RÉSUMÉ
Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides characterized by infiltration of hair follicle epithelium by neoplastic lymphoid cells. Generally, it is usually typified by indurated plaques and tumours mainly on the head and neck. However, a wide range of clinical signs have been noted. The clinical presentation of FMF may include prurigo-like lesions, acneiform lesions, cysts, nodules, areas of scarring alopecia, and hypopigmented plaques or papules with follicular prominences. The average age of diagnosis is 60 years while it is rare in childhood and adolescence. We discuss the case of a 12-year-old male patient who had an asymptomatic, erythematous, infiltrating plaque across his left nasolabial fold for three months prior to presentation. Histological assessment of lesion showed characteristic findings of follicular mucinosis with predominance of CD4+ lymphocytes and immunohistochemical studies were positive for CD3+ stains. An increased CD4:CD8 ratio and negative CD20 was also shown. Both findings were consistent with diagnosis of FMF.
Sujet(s)
Mycosis fongoïde , Tumeurs cutanées , Mâle , Adolescent , Humains , Enfant , Adulte d'âge moyen , Tumeurs cutanées/diagnostic , Tumeurs cutanées/anatomopathologie , Mycosis fongoïde/diagnostic , Mycosis fongoïde/composition chimique , Mycosis fongoïde/anatomopathologie , Follicule pileux/composition chimique , Follicule pileux/anatomopathologieRÉSUMÉ
Mycosis fungoides with large cell transformation (MFLCT) can be difficult to distinguish from primary cutaneous CD30+ T cell lymphoproliferative disorders (PC CD30+ LPD), especially primary cutaneous anaplastic large cell lymphoma (PC-ALCL). This diagnostic distinction is critical for appropriate patient management. GATA3 has been proposed to be useful in the discrimination between these two entities. We identified 25 cases of MFLCT and 24 cases of PC CD30+ LPDs (including lymphomatoid papulosis (n=14), PC-ALCL (n=6), and CD30+ LPD, not otherwise specified (n=4)) diagnosed at our institution from 2002 to 2019. Sections from archived specimens were stained to evaluate for GATA3 expression by immunohistochemistry and compared among cutaneous CD30+ T cell LPDs. The majority of the MFLCT cohort had strong, diffuse expression of GATA3 ranging from 0 to 100% of dermal T cells (mean 53.20%) with 15/25 cases (60%) showing GATA3 expression greater than 50%, while the PC CD30+ LPD group showed variable, moderate GATA3 labeling ranging from 0 to 60% of dermal T cells (mean 23.26%), with 5/6 cases (83%) showing GATA3 expression less than 40% (p =0.003). The calculated sensitivity and specificity were 56% and 74%, while positive and negative predictive values were 70% and 61%, respectively. Based on the percent staining of positive cells, using 50% as a cutoff value for expression, GATA3 might be a useful immunohistochemical marker to discriminate MFLCT from PC CD30+ LPDs, including PC-ALCL.
Sujet(s)
Marqueurs biologiques tumoraux/analyse , Facteur de transcription GATA-3/analyse , Immunohistochimie , Antigènes CD30/analyse , Lymphome à grandes cellules anaplasiques/composition chimique , Papulose lymphomatoïde/métabolisme , Mycosis fongoïde/composition chimique , Tumeurs cutanées/composition chimique , Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Diagnostic différentiel , Femelle , Humains , Lymphome à grandes cellules anaplasiques/immunologie , Lymphome à grandes cellules anaplasiques/anatomopathologie , Papulose lymphomatoïde/immunologie , Papulose lymphomatoïde/anatomopathologie , Mâle , Adulte d'âge moyen , Mycosis fongoïde/immunologie , Mycosis fongoïde/anatomopathologie , Valeur prédictive des tests , Études rétrospectives , Tumeurs cutanées/immunologie , Tumeurs cutanées/anatomopathologie , Jeune adulteRÉSUMÉ
BACKGROUND: Folliculotropic mycosis fungoides (FMF) is a variant of cutaneous T-cell lymphoma that has clinical overlap with a variety of inflammatory follicular unit disorders. However, we describe distinctive presentations of FMF with acneiform features that can be diagnostically challenging, leading to diagnostic delay. OBJECTIVE: To highlight the importance of histopathologic and immunohistochemical evaluation for diagnostic confirmation of presumed inflammatory follicular unit-based disorders that are unusual in presentation or unresponsive to standard therapies. METHODS: A cross-sectional retrospective study of 5 consecutive patients with a histopathologic diagnosis of FMF was conducted. The clinical, histopathologic, immunophenotypic, and molecular genetic features of cases are presented. RESULTS: We describe 5 patients with clinical and histopathologic presentations of FMF masquerading as hidradenitis suppurativa, furunculosis, or acne vulgaris (age range 34-66 years, 4:1 female to male). Clinical morphologies included open and closed comedones, inflammatory pustules, papules and nodules, follicular papules with keratotic plugging, cysts, and scarring involving the face, trunk, and intertriginous areas. All patients failed to respond to standard therapies, including topical and oral antibiotics, topical and oral retinoids, or topical corticosteroids, before receiving the diagnosis of FMF. Lesional skin biopsies showed a perifollicular CD4-positive T-lymphocytic infiltrate with pilotropism, intrafollicular mucin deposition, foreign-body granulomatous inflammation, acute inflammation, and follicular epithelial necrosis. None had concurrent systemic mycosis fungoides. LIMITATIONS: Small retrospective cohort study. CONCLUSION: We present these cases to expand the clinical and histopathologic spectrum of FMF that may strikingly resemble acneiform disorders and to highlight the importance of diagnostic reconsideration with histopathologic evaluation.
