PMID- 31233225 OWN - NLM STAT- MEDLINE DCOM- 20200324 LR - 20231213 IS - 1600-0560 (Electronic) IS - 0303-6987 (Linking) VI - 46 IP - 12 DP - 2019 Dec TI - BRCA1-associated protein (BAP1)-inactivated melanocytic tumors. PG - 965-972 LID - 10.1111/cup.13530 [doi] AB - Although discussed using variable terminology, cutaneous BRCA1-associated protein (BAP1)-inactivated melanocytic tumor (BIMT) has been considered a discrete diagnostic entity since 2011. Here, we review the initial genomic studies that identified these distinct melanocytic tumors and the clinical and histopathological features that define these tumors. These epithelioid, predominantly dermal, and melanocytic tumors present as erythematous nodules and histopathologically have features that may overlap with Spitz nevi and nevoid melanoma. There is no sex predilection, and cutaneous BIMTs can appear at any age; however, in most familial (germline mutant) cases patients have multiple cutaneous tumors with a first diagnosis in the second or third decade of life; ocular melanoma and other tumors are increasingly identified in these kindreds with germline BAP1 mutation. These tumors have been described with a myriad of terms including: Wiesner nevus, nevoid melanoma-like melanocytic proliferation (NEMMP), BAP1 mutant Spitz nevus, BAP1 mutant nevoid melanoma, cutaneous BAPoma, and most recently cutaneous BIMT. CI - (c) 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. FAU - Zhang, Arianna J AU - Zhang AJ AUID- ORCID: 0000-0002-4569-0557 AD - Pathology Service, Massachusetts General Hospital, Boston, Massachusetts. FAU - Rush, Patrick S AU - Rush PS AD - Dominion Pathology Associates, Roanoke, Virginia. FAU - Tsao, Hensin AU - Tsao H AD - School of Medicine, Harvard University, Boston, Massachusetts. AD - Department of Dermatology, Massachusetts General Hospital, Boston, Massachusetts. FAU - Duncan, Lyn M AU - Duncan LM AUID- ORCID: 0000-0003-3732-8256 AD - Pathology Service, Massachusetts General Hospital, Boston, Massachusetts. AD - School of Medicine, Harvard University, Boston, Massachusetts. LA - eng PT - Journal Article PT - Review DEP - 20190711 PL - United States TA - J Cutan Pathol JT - Journal of cutaneous pathology JID - 0425124 RN - 0 (BAP1 protein, human) RN - 0 (BRCA1 Protein) RN - 0 (BRCA1 protein, human) RN - 0 (Tumor Suppressor Proteins) RN - EC 3.4.19.12 (Ubiquitin Thiolesterase) SB - IM MH - BRCA1 Protein/*metabolism MH - Cell Proliferation MH - Eye Neoplasms/*genetics/pathology MH - Female MH - Genetic Predisposition to Disease MH - Germ-Line Mutation/genetics MH - Humans MH - Male MH - Melanocytes/*pathology MH - Melanoma/*genetics/pathology MH - Nevus, Epithelioid and Spindle Cell/*genetics/pathology MH - Skin/pathology MH - Skin Neoplasms/*genetics/pathology MH - Tumor Suppressor Proteins/*metabolism MH - Ubiquitin Thiolesterase/*metabolism MH - Melanoma, Cutaneous Malignant OTO - NOTNLM OT - BAP1-inactivated naevus/melanocytoma OT - BAP1 OT - BIMT OT - Spitz EDAT- 2019/06/25 06:00 MHDA- 2020/03/25 06:00 CRDT- 2019/06/25 06:00 PHST- 2019/02/25 00:00 [received] PHST- 2019/06/18 00:00 [revised] PHST- 2019/06/20 00:00 [accepted] PHST- 2019/06/25 06:00 [pubmed] PHST- 2020/03/25 06:00 [medline] PHST- 2019/06/25 06:00 [entrez] AID - 10.1111/cup.13530 [doi] PST - ppublish SO - J Cutan Pathol. 2019 Dec;46(12):965-972. doi: 10.1111/cup.13530. Epub 2019 Jul 11.