Pleomorphic T-cell lymphoma with chondropathia tuberosa; a case report and review

Pleomorphic T-cell-lymphoma (anaplastic IgA-plasma cell tumor) belongs to the group of malignant non-Hodgkin's lymphomas (NHL). The histological and immunophenotypical subtypes differ with genetic and environmental etiologic factors. Lymphomas arise from the clonal proliferation of precursor cells within lymphoid organs with acquired chromosomal abnormalities. Approximately 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 pleomorphic T-cell-lymphoma imitating a Chondropathia tuberosa (Tietze's syndrome): The tumor appeared with a tender tumescence over the sternum and a painful swollen left sterno-clavicular joint--as a rule a typical sign for Tietze's syndrome. Only sternal puncture followed by immune histology confirmed an anaplastic IgA-plasma cell tumor. The primary tumor, and later on an osteolysis of cervical vertebrae I-III with a complete destruction of the axis and an affection of the dens atlantis could not be detected by radiographic examinations. Moreover, this tumor infestation could only be depicted by the magnet resonance imaging and the computed tomography. Furthermore, other results of our report are the very rare manifestation of a T-cell lymphoma as a pedicled tumor near the pancreas or of pancreatic origin and the excellent result of an autologous stem cell transplantation. Reviewing the literature, we want to discuss the present scientific and clinical standards of diagnosis, progress and treatment of Chondropathia tuberosa and T-cell lymphoma, and we want to point out some new aspects of both diseases.