Sujet(s)
Acné juvénile/anatomopathologie , Follicule pileux/anatomopathologie , Mycosis fongoïde/anatomopathologie , Tumeurs cutanées/anatomopathologie , Adulte , Sujet âgé , Marqueurs biologiques tumoraux/analyse , Biopsie , Études transversales , Diagnostic différentiel , Femelle , Follicule pileux/composition chimique , Humains , Immunohistochimie , Mâle , Adulte d'âge moyen , Mycosis fongoïde/composition chimique , Mycosis fongoïde/thérapie , Stadification tumorale , Valeur prédictive des tests , Études rétrospectives , Tumeurs cutanées/composition chimique , Tumeurs cutanées/thérapieRÉSUMÉ
Papular mycosis fungoides (PMF) is a rare variant of mycosis fungoides (MF). The exact nosology and prognosis of PMF are still unclear. We retrospectively identified cases of PMF from the files of the Department of Dermatology of the Medical University of Graz, Austria, and checked the follow-up data. The patients comprised 13 men and 5 women (median age: 57.5 y; range 13 to 77 y). In 4 patients, an initial clinicopathologic diagnosis of atypical pityriasis lichenoides was made; these cases were subsequently reclassified as PMF due to the onset of conventional patches of MF during follow-up. Follow-up data of our cases showed that 2 patients died of disease progression 50 and 199 months after the first presentation, respectively. Two patients are alive with progressive disease after 215 and 300 months, respectively. Ten patients are alive with stable disease (median: 70 mo). Four patients were in complete remission at last follow-up visit (median: 215 mo; 2 of them died of unrelated causes). Our data confirm that PMF represents a clinicopathologic variant of early MF with prognosis similar to conventional presentations of the disease. Familiarity with PMF and distinction from other cutaneous papular lymphoid proliferations is necessary for a precise diagnosis and management of these patients.
