NED

  1. N/A, not available; WLE, wide local excision; MMS, Mohs micrographic surgery; LR, local recurrence; AWD, alive with disease; AWOD, alive without disease; NED, no evidence of disease).