Extracutaneous mycosis fungoides

The clinical course of 40 patients with histologically documented extracutaneous mycosis fungoides (ECMF) was reviewed. Thirty one patients had documentation of nodal disease only (Stage IVA). Nine patients had histologic evidence of visceral involvement (Stage IVB). A wide variety of topical, regional and/or systemic therapies were used in the management of these patients. The median survival for the entire group was 14.5 months. Eleven patients (28%) obtained a complete response (CR) and had a median survival of 21 months as compared with 8 months among 29 patients not obtaining a CR. One patient is without evidence of disease (NED) at 10 years. Multiple prognostic factors were examined with respect to survival. Prognostic factors found to be significant at the P = 0.05 level included ECMF at presentation of skin disease (versus at the time of relapse), the ability to achieve a CR, and management incorporating the use of topical therapy, especially electron beam treatment. Tabulation of all trials of chemotherapy either at diagnosis of ECMF or subsequently revealed that cyclophosphamide, vincristine and prednisone (CVP) and cyclophosphamide, vincristine, prednisone, and bleomycin (COP-Bleo) were the most effective chemotherapeutic combinations. In a multivariate analysis with survival as the endpoint, the best model consisted of only two covariates: ECMF at presentation of skin disease, and the use of topical therapy. Other covariates found not to be significant at the P = 0.05 level included age, gender, clinical extent of cutaneous and extracutaneous disease, Stage IVA versus Stage IVB disease, the presence of Sezary cells in the peripheral blood smear, and management incorporating the use of systemic therapy.     

Granulomatous reactions in mycosis fungoides

Granulomatous reaction characterized by the formation of noncaseating accumulations of epithelioid histiocytes and multinucleated giant cells of the foreign-body type is a rare, poorly understood, and generally ignored phenomenon seen in various types of lymphoma. Its presence in cutaneous infiltrates of mycosis fungoides is equally unusual, but some favorable prognostic significance has been ascribed to it previously. In this paper, four patients with typical mycosis fungoides and granulomas demonstrated histologically in their cutaneous infiltrates are presented. All four died of disseminated disease with evidence of central nervous system involvement in three of them. These clinical histories lend no support to the notion that granulomatous mycosis fungoides is a benign variant of this lymphoma. Relevant literature is reviewed and discussed.     

Acral necrotizing mycosis fungoides

A 65-year-old male with long-standing acral "eczema" and patches of mycosis fungoides suddenly developed a transformed, necrotizing ulcerative cutaneous T-cell lymphoma manifesting as necrotic lesions of the toe and lip. The tumor had brown and/or black eschars on the surface and grew multiple opportunistic organisms. Markers CD3, Epstein-Barr virus-encoded small RNA, and T-cell receptor g gene rearrangement were positive, with CD4, 8, 30, 56, and TIA-1 negative. In spite of initial response to radiation, the patient succumbed to infection within 3 months.     

Laryngeal mycosis fungoides.

Mycosis fungoides presented as a tumor of the arytenoid cartilage and epiglottis of the larynx of an 80-year-old female. The tumor was initially interpreted as an undifferentiated small cell malignant neoplasm and treated with radiation. Two years later the patient developed widespread cutaneous involvement with mycosis fungoides, including mycosis fungoides bullosum. The mycosis fungoides progressed to death over the next two years. At autopsy widespread visceral involvement was present. The larynx was extensively infiltrated with mycosis fungoides, and the histology was similar to the original laryngeal biopsy. Although mycosis fungoides is generally thought of as a cutaneous disease, it may rarely present in a squamous epithelium-lined organ other than the skin.     

Therapy for mycosis fungoides

Opinion statement Treatment of mycosis fungoides (MF) is indicated to reduce symptoms, improve clinical appearance, prevent secondary complications, and prevent progression of disease, all of which may have an impact on survival. Treatment of MF includes topical and systemic therapies, which can be administered alone or in combination. Psoralen and ultraviolet A radiation is effective in early-stage MF, inducing complete remissions in most patients. Psoralen and ultraviolet A radiation may also be combined with low doses of interferon (IFN)-a to treat stage I/II disease. However, early aggressive therapy with radiation and chemotherapy does not improve the prognosis. Local radiotherapy or total skin electron beam irradiation has been used with success to control advanced skin disease. Extracorporeal photopheresis may also be used successfully, but it is not generally available. Once the disease becomes refractory to topical therapy, IFN-a single-agent or combination chemotherapy may be administered, but the duration of response is often less than 1 year and ultimately all patients will relapse and become refractory. Among chemotherapeutic agents, pentostatin, gemcitabine, and liposomal doxorubicin seem to be particularly effective. Response rates after combined modality therapy with total skin electron beam irradiation and chemotherapy/IFN-a appear similar to response rates of chemotherapy alone. Therefore, there is a great need for the further development of novel emerging treatment modalities, such as retinoids (ie, bexarotene) and immunotherapeutic agents (ie, cytokines, tumor vaccines, and monoclonal antibodies), all of which appear to have significant therapeutic potential in patients with MF. Biologically based therapies may reduce the need for genotoxic therapies, such as cytostatics and radiotherapy.     

Spinal cord compression in mycosis fungoides

Symptomatic involvement of the central nervous system (CNS) with mycosis fungoides is rare, and usually characterized by involvement of meninges. We describe a patient with long-standing mycosis fungoides who developed acute spinal cord compression. Since tumor-related spinal cord compression requires early intervention for a successful outcome, it should be recognized as an additional form of CNS mycosis fungoides.     

