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PubMed1985

Mycosis fungoides with multiple oral mucosal lesions. A case report.

Mycosis fungoides is a cutaneous T-cell lymphoma which in its later stages affects extracutaneous sites, including the oral mucosa. A case is presented in a 69-year-old male in which oral involvement was widespread and rapidly progressive. Although gingival involvement, as seen in this patient, is relatively rare, it is useful for the periodontist to be aware of the oral manifestations of this disease since cutaneous lesions would most likely be covered if the patient were to present for consultation on an ambulatory basis.

Aged↗
PubMed1987

The value of morphometry in the ultrastructural diagnosis of Mycosis fungoides.

To assess the value of morphometry in the ultrastructural diagnosis of Mycosis Fungoides (MF), skin biopsy specimens from 43 patients with established MF and 28 with non-MF lymphoid skin infiltration were studied by transmission electron microscopy. Morphometrical measurements were done on the nuclei of the infiltrating lymphoid cells. The study showed that the nuclear contour index (NCI) taken alone has a very poor diagnostic value. A more reliable way to assess morphometrically the nucleus of MF cells is to combine two parameters: the nuclear surface area and the NCI, as proposed by McNutt and his group. Although the sensitivity of this method is only 43% (McNutt et al. obtained a sensitivity of 63%, analyzing a smaller number of patients), it surprisingly has a highly significant prognostic value. Most of the patients classified as positive by this method had a very aggressive course of their disease or died from it, while most of the patients who were considered as negative had an indolent course of disease or were at a stage of remission. It is suggested that other cellular expressions may be of importance in the recognition of MF cells. It is therefore proposed that, in combination with ultrastructural immunocytochemistry, such parameters be assessed. It is hoped that the results of such a study will determine the number of parameters necessary to better recognize MF cells, thus making early diagnosis of the disease possible.

Cell Nucleus↗
PubMed1979

Electron beam therapy of mycosis fungoides.

Sixteen patients with mycosis fungoides were treated with a 3.3 MeV skin electron beam to a dose of 30 Gy over 40 days. Nine patients achieved a complete remission which was generally short. Only two patients remained free of disease one year following treatment. All patients received palliative benefit from treatment, but no significant increase in survival can be anticipated.

Adult↗
PubMed2001

Composite lymphoma of Hodgkin lymphoma and mycosis fungoides: previously undescribed in the same extracutaneous site.

Mycosis fungoides (MF), diagnosed and limited to the skin, has been associated with the subsequent development of Hodgkin lymphoma (HL), most commonly of the nodular sclerosing (NS) subtype. In the previously described cases, there are none in which the extracutaneous tissue was simultaneously involved by HL and residual/relapsing MF. Here we report a case of HL, mixed cellularity (MC) subtype, arising in an inguinal lymph node in a patient with a previous diagnosis of MF. We describe the immunophenotypic, histologic and immunohistochemical findings of a composite lymphoma containing the HL, MC subtype and MT. The importance of detecting MF in addition to the HL in the extracutaneous site with available diagnostic modalities is highlighted.

Aged↗
PubMed1993

Total skin electron irradiation: efficacy in early mycosis fungoides.

The rare, indolent, but lethal malignancy, mycosis fungoides (MF), is amenable to durable remissions if treated topically at an early stage with nitrogen mustard, PUVA, or radiotherapy. A modification of conventional therapeutic irradiation which utilizes electron beams rather than photons, has been in use since 1951. This method, termed total skin electron irradiation (TSEI), has achieved consistently good CR rates (95-100%) at a variety of centres in the U.S.A., England, France, and Italy, despite troublesome differences in staging systems. In northern Israel we have treated 37 MF patients with TSEI during the past 13 years. All 21 of our early stage patients achieved CR, which is no longer regarded as an unusual result. However, most workers in the field acknowledge that issues of optimal dosing and curative potency remain unresolved.

Adult↗
PubMed1998

Total skin electron irradiation in mycosis fungoides dose and fractionation considerations.

