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PubMed1987

[Total cutaneous irradiation with low energy electrons and mycosis fungoides. Technic and dosimetry].

Mycosis fungoides initially involves the epidermis and the superficial layers of derma at a depth of about 1 cm. Wide field irradiation with low energy electrons is therefore the treatment of choice in the initial stages of the disease. In our Institute, total skin electron beam irradiation is delivered with Therac 20 linear accelerator: the lowest available energy is 6 MeV. A lucite sheet of 0.6 cm thickness is used to decrease the energy of the beam. We used film dosimetry to evaluate the homogeneity of dose distribution in an Alderson-Rando phantom with different arrangements of the fields and the lucite sheet: 4 and 6 fields techniques have been compared with different positions of the lucite filter, near the phantom and near the collimator. Six fields yield a better dose distribution: homogeneity is within +/- 3.7%, while with four fields it is within +/- 6%. X-rays contamination is less than 2%. "In vivo" dosimetry has been performed using thermoluminescent dosimeters: homogeneity is within +/- 15%.

Electrons↗
PubMed1979

Combined therapy for patients with mycosis fungoides.

In a comprehensive mycosis fungoides program, 60 patients have been seen with a pathologic diagnosis of this disease. Forty-four patients with advanced disease were referred for radiation therapy. Three treatment techniques were identified in which 14 patients were treated with localized fields using electrons or whole-body electron-beam therapy with doses of less than 3000 rads, 21 patients were treated using the Stanford technique with tissue doses of between 3000 and 4000 rads, and nine patients were treated with six cycles of mechlorethamine, vincristine, prednisone, and procarbazine or cyclophosphamide, vincristine, prednisone, and procarbazine following the electron-beam therapy. The actuarial survival rate was 45% at 1 year for the 14 patients with localized electron-beam therapy, whereas the actuarial survival rates were 83% for patients treated with whole-body electron-beam therapy and 100% for patients treated with whole-body electron-beam therapy followed by four-drug chemotherapy. The recurrence-free interval for these three groups correlates with these observations. A central nervous system recurrence has been observed in the combined-therapy group.

Adult↗
PubMed1998

Comparative analysis of histological and immunohistological features in mycosis fungoides and Sézary syndrome.

Mycosis fungoides (MF) and Sézary syndrome (SS) are closely related cutaneous T-cell lymphomas, which differ in several clinical aspects. We compared histological and immunophenotypical features of these two entities, which could be implicated in the dermotropism and epidermotropism, characteristic for both of them. Thirteen biopsy specimens from patients with established plaque-stage MF and 13 from SS patients were examined retrospectively, and 21 histological criteria were assessed. Further, 9 cryosections from MF lesions and 9 from SS lesions were stained for LFA, ICAM1, CD40, CD40-ligand, CD28, CD80, CTLA-4, CD86, FAS, FAS-ligand, CLA and CD15s. The only histological criteria that showed persistent differences were acanthosis (in 12 of 13 SS and in 7 of 13 MF specimens) and Pautrier collections (detected in 6 SS and 11 MF biopsies). Patterns of staining with the antibodies mentioned above were found to be similar. Our results indicate that these interaction molecules seem to be involved in the pathogenesis of MF and SS, but their immunohistochemical distribution does not contribute to the differentiation between the two entities.

Abatacept↗
PubMed2007

Does adjuvant alpha-interferon improve outcome when combined with total skin irradiation for mycosis fungoides?

BACKGROUND: Patients with mycosis fungoides (MF) experience frequent disease recurrences following total skin electron irradiation (TSEI) and may benefit from adjuvant therapy. OBJECTIVES: To review the McGill experience with adjuvant alpha-interferon (IFN) in the treatment of MF. METHODS: From 1990 to 2000, 50 patients with MF were treated with TSEI: 31 with TSEI alone and 19 with TSEI + IFN. Median TSEI dose was 35 Gy. In the TSEI + IFN group, IFN was given subcutaneously at 3 x 10(6) units three times per week starting 2 weeks prior to start of TSEI, continued concurrently with the radiation and for an additional 12 months following TSEI. The TSEI alone group included 16 men and 15 women with a median age of 61 years (range 31-84). The TSEI + IFN group included 14 men and five women with a median age of 51 years (range 24-83). Clinical stage was IA, IB, IIA, IIB, III and IVA in 2, 9, 4, 8, 1 and 7 patients of the TSEI group and 0, 3, 3, 7, 4 and 2 patients of the TSEI + IFN group. RESULTS: Median follow up for living patients was 70 months. All patients responded to treatment. Complete response (CR) rate was 65% following TSEI and 58% following TSEI + IFN (P = 0.6). Median overall survival (OS) was 61 months following TSEI and 38 months following TSEI + IFN (P = 0.4). Acute grade II-III dermatitis was seen in all patients. Fever, chills or myalgia were seen in 32% of patients treated with TSEI + IFN. CONCLUSIONS: Concurrent IFN and TSEI is feasible, with acceptable toxicity. Even when controlling for disease stage, the addition of IFN did not appear to increase CR rate, disease-free survival or OS.

