Francesco Sopracordevole 1 , Fabio Puglisi 2,3 , Michele Bartoletti 4,3 , Roberta Mazzeo 2,3 , Marco De Scordilli 2,3 , Anna Del Fabro 1 , Maria Grazia Vitale 2,5 , Lucia Bortot 2,5 , Milena Sabrina Nicoloso 6 , Serena Corsetti 3 , Marta Bonotto 5 , Simona Scalone 3 , Giorgio Giorda 1 , Roberto Sorio 3 , Claudia Andreetta 5 , Maria Luisa Meacci 7 , Rocco De Vivo 8 , Gianpiero Fasola 5 Show Affiliations »
Abstract
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. © IGCS and ESGO 2020. No commercial re-use. See rights and permissions. Published by BMJ.
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 . © IGCS and ESGO 2020. No commercial re-use. See rights and permissions. Published by BMJ.
Entities: Chemical
Disease
Gene
Species
Keywords:
extramammary; paget disease
Year: 2020
PMID： 32998859 DOI： 10.1136/ijgc-2020-001771
Source DB: PubMed Journal: Int J Gynecol Cancer ISSN： 1048-891X Impact factor: 3.437