This study was undertaken to analyze the influence of total skin dose and dose-fractionation schedules on the response rate, survival and skin toxicity of patients with mycosis fungoides [MF] treated with total skin electron irradiation [TSEI]. From 1979 to 1992, 40 patients with MF were treated with TSEI using a modified Christie Hospital technique. Mean follow-up time was 48 months [median 20 months]. 37/40 patients completed TSEI; three died due to non-treatment-related conditions during therapy. 34/37 [92%] treated patients achieved complete remission [CR] and 16/40 [40%] are alive with no evidence of disease. Over the years, changes in dose-fractionation schedules were made and correlated with the pattern of CR and skin toxicity. The 5-year actuarial survival [Stanford staging] was 84% in Stages IA-IB [all Stage IA patients are alive] and 59% in Stage II. The probability of survival of Stage III-IV patients was 30% at 30 months. Late skin toxicity was mild to moderate in 60% and severe in 25% of patients. A reduction of the total dose and dose-per-fraction resulted in an acceptable CR rate and a significantly lower toxicity. TSEI is effective in early stage MF. Skin control and late skin toxicity seem to be dose-fractionation-schedule related. For the early stages, the optimal treatment schedule seems to be 24-30 Gy to the whole skin surface in 2.4-3.0 Gy fractions, given twice weekly over a period of four to six weeks. Total doses of 24-30 Gy at 2.4-3.0 Gy per fraction yielded comparable skin control rates with lower skin toxicity.

Adult↗
PubMed2026

Established and emerging prognostic factors in mycosis fungoides and Sézary syndrome.

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common subtypes of cutaneous T-cell lymphoma (CTCL), characterized by heterogeneous clinical behavior and variable prognosis. Accurate prognostication is essential for risk-adapted management. This review synthesizes emerging evidence on prognostic factors in MF and SS, with a focus on recent genomic advances. Traditional prognostic frameworks are outlined, highlighting the prognostic impact of demographics, stage, clinical features, and histologic findings. More advanced prognostics are then outlined, including genomic alterations and impact of the tumor microenvironment. We highlight realms in which integration of traditional and more biological prognostic frameworks can support more individualized treatment strategies.

Sézary syndrome↗
PubMed2005

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.

Aged↗
PubMed1994

Systemic therapy of cutaneous T-cell lymphomas (mycosis fungoides and the Sézary syndrome).

OBJECTIVE: To review recent studies of systemic therapy for mycosis fungoides and the Sézary syndrome (cutaneous T-cell lymphomas). DATA SOURCES: English-language articles indexed in MEDLINE from 1988 through 1994. STUDY SELECTION: All therapeutic studies were selected. DATA EXTRACTION: The data were abstracted without judgments on response criteria or patient numbers. Data quality and validity were assessed by independent author reviews. DATA SYNTHESIS: No systemic therapy cures patients with cutaneous T-cell lymphomas. Single and combined chemotherapeutic agents produce high response rates. Whether any of these is preferred is not established. A randomized trial comparing combination chemotherapy plus radiation therapy with topical therapy showed no survival benefit for the combination. Several adenosine analogs and retinoids were active, but their optimal use is uncertain. Interferons are as active as chemotherapeutic agents and may be less toxic. Interferon combined with psoralen plus ultraviolet A light therapy produces high complete response rates and long-lasting remissions. Combinations with other systemic therapies do not increase response rates. Photopheresis therapy should be regarded as experimental. Promising preliminary results were seen with interleukin-2 fusion toxins and several antibody conjugates. CONCLUSIONS: Systemic therapy should be considered effective and palliative. The principles of treating all low-grade lymphomas can be applied. Randomized trials are needed to evaluate new agents (such as a comparison of psoralen plus ultraviolet light with or without interferon), and large phase II trials are needed for new agents such as photopheresis, interleukin-2 fusion toxin, temozolomide, and others.

Combined Modality Therapy↗
PubMed1975

Cutaneous T-cell lymphomas: the Sézary syndrome, mycosis fungoides, and related disorders.

Substantial evidence has accumulated to indicate not only that mycosis fungoides and the Sézary syndrome are closely related malignancies, but to suggest that they are part of a larger spectrum of cutaneous lymphomas. The neoplastic cells of these disorders have membrane features of thymus-derived (T) lymphocytes, a characteristic tissue distribution (skin infiltration, marrow sparing, localization in T-cell regions of lymphoid tissue), and distinctive morphology. For these reasons, we suggest that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas". The anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity. Leukapheresis has been particularly effective in the management of selected patients. The homogeneous T-cell populations in the patients with leukemia also provide important opportunities to study many aspects of lymphocyte physiology that are of broad biologic significance.

