Clinical Characteristics of an Italian Patient Population with Advanced BCC and Real-Life Evaluation of Hedgehog Pathway Inhibitor Safety and Effectiveness.


Journal

Dermatology (Basel, Switzerland)
ISSN: 1421-9832
Titre abrégé: Dermatology
Pays: Switzerland
ID NLM: 9203244

Informations de publication

Date de publication:
2023
Historique:
received: 01 03 2023
accepted: 22 05 2023
medline: 7 12 2023
pubmed: 14 6 2023
entrez: 13 6 2023
Statut: ppublish

Résumé

Advanced basal cell carcinoma (aBCC) represents a complex and clinically heterogeneous group of lesions for which curative surgery and/or radiotherapy is unlikely. Systemic therapy with hedgehog pathway inhibitors (HHIs) changed the treatment landscape for this complex patient population. The aims of the present study are to describe the clinical characteristics of a real-life Italian cohort diagnosed with aBCC and to investigate effectiveness and safety of HHI. A multicenter observational study was performed by twelve Italian centers in the period January 1, 2016 - October 15, 2022. Patients aged ≥18 years and diagnosed with aBCC (locally advanced [laBCC] and metastatic BCC [mBCC]) were eligible for the study. Methods for investigating tumor response to HHI included clinical and dermatoscopic evaluation, radiological imaging, and histopathology. For HHI safety assessment, therapy-related adverse events (AEs) were reported and graded according to the Common Terminology Criteria for Adverse Events (CTCAE) version 5.0. We enrolled 178 patients under treatment with HHI: 126 (70.8%) and 52 patients (29.2%) received sonidegib and vismodegib, respectively. Comprehensive data on HHI effectiveness and disease outcome were available for 132 (74.1%) of 178 patients: 129 patients had a diagnosis of laBCC (n = 84, sonidegib; n = 45, vismodegib) and 3 patients of mBCC (n = 2, vismodegib; n = 1, sonidegib, off-label). Objective response rate was 76.7% (95% confidence interval [CI]: 82.3-68.7) and 33.3% (95% CI: 88.2-1.7) for laBCC (complete response [CR]: 43/129; PR: 56/129) and mBCC (CR: 0/3; PR: 1/3), respectively. High-risk aBCC histopathological subtypes and occurrence of >2 therapy-related AEs were significantly associated with nonresponse to HHI therapy ([OR: 2.61; 95% CI: 1.09-6.05; p: 0.03] and [OR: 2.74; 95% CI: 1.03-7.9; p: 0.04]), respectively. Majority of our cohort (54.5%) developed at least 1 therapy-related AE, most of which were mild-moderate in severity. Our results demonstrate the effectiveness and safety profile of HHI and confirm the reproducibility of pivotal trial results in real-life clinical setting.

Sections du résumé

BACKGROUND BACKGROUND
Advanced basal cell carcinoma (aBCC) represents a complex and clinically heterogeneous group of lesions for which curative surgery and/or radiotherapy is unlikely. Systemic therapy with hedgehog pathway inhibitors (HHIs) changed the treatment landscape for this complex patient population.
OBJECTIVES OBJECTIVE
The aims of the present study are to describe the clinical characteristics of a real-life Italian cohort diagnosed with aBCC and to investigate effectiveness and safety of HHI.
METHODS METHODS
A multicenter observational study was performed by twelve Italian centers in the period January 1, 2016 - October 15, 2022. Patients aged ≥18 years and diagnosed with aBCC (locally advanced [laBCC] and metastatic BCC [mBCC]) were eligible for the study. Methods for investigating tumor response to HHI included clinical and dermatoscopic evaluation, radiological imaging, and histopathology. For HHI safety assessment, therapy-related adverse events (AEs) were reported and graded according to the Common Terminology Criteria for Adverse Events (CTCAE) version 5.0.
RESULTS RESULTS
We enrolled 178 patients under treatment with HHI: 126 (70.8%) and 52 patients (29.2%) received sonidegib and vismodegib, respectively. Comprehensive data on HHI effectiveness and disease outcome were available for 132 (74.1%) of 178 patients: 129 patients had a diagnosis of laBCC (n = 84, sonidegib; n = 45, vismodegib) and 3 patients of mBCC (n = 2, vismodegib; n = 1, sonidegib, off-label). Objective response rate was 76.7% (95% confidence interval [CI]: 82.3-68.7) and 33.3% (95% CI: 88.2-1.7) for laBCC (complete response [CR]: 43/129; PR: 56/129) and mBCC (CR: 0/3; PR: 1/3), respectively. High-risk aBCC histopathological subtypes and occurrence of >2 therapy-related AEs were significantly associated with nonresponse to HHI therapy ([OR: 2.61; 95% CI: 1.09-6.05; p: 0.03] and [OR: 2.74; 95% CI: 1.03-7.9; p: 0.04]), respectively. Majority of our cohort (54.5%) developed at least 1 therapy-related AE, most of which were mild-moderate in severity.
CONCLUSIONS CONCLUSIONS
Our results demonstrate the effectiveness and safety profile of HHI and confirm the reproducibility of pivotal trial results in real-life clinical setting.