Adult↗
PubMed1997

Total skin electron beam therapy followed by adjuvant psoralen/ultraviolet-A light in the management of patients with T1 and T2 cutaneous T-cell lymphoma (mycosis fungoides).

PURPOSE: Patients with mycosis fungoides [cutaneous T-cell lymphoma (CTCL)] may benefit from adjuvant therapy after completing total skin electron beam therapy (TSEBT). We report the results for T1/T2 CTCL patients treated with adjuvant oral psoralen plus ultraviolet light (PUVA) with respect to overall survival (OS), disease-free survival (DFS), salvage of recurrence, and toxicity. METHODS AND MATERIALS: Between 1974 and 1993, TSEBT was administered to a total of 213 patients with CTCL. Records were reviewed retrospectively, and a total of 114 patients were identified as having T1 or T2 disease. Radiotherapy was provided via a 6-MeV linac to a total of 36 Gy, 1 Gy/day, 4 days/week, for 9 weeks. Beginning in 1988, patients were offered adjuvant PUVA within 2 months of completing TSEBT. This was started at 0.5-2 J/m2, 1-2 treatments/week, with a taper over 3-6 months. Therapy then continued once per month. There were 39 T1 and 75 T2 patients. Six T1 (15%) and eight T2 (11%) patients were treated with adjuvant PUVA. A further 49% of the 114 patients received adjuvant systemic therapy, 3% received spot external beam, 4% received adjuvant ECP, 2% received topical nitrogen mustard, 22% received a combination of therapies exclusive of PUVA, and 9% received no adjuvant therapy. Patients were balanced in all subgroups based on pre-TSEBT therapy. The median age of the cohort was 58 (range 20-88), with a median follow-up time of 62 months (range 3-179). RESULTS: Within 1 month after completing of TSEBT, 97% of T1, and 87% of T2 patients had achieved a complete remission. Stratified by adjuvant therapy, none of six T1 and one of eight T2 patients who received adjuvant PUVA failed within the first 3 years after completion of TSEBT. A total of 43% of the T1 and T2 patients receiving other or no adjuvant treatment failed within the same time course. The 5-year OS for the entire cohort was 85%. Those who received PUVA had a 5-year OS of 100% versus a 5-year OS for the non-PUVA group of 82% (p < 0.10). The 5-year DFS for the entire cohort was 53%. Those who received PUVA had a 5-year DFS of 85% versus a 5-year DFS for the non-PUVA group of 50% (p < 0.02). By T stage, those with T1 receiving PUVA exhibited no relapses, whereas those with T1 not treated with PUVA had a crude relapse rate of 36%. Median DFS was not reached at 103 months for the T1 adjuvant PUVA patients versus 66 months for the non-PUVA patients (p < 0.01). For those with T2, crude relapse rates were 25% and 55%, respectively, with DFS of 60 (median DFS not reached) and 20 months (p < 0.03). The 5-year DFS for patients salvaged with PUVA was 50%. Toxicity of adjuvant and salvage PUVA therapy was acceptable, with only two patients requiring a reduction in PUVA dosage. CONCLUSION: PUVA can maintain remissions in patients with CTCL after TSEBT. There is a significant benefit in DFS but no statistically significant improvement in OS. Prospective, randomized data are needed to confirm these results. PUVA is also effective as a salvage therapy after TSEBT in early-stage patients with recurrence, with acceptable toxicity.

Administration, Oral↗
PubMed1975

Immunoblastic sarcoma with leukemic blood picture in the terminal stage of mycosis fungoides.

A 76 year old man with mycosis fungoides developed an immunoblastic sarcoma and a leukemic blood picture in the final tumor stage after 6 years, in which the disease had clinically progressed in a typical manner. The results of histological and cytochemical studies of autopsy material are presented. Based on these findings and evidence of the T cell nature of mycosis fungoides, the immunoblastic sarcoma observed in the terminal stage of this case of mycosis fungoides might be of the rare T cell type.