Cell Membrane↗
PubMed1992

Parathyroid hormone related protein is localized in the granular layer of normal skin and in the dermal infiltrates of mycosis fungoides but is absent in psoriatic lesions.

Biopsies of normal and diseased skin were immunohistochemically investigated for the presence of parathyroid hormone-related protein (PTH-rp). In normal skin and several skin disorders a monoclonal antibody against the 34-68 sequence of PTH-rp was found to be exclusively located in the granular layer. PTH-rp could not be detected in untreated psoriatic plaque lesions even when a granular layer was present. Psoriatic lesions improving after 1-2 weeks' treatment with betamethasone or vitamin D3 analogue revealed PTH-rp reactivity just above the granular layer. These findings substantiate a possible role for PTH-rp as a growth inhibitor. In the dermis the granular layer in the upper part of the hair follicles was stained for PTH-rp and the dermal infiltrates in 5 of 10 patients were stained with mycosis fungoides.

Humans↗
PubMed1998

Lymphomatoid papulosis in association with mycosis fungoides: a study of 15 cases.

We report clinical findings in 15 patients with lymphomatoid papulosis (LyP) associated with mycosis fungoides (MF). LyP either preceded (n = 4), followed (n = 5) or occurred concurrently with the MF lesions (n = 6). Twenty-eight LyP lesions were classified histologically and analysed further with immunostaining for CD3 and CD30. Five biopsies contained a predominance of type A cells, six biopsies contained a predominance of type B cells. and six were mixed (A + B). However, 11 biopsies contained a population of atypical mononuclear cells with large hyperchromatic nuclei that we have termed indeterminate cells. These cells contained a thin rim of eosinophilic cytoplasm and showed strong CD30 but absent, faint or normal CD3 staining. In seven biopsies from five separate patients these cells represented the predominant cell type and we have termed this the pleomorphic variant of LyP. Analysis of T-cell receptor genes using Southern blot analysis and polymerase chain reaction/single strand conformational polymorphism analysis identified a T-cell clone in six of 16 LyP lesions and nine of 16 MF lesions. In the three patients who had clones in both types of skin lesions, the clones were identical. Only two of 10 blood samples, both of which were from the same patient, had a T-cell clone and none of two lymph nodes showed evidence of a clonal population. To date all patients are alive with a median follow-up of 15 years from the onset of the first lesion. One patient has developed Lin anaplastic large cell lymphoma of the nasopharynx. These data augment the current literature on the association of LyP and MF and suggest that the

Adolescent↗
PubMed1994

Mycosis fungoides--an underwriting prospective with emphasis on staging and risk selection.

Mycosis Fungoides is a T-cell lymphoma having a broad clinical spectrum ranging from localized cutaneous to rapidly fatal systemic disease. Early clinical presentation is non specific, delaying correct diagnosis. Compared to clinical, the insurable spectrum is narrow. Staging for skin, lymph node and other organ manifestations is presented. Factors which influence mortality within each stage are elucidated. The survival curves of stages and stage groupings are illustrated and discussed to facilitate risk classification. Cutaneous (T) and lymph node (LN) stages are the most important prognosticators. Substages T1/T2, LN1/LN2 without associated palpable adenopathy, eosinophilia, visceral and blood positive findings are insurable. It would be most appropriate to place them in a tumor class of mild/moderate risk after the initial excessive mortality period ends. Higher T and LN substages, adenopathy and visceral disease have highly adverse mortality. These ultimately reveal a flattening of survival curves at 8-10 years. Although numerous treatment modalities have been used, none appear to consistently prolong life expectancy except in the earliest stage skin disease.

Actuarial Analysis↗
PubMed1999

Multinucleate-cell angiohistiocytoma occurring in a patient with mycosis fungoides.

Multinucleate-cell angiohistiocytoma (MCAH) is a benign vascular proliferation of unknown etiology. Clinically, MCAH presents as grouped, erythematous, or violaceous papules on the extremities in older women. These lesions often resemble Kaposi's sarcoma. Histologic examination reveals characteristic bizarre-shaped, multinucleate giant cells, some of which contain three to six nuclei arranged in a ring-like or overlapping pattern, which stain positively for Factor XIIIa. In addition, there is a proliferation of dermal capillaries and venules with a mild lymphohistiocytic infiltrate. To our knowledge, this is the first reported case of MCAH occurring in a patient with mycosis fungoides.

