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 ijgc-2020-001771
DO 10.1136/ijgc-2020-001771
A1 Bartoletti, Michele
A1 Mazzeo, Roberta
A1 De Scordilli, Marco
A1 Del Fabro, Anna
A1 Vitale, Maria Grazia
A1 Bortot, Lucia
A1 Nicoloso, Milena Sabrina
A1 Corsetti, Serena
A1 Bonotto, Marta
A1 Scalone, Simona
A1 Giorda, Giorgio
A1 Sorio, Roberto
A1 Andreetta, Claudia
A1 Meacci, Maria Luisa
A1 De Vivo, Rocco
A1 Fasola, Gianpiero
A1 Sopracordevole, Francesco
A1 Puglisi, Fabio
YR 2020
UL http://ijgc.bmj.com/content/early/2020/09/29/ijgc-2020-001771.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.