Histopathologic Diagnosis Of Mycosis Fungoides Research Articles

Micosis fungoide. Experiencia en un hospital pediátrico

Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. ObjectivesWe aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. Material and methodData for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. ResultsA total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8–15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months–7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. ConclusionsMF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary.

Mycosis fungoides with epidermal mucinosis: a variant of mycosis fungoides with a spongiosis-like pattern.

The histopathologic diagnosis of mycosis fungoides (MF) has classically relied on the presence of atypical epidermotropic T-lymphocytes predominating over spongiosis. However, in some cases of MF, prominent epidermal mucinosis in a spongiosis-like pattern mimics a spongiotic dermatitis. To our knowledge, only one series in the literature has thus far recognized the presence of epidermal mucinosis in MF. We evaluated 30 skin biopsies from 18 patients with the clinical diagnosis of MF, which fulfilled all histopathologic criteria for patch- or plaque-stage MF, but also showed epidermal mucinosis in a spongiosis-like pattern. A total of 15 specimens were studied by immunohistochemistry, and seven were tested for T-cell receptor (TCR) gene rearrangements. Twenty biopsies of spongiotic dermatitides were included as controls. We confirmed the presence of epidermal mucinosis in all 30 cases of MF with a spongiosis-like pattern based on histopathologic criteria and the colloidal iron stain for mucin. Immunohistochemistry in 15 specimens showed significant loss of pan-T-cell antigens CD5 (10/15) and CD7 (14/15); and TCR clonality was detected in 7 specimens from 6 patients, supporting the diagnosis of MF. We report helpful histopathologic criteria for distinguishing MF with epidermal mucinosis in a spongiosis-like pattern from spongiotic dermatitis.

Influence of clinical and pathologic features on the pathologist's diagnosis of mycosis fungoides: a pilot study.

Although clinicopathologic correlation is critical in the diagnosis of early mycosis fungoides (MF), how clinical information directly affects the pathologist's interpretation is unknown. This pilot study aimed to assess the influence of provided clinical information and specific histopathologic features on the histopathologic diagnosis of MF vs. its inflammatory simulants. A computerized survey recorded diagnostic impressions by 24 dermatopathologists of 30 hematoxylin-eosin stained images, including 15 MF images and 15 dermatitis images. Images were accompanied by concordant clinical descriptions (33%), no clinical information (33%) or discordant clinical descriptions (33%). Percentage of correctly classified MF histopathologic images for the three scenarios of concordant clinical information, no clinical information or discordant clinical information were 32% (kappa 0.19), 56% (kappa 0.12) and 16% (kappa 0.33), respectively. The percentage of correctly classified slides presented with no clinical information was different from the other two groups (p < 0.0001). Pautrier collections were most associated with correct classification. Clinical information may play a significant role in the histopathologic diagnosis of MF, although there may be some value in initial blinded histopathologic interpretation. Specific histopathologic features differ in relative importance in the diagnosis of MF.

T-cell receptor gene rearrangement studies using the GeneScan technique as an adjunct to the histopathological diagnosis of mycosis fungoides.

T-cell receptor gene rearrangement studies are considered to be an adjunct to the histopathological diagnosis of mycosis fungoides (MF). Fluorophore-coupled primers in polymerase chain reactions followed by fragment analysis with a capillary electrophoresis device (GeneScan analysis) have been recently advocated and widely used. This study was performed to assess T-cell receptor γ-gene rearrangement GeneScan as an adjunct to the histopathological diagnosis of MF. We studied 163 cases. The rates of clonality using this technique were found to be 22/37 (59.5%) in early patch MF, 30/46 (65.2%) in more advanced stages, 13/34 (38.2%) in inconclusive cases, and 10/46 (21.7%) in benign inflammatory dermatoses. Our results support the histopathological criteria for the diagnosis of early patch MF, because of the close rates of clonality obtained in early patch MF with subtle histopathological findings, and in the more advanced subtypes of MF with more overt and specific histopathological criteria.