Diagnosis, Differential↗
PubMed1999

Allergic contact dermatitis from transdermal clonidine in a patient with mycosis fungoides.

Although therapy with transdermal clonidine is considered an effective method of hypertension control, this mode of delivery has been associated with localized dermal reactions in numerous patients. We present a patient with coexistent mycosis fungoides and allergic contact dermatitis from transdermal clonidine. The association of these two dermatologic processes has not been previously reported.

Administration, Cutaneous↗
PubMed1999

Prognostic factors in primary cutaneous lymphomas other than mycosis fungoides and the Sézary syndrome. The French Study Group on Cutaneous Lymphomas.

Prognostic studies of primary cutaneous lymphomas (PCL) other than mycosis fungoides (MF) and the Sézary syndrome (SS; non-MF/SS PCL) have been mainly performed on subgroups or on small numbers of patients by using univariate analyses. Our aim was to identify independent prognostic factors in a large series of patients with non-MF/SS PCL. We evaluated 158 patients who were registered in the French Study Group on Cutaneous Lymphomas database from January 1, 1986 to March 1, 1997. Variables analyzed for prognostic value were: age; sex; type of clinical lesions; maximum diameter, location, and number of skin lesions; cutaneous distribution (ie, local, regional, or generalized); prognostic group according to the European Organization for Research and Treatment of Cancer (EORTC) classification for PCL; B- or T-cell phenotype; serum lactate dehydrogenase (LDH) level; and B symptoms. Univariate and multivariate analyses were performed using a model of relative survival. Forty-nine patients (31%) died. The median relative survival time was 81 months. In univariate analysis, EORTC prognostic group, serum LDH level, B symptoms, and variables related to tumor extension (ie, distribution, maximum diameter, and number of skin lesions) were significantly associated with survival. When these variables were considered together in a multivariate analysis, EORTC prognostic group and distribution of skin lesions remained statistically significant, independent prognostic factors. This study confirms the good predictive value of the EORTC classification for PCL and shows that the distribution of skin lesions at initial evaluation is an important prognostic indicator.

Adolescent↗
PubMed1999

Mycosis fungoides and the Sézary syndrome.

Mycosis fungoides (MF) and the Sézary syndrome are a group of extranodal non-Hodgkin's lymphomas of T-cell origin with primary cutaneous involvement. The group distinguishes itself from other primary cutaneous T-cell lymphomas (CTCLs) by its unique clinical features and histopathology. In its early stages, it often resembles common benign dermatoses, and therefore, a definitive diagnosis can be delayed. The affected T cells are characterized by a predominant CD4+ phenotype with frequent loss of CD7 (pan-T-cell antigen) and often demonstrate T-cell receptor (TCR) rearrangement. The prognosis of patients with MF is highly dependent on the extent and type of skin involvement. The initial cutaneous presentation of MF can be patches, plaques, tumors, or erythroderma. Patients who present with limited patch/plaque disease have an outstanding prognosis with an overall long-term survival that is similar to the expected survival of a matched control population. It is exceedingly rare for patients who present with limited or generalized patch/plaque disease without peripheral lymphadenopathy to have extracutaneous involvement. Therefore, the staging evaluation differs for patients with MF versus patients with other non-Hodgkin's lymphomas and should be tailored to the clinical presentation. Patients who have tumorous or erythrodermic skin involvement have a less favorable prognosis, and patients who present with extracutaneous disease have a poor prognosis. There are multiple therapeutic options for patients with MF and the Sézary syndrome. Selection of a specific treatment plan is based primarily on the clinical stage of the disease. The primary therapy for patients with patch/plaque disease without extracutaneous involvement is a topical regimen, whereas chemotherapy or other aggressive systemic regimens are reserved for those with recalcitrant disease or extracutaneous involvement. There is no evidence that early aggressive systemic therapy is preferable to conservative therapy in the management of limited disease. There are newer combination topical and/or systemic regimens that result in an improved clinical response and possibly a prolonged response duration. For advanced disease, standard therapies are often palliative and successful clinical response is often very short-lived. Therefore, all patients with recalcitrant or extracutaneous disease should be considered for newer investigative therapies.

Humans↗