Radiotherapy for unilesional mycosis fungoides

Purpose: To evaluate the treatment outcome and natural history of patients with the diagnosis of unilesional mycosis fungoides, treated according to a prospective radiotherapy protocol in our institution since July 1975.Methods and Materials: A total of 325 patients with the diagnosis of mycosis fungoides have been referred to the Department of Radiation Oncology at Allegheny University of Health Sciences from July 1975 through September 1996. Of these, 18 patients (5%) were classified as having unilesional mycosis fungoides and were irradiated with a curative intent using local electron fields. One patient received 22 Gy; 1 patient received 40 Gy, and the rest of the patients 30.6 Gy. Daily fractions ranged from 1.8 to 2.0 Gy. Treatments prior to radiation consisted of topical steroids and/or antifungal creams in the majority of patients, with temporary partial responses. One patient had received 2 years of topical mechlorethamine (HN2) and another patient had received topical carmustine solution (BCNU) without response prior to irradiation.Results: The responses were measured clinically; posttreatment skin biopsy was not performed routinely unless there was clinical evidence of disease persistence. Complete response rate was 100%; all treated lesions cleared completely within 4 to 8 weeks after the completion of radiation. With a median follow-up of 43 months (range 12 to 240 months), 2 relapses have occurred, 2 and 71 months after the completion of radiation. Both relapses were confined to the skin and were remote from the original site. Both relapses responded to topical application of HN2. There have been no recurrences in the irradiated field nor systemic dissemination. No long-term side effects were found related to treatment, and all the patients are currently alive and without evidence of disease. Actuarial relapse-free and overall survival at 10 years are, respectively, 86.2% and 100%.Conclusion: Unilesional mycosis fungoides has a long natural history, is possibly the earliest manifestation of a malignant process, and local treatments, including local radiotherapy, result in long-term disease-free intervals and, possibly, cure. Total skin electron beam radiotherapy is not indicated for this disease entity.     

Tumor cell characterization in mycosis fungoides.

Five patients with tumor stage mycosis fungoides had tumor lesions excised and examined by light and electron microscopy. The tumor cells were also isolated and the percentages of E rosettes (T cells) and EAC rosettes (B cells) were determined and the rosettes examined by electron microscopy. The predominant cell type isolated from four of the five tumors was a T cell. Peripheral lymphocyte function measured by LTT to phytohemagglutinin and pokeweed mitogen was normal. Serum immunoglobulins gave variable results. The results support the concept that mycosis fungoides is primarily a T cell tumor involving the skin and morphologically similar to the Sézary cell.     

Aberrant microRNA expression in tumor mycosis fungoides

Tumor Biology - Herein, miRNA candidates relevant to mycosis fungoides were investigated to provide data on the molecular mechanisms underlying the pathogenesis of the disease. The miRNA expression...     

T-cell leukemia-lymphoma and mycosis fungoides.

Notable efforts have been made to relate aspects of the cell biology of T cells to the pathology, diagnosis, and treatment T-cell neoplasms. In particular, the application of molecular biologic tools to these areas has already allowed the generation of patient-specific markers for disease. A case can be made that a knowledge of the distinctive natural history of T-cell neoplasms should influence choices of treatment. Additional insights into the relevance of the human T-cell leukemia-lymphoma virus family to human disease have been recorded, and an important association of cutaneous T-cell lymphoproliferative disorders with human immunodeficiency virus infection has been documented.     

Electron-beam therapy for mycosis fungoides.

The lesions of mycosis fungoides are radiosensitive and because extensive areas of the body are usually involved, the treatment of choice is with the electron beam. Since 1971, a fractionation scheme of weekly doses for irradiation of the entire skin surface has been practiced with a 6 MeV linear accelerator that generates 3.5 MeV electrons. One hundred and five patients were treated in this way by delivering 400 rads once weekly for six consecutive weeks. All patitents enjoyed complete disappearance of the skin lesions at once. Of the 62 patients whom we were able to follow-up after electron-beam therapy, for periods ranging from one to nearly seven years (median, two years), the duration of remission was one-half to nearly five and a half years (median, one and one-half years). The treatment was well tolerated and no unusual skin reactions or hematopoietic depression occurred.     

Ointment-based mechlorethamine treatment for mycosis fungoides

Atypical immature squamous metaplasia (AIM) is a poorly understood lesion with uncertain biological and clinical significance. This report reviews 170 cases of cervical condylomata and 60 cases of high-grade cervical intraepithelial neoplasia (CIN II/III); AIM was found in association with 34% of condylomata and 16% of high-grade CIN. Thirty-seven cases of AIM alone were reviewed and nearly all presented with a cytologic diagnosis of CIN I or condyloma and a colposcopic appearance of white epithelium with or without punctation and mosaic structure. The mean ages of patients with condylomata, AIM, CIN II, and CIN III were 27, 27, 32, and 37 years, respectively. By immunoperoxidase techniques 75% of condylomata, 16% of AIM, and 0% of CIN lesions were positive. The histologic criteria for the diagnosis of AIM and it's morphologic distinction from CIN and relationship to condylomata, are outlined. AIM is a distinct histologic entity that shares similar epidemiologic, morphologic and biologic characteristics with condyloma. When AIM is found alone in biopsy material, careful correlation of cytology, and biopsy results should be performed and therapy should be based on the size and distribution of the lesion.     

Treatment of mycosis fungoides with isotretinoin

A 56-year-old man with a 7-year history of well-documented mycosis fungoides is reported. Because the patient was a treatment failure with topical nitrogen mustard due to severe allergic contact dermatitis, and because of recent reports of the efficacy of retinoid compounds, he was treated with a 6-month course of isotretinoin with total clearing of his skin lesions. Previous case reports and possible mechanisms of action are reviewed.