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PubMedDate not supplied

Oral involvement in mycosis fungoides: report of two cases and a literature review.

Oral involvement is uncommon in cutaneous T-cell lymphomas and usually associated with poor prognosis. The clinicopathological and immunohistochemical findings of 2 new cases are described along with a literature review. The first patient had a 10-year history of mycosis fungoides when she developed lesions in the oral tissues. She died 6 months later despite treatment. The immunophenotype was CD3+, CD4+, CD8-, CD30-. The second patient had a mycosis fungoides for 5 years when she developed lesions in the uvula and oropharynx. She was treated with polychemotherapy and she is alive 5 years after oral involvement. The immunophenotype was CD3+, CD4-, CD8+, CD30-. There are conflicting reports about the prognosis in the CD8+ phenotype. The present cases and the literature review seem to indicate that in oral involvement the CD8- phenotype is not associated with a worse prognosis than the CD4+ subtype. However, it is necessary to study new cases to confirm this statement.

Aged↗
PubMed1986

Leu-M1 antigen expression in advanced (tumor) stage mycosis fungoides.

The authors describe two patients with clinically and histopathologically documented advanced (tumor) stage mycosis fungoides. In each case the large, pleomorphic neoplastic cells lacked the monoclonal antibody-defined cell surface antigens commonly associated with immature and mature T-cells, i.e., T11, Leu-1, T3, T4, T6, T8, and T10, but expressed various T-cell-associated activation antigens, such as HLA-DR, Tac, and T21. Leu-M1, a monocyte-associated antigen, was not expressed by the small, cerebriform neoplastic cells in the plaque stage lesions of either patient. However, Leu-M1 was expressed by most of the large, pleomorphic neoplastic cells present in the nodular lesions of both patients. The pattern of Leu-M1 antigen expression was identical to that previously reported in the Reed-Sternberg cells of Hodgkin's disease. Identification of these two patients suggests using caution in the interpretation of the results of immunophenotypic analysis of cutaneous lymphoid neoplasms and that Leu-M1 should not be used as a diagnostic indicator of Hodgkin's disease or a histiocytic-derived neoplasm. These studies also suggest that Leu-M1 may be preferentially expressed on a subpopulation of activated, rapidly proliferating, and/or dedifferentiated neoplastic T-cells that proliferate in the advanced (tumor) stages of mycosis fungoides.

Aged↗
PubMed1998

Validation of cause-of-death certification for outpatient cancers: the contrasting cases of melanoma and mycosis fungoides.

The reported incidence of cancers typically diagnosed in the outpatient setting may underestimate their true frequency, but the validity of mortality estimates from many of these causes has not been studied in detail. The authors sought to evaluate the validity of mortality estimation from death certificates for two such cancers, melanoma and mycosis fungoides (a cutaneous lymphoma), using routinely collected cancer registry data for 1973-1994 from the Surveillance, Epidemiology, and End Results program. Their method estimates that 93% of the deaths attributable to melanoma were certified as due to melanoma but that only 60% of the deaths attributable to mycosis fungoides were so certified. Evaluation of the accuracy of cause-of-death certification in this manner is helpful in the interpretation of mortality statistics.

Death Certificates↗
PubMed1985

Mycosis fungoides simulating acanthosis nigricans.

In this paper, an unusual papillomatous variant of the plaque stage of mycosis fungoides with clinical similarities to acanthosis nigricans is described. In contrast to the classic plaque stage of mycosis fungoides, the dermal infiltrates in this patient showed a rather monomorphous proliferation of blast-like cells that had little or no tendency to infiltrate the epidermis.

Acanthosis Nigricans↗
PubMed1988

Light-microscopic assessment of 100 patients with patch/plaque-stage mycosis fungoides.

