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

From: Clinical features and treatment of vulvar Merkel cell carcinoma: a systematic review

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