Acid Phosphatase↗
PubMed1997

Spontaneous regression of granulomatous mycosis fungoides in an HIV positive patient.

The development of mycosis fungoides in HIV-positive patients is uncommon. We describe the granulomatous type of mycosis fungoides in an HIV-positive patient whose cutaneous lesions regressed without treatment. In addition to HIV seropositivity and mycosis fungoides, he had a long history of pulmonary sarcoidosis. Immunophenotyping of the cutaneous lymphoid infiltrates revealed a predominance of CD4+ T cells, and monoclonality was demonstrated by T-cell gene rearrangement studies. The regression of mycosis fungoides in this patient was associated with a falling peripheral blood CD4 T-cell count.

Adult↗
PubMed1989

[The significance of epidermal changes in the early diagnosis and development of mycosis fungoides].

In 47 patients with stage I mycosis fungoides, biopsy of skin has revealed substantial changes in epidermis acanthosis with confluent epidermal processes, focal dystrophy of basal cells, mitoses in various epidermal layers, parakeratotic foci without a granular layer. Histoautoradiographic and immunomorphological studies demonstrated that enhanced proliferative activity of keratinocytes that was more pronounced in stages I and II mycosis fungoides and impaired epidermal differentiation that was predominant in neoplastic stage III underlay the above changes. There was ultrastructural evidence for abnormal epidermal keratinization. It was proposed that epidermal abnormalities made an important contribution to the development of mycosis fungoides.

Fluorescent Antibody Technique↗
PubMed1987

Light microscopic, S-100 immunostaining, and ultrastructural analysis of dermatopathic lymphadenopathy, with and without associated mycosis fungoides.

Thirty-five patients with dermatopathic lymphadenopathy, 12 with (9 patients and 3 follow-up) and 23 without associated mycosis fungoides, and 7 controls with 26 lymph nodes were studied by light microscopic examination and immunostaining for S-100 protein. Fourteen of these cases were examined ultrastructurally. The group of cases studied included lymph nodes obtained at postmortem examination from three patients with mycosis fungoides and evidence of nodal involvement by light microscopic examination. Lymph nodes from patients with dermatopathic lymphadenopathy and paracortical hyperplasia (but no effacement of architecture by recognizable mycosis fungoides cells by light microscopic examination) associated with biopsy-proven mycosis fungoides showed well-defined, diffusely distributed sheets of S-100-positive cells. Cases of dermatopathic lymphadenopathy unassociated with a skin lymphoma (23 cases), with the exception of 3 cases (12.5%), only showed scattered S-100-positive cells, a similar pattern to that noted in normal or reactive lymph nodes from the control cases and reported literature. In the three cases with diffuse sheets without associated mycosis fungoides, the intensity of S-100 staining was the same in germinal centers as in parafollicular areas, while in cases associated with mycosis fungoides, the staining was predominantly in parafollicular zones. Ultrastructural immunolabeling for S-100 protein also revealed different patterns in both subsets of patients. Interestingly, in cases in which the lymph nodes were identified by light microscopic examination to be replaced by mycosis fungoides, the sheets of S-100-positive cells disappeared. The corresponding ultrastructural evaluation showed cellular aggregates with features of T-cells. A combination of S-100 immunocytochemistry and morphologic ultrastructural assessment can be of help in evaluation of lymphadenopathy in patients with mycosis fungoides. Although this study indicates that the finding of sheets of S-100-positive cells in patients with dermatopathic lymphadenopathy is not always associated with mycosis fungoides, the identification of diffuse sheets of S-100-positive cells, especially with parafollicular distribution, in a patient without known T-cell lymphoma should probably be considered an indication to suggest a complete clinical evaluation to rule out the possibility of an undiagnosed mycosis fungoides. The finding of sheets of S-100-positive cells in patients with proven mycosis fungoides may be a morphologic indicator of impending extracutaneous dissemination.

Dermatitis↗
PubMed2003

Management of mycosis fungoides. Part 1. Diagnosis, staging, and prognosis.

