Mycosis Fungoides of the Oral Cavity: Fungating Tumor Successfully Treated with Electron Beam Radiation and Maintenance Bexarotene

Abstract

Oral involvement in mycosis fungoides is unusual and portends a poor prognosis. The clinical findings of three new cases are described along with a differential diagnosis and review of the literature. For brevity, only one patient is discussed in detail below whereas the other two cases are solely described in table form. The patient had a four-year history of mycosis fungoides before developing an exophytic tongue tumor. He was treated with local electron beam radiation and is disease-free to date while being on maintenance therapy with oral bexarotene. Analysis of the data collected from our review of the literature and the present cases reveal key insights.

Large Cell Transformation of Oral Mycosis Fungoides

“…Although it is the most common cutaneous T-cell lymphoma, MF is relatively rare (~1200 new cases annually in the United States) [5]. Furthermore, oral MF is extremely rare and represents ~1% of all MF cases [5][6][7][8]. MF usually occurs between the fifth and sixth decades of life and is twice as common in males than in females [4,5,9].…”

“…In most cases, oral MF is preceded by cutaneous involvement by an average of 8 years [5,9,10]. Approximately 45 cases of MF have been reported in the oral cavity to date [5][6][7][8]. Intra-orally, MF can present at any clinical stage similar to cutaneous MF with erythema, indurated plaques or ulcerated nodules [5,13].…”

“…Sezary syndrome incurs a relatively guarded prognosis with a median survival of 2-3 years from the time of diagnosis [5]. Furthermore, LCT-MF (defined as the presence of >25% infiltrating large lymphoid cells) is an adverse prognostic sign [1,4] and to our knowledge, there have only been four cases of oral LCT-MF reported in the English literature [8,23,24].…”

Combination of low-dose total skin electron beam therapy and subsequent localized skin electron beam therapy as a therapeutic option for advanced-stage mycosis fungoides

“…Second, concomitant use of systemic retinoid may have enhanced the therapeutic effects. The effects of retinoids in combination with radiation have not been fully evaluated, but some otherwise refractory cases have reportedly achieved substantial improvements with this combination …”

Oral mycosis fungoides with CD30+ large cell transformation successfully treated with brentuximab vedotin

“…Prior cases of oral large cell transformed MF (LCT-MF) have also been predominantly treated with radiation therapy (Table I). 2, 5, 6, 7 However, this therapeutic approach is not ideal, as it can lead to significant adverse effects, including salivary gland dysfunction, severe mucositis, and osteoradionecrosis 8 . We describe a new case of oral mycosis fungoides with CD30 + large cell transformation that demonstrated a complete response to 2 doses of brentuximab vedotin without significant side effects.…”