Table 3.
Summary of Clinical Presentation, Treatment, and Outcome of Vulvar Merkel Cell Carcinoma Cases
| Case | Age | Location/Size | Presentation | Treatment | Outcome + Survival |
|---|---|---|---|---|---|
| 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 |