Relapse of primary extranodal marginal-zone B-cell lymphoma of the dura mater

Abstract

Lymphoedema manifesting with sudden onset of swelling of one whole leg suggests proximal obstruction of lymphatic drainage. In the developed world, lymphoedema due to venous or lymphatic obstruction in the pelvic area commonly results from damage or removal of regional lymph nodes through surgery or radiotherapy. By contrast, the most common cause worldwide is filariasis (the direct infestation of lymph nodes by the parasite Wuchereria bancrofti). Cancer rarely presents with lymphoedema except in advanced examples that present late, such as prostate cancer, in which venous obstruction might coexist. We report a rare case of extranodal marginal-zone B-cell lymphoma of the dura mater, which relapsed 28 years later in the form of a massive unilateral lymphoedema of the left leg. A 52-year-old man complained of an enormous reduction in mobility caused by increasing swelling of the left leg over 4 weeks. 28 years previously (1971), a reticulosarcoma of the dura mater was diagnosed and the cerebral tumour had a complete resection and radiated focally. 11 years after the initial diagnosis, a relapse occurred in a left-sided cervical lymph node; this was treated by resection and radiation. Staging investigations did not show any further disease. No overt recurrence of a lymphoma was detected until the patient re-presented 17 years later with lymphoedema of the leg. Physical examination showed an enlarged girth of the left thigh (79 cm) and of the left lower leg (52 cm),