Sujet(s)
Mycosis fongoïde/anatomopathologie , Tumeurs cutanées/anatomopathologie , Adolescent , Adulte , Sujet âgé , Autriche , Marqueurs biologiques tumoraux/analyse , Évolution de la maladie , Femelle , Études de suivi , Humains , Mâle , Adulte d'âge moyen , Mycosis fongoïde/composition chimique , Mycosis fongoïde/mortalité , Mycosis fongoïde/thérapie , Études rétrospectives , Tumeurs cutanées/composition chimique , Tumeurs cutanées/mortalité , Tumeurs cutanées/thérapie , Facteurs temps , Résultat thérapeutiqueRÉSUMÉ
Hypopigmented interface T-cell dyscrasia (HITCD) is a distinct form of lymphoid dyscrasia that may progress to hypopigmented mycosis fungoides (HMF). We compared both diseases as regards their CD4/CD8 phenotype and expression of granzyme B and tumor necrosis factor-alpha (TNF-α) and how these are affected by narrow-band UVB (nb-UVB). The study included 11 patients with HITCD and 9 patients with HMF. They received nb-UVB thrice weekly until complete repigmentation or a maximum of 48 sessions. Pretreatment and posttreatment biopsies were stained using anti CD4, CD8, TNF-α, and granzyme B monoclonal antibodies. Epidermal lymphocytes were CD8 predominant in 54.5% and 66.7% of HITCD and HMF cases, respectively, whereas dermal lymphocytes were CD4 predominant in 63.6% and 66.7%, respectively. Significantly, more dermal infiltrate was encountered in HMF (P = 0.041). In both diseases, granzyme B was only expressed in the dermis, whereas TNF-α was expressed both in the epidermis and dermis. No difference existed as regards the number of sessions needed to achieve repigmentation or cumulative nb-UVB dose reached at end of study. (P > 0.05). Narrow-band UVB significantly reduced only the epidermal lymphocytes in both diseases (P ≤ 0.05) with their complete disappearance in 8 (72.7%) HITCD and 6 (66.7%) HMF cases. In both diseases, nb-UVB did not affect granzyme B or TNF-α expression (P > 0.05). In conclusion, both diseases share the same phenotype, with HITCD being a milder form of T-cell dysfunction. In both diseases, epidermal lymphocytes are mainly CD8-exhausted cells lacking cytotoxicity, whereas dermal cells are mostly reactive cells exerting antitumor cytotoxicity. Tumor necrosis factor-alpha mediates hypopigmentation in both diseases and prevents disease progression. Repigmentation after nb-UVB in both diseases occurs before and independently from disappearance of the dermal infiltrate.
Sujet(s)
Lymphocytes T CD4+/anatomopathologie , Lymphocytes T CD8+/anatomopathologie , Hypopigmentation/anatomopathologie , Syndromes immunoprolifératifs/anatomopathologie , Mycosis fongoïde/anatomopathologie , Tumeurs cutanées/anatomopathologie , Pigmentation de la peau , Peau/anatomopathologie , Adolescent , Adulte , Biopsie , Lymphocytes T CD4+/composition chimique , Lymphocytes T CD4+/effets des radiations , Lymphocytes T CD8+/composition chimique , Lymphocytes T CD8+/effets des radiations , Enfant , Études transversales , Femelle , Granzymes/analyse , Humains , Hypopigmentation/métabolisme , Hypopigmentation/radiothérapie , Immunohistochimie , Syndromes immunoprolifératifs/métabolisme , Syndromes immunoprolifératifs/radiothérapie , Mâle , Adulte d'âge moyen , Mycosis fongoïde/composition chimique , Mycosis fongoïde/radiothérapie , Phénotype , Peau/composition chimique , Peau/effets des radiations , Tumeurs cutanées/composition chimique , Tumeurs cutanées/radiothérapie , Pigmentation de la peau/effets des radiations , Résultat thérapeutique , Facteur de nécrose tumorale alpha/analyse , Traitement par ultraviolets , Jeune adulteRÉSUMÉ
BACKGROUND: Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma. Early-stage MF patches or plaques often resemble inflammatory skin disorders (ISDs), including psoriasis and atopic dermatitis. Cell adhesion molecule 1 gene (CADM1), which was initially identified as a tumor suppressor gene in human non-small cell lung cancer, has been reported as a diagnostic marker for adult T-cell leukemia/lymphoma. OBJECTIVE: We investigated CADM1 expression in MF neoplastic cells, especially during early stages, and evaluated its usefulness as a diagnostic marker for MF. METHODS: We conducted a retrospective study by using immunohistochemical staining and confirmed the expression of CADM1 in MF. In addition, we compared CADM1 messenger RNA expression in microdissected MF samples and ISD samples. RESULTS: In the overall study period, 55 of 58 MF samples (94.8 %) stained positive for CADM1. None of the 50 ISD samples showed positive reactivity (P < .0001). We found CADM1 messenger RNA expression in the intradermal lymphocytes of patients with MF but not in those of patients with an ISD. LIMITATIONS: We did not conduct a validation study for MF cases in other institutions. CONCLUSIONS: CADM1-positive cells can be identified in early stages with fewer infiltrating cells and may be useful as a diagnostic marker for early-stage MF.