The light-microscopic recognition of patch/plaque stages of mycosis fungoides is difficult, but remains as the "gold standard" for the diagnosis of mycosis fungoides (MF). A review of previous publications concerning the light-microscopic histological criteria for recognition of MF is followed by a summary of our recent histological findings involving 100 patients. The quantitative histological assessment included 13 different parameters for each case of patch/plaque-stage disease. The different criteria included parakeratosis, acanthosis, density of upper dermal mononuclear cell infiltrate, degree of lymphocyte atypia, degree of epidermotropism, Pautrier microabscess formation, band-like pattern, presence of basal vacuolar change, spongiosis, papillary dermal fibrosis, presence of eosinophils, and presence of plasma cells. The most significant histological features of patch/plaque-stage MF are: 1. Broad zones of epidermis in which lymphocytes, which are usually not markedly atypical, are in close apposition to basal and lower-level keratinocytes predominantly as single cells in a somewhat linear configuration on the epidermal side of the dermal-epidermal junction. In general, the surrounding keratinocytes do not display any cytopathic changes, i.e., basal vacuolar degeneration or apoptosis. 2. The papillary dermis contains a variably dense mono-nuclear cell infiltrate that is usually polymorphous and in which there is papillary dermal fibrosis with plasma cells and eosinophils. 3. Pautrier microabscesses are only present in the minority of cases of MF. Representative "threshold" cases of the earliest recognizable diagnostic cases of MF are illustrated. A pragmatic diagnostic approach to patients presenting with a clinical picture suggesting MF is outlined. The potential usefulness of additional ancillary diagnostic techniques such as immunoperoxidase frozen-section stains and T-cell-receptor gene rearrangements are briefly reviewed.

Adult↗
PubMed1990

Mycosis fungoides bullosa.

A case of mycosis fungoides bullosa is presented. The results in our study confirmed that the predominant atypical lymphoid cells in the bullae, peripheral blood, and involved lymph nodes expressed the T-cell helper phenotype using immunophenotyping techniques. The literature is reviewed, confirming that our case demonstrated cells of the T-helper phenotype, not only in the skin but also in the blood and lymph node tissue. Bullous lesions in mycosis fungoides are rare.

Aged↗
PubMed1992

Demonstration of clonal disease in early mycosis fungoides.

Cutaneous T-cell lymphomas (CTCLs) are known to show monoclonal T-cell infiltrates late in the course of the disease. However, detection of monoclonal T-cell proliferation in early stages is difficult. To investigate the possibility that clones might appear only as minor subpopulations, we analyzed the proliferative activity of T cells expression certain variable (V) regions of the T-cell receptor (TCR). In biopsy specimens from 27 patients with CTCLs, all T cells expressing the V regions V alpha 2, V beta 5a, V beta 5b, V beta 6, V beta 8 and V beta 12 accounted for less than 5% of the total infiltrate. In the vast majority of the cases, less than 3% of the cells expression one TCR V region proliferated. In one case of mycosis fungoides, however, an epidermotropic clone expressing V beta 8 was detected. This clone was present in two distinct lesions from different anatomic sites and was found in an early relapse 1 year after complete remission following PUVA therapy. The case described here documents the possibility of clonal disease early in the course of mycosis fungoides.

Aged↗
PubMed1992

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↗
PubMed1994

Estimates of nuclear volume in plaque and tumor-stage mycosis fungoides. A new prognostic indicator.

It is well documented that mycosis fungoides (MF), a cutaneous T-cell lymphoma, has a variable clinical course. Unbiased stereological estimates of three-dimensional volume-weighted mean nuclear size (nucl vV) of mycosis cells were obtained in a retrospective study of 18 patients with a total of 34 biopsies of cutaneous plaque and tumor-stage MF. The value of nucl vV in the first sampled biopsy, as well as the average and highest values, were determined in biopsies from each patient. The patients were divided into two groups, either above or below the group median. There was a strong positive correlation between shorter survival and an average value of nucl vV > 104 microns 3 or a highest value of nucl vV > 126 microns 3 (2p = 0.002 and 0.003, respectively). A nucl vV > 91.6 microns 3 in the first biopsy was also suggestive of a shorter survival (2p = 0.07). There was no clear pattern of nucl vV evolution in the patients with multiple biopsies, but the impact of various therapeutic regimens cannot be assessed. Certain estimates of nucl vV appear to be good prognostic indicators in plaque and tumor-stage MF, but further study of a larger series of patients is needed to corroborate these results and assess the impact of differing therapeutic regimes.

