Extracutaneous Tumour Mass Following Bexarotene and Interferon Therapy of Primary Cutaneous CD30+ Large Cell Lymphoma

Abstract

) no prior or concurrent lymphomatoid papulosis (LyP), mycosis fungoides (MF), or other type of cutaneous lymphoma or extracutaneous localization at presentation (1, 2).Lesions of primary cu-taneous CD30+ ALCL tend to be large nodules, tumours, or subcutaneous masses that often ulcerate. Primary cu-taneous CD30+ lymphoproliferative disorders, including CD30+ ALCL, LyP and borderline cases, represent a clinical and histological continuum. In contrast to primary systemic and secondary CD30+ ALCL (e.g. large-cell transformation of MF), the primary cutaneous variant is characterized by an indolent clinical course with 5- and 10-year survival rates exceeding 95% and disseminated or extracutaneous disease in less than 10% (1). There is no uniform therapeutic approach for CD30+ ALCL, but most patients receive combinations of surgery, local radiation and/or chemotherapy (2, 3). Bexarotene, a retinoid X-receptor (RXR)-selective reti-noid, has recently been approved by the Food and Drug Administration (FDA) for cutaneous T-cell lymphoma including refractory MF and Sezary syndrome (4). There are only anecdotal reports on the treatment of CD30+ ALCL with bexarotene (5, 6). We describe here a patient with a predominantly inter-triginous manifestation of primary cutaneous ALCL, who rapidly developed extracutaneous tumour mass following bexarotene and interferon-α (IFN-α).CASE REPORTA 41-year-old Caucasian woman was referred to our hospital with a 10-month history of progressive cuta-neous tumours located mainly in the intertriginous areas such as the axillae, submammary region and inguinal folds (Fig. 1). Apart from slight pruritus she felt other-wise healthy, and had no concomitant diseases except obesity. Several skin biopsies showed mainly dermal and in part subcutaneous infiltration of atypical large lymphoid cells with pleomorphic nuclei and prominent nucleoli. Immunophenotyping revealed that these cells were strongly positive for CD30, CD4 and CD3, and negative for CD20 and CD56. A complete work-out, including clinical and bone marrow examinations, sonography and computerized tomography (CT), showed no evidence for systemic disease. A diagnosis of primary cutaneous CD30+ ACLC was made. Surgi-cal treatment was not considered because of the number of skin lesions. Hence, local radiotherapy with a total dose of 40 Gy given as 20 daily 2-Gy fractions was performed, resulting in a marked decrease of tumour load. However, she experienced new lesions shortly after finishing radiotherapy, and therefore monthly cycles of extracorporeal photophoresis (ECP) in combination with 3×4.5 MIU/week IFN-α-2b were initiated. After a stable outcome for 9 months, new lesions appeared, so bexarotene was added at a dosage of 300 mg/m