Mycosis fungoides is a low-grade lymphoproliferative disorder caused by CD4+ lymphocytes. It is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumors, or erythroderma. Diagnosis of early mycosis fungoides can be difficult due to the nonspecific nature of cutaneous and histologic findings. However, recent advances in the application of histologic criteria, coupled with molecular biology tools such as immunophenotyping and polymerase chain reaction, have improved diagnostic accuracy. Independent prognostic factors include the extent and nature of skin involvement, the presence of extracutaneous disease, blood involvement, age > or = 60 years, and lactate dehydrogenase elevation. Accordingly, patients with limited patches and/or plaques (stage IA or IIA) experience long-term survival comparable to that of matched controls. The median survival is 11 years for patients with extensive patch/plaque (stage IB or IIA), 3.2 years for those with cutaneous tumors (stage IIB), 4.6 years for those with erythroderma (stage III), 1.2 years for those with pathologic nodal involvement (stage IVA), and 0.9 years for those with visceral disease (stage IVB). Over time, mycosis fungoides may progress to Sézary syndrome or transform to large-cell histology.

CD4-Positive T-Lymphocytes↗
PubMed1991

Mycosis fungoides metastatic to the orbit.

We report a case of mycosis fungoides metastatic to the anterior orbit in an 83-year-old woman. The patient had a history of several years' duration of well-controlled primary mycosis fungoides with localized dermatologic T-cell lymphomas, mainly on the lower extremities. She developed gradual proptosis, hyperophthalmos, and vertical diplopia. Computed tomography demonstrated a solid, extraconal mass in the right lower anterior orbit. Biopsy revealed a gristly, yellowish, infiltrating mass at the level of the inferior orbital septum. Pathologic examination revealed anaplastic T lymphocytes with multiloculated, cerebriform nuclei that matched those of the primary mycosis fungoides skin tumors. Mycosis fungoides rarely spreads to the ocular and periocular structures, and this case was unique in that it presented as a pure orbital lesion with sparing of the overlying lids and adnexal structures.

Aged↗
PubMed1981

Ocular findings in mycosis fungoides.

Thirty consecutive patients with biopsy-proved mycosis fungoides were examined ophthalmologically. Specific ocular changes, judged directly related to the mycosis fungoides, were found in 11 of the 30 subjects, usually in the late plaque or tumor stages of the disease. Tumors, especially involving the lids, were the most common ophthalmic finding. Keratitis, uveitis, and optic atrophy were also described. A review of the literature is given, and changes found in patients in this series correlate well with ophthalmic findings presented in diverse isolated case reports. It seems that the frequency of ophthalmic pathologic findings in mycosis fungoides is much more common than previously assumed.

Aged↗
PubMed2003

Juvenile mycosis fungoides diagnosed before 18 years of age.

The literature regarding mycosis fungoides in children is sparse. To shed further light on the characteristics of mycosis fungoides in the paediatric population we analysed the clinicopathological features of 10 patients in whom this malignancy was diagnosed before the age of 18 years. All were Jews and Arabs with histologically proven patch/early plaque stage disease: 4 in stage IA, 4 in IB and 2 with unilesional disease. Seven patients had hypopigmented lesions either constituting the sole manifestation (2 patients) or in combination with classic lesions (5 patients); of these, 3 had light skin and 4 pigmented skin. Most patients had immunohistochemical features characteristic of mycosis fungoides, with a predominance of CD4+ T cells. Some had deletion of CD7+ cells. In 3 patients, however, the epidermotropic cells were exclusively or predominantly CD8+ cells. All patients responded to conventional therapy and during an average follow-up of 3.4 years only one patient showed stage progression, but without extracutaneous involvement. It is concluded that juvenile mycosis fungoides is characterized by early stage disease, occasionally with unilesional disease, usually with hypopigmented lesions irrespective of skin colour, and a good response to therapy. On the basis of our experience and review of the literature, it appears that the CD8+ phenotype is over-represented in juvenile disease.

Adolescent↗
PubMed1998

Mycosis fungoides metastasizing to the brain parenchyma: case report.

OBJECTIVE AND IMPORTANCE: Mycosis fungoides is a rare T-cell lymphoma of the skin that can, in one-half to three-quarters of patients suffering from this disease, involve the viscera in late stages of the disease. Although autopsy series performed more than 2 decades ago showed that the incidence of metastatic mycosis fungoides to the central nervous system is approximately one of seven, a total of only several dozen cases have been reported to date. As compared to meningeal involvement, intraparenchymal metastases are even rarer. We describe a biopsy-proven case of intraparenchymal central nervous system mycosis fungoides in a patient with nonprogressive skin involvement and no detectable visceral involvement, and we present a review of the relevant literature. CLINICAL PRESENTATION: A 68-year-old man, 3 years after the diagnosis of his skin disease, developed fatigue, confusion, and frontal lobe signs without the presence of cerebriform cells in the peripheral blood or any other clinical evidence of visceral involvement. Magnetic resonance imaging revealed a diffuse area of increased T2-weighted signal involving the white matter of both cerebral hemispheres as well as a focal area of T2 abnormality along the body of the corpus callosum. The radiological differential diagnosis was either leukodystrophy caused by chemotherapy, progressive multifocal leukoencephalopathy, or glioma with associated white matter changes. INTERVENTION: A stereotactic serial brain biopsy revealed diffuse perivascular infiltrates of atypical lymphocytes, as well as several large cells with cerebriform nuclei consistent with mycosis fungoides. The cells were immunoreactive for LCA, MT1, UCHL1, and CD3. CONCLUSION: We stress the importance of including mycosis fungoides as part of the differential diagnosis for a brain lesion in patients with cutaneous T-cell lymphoma, because treatments do exist, and we conclude that a serial stereotactic biopsy may be necessary to provide a definitive diagnosis.

