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PubMed2003

Treatment of patch-stage mycosis fungoides with topical corticosteroids.

Experience at the University of California, San Francisco (UCSF), in the management of patch-stage mycosis fungoides (MF) with topical, predominantly high-potency, corticosteroids is reviewed. The technique of applications is discussed in detail. Approximately 200 patients have been treated. The results are very favorable. The response rate is over 90%. Side-effects are minor. Topical clobetasol is the first-line treatment for early stage MF at UCSF.

Administration, Topical↗
PubMed2003

Management with topical nitrogen mustard in mycosis fungoides.

Topical nitrogen mustard (mechlorethamine, NM) has been used as primary therapy for management of patients with mycosis fungoides (MF) since the 1950s. Many investigators have demonstrated the efficacy of topical NM in patch and/or plaque disease of MF. Updated results from Stanford also confirm the clinical efficacy. The complete response (CR) rates reported are 76-80% for patients with limited patch/plaque (stage IA), and 35-68% for those with generalized patch/plaque (stage IB) disease. Topical NM can be used as an aqueous (water) or ointment-based preparation. The efficacy results are similar in patients who were treated with aqueous versus ointment preparations. Maintenance regimens used are variable, but there is no data to suggest that a longer maintenance duration results in greater potential for long-term remission. Most patients who achieve initial CR with topical NM tend to require NM-only for disease management. Topical NM is equally effective when used as salvage therapy with disease relapse. The most common toxicity of topical NM therapy is contact irritant or allergic reaction. The potential for allergic reaction is significantly reduced (< 10%) when NM is used as an ointment preparation. The potential for secondary skin cancer development is increased in patients who have used multiple sequential topical skin-damaging therapies or NM in the genital skin, but not in patients who have used NM as monotherapy (avoiding genital skin application). Topical NM is used safely in pediatric patients and there is no evidence of any clinically significant systemic absorption of topically applied NM.

Administration, Topical↗
PubMed2003

Topical carmustine (BCNU) in the treatment of mycosis fungoides.

The University of California, San Francisco (UCSF), experience with topical carmustine (BCNU) in the treatment of approximately 200 patients with mycosis fungoides (MF) is summarized. Topical BCNU is an effective treatment for patch- and early plaque-stage MF. Side-effects are usually not a major deterrent to continuation of therapy.

Administration, Topical↗
PubMed2003

Mycosis fungoides: radiation therapy.

Radiation therapy is the most effective single agent for the treatment of mycosis fungoides. There are well-defined dose-response relationships for achieving a complete response as well as the durability of this response. Techniques of electron beam therapy have been developed that permit treatment of the entire skin. Total-skin electron beam therapy is an important form of management, especially for patients who have thick generalized plaque or tumorous disease. Radiation therapy may also be used selectively for treatment of extracutaneous disease.

Clinical Trials as Topic↗
PubMed2003

Systemic chemotherapy for the treatment of mycosis fungoides and Sézary syndrome.

The treatment of mycosis fungoides and Sézary syndrome is unique. The treatments of choice are highly stage-and practitioner-dependent. For early stage patients, treatment with palliative topical therapies is often adequate to yield excellent, high-quality and durable responses. For the more advanced stages, systemic approaches are more appropriate, either alone or in combination, to palliate patients. There is still little convincing evidence from randomized trials that any single approach is favorable for improving survival. Many practitioners believe the disease can be controlled with the host immune system, but there again is little scientific support for this conclusion. It has been hypothesized that some of our therapies work through this mechanism, such as photopheresis and perhaps even psoralen and ultraviolet A. Unfortunately, even the use of biologic response modifiers, such as interferon and the interleukins, is not absolutely supportive of an active role for immune surveillance since these agents have been shown to have cytotoxic and differentiating effects. Because of the above, many practitioners have actively discouraged the use of chemotherapy because it may impair the host immune system; certainly, the purine analogs would fall into this category. However, there is little evidence supporting significant immune effects for other chemotherapeutics, and clearly, the responses seen would support their use in appropriate patients. As the present authors will detail in this paper, the advances in understanding cancer biology and mechanisms of resistance should, in the future, lead to optimal selection of agents that are predicted to be optimally active, limiting the toxicity and waste associated with ineffective drug usage.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

Mycosis fungoides with fatal brain involvement.

