Abstract

This 39-year-old Korean woman has shown typical features of the von Recklinghausen’s neurofibromatosis, which is associated with a malignant schwannoma on the anti-mesenteric border of the jejunum. The presented case is characterized by the progressive generalized brown-pigment deposits over the skin and multiple cutaneous varying indurative masses, which have started to develop since about 10 years. Additionally, considerable deformities and changes in the skeletal system were demonstrated. The discussion about the clinicopathologic characteristics of the case, with literature review is described.