Aged↗
PubMedDate not supplied

Central neurologic involvement in mycosis fungoides: ten cases, actuarial risk assessment, and predictive factors.

BACKGROUND: Neurologic involvement in mycosis fungoides is rare. Isolated case reports in the literature suggest the pattern and the natural history for such occurrences, while a literature summary can provide direction on diagnosis and management. Although case series may confirm such information, cohort data are required to establish an overall risk of occurrence and to evaluate possible predictive factors. METHODS: We presented a case of central nervous system involvement in mycosis fungoides from Haifa, Israel and tabulated a series of nine cases from Canada. To estimate the risk of neurologic involvement, a cohort of 680 consecutive patients with newly diagnosed mycosis fungoides, of which the nine cases of neurologic involvement emerged during follow up, was analyzed using the Kaplan-Meier method. The actuarial risk of developing neurologic involvement was related to the baseline tumor-node-metastasis-blood classification factors. RESULTS: The pattern of disease in these 10 additional cases confirms the overall pattern in the approximately 40 patients described in the literature. The main symptoms are fluctuating higher cognitive functions and cranial nerve dysfunction, with fairly rapid clinical onset of symptoms. Most cases of central neurologic involvement with mycosis fungoides emerge within a setting of advanced disease. In patients with newly diagnosed mycosis fungoides, the greatest risk of developing neurologic involvement is within the first several years after diagnosis and is associated with the initial stage of disease. Patients with two or more of the T3-4, N3, M1, and B1 classification factors have a one in six chance of developing central neurologic involvement, while there is about a one in a hundred chance for the corresponding control group. CONCLUSIONS: Neurologic involvement with mycosis fungoides is indeed rare, but it is associated with a more advanced stage at diagnosis and with other visceral disease that can precede it. Although the role of low-dose prophylactic cranial radiation is uncertain, overt neurologic involvement requires urgent palliative treatment.

Actuarial Analysis↗
PubMed1991

Thy-1 and T-cell receptor antigen expression in mycosis fungoides and benign inflammatory dermatoses.

We have studied human Thy-1 and T-cell receptor (TCR) antigen expression in mycosis fungoides and benign inflammatory dermatoses. The study included 24 biopsy specimens from 21 patients with mycosis fungoides (nine patch stage from eight patients, 13 plaque stage from 11 patients, and two tumor stage from two patients), six specimens from five patients with premycotic parapsoriasis (pre-mycosis fungoides), three specimens from three patients with lichen planus, 11 specimens from 11 patients with lupus erythematosus, 13 specimens from 13 patients with dermatitis, six specimens from six patients with drug eruptions, nine normal skin specimens from nine subjects, and three specimens from three patients with small plaque (benign) parapsoriasis. Immunoperoxidase studies using the avidin-biotin complex technique on serial frozen sections were performed. Primary antibodies were anti-human Thy-1, anti-alpha heterodimer of the TCR, anti-beta heterodimer of the TCR, and anti-delta heterodimer of the TCR. An extensive dendritic network of Thy-1+ cells was seen in all cases of mycosis fungoides. Epidermotropic cells were Thy-1 negative, and Thy-1 was expressed perivascularly in normal individuals and patients as previously reported. Epidermal gamma/delta cells were seen only in mycosis fungoides, where up to 60% of the epidermal lymphocytes expressed this TCR. The increased numbers of Thy-1 and gamma/delta T cells in mycosis fungoides were statistically significant when compared with normal skin or benign inflammatory dermatoses. The role of these dendritic dermal Thy-1+ cells and epidermal gamma/delta T cells in mycosis fungoides is unclear. The significant numbers of these potentially immunomodulating cells that were seen suggest that they are involved in the pathogenesis of mycosis fungoides.

Antigens, Surface↗