The clinical course, therapy and investigations in a man with lichenoid mycosis fungoides which resulted in fatal spread to the brain is described. Immunological studies revealed a significant reduction in T cell numbers. Brain lesions were confirmed by CT Scan and drill biopsy. The literature on the subject is discussed.

Brain Neoplasms↗
PubMed1997

Photochemotherapy for mycosis fungoides: current status.

Phototherapy for mycosis fungoides is reviewed with particular emphasis on PUVA (psoralen ultraviolet A-range) therapy and its combination with other topical and systemic treatments. PUVA therapy has the advantages of being available in all major centres, is simple to administer, relatively inexpensive and has relatively low toxicity. Medium-term results suggest that PUVA is as effective as any of the other topical therapies for stage I disease. Its combination with alpha-interferon produces significant benefit in stage II or more advanced disease. Long-term non-melanoma skin cancer is increased with PUVA and combination PUVA usage, but melanoma is not increased. Its long-term position in comparison to other therapies with regard to morbidity, mortality and quality of life needs further evaluation.

Evaluation Studies as Topic↗
PubMed2004

Granulomatous mycosis fungoides with extensive chest wall involvement.

A 40-year-old woman presented with a 5-year history of a mass overlying her right pectoralis major muscle. Histopathology of the lesion revealed a florid granulomatous infiltrate including an atypical lymphocytic component with marked epidermotropism consistent with granulomatous mycosis fungoides. Staging investigations demonstrated the tumour to be localized to the right chest. Consequently, the patient was treated with radiotherapy (50 Gy) to the lesion with good clinical effect. However, she soon developed a clinically palpable lesion on the left chest outside the radiotherapy field. Positron emission tomography scanning demonstrated an extensive left-sided chest wall tumour and also residual tumour on the right. This left-sided lesion failed to respond to systemic chemotherapy. Further radiotherapy (50 Gy) has recently been administered to the left chest lesion; the response is being monitored. While granulomatous inflammation has been previously described in cutaneous T-cell lymphomas, it is rare and is often associated with a delay in the diagnosis and difficulty with clinical staging. The clinical presentation can be extremely variable and consequently, diagnosis rests with histological features, immunohistochemical studies and gene rearrangement analysis.

Adult↗
PubMed2004

Follicular mycosis fungoides mimicking a cutaneous B-cell lymphoproliferative disorder.

Follicular mycosis fungoides (MF) is an uncommon histological variant of MF characterized by infiltrates of atypical lymphocytes around and within the epithelium of the hair follicles (folliculotropism). Here we report a patient with rapidly progressive follicular MF on the face, associated with concurrent typical MF lesions elsewhere. The histology was unusual, as apart from dense lymphoid infiltrates showing folliculotropism and epidermotropism, there was a prominent B-cell component with germinal centres, leading to an initial diagnosis of cutaneous B-cell lymphoma. The final diagnosis of follicular MF was established on demonstration of clonal T-cell receptor gene arrangements and lack of clonality for heavy chain gene rearrangements. This case illustrates a variant of MF that has a more rapid progression than the otherwise indolent course of classical MF over many years, and the diagnostic pitfalls, whereby the histology can mimic a B-cell proliferative disorder.

B-Lymphocytes↗
PubMed2007

A 30-year history of CD4+ vesiculo-bullous mycosis fungoides and multiple visceral malignancies.