Delay in the Histopathologic Diagnosis of Mycosis Fungoides

The diagnosis of mycosis fungoides (MF) is difficult in early stages and is based on a combination of clinical findings and histopathologic criteria. The aim of this study was to assess the diagnostic delay in MF and to investigate the rationale for multiple biopsies in a single-centre, retrospective study of 157 patients with MF. The first biopsy was diagnostic for MF in 25% of cases. The median diagnostic delay was 2.3 years and depended on whether the diagnosis was established after one or multiple biopsies. The chance of a biopsy resulting in a diagnosis of MF was 25% irrespective of the number of the biopsy in the sequence. There was a significant diagnostic delay, especially in patients in whom the initial biopsy was not specific. Sampling error and unnecessary postponement of subsequent biopsies are likely factors and therefore multiple biopsies should be considered in patients with skin lesions suggesting MF.

Coexistence of Patch Stage Mycosis Fungoides and Interstitial Granuloma Annulare in the Same Patient

Several clinical and histopathologic variants of mycosis fungoides (MF) have been well described, including the often elusive interstitial MF. Differentiation from other inflammatory disorders, such as interstitial granuloma annulare (GA) and inflammatory morphea, may be extremely difficult. We report a case of MF and GA coexisting in a 54-year-old woman who initially presented to clinic in 2000 with slightly scaly patches on the trunk and extremities, histopathologically diagnostic of MF. A second biopsy taken a few months later revealed an interstitial infiltrate that was initially interpreted as interstitial MF. Over the following 10 years, additional biopsies revealed features of conventional MF. In 2009, a new biopsy showed unequivocal features of interstitial GA. Reevaluation of the original biopsy, diagnostic of "interstitial MF," revealed that this, too, could be better classified as interstitial GA than interstitial MF. Our case illustrates that MF and interstitial GA may coexist simultaneously, thus representing a pitfall in the histopathologic diagnosis of MF. Given the similarities in clinicopathologic presentation, dermatologists and dermatopathologists should be cautious not to inadvertently misinterpret GA as interstitial MF.

The Histological Spectrum of Early Mycosis Fungoides: A Study of 58 Saudi Arab patients

The histopathological diagnosis of Mycosis Fungoides (MF) is challenging in its early stages and can easily be confused with inflammatory dermatoses. This study aims to; (i) assess the frequency and significance of different histopathological parameters in early MF, seen in Saudi patients, and (ii) to study the utility of these parameters in differentiating between early MF and inflammatory dermatoses. This is a five years study of 66 skin biopsies generated from 58 patients with clinically suspicious MF lesions or early patch stage MF. These cases were retrieved from the archives of the Department of Pathology, King Khalid University Hospital, Riyadh from the year 2002 to 2006. Histological criteria were assessed and graded semi-quantitatively on a four-point scale by a dermatopathologist and two pathologists independently. The histological parameters suggesting the diagnosis in early stages MF include epidermotropism, dermal fibrosis, Pautrier's micro abscesses, Basal alignment of neoplastic lymphocytes, hyperconvoluted dermal and epidermal lymphocytes and grandiosity sign (size of lymphocytes becoming larger as they migrate towards granular layer of epidermis). These parameters on univariate analysis achieved statistical significance (p<0.05) in differentiating MF from non-MF cases. In addition, hyperconvoluted dermal and epidermal lymphocytes proved to be highly reliable with high specificity and sensitivity. The histopathological diagnosis of early MF lesions and their discrimination from inflammatory simulators can be achieved by carefully observing the hyperconvoluted dermal and epidermal lymphocytes along with the constellation of the other parameters.

Value of the CD8-CD3 Ratio for the Diagnosis of Mycosis Fungoides

Histopathological diagnosis of mycosis fungoides is difficult, especially in early lesions that may be indistinguishable from inflammatory dermatoses. Mycosis fungoides is a clonal proliferation of mature epidermotropic CD4+ lymphocytes. The aim of this study was to determine the contribution of the CD8-CD3 ratio to the diagnosis of mycosis fungoides. We retrospectively compared the immunophenotypic characteristics of 30 mycosis fungoides with 28 inflammatory dermatoses. The diagnosis of mycosis fungoides was reinforced in all cases by the presence of a cutaneous dominant T-cell clonal population. To analyze exclusively the lymphocytic infiltrates, CD4, which is also expressed by histiocytes, was not considered. The CD8-CD3 ratio was determined separately in the epidermis and the dermis using two methods, one quantitative and the other semiquantitative. Concordance rates between the two methods were higher in epidermal than dermal infiltrates. The mean CD8-CD3 ratio was significantly lower for mycosis fungoides than control cases, with the difference being greater in the epidermal than the dermal component. Although not absolutely specific, a low CD8-CD3 ratio in the epidermal component of a lymphocytic infiltrate supports the diagnosis of mycosis fungoides. It can be evaluated in routine practice using a semiquantitative approach.