Identifiants

pubmed: 37311439
pii: 000531280
doi: 10.1159/000531280
doi:

Substances chimiques

sonidegib 0RLU3VTK5M
HhAntag691 0
Hedgehog Proteins 0
Anilides 0
Antineoplastic Agents 0

Types de publication

Multicenter Study Observational Study Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

868-876

Informations de copyright

© 2023 S. Karger AG, Basel.

Auteurs

Maria Mannino (M)

UOC di Dermatologia, Dipartimento di Scienze Mediche e Chirurgiche Addominali ed Endrocrino Metaboliche, Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy, mariamannino04@yahoo.it.
Dermatologia, Università Cattolica del Sacro Cuore, Rome, Italy, mariamannino04@yahoo.it.

Alfredo Piccerillo (A)

Dermatologia, Università Cattolica del Sacro Cuore, Rome, Italy.

Gabriella Fabbrocini (G)

Section of Dermatology - Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.

Pietro Quaglino (P)

Dermatologic Clinic, Department of Medical Sciences, University of Turin, Turin, Italy.

Giuseppe Argenziano (G)

Dermatology Unit, University of Campania "Luigi Vanvitelli", Naples, Italy.

Emi Dika (E)

Dermatology - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.
Department of Experimental, Diagnostic and Specialty Medicine (DIMES) Alma Mater Studiorum University of Bologna, Bologna, Italy.

Paolo Antonio Ascierto (PA)

Melanoma, Cancer Immunotherapy and Development Therapeutics, Istituto Nazionale Tumori Istituto di Ricovero e Cura a Carattere Scientifico Fondazione Pascale, Naples, Italy.

Giovanni Pellacani (G)

Department of Dermatology, University of Rome La Sapienza, Rome, Italy.

Caterina Longo (C)

Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
Azienda Sanitaria Locale, IRCCS di Reggio Emilia, Centro Oncologico ad Alta Tecnologia Diagnostica-Dermatologia, Reggio Emilia, Italy.

Maria Concetta Fargnoli (MC)

Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.

Luca Bianchi (L)

Dermatology Unit, Tor Vergata University Hospital, Rome, Italy.

Piergiacomo Calzavara-Pinton (P)

Department of Dermatology, ASST-Spedali Civili, University of Brescia, Brescia, Italy.

Iris Zalaudek (I)

Department of Dermatology, University of Trieste, Trieste, Italy.

Paolo Fava (P)

Dermatologic Clinic, Department of Medical Sciences, University of Turin, Turin, Italy.

Massimiliano Scalvenzi (M)

Section of Dermatology - Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy.

Enrico Bocchino (E)

UOC di Dermatologia, Dipartimento di Scienze Mediche e Chirurgiche Addominali ed Endrocrino Metaboliche, Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy.
Dermatologia, Università Cattolica del Sacro Cuore, Rome, Italy.

Alessandro Di Stefani (A)

UOC di Dermatologia, Dipartimento di Scienze Mediche e Chirurgiche Addominali ed Endrocrino Metaboliche, Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy.
Dermatologia, Università Cattolica del Sacro Cuore, Rome, Italy.

Ketty Peris (K)

UOC di Dermatologia, Dipartimento di Scienze Mediche e Chirurgiche Addominali ed Endrocrino Metaboliche, Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy.
Dermatologia, Università Cattolica del Sacro Cuore, Rome, Italy.

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