Table 1

Summary of HER2 positive cases of vulvar Paget’s disease reported

CasePrevious therapiesSurgeryAnti-HER2 therapyResponsePFSMain ADRs
1Laser therapy; radiotherapy; imiquimod; photodynamic therapy
  1. Wide vulvo- perianal resection with packaging of a left colostomy

  2. Ileo-inguinal lymphadenectomy and enlargement of previous perineal resection

  1. Weekly paclitaxel plus weekly trastuzumab for 9 months, then maintenance with 3-weekly trastuzumab for 1 year

  2. Re-challenge with weekly paclitaxel and trastuzumab for 7 months, then maintenance with 3-weekly trastuzumab for 7 months

  3. Trastuzumab emtansine for 2 months, ongoing

  1. CR

  2. PR

First-line therapy: 36 months
Second-line therapy: 14 months
G3: neutropenia
2Cisplatin; pelvic radiotherapy
  1. Simple vulvectomy

  2. Vulvar resection

  3. Excision of urethral relapse

  4. New resection of urethral meatus

Weekly paclitaxel plus weekly trastuzumab for 5 monthsPR10 MonthsG2: anemia, onychopathy.
3Laser vaporization; testosterone propionate
  1. Simple vulvectomy with positive margins

  2. Radical vulvectomy with the excision of perineum, perianus, and part of the gluteal muscle

  3. Excision of bulky inguino-femoral lymph nodes

  1. Weekly pacli­taxel for four total cycles plus weekly trastuzumab for 4 months, then 3-weekly trastuzumab for 11 months

  2. 3-Weekly trastuzumab and carboplatin

1. CR than PD (brain) during trastuzumab12 Months until encephalic PD
16 Months until liver and bone PD
G1: fatigue, constipation, nausea, anemia and alopecia
G2: neurotoxicity
4Steroids, hyaluronic acid, tocopherol, anti-fungal, for vulvar lichen
Topical treatment with imiquimod (VPD)
  1. Laser vaporization of the anal and perianal mucosa and a simple vulvectomy with skin grafts, with two large triangular flaps bilaterally (V-Y plastic)

  2. Skinning vulvectomy of the anterior hemi-vulva with plastic reconstruction and perianal/anal excision

  1. 3-weekly trastuzumab

  2. Weekly paclitaxel and 3-weekly trastuzumab

Objective local response and subjective relief in symptomsNot evaluableG2: neurotoxicity
  • ADR, adverse drug reaction; CR, complete response; G, toxicities grade according to NCI CTCAE v4.0; HER2, human epidermal growth factor receptor 2 ; PD, progression disease; PFS, progression free survival defined as the time from treatment start to date of radiological or clinical progression; PR, partial response.