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Wide, local excision with clear margins, of 1-2 cm, with or without lymph node dissection is the standard treatment.7 Makela I et al i .8 described in 2021 the first CAA case with a BRCAness genetic background and a I PALB2 i biallelic inactivation at the tumour level. There was neither clinical nor radiologic/pathology findings favouring male breast cancer as a primary tumour. About 200 cases are reported to date with less than 30 metastatic cases.1 Partner and localizer of BRCA2 ( I PALB2 i ) is a tumour suppressor gene mainly involved in breast adenocarcinoma predisposition. [Extracted from the article]

Patient is still in complete remission of her SUSCC after 11 years and did not show any other tumour of the I BAP1 i -TPDS whereas several I BAP1 i -inactivated melanocytic tumour (BIMT) were treated in her children. (d) Immunohistochemical study: Loss of nuclear BAP1 expression in tumour cells but not in adjacent uninvolved ones (C4, monoclonal, mouse, Diagomics). gl I BAP1 i is a tumour suppressor gene, located on chromosome 3 (3p21.1). Here, we report a case of unusual SUSCC in a patient carrier of a germline I BAP1 i pathogenic variant, therefore, extending the spectrum of skin tumours in this Tumour Predisposing Syndrome ( I BAP1 i -TPDS). [Extracted from the article]