Sujet(s)
Marqueurs biologiques tumoraux/analyse , Molécule-1 d'adhésion cellulaire/analyse , Mycosis fongoïde/composition chimique , Protéines tumorales/analyse , Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Molécule-1 d'adhésion cellulaire/biosynthèse , Molécule-1 d'adhésion cellulaire/génétique , Dermatite/métabolisme , Diagnostic précoce , Femelle , Régulation de l'expression des gènes tumoraux , Humains , Japon/épidémiologie , Lymphocytes/métabolisme , Lymphocytes/anatomopathologie , Adulte d'âge moyen , Mycosis fongoïde/diagnostic , Mycosis fongoïde/génétique , Mycosis fongoïde/anatomopathologie , Protéines tumorales/biosynthèse , Protéines tumorales/génétique , Stadification tumorale , ARN messager/biosynthèse , ARN tumoral/biosynthèse , Études rétrospectivesRÉSUMÉ
This pathology PILOT study aims to define the role and feasibility of centralized pathology review in a cohort of 75 patients from different centers in the United States and Europe using digital slide scanning. The pathologic material from 75 patients who had been diagnosed with mycosis fungoides/Sézary syndrome and were clinically staged as IIb or above was retrieved from 11 participating centers. Each pathology reviewer was provided with the pathologic diagnosis (by the referring pathologist), and the following list of histopathologic criteria (presence or absence) from the initial report: epidermotropism, folliculotropism (FT), large cell transformation, syringotropism, and granulomas. Patients with advance stage were selected for this study as this is a population where there is significant variability in the diagnosis of pathologic prognostic and predictive biomarkers. The slides were digitally scanned with an Aperio scanner and consensus review of cases occurred when major or minor discrepancies between the referral diagnosis and central pathology review occurred. Among the 75 cases, 70 (93.3%) had a final consensus diagnosis between the 3 central review pathologists. The overall agreement between the consensus review and the referring pathologist was 60%. The overall agreement was also higher between the reviewers and consensus review, compared with the referring pathologist and consensus. 65.3% of cases had some type of discrepancy (major or minor) between the outside and consensus review. Major discrepancies were seen in 34 of 73 cases (46.6%; 73 cases indicated a yes or no response). Minor discrepancies were seen in 32 of 75 (42.7%) of cases. Most of the major discrepancies were accounted by a difference in interpretation in the presence or absence of large cell transformation or FT. Most minor discrepancies were explained by a different interpretation in the expression of CD30. We found digital slide scanning to be a beneficial, reliable, and practical for a methodical approach to perform central pathology review in the context of a large clinical prospective study.
Sujet(s)
Interprétation d'images assistée par ordinateur/méthodes , Microscopie/méthodes , Mycosis fongoïde/anatomopathologie , Syndrome de Sézary/anatomopathologie , Tumeurs cutanées/anatomopathologie , Marqueurs biologiques tumoraux/analyse , Biopsie , Europe , Études de faisabilité , Humains , Immunohistochimie , Mycosis fongoïde/composition chimique , Stadification tumorale , Biais de l'observateur , Valeur prédictive des tests , Études prospectives , Reproductibilité des résultats , Syndrome de Sézary/composition chimique , Tumeurs cutanées/composition chimique , États-UnisRÉSUMÉ
Folliculotropic mycosis fungoides (FMF), a variant of mycosis fungoides (MF) with distinct clinical features, is characterized by infiltration of malignant T cells in hair follicles. This raises the hypothesis that antigens in the hair follicle may contribute to the pathogenesis of FMF. T-cell receptor ß gene (TRB) sequences as well as dendritic cell subsets in patients with FMF (n = 21) and control patients with MF (n = 20) were studied to explore this hypothesis. A recurrent usage of the TRB junctional genes TRBJ2-1 and TRBJ2-7 was found in patients with FMF compared with those with MF. These genes contribute to an amino acid motif in the complementarity-determining region 3 (CDR3) of the T-cell receptor. This motif was previously found in T cells stimulated by lipids bound to CD1 on antigen-presenting cells. Additional immunohistochemical analysis revealed abundant CD1c- and CD1a- expressing dendritic cells in FMF. The combined findings support a role for lipid-antigen stimulation in FMF.
Sujet(s)
Antigènes CD1/analyse , Glycoprotéines/analyse , Mycosis fongoïde/composition chimique , Mycosis fongoïde/anatomopathologie , Études cas-témoins , Régions déterminant la complémentarité/analyse , Cellules dendritiques/composition chimique , Gènes de la chaine bêta du récepteur des lymphocytes T , Follicule pileux/composition chimique , Follicule pileux/anatomopathologie , Humains , Immunohistochimie , Lymphome T cutané/composition chimique , Lymphome T cutané/génétique , Lymphome T cutané/anatomopathologie , Mycosis fongoïde/génétique , Tumeurs cutanées/composition chimique , Tumeurs cutanées/génétique , Tumeurs cutanées/anatomopathologieRÉSUMÉ
is missing (Short communication).
