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We report 3 cases of congenital multiple glomus tumors seen during the last 5 years. One of them showed autosomal dominant inheritance with male-to-male transmission. The remaining patients had no family history of similar lesions. The clinical and histopathological aspects of our patients support the recently described type 2 segmental manifestation of multiple glomus tumors. One of the cases showed associated multiple and giant trichilemmal cysts with a linear distribution in the scalp., (Copyright (R) 2000 S. Karger AG, Basel)

Malignant neoplasms from natural killer (NK) cells are characterized by their positivity for CD56 and absence of monoclonal TCR gene rearrangement. Recently, they have been classified into four main types (nasal and nasal-type NK cell lymphoma, aggressive NK cell leukemia/lymphoma, and blastoid NK cell leukemia/lymphoma), based on clinical features, racial predisposition, presence of azurophilic granules, immunophenotype and association with Epstein-Barr virus (EBV) infection. A 72-year-old Caucasian man presented with a malignant neoplasm comprised of blastoid cells without azurophilic granules in the Giemsa stain, with positivity for CD2, CD4, HLA-DR, CD45 and CD56, and negativity for CD3 (surface and cytoplasmic) and CD5. In situ hybridization for EBV and PCR analysis of rearrangement of the T cell receptor gene were negative. Based on these results, a diagnosis of blastoid NK cell lymphoma was made. In this case the first clinical manifestations were the cutaneous lesions, and, although the disease was already advanced at the diagnosis, the patient responded completely to the treatment and remains asymptomatic 14 months after diagnosis., (Copyright 2000 S. Karger AG, Basel.)