An 83-year-old Caucasian woman presented with a 25-year history of an itchy, eczematous blistering eruption affecting her trunk and acral sites. She had a past history of adenocarcinoma of the lung, colorectal carcinoma and bladder carcinoma. Several skin biopsies consistently showed features of a spongiotic process. Direct and indirect immunofluorescence studies were repeatedly negative, excluding the possibility of an autoimmune blistering disorder. A skin biopsy several years later, however, showed histological and immunophenotypic features of mycosis fungoides. The literature on this rare phenotype of cutaneous T-cell lymphoma generally portrays a negative prognosis. Our case illustrates an excellent prognosis with stable disease 30 years after onset.

Adenocarcinoma↗
PubMed1987

A comparative microphotometric analysis of adult T-cell leukemia/lymphoma (ATLL) in the skin and mycosis fungoides (MF).

In the skin lesions of 16 adult T-cell leukemia/lymphoma (ATLL) and 13 mycosis fungoides (MF), morphological features of cutaneous infiltrates and their cell kinetics were evaluated through microphotometry of nuclear DNA content and nuclear size on Feulgen-stained sections. Lymphomas comprising some giant cells with irregular-shaped nuclei were found only in ATLL and these cells revealed aneuploid high DNA values. In contrast, neoplastic cells with pale cytoplasm in the Giemsa stain were seen more frequently in MF (69.5%) than in ATLL (12.5%). Patterns of the cutaneous infiltration according to skin microenvironments such as perivascular areas, dermal papillae, and periadnexal areas, were similar in the both entities. In the perivascular areas a mixed proliferation of lymphoma cells and non-neoplastic lymphocytes was conspicuous. The lymphoma cells were determined by high DNA values and large nuclear sizes. But the ratio of lymphoma cells to non-neoplastic cells in the infiltrates in the perivascular areas was higher in ATLL than in MF. In the dermal papillae and around the skin appendages, lymphoma cells predominated. Lymphoma cells which formed a plaque or tumor in the dermis contained many actively proliferating cells having higher DNA values and larger nuclear sizes than the lymphoma cells without tumor formation. One case of ATLL intermediate cell type showed, however, few proliferating cells inspite of tumor formation, suggesting a pooling or a long life-span in the skin.

Cell Cycle↗
PubMed2006

UVB in the management of early stage mycosis fungoides.

BACKGROUND: Several options for treatment of early mycosis fungoides (MF) offer similar success rates. Previous small studies have shown UVB to be at least as effective as PUVA. OBJECTIVE: To summarize our experience with UVB treatment of early MF. METHODS: A retrospective analysis of early-stage MF patients treated by narrow band (NB) or broad band (BB) UVB in our institution between 1996 and 2002. Most patients achieving complete response (CR) were put on maintenance until natural sun exposure was possible and followed up every 3-6 months. The results were compared to those previously reported regarding PUVA. RESULTS: Sixty-eight and 43 patients were treated by NB and BB UVB, respectively. Eighty-six per cent (84 and 89% in NB and BB UVB groups, respectively) of IA and 71% (78 and 44% in NB and BB UVB groups, respectively) of IB patients achieved CR within a mean of 12.8 and 10.6 weeks, respectively. When maintenance was stopped, 65 and 30% had not relapsed after an average follow up of 27 and 222 weeks, respectively. Non-relapse rate was 33 and 48% for those having had vs. those not having had maintenance, respectively. CONCLUSIONS: Our results are comparable to all previously reported for skin-targeted treatments, including PUVA and, to our belief, reflect the nature of early MF, in which CR can probably be achieved in most of the patients. Among the responding patients there is no relapse during prolonged follow-up in about one third of the cases. Thus, we believe treatment should be stopped completely following first CR induction and maintenance treatment should be considered for relapsing patients only. Both broad and narrow UVB options are good and future choices should be made on the basis of short- and long-term side-effects.

Adolescent↗
PubMed2006

Narrowband UVB phototherapy for early-stage mycosis fungoides: evaluation of clinical and histopathological changes.