Sujet(s)
Séquençage nucléotidique à haut débit , Mycosis fongoïde/composition chimique , ARN viral/analyse , Adulte , Sujet âgé , Biopsie , Humains , Mâle , Adulte d'âge moyen , Peau/composition chimique , Peau/anatomopathologieRÉSUMÉ
Adult T-cell leukemia/lymphoma (ATLL) is a rare and often aggressive lymphoid malignancy known to be associated with human T-cell lymphotropic virus type 1. There are 2 broad categories: acute and chronic. In the acute category, there is a leukemic and a lymphomatous variant, whereas in the designated "chronic" form, there is mild peripheral blood lymphocytosis. The intermediate "smoldering" category is without peripheral blood lymphocytosis with only discernible skin involvement. We present a 68-year-old human T-cell lymphotropic virus type 1 seropositive female with a mild peripheral blood atypical lymphocytosis who had indurated nodules on her hands of 2 years duration and a new scaly ichthyosiform eruption on her lower extremities. Histopathologic examination of the hand biopsy revealed coalescing nodules of large atypical noncerebriform lymphocytes with focal areas of epidermotropism. Phenotypically, the infiltrate was positive for ß-F1, CD2, CD4, CD5, CD7, Foxp3, and CD25. In both biopsies, there was striking upregulation of TOX (thymocyte selection-associated high mobility group box factor) in the nuclei of neoplastic cells. The second biopsy taken from the ichthyotic patch on the patient's left leg showed a subtle pattern of epidermal infiltration by atypical noncerebriform lymphocytes and a distinct compact scale consistent with the clinical picture of ichthyosis. The histopathologic appearance was that of a yet undescribed ichthyosiform mycosis fungoides-like presentation of chronic ATLL. In addition, the observed upregulation of nuclear TOX may play an oncogenic role in ATLL. The course to date in this patient has been relatively indolent, although the patients believe that large cell transformation could portend more aggressive disease.
Sujet(s)
Marqueurs biologiques tumoraux/analyse , Protéines HMG/analyse , Ichtyose/métabolisme , Leucémie-lymphome à cellules T de l'adulte/métabolisme , Mycosis fongoïde/composition chimique , Tumeurs cutanées/composition chimique , Sujet âgé , Protocoles de polychimiothérapie antinéoplasique/usage thérapeutique , Biopsie , Femelle , Humains , Ichtyose/traitement médicamenteux , Ichtyose/anatomopathologie , Immunohistochimie , Leucémie-lymphome à cellules T de l'adulte/traitement médicamenteux , Leucémie-lymphome à cellules T de l'adulte/anatomopathologie , Mycosis fongoïde/traitement médicamenteux , Mycosis fongoïde/anatomopathologie , Tumeurs cutanées/traitement médicamenteux , Tumeurs cutanées/anatomopathologie , Résultat thérapeutique , Régulation positiveRÉSUMÉ
The aim of this study was to investigate the role of lymphangiogenesis in the clinical progression and outcome of mycosis fungoides. Immunohistochemistry and Western blot techniques were used to assess the expression of podoplanin and vascular endothelial growth factor C in mycosis fungoides. Expression of vascular endothelial growth factor C measured by immunohistochemistry was significantly higher in mycosis fungoides samples in comparison with control cases (chronic benign dermatoses) (p = 0.0012). Increased expression of podoplanin was found in advanced vs. early mycosis fungoides (p < 0.0001), and was positively correlated with cutaneous and nodal involvement (p < 0.001, p < 0.0001; respectively). Higher podoplanin expression was also significantly associated with shorter survival (p < 0.001). Strong positive correlation was observed between expression of podoplanin analysed by immunohistochemistry and Western blot (r = 0.75, p < 0.0001). A similar association was shown regarding expression of vascular endothelial growth factor C (r = 0.68, p = 0.0007). In conclusion, these results suggest that increased expression of podoplanin is associated with poor clinical course, as well as shorter survival, of patients with mycosis fungoides.
