Primary cutaneous CD8-positive medium/large cell pleomorphic T-cell lymphoma of the pinna - Various therapeutic concepts - A case report

Primary cutaneous T-cell lymphomas (CTCL) are mycosis fungoides, Sezary syndrome and other Fare forms. We report about a 56-year old patient with primary cutaneous, CD8-positive, medium/large cell pleomorphic T-cell lymphoma of the pinna. This histological variant is rare (3 % of all CTCL). There are scarce reports regarding primary manifestation on the ear. No standardised therapeutic concept has yet been established for rare forms of CTCL. Therapeutic concepts had to be adjusted several times according to the course of the disease due to progressive manifestation of the lymphoma. After application of a combination therapy with retinoid, interferon a Za and PUVA and subsequently localised photodynamic therapy a localised photon beam radiotherapy was carried out on the ear (43,4 Gy). After radiotherapy a complete remission could be observed, and it now lasts 15 months. Radiotherapy was therefore the treatment modality that was most effective, showed only minor side effects and was cost efficient.