BACKGROUND: Early-stage (IA, IB, IIA) mycosis fungoides (MF) has long been treated with various agents including topical potent steroids, nitrogen mustard, carmustine, oral psoralen plus UVA (PUVA), broadband UVB, electron-beam radiotherapy, interferon-alpha and retinoids. However, each of these modalities is associated with various side-effects. Narrowband UVB (NB-UVB) therapy has the same effect but is safer to use than the other methods. OBJECTIVE: Our purpose in this prospective study was to determine the effects of NB-UVB in early-stage MF both clinically and histopathologically. MATERIALS AND METHODS: Twenty-three patients (20 men, three women, aged 27-78 years) with clinically and histologically confirmed MF were enrolled. Patients received NB-UVB therapy three times a week. Clinical and histological responses, cumulative doses, total number of treatments, side-effects and duration of remission period were noted. RESULTS: Six patients had stage IA MF, 15 patients stage IB and two patients stage IIA. Eighteen patients had patch stage and five patients had plaque stage histopathologically. All of the patients in the patch group had a complete response (CR). In the plaque group, three patients (60%) had a CR and two (40%) had partial (PR) or no clinical response (NR). The clinical response between patch and plaque groups was statistically significant. Regarding the histopathological findings, 17 (94.4%) had complete clearing and only one (5.6%) patient had a partial improvement in the patch group. In the plaque group, one (20%) patient had complete clearing and four (80%) patients had partial or no improvement. The difference between the two groups was statistically significant. In the patch group, the mean cumulative dose was 90.15 J/cm(2) and the mean number of treatments was 35.33. In the plaque group, the mean cumulative dose was 90.67 J/cm(2) and the mean total number of treatments was 39.40. The differences were not statistically significant, either between the mean cumulative dose or the mean number of treatments. The mean duration of follow-up was 10.87 months (range 1-25 months). Only one of the patients had a relapse. CONCLUSIONS: NB-UVB therapy for patients with early-stage MF is an effective and safe treatment with the effect lasting for months. We suggest that clinical clearance correlates with histological improvement except for patients in the plaque stage.

Adult↗
PubMed1978

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.

Adult↗
PubMed1978

Teleroentgen therapy for mycosis fungoides.

Different techniques of teleroentgen therapy with soft X rays for total body treatment of cutaneous lymphomas are described. Therapeutic results in 26 patients with mycosis fungoides are reported.

Adult↗
PubMed1978

Radiotherapy of mycosis fungoides: twenty years of experience with teleroentgen and low-voltage X-ray therapy.

Of 98 patients with mycosis fungoides, 55 have been treated with teleroentgen therapy and 43 with low voltage radiation at a shorter distance. Teleroentgen therapy is indicated in the stage of generalized eczematous lesions or superficial infiltration. According to the Hamburg method, a dose of 300 R was delivered to eight fields at a target distance of 120 cm. After a series of one to three treatments, 300 R, administered at three-week intervals, all lesions usually cleared. Small patches and deeply infiltrated lesions and tumors were treated with the same doses and intervals of low voltage X rays, the half-value depth corresponding to the depth of infiltration. After five years, 54% of all patients were still alive and after 10 years, 29% were still living.

Adult↗
PubMed1986

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.

Humans↗
PubMed1986

Tumor stage mycosis fungoides in a patient treated with long-term corticosteroids for asthma and atopic-like dermatitis.

An association between atopy and the Sézary syndrome has been recognized in some patients. Our case represents an association between adult-onset asthma and atopic-like dermatitis, markedly elevated IgE levels, and the development of tumor stage mycosis fungoides (MF) in a patient receiving long-term corticosteroid therapy. The chronic eczematous dermatitis may have represented evolving MF in which the specific diagnosis was delayed by treatment with topical and systemic corticosteroids until advanced tumor stage disease. This case, in conjunction with prior reports, suggests that an atopic diathesis can be associated with both leukemic and nonleukemic forms of cutaneous T-cell lymphoma.

Adrenal Cortex Hormones↗