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Multiple courses of high-dose total skin electron beam therapy in the management of mycosis fungoides.

PURPOSE: A retrospective analysis was undertaken to determine the indications for, the efficacy of, and the long-term complications of two courses of total skin electron beam therapy for mycosis fungoides. METHODS AND MATERIALS: A retrospective analysis of 15 patients with the pathologic diagnosis of mycosis fungoides treated in the Department of Radiation Oncology at Stanford University Medical Center between 1968 and 1990 was performed. All patients received two courses of high-dose electron beam therapy to the skin. The mean dose for the total skin treatment for the first course was 32.6 Gy and 23.4 Gy for the second course of treatment. RESULTS: Following the first course of total skin electron beam therapy, 11 of 15 had a complete response, with a mean duration of 11.6 months. All patients received adjuvant therapies between the first and second courses of high-dose total skin electron beam therapy. The mean interval between the first and the second courses of therapy was 41.3 months. Patients were restaged prior to commencement of their second course of high-dose total skin electron beam therapy, resulting in upstaging in six. The second course of therapy resulted in six complete responses and nine partial responses. Twelve of these patients have since died, 1 is lost to follow-up, and 2 are living with disease. The long-term side effects in the two living patients include pigmentation changes, alopecia, and diffuse xerosis. CONCLUSION: Delivery of two courses of total skin electron beam therapy is technically feasible, tolerable, and efficacious. The dose to the total skin was reduced for the second course of therapy in all cases. The criteria used to screen patients included initial good response to total skin electron treatment, long disease-free interval, exhaustion of other therapeutic modalities, and generalized skin involvement at relapse. Long-term toxicities were mild in severity and generally consisted of generalized xerosis, scattered telangiectasias, pigmentation changes, and partial alopecia.

Adult↗

Unusual presentation of mycosis fungoides as pigmented purpura with malignant thymoma.

Thymoma is known to be associated with many lymphoreticular and nonlymphoreticular tumors. A woman who presented with patch stage mycosis fungoides with skin lesions resembling a pigmented purpura was found to have an anterior mediastinal mass on routine staging evaluation. A core needle biopsy of the mass revealed a lymphocyte predominant malignant thymoma. Review of the literature reveals many diseases with dermatologic manifestations associated with thymoma; however, malignant thymoma coexisting with mycosis fungoides has not previously been reported.

Adult↗

The treatment of mycosis fungoides: adjuvant topical mechlorethamine after electron beam therapy.

The feasibility of employing adjuvant topical mechlorethamine after electron beam therapy in the treatment of patients with mycosis fungoides is demonstrated. Patients treated with a planned adjuvant topical mechlorethamine schedule had a median disease-free interval of 25 months compared to 17 months for the group treated with electron beam therapy alone. Projected relapse-free survivals are slightly better in the adjuvant group--37% versus 29%. Patients receiving adjuvant topical mechlorethamine after the electron beam were observed to have a low incidence of contact allergy to the medication. The topical medication can be continued if a contact allergy develops by using a planned desensitization program. We currently treat all mycosis fungoides patients with electron beam therapy, randomizing half to receive adjuvant topical mechlorethamine.

Administration, Topical↗

Mycosis fungoides and Sézary syndrome in Japan. Clinicopathologic study of 107 autopsy cases.

We reviewed 111 clinical and pathological materials of patients who had been entered as mycosis fungoides (MF) or Sézary syndrome (SS) in the Annuals of the Pathological Autopsy Cases in Japan during the 29-year-period since the Annuals' inception in 1958. One hundred and seven patients were classified. Of these, 64 were in Group 1 (mycosis fungoides, MF), 23 in Group II (Sézary syndrome, SS), and 20 in Group III (non-Hodgkin's lymphoma, NHL). The clinicopathologic features of Groups I and II (MF, SS) were similar to those of cutaneous T cell lymphoma (CTCL) in Europe and the United States. Those of Group III (NHL) were indistinguishable from primary cutaneous non-Hodgkin's lymphoma. It was suggested that some patients with adult T cell leukemia/lymphoma (ATLL) with skin lesions used to be misdiagnosed as MF or SS in earlier years when ATLL was still unknown. It was concluded that CTCL in Japan has clinicopathologic features quite like those of CTCL in Europe and the United States and that they are probably the same disease.

Adolescent↗

The immunologic and clinicopathologic heterogeneity of cutaneous lymphomas other than mycosis fungoides.

Twenty-one cases of non-Hodgkin's lymphoma with cutaneous involvement, other than mycosis fungoides, were evaluated immunologically, histologically, and clinically. Ten patients presented with skin disease alone, seven with concurrent cutaneous and extracutaneous disease, and four with extracutaneous disease only. Twenty of the 21 cases were nonepidermotropic tumors and were equally likely to express B or T phenotypes. None of the cases expressed a true histiocytic phenotype. Almost all cases expressed la and class 1 HLA determinants. Immunophenotypes were stable regardless of time interval, therapy, or body site sampled in seven of eight patients studied serially. In contrast to mycosis fungoides, the T lymphomas exhibited noncerebriform cytology, tumor la expression, lack of mature helper T-cell phenotype, nonepidermotropic histology, a tendency for marrow involvement, and presented as nodules rather than patches or plaques. Since each T lymphoma expressed an abnormal but uniform T-cell phenotype other than mature cytotoxic/suppressor or helper, the neoplastic population could be distinguished from reactive T cells. Reactive elements averaged one-third of the cellular infiltrates and were mainly T cells and macrophages. Langerhans cells were generally normal in number and distribution. Several histopathologic subtypes were identified with diffuse large cell lymphomas, including immunoblastic lymphomas, comprising 71% of cases (15/21). Prediction of the immunophenotype based on cytologic criteria was correct in 67% of cases (14/21). All errors occurred among the 13 high-grade lymphomas. Survival data were consistent with those of prior studies that have indicated that clinical course is dependent on stage and histologic subtype. Non-Hodgkin's cutaneous lymphomas constitute an immunologically, histologically, and clinically heterogeneous group of neoplasms.