Adult↗
PubMed1996

Atypical cutaneous changes after topical treatment with nitrogen mustard in patients with mycosis fungoides.

Side effects in the treatment of mycosis fungoides with topical nitrogen mustard include allergic contact dermatitis, hyperpigmentation, urticaria, and erythema multiforme-like dermatitis. We reviewed biopsy specimens from 10 patients with mycosis fungoides who were treated with topical nitrogen mustard for 10-76 months. There was no history of oral psoralen with long-wave UV radiation treatment, radiotherapy, or systemic chemotherapy. Control biopsies taken from erythematous or poikilodermatous patches on the trunk or proximal extremities showed epidermal and dermal changes associated with cytologic atypia that were not present before treatment. These changes included slight epidermal hyperplasia with foci of flat rete ridges, atypical keratinocytes with large nuclei, mostly in the lower portion of the epidermis; suprabasal mitotic figures; a few dyskeratotic cells, focal vacuolar alteration of the epidermal basal layer; increased number of slightly enlarged junctional melanocytes; melanophages in the papillary dermis; dilated blood vessels lined by plump, atypical endothelial cells; and large fibroblasts with atypical nuclei. These atypical histologic changes resemble, in part, those described in association with systemic chemotherapeutic agents, such as etoposide, busulfan, and bleomycin. We conclude that topical nitrogen mustard should be added to the list of chemotherapeutic agents that can produce atypical histologic changes in the skin.

Administration, Topical↗
PubMed1999

Solitary skin lesions with histopathologic features of early mycosis fungoides.

Mycosis fungoides (MF) is a cutaneous T-cell lymphoma that usually begins with cutaneous patches that evolve into plaques and tumors. A few recent reports describe a solitary variant of MF distinct from localized pagetoid reticulosis, a disease in which solitary verrucous lesions occur on acral skin. Solitary skin lesions with some of the histopathologic features of MF rarely occur during treatment with several drugs, especially antidepressants or antihistamines. We analyzed the clinicopathologic features of 20 patients with solitary skin lesions showing histopathologic features of patch- or early plaque-stage MF. Eight men and 12 women (mean age 50.6, range 23-82, median 49) had solitary, small erythematous patches or plaques located on the trunk (16 cases, 6 of them on the breast), upper extremities (3 cases), and inguinal region (1 case). Ten patients were treated with one or more drugs; only two of them received antidepressants or antihistamines. Histopathologic examination revealed in all cases a band-like infiltrate in the upper dermis, frequently with epidermotropism of solitary lymphocytes. Atypical lymphocytes were present in a minority of cases. Immunohistology showed a predominance of CD3+ T lymphocytes, in most cases admixed with clusters of CD20+ B-cells. Only a small proportion of the infiltrate was CD8+. Molecular analysis of the rearrangement of the T-cell receptor genes was performed in 16 cases using the polymerase chain reaction (PCR) technique and revealed a monoclonal band in 8 of them. After surgical excision, 2/14 patients had a recurrence near the surgical scar. In 18 patients with complete follow-up data, no evidence of "classic" MF could be observed after a mean follow-up of 31.9 months. Solitary skin lesions with the histopathologic features of MF can be considered as a distinct clinicopathologic entity, probably representing a solitary variant of mycosis fungoides.

Adult↗
PubMed1992

Total skin electron beam radiation therapy for mycosis fungoides.

