RT Journal Article
SR Electronic
T1 Human epidermal growth factor receptor-2 (HER2) is a potential therapeutic target in extramammary Paget’s disease of the vulva
JF International Journal of Gynecologic Cancer
JO Int J Gynecol Cancer
FD BMJ Publishing Group Ltd
SP 1672
OP 1677
DO 10.1136/ijgc-2020-001771
VO 30
IS 11
A1 Michele Bartoletti
A1 Roberta Mazzeo
A1 Marco De Scordilli
A1 Anna Del Fabro
A1 Maria Grazia Vitale
A1 Lucia Bortot
A1 Milena Sabrina Nicoloso
A1 Serena Corsetti
A1 Marta Bonotto
A1 Simona Scalone
A1 Giorgio Giorda
A1 Roberto Sorio
A1 Claudia Andreetta
A1 Maria Luisa Meacci
A1 Rocco De Vivo
A1 Gianpiero Fasola
A1 Francesco Sopracordevole
A1 Fabio Puglisi
YR 2020
UL http://ijgc.bmj.com/content/30/11/1672.abstract
AB Background Invasive vulvar Paget’s disease with over-expression of the human epidermal growth factor receptor 2 (HER2) protein is potentially suitable for targeted therapy, especially in a metastatic setting where no effective treatments are available.Methods Four consecutive patients with HER2 positive advanced vulvar Paget’s disease, treated with weekly trastuzumab (loading dose 4 mg/kg, then 2 mg/kg) and paclitaxel (80 mg/m2) followed by 3-weekly trastuzumab maintenance (6 mg/kg), are reported.Results Median age and follow-up of patients were 62.5 years (45–74) and 16 months (6-54), respectively. Complete or partial responses were observed in all patients. Median time to response was 3 months (range 2–4), while median duration of response was 10 months (range 2–34). Case 1 presented with pulmonary and lymph nodes involvement. She experienced a radiological complete response after 24 treatment administrations, and a progression-free survival of 36 months. At disease progression, treatment re-challenge achieved partial response. She is currently receiving treatment with trastuzumab–emtansine. Case 2 was a 74-year-old woman who developed pulmonary metastasis after first-line cisplatin treatment. She had a partial response and a progression-free survival of 10 months. Case 3 had inguinal and para-aortic lymphadenopathy in complete response after 18 treatment administrations. She developed brain metastasis while receiving trastuzumab maintenance. Case 4 was treated for locally advanced disease and experienced a subjective benefit with relief in perineal pain and itching. No unexpected treatment-related side effects were reported.Conclusions Advanced vulvar Paget’s disease is a rare disorder and no standard treatment is available. In the sub-group of HER2 positive disease, weekly paclitaxel–trastuzumab appears to be active and safe, and may be considered a therapeutic option in these patients.