Mycosis fungoides with follicular mucinosis displaying aggressive tumor-stage transformation : successful treatment using radiation therapy plus oral bexarotene combination therapy.

Abstract

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.

• Authors: Narin Apisarnthanarax, Chul S Ha, Madeleine Duvic
• Journal: American journal of clinical dermatology (Am J Clin Dermatol) Vol. 4 Issue 6 Pg. 429-33 ( 2003) ISSN: 1175-0561 [Print] New Zealand
• PMID: 12762834 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Anticarcinogenic Agents
• Tetrahydronaphthalenes
• Bexarotene

Topics

• Administration, Oral
• Adult
• Anticarcinogenic Agents (therapeutic use)
• Bexarotene
• Cell Transformation, Neoplastic (drug effects, pathology, radiation effects)
• Chemotherapy, Adjuvant
• Female
• Humans
• Mucinosis, Follicular (etiology, pathology, therapy)
• Mycosis Fungoides (complications, pathology, therapy)
• Skin Neoplasms (complications, pathology, therapy)
• Tetrahydronaphthalenes (therapeutic use)