Sujet(s)
Évolution de la maladie , Lymphangiogenèse , Glycoprotéines membranaires/analyse , Mycosis fongoïde/composition chimique , Mycosis fongoïde/anatomopathologie , Tumeurs cutanées/composition chimique , Tumeurs cutanées/anatomopathologie , Facteur de croissance endothéliale vasculaire de type C/analyse , Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Technique de Western , Cytoplasme/composition chimique , Cellules endothéliales/composition chimique , Femelle , Humains , Immunohistochimie , Métastase lymphatique , Mâle , Adulte d'âge moyen , Stadification tumorale , Taux de survie , Lymphocytes T/composition chimique , Jeune adulteSujet(s)
Mycosis fongoïde/anatomopathologie , Tumeurs cutanées/anatomopathologie , Administration par voie cutanée , Sujet âgé de 80 ans ou plus , Antinéoplasiques alcoylants/administration et posologie , Marqueurs biologiques tumoraux/analyse , Biopsie , Humains , Immunohistochimie , Mâle , Chlorméthine/administration et posologie , Mycosis fongoïde/composition chimique , Mycosis fongoïde/traitement médicamenteux , Stadification tumorale , Induction de rémission , Tumeurs cutanées/composition chimique , Tumeurs cutanées/traitement médicamenteux , Facteurs temps , Résultat thérapeutiqueSujet(s)
Marqueurs biologiques tumoraux/analyse , Sous-unité p35 de l'interleukine-12/analyse , Mycosis fongoïde/diagnostic , Tumeurs cutanées/diagnostic , Cellules dendritiques/composition chimique , Derme/anatomopathologie , Eczéma/anatomopathologie , Femelle , Humains , Nourrisson , Cellules de Langerhans/composition chimique , Mâle , Adulte d'âge moyen , Mycosis fongoïde/composition chimique , Mycosis fongoïde/anatomopathologie , Psoriasis/anatomopathologie , Tumeurs cutanées/composition chimique , Tumeurs cutanées/anatomopathologie , Microenvironnement tumoralRÉSUMÉ
Mycosis fungoides (MF) is a cutaneous T-cell lymphoma that can undergo local progression with possible systemic dissemination. We report a case of a patient affected by MF with a pancreatic mass that was a diagnostic challenge between primitive tumor and pancreatic metastasis from MF. Clinical setting findings and imaging studies raised the suspicion of a pancreatic primary neoplasm. A diagnostic clue was provided by the combined histomorphologic/immunohistochemical study of pancreatic and cutaneous biopsies, which revealed a pancreatic localization of MF. Considering the rarity of metastatic localization of MF to the pancreas, we next investigated whether chemokine-chemokine receptor interactions could be involved in the phenomenon to provide new insight into the possible mechanisms underlying metastatic localization of MF to the pancreas. Histological analyses of archival pancreatic tissue demonstrated that glucagon-secreting cells of the pancreatic islets expressed the CCL27 chemokine, which may have attracted in our case metastatic MF cells expressing the complementary receptor CCR10.
Sujet(s)
Mycosis fongoïde/anatomopathologie , Tumeurs du pancréas/secondaire , Tumeurs cutanées/anatomopathologie , Marqueurs biologiques tumoraux/analyse , Biopsie , Diagnostic différentiel , Humains , Immunohistochimie , Mâle , Adulte d'âge moyen , Mycosis fongoïde/composition chimique , Mycosis fongoïde/imagerie diagnostique , Mycosis fongoïde/thérapie , Tumeurs du pancréas/composition chimique , Tumeurs du pancréas/imagerie diagnostique , Tumeurs du pancréas/thérapie , Valeur prédictive des tests , Tumeurs cutanées/composition chimique , Tumeurs cutanées/thérapie , TomodensitométrieRÉSUMÉ
Phenotypic variants of mycosis fungoides (MF) include mainly the expression of cytotoxic markers by neoplastic cells (either α/ß or γ/δ cytotoxic). To manage the patient properly, distinction from other cutaneous cytotoxic natural killer/T-cell lymphomas is paramount. Particularly for cutaneous γ/δ T-cell lymphoma, distinction is often based on clinicopathologic correlation (presence of tumors at first diagnosis as opposed to patches only in MF). The authors report a case of cytotoxic MF characterized by expression of TCRγ in two of three biopsies performed within a time frame of 1 week. The patient presented with patches, plaques, and 1 tumor at the time of first diagnosis; thus, distinction from cutaneous γ/δ T-cell lymphoma was not possible on clinical grounds alone. The diagnosis of MF was established, thanks to the phenotypic variations revealed by the three biopsies, with 1 lacking expression of cytotoxic proteins (TIA-1 and granzyme B) and of TCRγ. This case shows the importance to perform several biopsies in cases of cutaneous lymphoma, as morphologic and phenotypic features are variable and information gathered from a single biopsy may result in a wrong diagnosis.