Adolescent↗

[Topical application of ACNU for the treatment of mycosis fungoides].

The effects of topical chemotherapy of 1-(4-amino-2-methyl-5-pyrimidinyl) methyl-3-(2-chloroethyl)-3-nitrosourea hydrochloride (ACNU) on the lesions of mycosis fungoides were evaluated in 7 patients, ranging in age from 44 to 79 years old. Either 0.2% or 0.4% concentration of ACNU, in ointment and ethanol was used. 0.4% ACNU ethanol solution was effective in bringing the plaque lesions under satisfactory control with a complete clearance. ACNU was painted two to three times a week with a maximum dose of 50mg. Irritation and erosion of the applied areas were the major side effects, which were however, controlled by topical steroid ointment. No serious side effects of marrow and liver function were found even when ACNU was applied as long as 40 months (total ACNU dose: 16 gm). Histologically the cleared lesions revealed the thinning of epidermis, almost complete loss of lymphocytic infiltrates and fibrosis of dermis which was density infiltrated by lymphocytes prior to the ACNU therapy. Thus, topical chemotherapy of ACNU appears to be encouraged for modifying the plaque lesions of mycosis fungoides.

Administration, Topical↗

[Association of mycosis fungoides and Jaccoud syndrome].

We report here a case of a 49 year-old woman who developed a unilateral Jaccoud's arthropathy 9 years after a mycosis fungoides of the left hand. This rheumatic affection, described in 1866, is a deformation of the hands or of the feet linked to a periarticular disease without any bone lesion. Many causes have been registered but the most frequent etiology is the systemic lupus erythematosus. The mycosis fungoides had never been reported as yet to be responsible to the Jaccoud's arthropathy.

Female↗

Hyperkeratotic mycosis fungoides restricted to the palms.

The case of a 45-year-old Latin American man, who presented to the Dermatology Clinic with a 6-month history of hyperkeratotic lesions confined to the palms and the palmar aspects of the digits of both hands, is discussed. Biopsy of these lesions revealed the classic histologic findings of mycosis fungoides. The clinical and histologic differential diagnosis of mycosis fungoides is considered.

Biopsy↗

Guttate parapsoriasis/digitate dermatosis (small plaque parapsoriasis) is mycosis fungoides.

Authors of most textbooks of dermatology and dermatopathology consider guttate parapsoriasis and digitate dermatosis to be variants of small plaque parapsoriasis which, they aver, is not related to mycosis fungoides. On the basis of a study of clinical and histopathologic findings in guttate parapsoriasis and digitate dermatosis, we conclude that those conditions actually represent two of the many clinical faces of mycosis fungoides.

Adolescent↗

Pityriasis lichenoides-like mycosis fungoides in children.

We report three children with clinical features of pityriasis lichenoides (scaly red to brown papules and macules) in whom there were histopathological findings of mycosis fungoides (disproportionate epidermotropism, Pautrier's microabscesses, and wiry and coarse collagen bundles). Immunohistochemical staining revealed a prevalence of T lymphocytes in the infiltrate. T-cell receptor gene rearrangement analysis in lesional skin demonstrated rearrangement of the gamma chain in all cases. Human T-cell lymphotropic virus type 1 serology was negative in the two patients in whom this test was performed. Thus, lesions resembling pityriasis lichenoides can be an unusual and potentially misleading presentation of mycosis fungoides.

Child↗

Pustular mycosis fungoides.

The clinical presentations of cutaneous T-cell lymphoma (mycosis fungoides) are quite variable. Atypical manifestations of cutaneous T-cell lymphoma include bullous, granulomatous, hypopigmented, verrucous, and pustular variants. We report a patient who had a psoriasiform dermatitis of the palm and soles which showed pustulation.

Combined Modality Therapy↗

Widespread cutaneous candidiasis and tinea infection masking mycosis fungoides.

A 71-year-old female with a widespread double mycotic infection caused by C. Albicans and T. rubrum was discovered to be suffering from mycosis fungoides. Clinically she was found to have large, polycyclic erythematous plaques with scaly, slightly infiltrated borders, covering almost all areas of the glabrous skin, and also involving the scalp (with no hair penetration), the soles and palms, toe-webs, finger and toe nails; there was also perleche and oral thrush. Cultures yielded C. albicans from most of the skin lesions, from the scalp, mouth, finger nails and urine and stool specimens, and T. rubrum from intermingled skin specimens, from the palms and soles and toe-nails. Blood culture was negative as were intracutaneous tests with fungal antigens and tuberculin. Histological examination confirmed the fungal invasion of the horny layer and at the same time revealed an underlying pathologic picture of mycosis fungoides, the lesions having been masked by the mycotic eruption. Intensive cytostatic and antifungal therapy led to a transient improvement but shortly thereafter there was a relapse of the fungal and lymphoproliferative manifestations and the patient died in septic shock.

Aged↗