Forty-nine patients with biopsy-proven mycosis fungoides, Stages I-IV were treated using total skin electron beam irradiation (TSEBI). Total dose ranged from 600 cGy to 3,200 cGy. To evaluate the dose response relationship, patients were retrospectively divided into two groups. In Group I, 18 patients received a dose of 2,000 cGy or less, and in Group II, 31 patients received more than 2,000 cGy. The overall response rate was 87.7% with a 75.7% complete response and 12.2% partial response. Complete response was higher among patients with early stage disease: (Stage IA 1/1, Stage IB 23/35 (92%), Stage IIA 3/4 (75%), Stage IIB 4/8 (50%), Stage III 3/6 (50%), Stage IVA 1/1, Stage IVB 0/1, and unstaged group 2/3 (66.6%)). Patients treated with a higher total dose had a higher overall 5-year survival rate (Group I 38%, Group II 68%), longer median duration of complete response (Group I, 27 months; Group II, 35.3 months), slightly better complete response rate (72.2% for Group I, 77.4% for Group II), and lower recurrence rate (Group I, 94%; Group II, 83.9%) compared to patients with lower total dose. Complications from TSEBI were minimal. Total skin electron beam irradiation is effective in controlling early stage mycosis fungoides; however, a prospective study to evaluate optimum total dose is needed.

Adult↗
PubMed1995

Reassessment of histologic parameters in the diagnosis of mycosis fungoides.

The histologic diagnosis of mycosis fungoides (MF) can be difficult to establish and is based on interpretation of numerous subtle changes, most of which may be present to some degree in many inflammatory and neoplastic cutaneous conditions. To reassess the diagnostic criteria for making a histologic diagnosis of MF, we retrospectively reviewed histologic sections from 64 patients with mycosis fungoides (MF+) and compared the findings with sections from 47 patients who were biopsied to exclude MF and were shown not to have the disease (MF-). Patients were selected as MF+ or MF- independent of histologic findings based on the clinical course with at least 3 years of follow-up and immunophenotyping results. Following patient selection, at least two observers reviewed each slide without knowledge of final diagnosis and graded the intensity of approximately 25 histologic parameters. On univariate analysis, the following parameters were significant at beyond the p = 0.01 level: Pautrier's abscesses, haloed lymphocytes, exocytosis, disproportionate epidermotropism, epidermal lymphocytes larger than dermal lymphocytes, hyperconvoluted intraepidermal lymphocytes, and lymphocytes aligned within the basal layer. Haloed lymphocytes proved to be the most robust discriminator of MF from non-MF on multivariate analysis. These findings show that whereas many previously described features do discriminate between MF and inflammatory mimics, others are much less specific. Furthermore, few cases demonstrate all histologic features; for example, Pautrier's microabscesses were seen in only 37.5% of our cases. We conclude that a combination of specific histologic parameters can be used to establish a microscopic diagnosis of MF without the necessity of confirmatory immunophenotyping in the vast majority of cases.

Abscess↗
PubMed1984

The treatment of ulcers in patients with mycosis fungoides with split-thickness skin grafts.

Five patients have recently been treated at this institution for skin ulcerations caused by mycosis fungoides and its treatment with total body electron beam radiation therapy. Both the disease and its treatment can injure epidermal appendages as well as the blood supply to the skin. As a result of this potential damage, there was initial concern that skin grafts might not take and donor sites might not heal in these patients. Though skin grafting has not been universally successful, we have been able to skin graft the majority of the ulcerated lesions in these patients with good results. Donor sites have healed, though more slowly than usual. Skin grafting can be effective for treating ulcers in patients who have undergone total body electron beam radiation therapy for mycosis fungoides.

Adult↗
PubMed2004

Mycosis fungoides and the Sézary syndrome.