Sujet(s)
Mycosis fongoïde/anatomopathologie , Tumeurs cutanées/anatomopathologie , Antihelminthiques antinématodes/usage thérapeutique , Bras , Marqueurs biologiques tumoraux/analyse , Biopsie , Diagnostic différentiel , Erreurs de diagnostic/prévention et contrôle , Femelle , Granzymes/analyse , Humains , Immunohistochimie , Immunophénotypage , Interféron alpha-2 , Interféron alpha/usage thérapeutique , Adulte d'âge moyen , Mycosis fongoïde/composition chimique , Mycosis fongoïde/immunologie , Mycosis fongoïde/thérapie , Puvathérapie , Phénotype , Protéines de liaison au poly(A)/analyse , Valeur prédictive des tests , Récepteur lymphocytaire T antigène, gamma-delta/analyse , Protéines recombinantes/usage thérapeutique , Tumeurs cutanées/composition chimique , Tumeurs cutanées/immunologie , Tumeurs cutanées/thérapie , Antigène intracellulaire-1 des lymphocytes T , Facteurs temps , Résultat thérapeutiqueRÉSUMÉ
BACKGROUND: Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma (CTCL), followed by CD30+ lymphoproliferative disorders, including lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (pcALCL). The objective was to report on a series of patients with different types of CTCL at different times in their clinical course, with a focus on clonality studies. METHODS: Four patients with multiple diagnoses of CTCLs were identified. The clinical information, treatment interventions, and histopathology were reviewed. T-cell receptor (TCR) gene rearrangement studies were performed on all available specimens. RESULTS: The four patients carried diagnoses of: (1) pcALCL and MF; (2) pcALCL, LyP, and pcALCL; (3) LyP, MF, and pcALCL; (4) LyP, pcALCL, and MF; each with characteristic presentation and histopathologic findings. The results of the TCR polymerase chain reaction showed that all tumors expressed and retained a TCR clone(s) as follows: (1) biallelic clone; (2) single clone; (3) biallelic clone with additional clone; and (4) single clone, respectively. CONCLUSION: We report a series of four cases of individual patients with coexisting diagnoses of some combination of MF, LyP, and pcALCL, whose lesions presented in nontraditional sequence and demonstrated a retained clone by gene rearrangement analysis.
Sujet(s)
Gènes du récepteur des cellules T , Lymphome cutané primitif à grandes cellules anaplasiques/génétique , Papulose lymphomatoïde/génétique , Mycosis fongoïde/génétique , Seconde tumeur primitive/génétique , Tumeurs cutanées/génétique , Sujet âgé de 80 ans ou plus , Allèles , Femelle , Réarrangement des gènes , Humains , Antigènes CD30/analyse , Lymphome cutané primitif à grandes cellules anaplasiques/composition chimique , Lymphome cutané primitif à grandes cellules anaplasiques/anatomopathologie , Papulose lymphomatoïde/métabolisme , Papulose lymphomatoïde/anatomopathologie , Mâle , Adulte d'âge moyen , Mycosis fongoïde/composition chimique , Mycosis fongoïde/anatomopathologie , Seconde tumeur primitive/composition chimique , Seconde tumeur primitive/anatomopathologie , Tumeurs cutanées/composition chimique , Tumeurs cutanées/anatomopathologie , Jeune adulteRÉSUMÉ
Mycosis fungoides (MF) simulates a variety of dermatologic disorders histopathologically and clinically, well deserving the designation of a great mimicker. Interstitial MF is a rare, but well-recognized histopathological variant resembling the interstitial form of granuloma annulare or the inflammatory phase of morphea. From a clinical standpoint, MF can have a wide array of manifestations, including an anecdotal presentation with lesions clinically suggestive of lichen sclerosus (LS). We herein report a 25-year-old man with a history of patch-stage MF who later developed widespread LS-like lesions histopathologically consistent with interstitial MF. In some biopsies, additional features resembling LS were discerned. We think that our case might represent a unique variant of interstitial MF presenting with LS-like lesions. The diagnostic challenge arising from this uncommon presentation is discussed together with review of the literature.