Liubov K, Irena EB., Michal M, et al. - Researchers reported a rare case of cutaneous Primary effusion lymphoma (PEL) with an unusual intravascular presentation, combined with Kaposi sarcoma including the skin, lung, and gastrointestinal tract. An individual 67-year-old man who presented with multiple cutaneous tumors and mass in the left lung. Autopsy findings shown in addition to the cutaneous lesions, tumors in the left lung and gastrointestinal mucosa were identified, and no effusions in the body cavities were noticed. The biopsy from the cutaneous lesions, pulmonary, and intestinal tumors showed histological and immunohistochemical characteristics of Kaposi sarcoma. Moreover, the skin biopsy specimens included a diffuse infiltrate made up of large pleomorphic cells, with focal intravascular growth that was negative for pan B-cell markers, weakly positive for CD38 and CD138 however, expressed CD3, HHV-8, and Epstein–Barr virus. Molecular genetic studies in this specimen showed monoclonal rearrangements of the IgH gene. The diagnosis of PEL, a solid variant, was made. Overall, it was found that next-generation sequencing analysis of the tumorous and normal tissue detected a pathogenic germline mutation of the FAM175A gene and somatic mutations in BRCA2 and RAD51B, and INPP4B and RICTOR.