Mycosis fungoides with follicular mucinosis displaying aggressive tumor-stage transformation : successful treatment using radiation therapy plus oral bexarotene combination therapy.
Follicular mucinosis is a tissue reaction pattern characterized by mucin deposition with follicular sebaceous units and is found as an idiopathic, primary, benign process (alopecia mucinosa), or as a secondary process due to inflammatory and neoplastic disorders (mycosis fungoides). When associated with follicular mucinosis, mycosis fungoides commonly pursues an aggressive course, often undergoing large-cell transformation, which is associated with resistance to therapy and poor prognosis. We present a case of mycosis fungoides with follicular mucinosis that was treated with incomplete courses of interferon, isotretinoin, and polychemotherapy with subsequent rapid progression to tumor-stage mycosis fungoides with large cell transformation and nodal and bone marrow involvement. In this setting, the patient was treated with local radiation therapy, total-skin electron beam therapy, and therapy and maintenance with the oral retinoid-X-receptor retinoid bexarotene, and achieved a durable complete remission.
Apisarnthanarax
Narin
N
Department of Dermatology, University of Texas MD Anderson Cancer Center, Houston, Texas 77030-4095, USA.
Ha
Chul S
CS
Duvic
Madeleine
M
D000284:Administration, Oral; D000328:Adult; D016588:Anticarcinogenic Agents / Q000627:therapeutic use; D000077610:Bexarotene; D002471:Cell Transformation, Neoplastic / Q000187:drug effects / Q000473:pathology* / Q000528:radiation effects; D017024:Chemotherapy, Adjuvant; D005260:Female; D006801:Humans; D000507:Mucinosis, Follicular / Q000209:etiology / Q000473:pathology / Q000628:therapy*; D009182:Mycosis Fungoides / Q000150:complications / Q000473:pathology / Q000628:therapy*; D012878:Skin Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy*; D013764:Tetrahydronaphthalenes / Q000627:therapeutic use
D016588:Anticarcinogenic Agents; D013764:Tetrahydronaphthalenes; D000077610:Bexarotene