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BAP1 ‐deficient and VE1 ‐negative atypical Spitz tumor
Author(s) -
Requena Celia,
Sanz Virginia,
Nagore Eduardo,
GarcíaCasado Zaida,
Rubio Luis,
Guillén Carlos,
Kutzner Heinz
Publication year - 2015
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.12519
Subject(s) - bap1 , spitz nevus , pathology , medicine , mutation , biology , nevus , cancer research , melanoma , gene , genetics
Atypical Spitz tumor with loss of BAP1 or Wiesner nevus is a peculiar variant of intradermal spitzoid melanocytic neoplasm composed of epithelioid melanocytes with a sheet‐like growth pattern, abundant infiltrating lymphocytes and rare or absent mitotic activity. This subset of atypical spitzoid tumors is characterized by the BRAF V600E mutation and loss of BAP1 expression. Recognition of these lesions is important because they can be a marker for a hereditary BAP1 ‐associated cancer syndrome. We present an unusual case of sporadic Wiesner nevus that had typical histopathologic features and a BAP1 but not a BRAF mutation. The biological significance of Wiesner nevus is controversial, and little is known about prognosis, particularly in atypical cases like this one.