Pleomorphic T-cell lymphoma with chondropathia tuberosa; A case report andreview

Pleomorphic T-cell-lymphoma (anaplastic IgA-plasma cell tumor) belongs to t he group of malignant non-Hodgkin's lymphomas (NHL). The histological and i mmunophenotypical subtypes differ with genetic and environmental etiologic factors. Lymphomas arise from the clonal proliferation of precursor cells w ithin lymphoid organs with acquired chromosomal abnormalities. Approximatel y 5% of all,primary malignant bone tumors are NHL, the majority of diffuse large B-cell type. Our case history can be regarded as the first published in English language reporting on a pheomorphic T-cell-lymphoma imitating a Chondropathia tuberosa (Tietze's syndrome): The tumor appeared with a tende r tumescence over the sternum and a painful swollen left sterno-clavicular joint - as a rule a typical sign for Tietze's syndrome. Only sternal punctu re followed by immune histology confirmed an anaplastic IgA-plasma cell tum or. The primary tumor, and later on an osteolysis of cervical vertebrae I-I II with a complete destruction of the axis and an affection of the dens atl antis could not be detected by radiographic examinations. Moreover, this tu mor infestation could only be depicted by the magnet resonance imaging and the computed tomography. Furthermore, other results of our report are the v ery rare manifestation of a T-cell lymphoma as a pedicled tumor near the pa ncreas or of pancreatic origin and the excellent result of an autologous st em cell transplantation. Reviewing the literature, we want to discuss the p resent scientific and clinical standards of diagnosis, progress and treatme nt of Chondropathia tuberosa and T-cell lymphoma, and we want to point out some new aspects of both diseases.