PURPOSE OF REVIEW: Mycosis fungoides and the Sézary syndrome represent a heterogeneous group of good-to-intermediate-risk non-Hodgkin lymphomas that have recently been identified as distinct histopathologic and clinical entities by the World Health Organization and European Organization for Research on the Treatment of Cancer lymphoma classification systems. Significant progress has been made in identifying and categorizing patients based on clinical prognostic factors, but there is little information regarding the etiology, molecular biology, and molecular genetics of these diseases. This review outlines recent advances in clinical diagnosis and prognosis as well as novel therapeutic approaches. RECENT FINDINGS: A number of reports have further defined clinical prognostic subgroups among early-stage patients and those with circulating Sézary cells. The recent availability and demonstrated efficacy of the oral RXR retinoid, bexarotene, has altered the treatment paradigm of early-stage patients who would not otherwise be exposed to systemic therapies. Novel targeted agents and receptor-directed therapies, including the fusion toxin, denileukin diftitox, histone deacetylase inhibitors, and novel nucleoside analog therapies, have demonstrated promising activity and are undergoing further clinical evaluation. The evolution of immunotherapy has been augmented by studies demonstrating the efficacy of peptide-loaded dendritic cells as well as the use of photopheresis to generate an anti-idiotype cytotoxic T-cell response. SUMMARY: This review will enumerate the most recent findings with respect to clinical staging, prognosis, and treatment of patients with mycosis fungoides and the Sézary syndrome. Novel treatment options will be reviewed and treatment paradigms will be outlined.

Anticarcinogenic Agents↗
PubMedDate not supplied

Toxic epidermal necrolysis syndrome versus mycosis fungoides.

We present a case in which our patient was first seen with biopsy and histologically confirmed toxic epidermal necrolysis syndrome (TENS). Subsequent to recovery and discharge from the hospital, the patient reappeared within 2 months of her discharge with a rash over her neck and back, with a central area of superficial breakdown. Biopsy results confirmed this lesion to be mycosis fungoides, not a recurrent case of TENS. Given the time lag between these two clinical courses, it is easy to speculate that this patient only had one disease entity, which we failed to diagnose on initial presentation. Once the skin becomes manifest with mycosis fungoides, the lesions are those typically beginning as an erythrodermic rash, it then progresses to indurated and infiltrated purple plaques. These symptoms may be confused with TENS, particularly in a patient with a preexisting diagnosis of TENS. It is critical that histologic confirmation of the clinical diagnosis be confirmed so that the treatment of the patient can be correctly instituted.

Biopsy↗
PubMed2002

Diffuse pulmonary involvement by mycosis fungoides: high-resolution computed tomography and pathologic findings.

The authors report the high-resolution computed tomography (HRCT) appearance of diffuse pulmonary involvement by mycosis fungoides in a 74-year-old woman whose clinical and radiographic manifestations simulated pneumonia. The HRCT showed multiple, dense, peribronchovascular nodules with surrounding ground-glass opacity and several wedge-shape peripheral opacities. The autopsy specimen revealed angiocentric and peribronchovascular involvement of mycosis fungoides and pulmonary infarctions distal to angiocentric infiltration of the tumor cells.

Aged↗
PubMedDate not supplied

Primary peripheral T-cell lymphoma of the face other than mycosis fungoides. Computed tomography and magnetic resonance findings.

OBJECTIVE: To describe the radiologic findings of primary peripheral T-cell lymphoma (PTCL) of the face other than mycosis fungoides. METHODS: Computed tomography (CT) and magnetic resonance imaging (MRI) findings of 5 consecutive patients with pathologically proven primary facial PTCL other than mycosis fungoides were retrospectively evaluated. Patients with PTCL involving the sinonasal cavity or lymph nodes were excluded. RESULTS: Diagnoses of patients included in this study consisted of natural killer/T-cell lymphoma (n = 2), subcutaneous panniculitis-like T-cell lymphoma (n = 1), anaplastic large cell lymphoma (n = 1), and PTCL not otherwise specified (n = 1). Infiltration or swelling of the superficial space of the face was noted on both CT and MRI, mimicking inflammation or infection. Also seen were well-enhancing small nodular (n = 2) or infiltrative mass-like lesions (n = 2) within the areas of infiltration, which showed intermediate signal intensity on T2-weighted images. One patient demonstrated infiltration and swelling alone. CONCLUSIONS: Primary facial PTCL is a rarely encountered tumor and demonstrates infiltration or swelling mimicking inflammation or infection. Nodular or infiltrative mass-like lesions may be helpful for its diagnosis.

Adult↗