Table 3 Summary of Clinical Presentation, Treatment, and Outcome of Vulvar Merkel Cell Carcinoma Cases

Bottles et al. 1984 [27]

73

Left labia majora.

Minute ulcer w/chronic ulceration

Initial: Testosterone + hydrocortisone cream to heal initial ulcer.

10 months, 3 weeks: Vulvectomy + Left Inguinal lymphadenectomy

9 Months: Local raised, nodular, erythematous tumor 3 x 2 cm + Left Inguinal LN metastases

11 months (11 days post operation): death due to acute MI + cardiopulmonary failure. Inguinal and paraaortic nodes, bone, liver, pulmonary vessel metastases.

Copeland et al. 1985 [26]

59

Left labium majus 6 x 8 cm

18 month history of painful lump + Local tumor + Left Inguinal LN metastases

Initial: Left hemivulvectomy + lymphadenectomy + Radiotherapy

8 months: Vulvar lesion excision.

8 months: Vulvar + several pulmonary metastases.

12 months: Death

Husseinzadeh et al. 1988 [25]

47

Right labium majus + vaginal introit. 4.2 x 3 cm

3 month history of right labial/groin swelling with brown vaginal discharge and pain on sitting. Local tumor + bilateral inguinal LN metastases

Initial: Vulvectomy + Bilateral lymphadenectomy + Radiotherapy

3 months: Excision + Chemotherapy

3 months: right thigh nodule, forehead nodule, single nodular lesion in left hilar region.

6 months: Death. Autopsy: hilar, lung, liver, pancreas metastases.

Chandeying et al. 1989 [24]

28

Right labium majus 4 cm

1 month history of painless lump. Local tumor + bilateral inguinal LN metastases

Initial: Vulvectomy + bilateral lymphadenectomy + radiotherapy

3 months: Right leg pain improved with symptomatic treatment.

4 months out: Alive. No subsequent follow up.

Loret de Mola et al. 1993 [23]

28

Left fourchette 1.5 x 2 cm

3 month history of Vulvar growth and irritation. Local tumor

Initial: local excision.

2 months: Wide local excision + left inguinal lymphadenectomy

8 months: chemotherapy

8 months: liver metastases.

20 months: Death.

Chen 1994 [22]

68

Left paraclitoral 3 x 2.5 cm

1 month history of mass. Local tumor.

Initial: Local excision

10 months: Chemotherapy.

9 months: bilateral Inguinal LN and liver metastases. 10 months = Vulva, scalp, bone and paraaortic LN. 17 months: Death.

Scurry et al. 1996 [21]

68

Left labium minus + fourchette 4 x 3 cm.

5 month history of painless lump with rapid growth in last 2 weeks. Local tumor + overlying discolored purplish skin. bilateral inguinal LN metastases

Initial: Vulvectomy + bilateral inguinal and Left pelvic Lymphadenectomy 2 months: Radiotherapy

Residual pelvic nodes post treatment.

2 months: para aortic LN.

5 months: Alive with residual disease.

Gil et al. 1997 [19]

74

Right labium majus 9 cm

3–4 month history of local tumor

Initial: Wide Local excision

13 months: free of disease

Fawzi et al. 1997 [20]

78

Right vulvar mass 5.5 x 4 cm

1 month history of perineal itching and discomfort. Pulmonary LN metastases.

Initial: Radical vulvectomy + bilateral inguinal LN dissection

20 days postoperative: break down of right groin site and subsequent death due to bleeding. No autopsy.

Hierro et al. 2000 [18]

79

Left labium minus 2.5 cm

Local tumor

Initial: local excision.

2 months: Radiotherapy

2 months local recurrence and regional LN metastases.

10 months: Death

Nuciforo et al. 2004 [17]

62

Right labia majora 20 mm

Local painful tumor.

Initial: local excision.

3 months: Radical vulvectomy + Radiotherapy.

3 months: bilateral inguinal LN metastases.

11 months: abdominal and mediastinal LN.

19 months: Alive with Several abdominal and thoracic metastases.

Khoury et al. 2005 [16]

49

Right vulvar mass 2 cm

Spontaneously ruptured Bartholin’s gland abscess with small induration at the site.

Initial: Drained abscess + wide local excision + bilateral LN dissection + Radiation therapy

24 months: Alive with no evidence of recurrence.

Pawar et al. 2005 [15]

35

Left labium majus 4 x 6 cm

One week history of painful swelling of the vulva + purulent discharge + LN mass

Initial: Drained abscess + antibiotics + partial excision

No follow up, patient planned to receive radiotherapy in her home country.

Mohit et al. 2009 [14]

50

Left labia majora 3–4 cm

3 month history of palpable mass.

Initial: local excision

2 months: Radiotherapy

2 months, 3 weeks: radical vulvectomy

9 months: Chemotherapy

2 months: Recurrent mass 10 x 12 cm w/spontaneously bleeding ulcerations

9 months: left hip pain

10 months: no evidence of metastases

11 months: death due to Pulmonary embolism secondary to DVT of LLE.

Sheikh et al. 2010 [13]

63

Right labium majus 5 x 7 cm

Post menopausal bleeding with fungating primary lesion.

Initial: wide local excision.

2 months: local + distant recurrence with multiple firm inguinal LN bilaterally + death before follow up treatment

Iavazzo et al. 2011 [12]

63

Left Labium 9 cm

6 month history of pruritus treated w/corticosteroid cream. 5 cm inguinal LN metastases.

Initial: radical vulvectomy + radiotherapy

No follow up

Winer et al. 2012 [11]

69

Right inguinal 3–4 cm

Patient noted Inguinal lesion.

Initial: Surgical excision

Future plans for adjuvant chemotherapy + radiotherapy

No follow up