Scleromyxedematous Changes in a Patient With Long-Standing Mycosis Fungoides Who Progressed to Sézary Syndrome
We report a case of scleromyxedematous changes arising in a patient with long-standing MF who progressed to Sézary syndrome (SS) shortly afterward. Total-skin electron-beam radiation therapy resulted in an excellent response, controlling both the MF/SS and the scleromyxedematous lesions; however, the patient died few months later. Although mucin deposition has been described in association with MF/SS (mainly follicular mucinosis in folliculotropic MF), there are limited reports in the literature on dermal mucinosis and scleromyxedematous changes in MF/SS. The mechanism of this association and its prognostic implications requires further investigation.
CONCLUSIONS: These findings demonstrate that LDRT can eradicate malignant T cells in MF, provides robust disease control, and is associated with improved survival in high-risk early stage patients. PMID: 31636100 [PubMed - as supplied by publisher]
We report here that low-dose radiation therapy (LDRT, 8 Gy) induced durable remissions of treated lesions. We hypothesized that treatment with LDRT may era dicate the malignant T cell clone within the treated site. To address this, we examined pre- and post-treatment biopsies from 20 lesional skin samples of 18 MF patients who received either 8 Gy LDRT (n=16) or topical steroids (n=4) with high-throughput T-cell receptor sequencing (HTS) of the TCRB ge ne to identify, quantify, and follow the malignant T cell clone.
Erica S. Tarabadkar† and Michi M. Shinohara*† Division of Dermatology, University of Washington, Seattle, WA, United States Skin directed therapies (SDTs) serve important roles in the treatment of early stage cutaneous T-cell lymphoma (CTCL)/mycosis fungoides (MF), as well as managing symptoms and improving quality of life of all stages. There are now numerous options for topical therapies that demonstrate high response rates, particularly in early/limited MF. Phototherapy retains an important role in treating MF, with increasing data supporting efficacy and long-term safety of both UVB and PUVA as ...
Conclusions: We observed high rates of radiographic response and palliation of neurological symptoms. Nonetheless, all patients succumbed to their disease shortly after treatment, confirming the poor prognosis of this condition. Our findings suggest that RT may play a valuable palliative role for these patients.Case Rep Oncol 2018;11:721 –728
CONCLUSIONS: Our results confirm that radiation therapy in the treatment of minimal stage MF contributes to afford good and durable results with negligible side effects and maintenance of good quality of life over the time. PMID: 29485254 [PubMed - as supplied by publisher]
Condition: Mycosis Fungoides Interventions: Drug: Mechlorethamine Hydrochloride Gel; Procedure: Total Skin Electron Beam Radiation Therapy Sponsors: Sidney Kimmel Cancer Center at Thomas Jefferson University; Actelion Not yet recruiting