How useful are T-cell receptor gene rearrangement studies as an adjunct to the histopathologic diagnosis of mycosis fungoides?

The diagnosis and differential diagnosis of mycosis fungoides (MF) is often difficult, clinically and histologically. Recent attempts to enhance diagnostic sensitivity have involved T-cell receptor (TCR) gene rearrangement studies, using Southern blot or polymerase chain reaction (PCR) technique. PCR is more widely used because of its increased sensitivity, lower labor- and cost-intensive analytic steps, lack of radioactive substances, and applicability to routinely processed biopsies. Several studies that use this technique detect clonal bands in 50% to 83% of the MF specimens from patch, patch/plaque, and erythrodermic stages, which often pose a diagnostic challenge. This compares with no clonality in the control groups of most of these studies, or with up to 13% in a few studies, although long-term follow-up reveal that some cases of so-called "clonal dermatitis" eventually progress into overt cutaneous T-cell lymphoma. Furthermore, retrospective studies on archival histologic material from patients with MF demonstrate similar rates of clonality in histologically "inconclusive," "borderline," and diagnostic MF biopsies. Therefore, in the proper clinicopathologic setting, and with consideration of the known limitations of this technique, TCR gene rearrangement studies on lesional skin using PCR may be helpful as an adjunct to the histopathologic diagnosis of MF.

Immunophenotyping and T-cell receptor γ gene rearrangement analysis as an adjunct to the histopathologic diagnosis of mycosis fungoides

Background: The histopathologic diagnosis of mycosis fungoides (MF) may be difficult. Objective: Our purpose was to evaluate the role of immunophenotyping and T-cell receptor (TCR) gene rearrangement studies as an adjunct to the histopathologic diagnosis of MF. Methods: Immunohistochemical studies with antibodies to CD4, CD5, CD7, and CD8 and TCR γ gene rearrangement analysis with a polymerase chain reaction were performed on fresh-frozen material of patients with “classic” histology of MF, “inconclusive” histology, and benign inflammatory dermatoses. Results: Clonal TCR γ gene rearrangements were found in 11 of 16 (69%) of classic MF cases, in 3 of 19 (16%) of inconclusive cases, and in none of the 12 inflammatory dermatoses cases ( P < .05 and P < .001, respectively). Only the mean CD7 counts were statistically significantly different between these 3 groups (MF < inconclusive < inflammatory). Conclusion: Inconclusive histology is probably a heterogeneous group in which CD7 counts and TCR γ gene rearrangement studies might help to differentiate the MF cases from the benign cases. (J Am Acad Dermatol 1998;39:554-9.)

Localization of Human T-Cell Lymphotropic Virus-1 tax Proviral Sequences in Skin Biopsies of Patients with Mycosis Fungoides by in situ Polymerase Chain Reaction

The histopathologic diagnosis of mycosis fungoides (MF), even when clinical manifestations of the disease seem convincing, is often tenuous. The observation that practically all patients with MF harbor human T cell lymphotropic virus type I (HTLV-I) proviral sequences in their circulating lymphocytes raised the possibility that such viral footprints could be detected in their cutaneous infiltrates. Application of in situ polymerase chain reaction (PCR) to skin biopsies of 11 of 12 patients demonstrated this assumption to be correct. In addition, cells suspected to be keratinocytes were also positive. None of 10 skin biopsies from a variety of sources used as controls, nor 3 lymph node biopsies from patients with B-cell lymphomas, showed any HTLV proviral sequences on in situ PCR. On the basis of these observations, it is concluded that in situ PCR carried out on skin biopsies of patients with presumptive MF may help to established the diagnosis.

Mycosis fungoides: a retrospective study.

The clinical course of ninety patients with the histopathologic diagnosis of mycosis fungoides is reviewed. An attempt is made to correlate sex, age, of onset, lesion type, and form of therapy with outcome of the disease. The data indicate that mycosis fungoides affects predominantly middle-aged males. Patients developing tumors or erythroderma in middle age or later tend to have a shorter survival. More aggressive therapy is associated with shortened survival. Most deaths were due to unknown or unrelated processes and therapeutic complications. The data support the theory that mycosis fungoides can be a relatively nonaggressive cutaneous lymphoma. In an attempt to treat aggressively, we may be exposing patients to increased mortality.