Article

4 Iconography
Access to the text (HTML) Access to the text (HTML)
PDF Access to the PDF text
Advertising


Access to the full text of this article requires a subscription.
  • If you are a subscriber, please sign in 'My Account' at the top right of the screen.

  • If you want to subscribe to this journal, see our rates



Journal of the American Academy of Dermatology
Volume 80, n° 6
pages 1585-1593 (juin 2019)
Doi : 10.1016/j.jaad.2018.09.014
accepted : 6 September 2018
Original Articles

Clinical and dermoscopic features of cutaneous BAP1 -inactivated melanocytic tumors: Results of a multicenter case-control study by the International Dermoscopy Society
 

Oriol Yélamos, MD a, b, , Cristián Navarrete-Dechent, MD a, c, Michael A. Marchetti, MD a, Tova Rogers, MD a, Zoe Apalla, MD d, Philippe Bahadoran, MD e, Nuria Blázquez-Sánchez, MD, PhD f, Klaus Busam, MD g, Cristina Carrera, MD b, Stephen W. Dusza, DrPH a, Arnaud de la Fouchardière, MD h, Gerardo Ferrara, MD i, Pedram Gerami, MD j, Harald Kittler, MD k, Aimilios Lallas, MD d, Josep Malvehy, MD b, José F. Millán-Cayetano, MD f, Kelly C. Nelson, MD l, Victor Li Quan j, Susana Puig, MD b, Howard Stevens, MD m, Luc Thomas, MD, PhD n, Ashfaq A. Marghoob, MD a
a Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York 
g Pathology Department, Memorial Sloan Kettering Cancer Center, New York, New York 
b Dermatology Department, Hospital Clínic, Institut d'Investigacions Biomediques August Pi i Sunyer, Universitat de Barcelona, and CIBER de Enfermedades Raras, Instituto de Salud Carlos III, Barcelona, Spain 
c Department of Dermatology, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile 
d First Department of Dermatology, Aristotle University, Thessaloniki, Greece 
e Dermatology Department, Centre Hospitalier Universitaire de Nice, Nice, France 
f Dermatology Department, Hospital Costa del Sol, Marbella, Spain 
h Département de Biopathologie, Centre Léon Bérard, Lyon, France 
i Anatomic Pathology Unit, Hospital of Macerata, Macerata, Italy 
j Dermatology Department, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 
k Department of Dermatology, Medical University of Vienna, Vienna, Austria 
l Dermatology Department, The University of Texas MD Anderson Cancer Center, Houston, Texas 
m Skin Care Network, Barnet, London, United Kingdom 
n Department of Dermatology, Lyon 1 University, Centre Hospitalier Lyon Sud and Lyon's Cancer Research Center INSERM U1052 – CNRS UMR5286, Lyon, France 

Reprint requests: Oriol Yélamos, MD, Dermatology Service, Memorial Sloan Kettering Cancer Center, 16 E 60th St, 4th fl, New York, NY 10022.Dermatology ServiceMemorial Sloan Kettering Cancer Center16 E 60th St, 4th flNew YorkNY10022
Abstract
Background

Multiple BRCA1-associated protein 1 (BAP1 )-inactivated melanocytic tumors (BIMTs) have been associated with a familial cancer syndrome involving germline mutations in BAP1.

Objectives

We sought to describe the clinical and dermoscopic features of BIMTs.

Methods

This was a retrospective, multicenter, case-control study. Participating centers contributed clinical data, dermoscopic images, and histopathologic data of biopsy-proven BIMTs. We compared the dermoscopic features between BIMTs and control patients.

Results

The dataset consisted of 48 BIMTs from 31 patients (22 women; median age 37 years) and 80 control patients. Eleven patients had a BAP1 germline mutation. Clinically, most BIMTs presented as pink, dome-shaped papules (n = 24). Dermoscopically, we identified 5 patterns: structureless pink-to-tan with irregular eccentric dots/globules (n = 14, 29.8%); structureless pink-to-tan with peripheral vessels (n = 10, 21.3%); structureless pink-to-tan (n = 7, 14.9%); a network with raised, structureless, pink-to-tan areas (n = 7, 14.9%); and globular pattern (n = 4, 8.5%). The structureless with eccentric dots/globules pattern and network with raised structureless areas pattern were only identified in BIMT and were more common in patients with BAP1 germline mutations (P  < .0001 and P  = .001, respectively).

Limitations

Limitations included our small sample size, retrospective design, the absence of germline genetic testing in all patients, and inclusion bias toward more atypical-looking BIMTs.

Conclusions

Dome-shaped papules with pink-to-tan structureless areas and peripheral irregular dots/globules or network should raise the clinical suspicion for BIMT.

The full text of this article is available in PDF format.

Key words : atypical Spitzoid tumor, BAP1 , BAP1 -inactivated melanocytic tumors, dermoscopy, melanoma, Wiesner nevus



 Supported by the National Institutes of Health/National Cancer Institute Cancer Center support grant P30 CA008748 and Beca Excelencia Fundación Piel Sana.
 Dr Gerami has served as a consultant to DermTech and Castle Biosciences and has received honoraria. The other authors have no conflicts of interest to disclose.
 Presented at the 2018 American Academy of Dermatology Meeting (San Diego, CA, February 15-20, 2018) and the 2018 World Dermoscopy Congress (Thessaloniki, Greece, June 14-16, 2018).



© 2018  American Academy of Dermatology, Inc.@@#104156@@
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Close
Article Outline