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Primary cutaneous T-cell lymphomas (CTCLs) other than mycosis fungoides (MF) and Sézary syndrome (SS) encompass a heterogenous group of non-Hodgkin lymphomas with variable clinical courses, prognoses, and management approaches. Given the morphologic and histologic overlap among the CTCL subtypes and other T-cell lymphomas with cutaneous manifestations, thorough evaluation with clinicopathologic correlation and exclusion of systemic involvement are essential prior to initiating therapy. Staging and treatment recommendations vary, depending on the subtype, clinical behavior, and treatment response. Generally, for subtypes in which staging is recommended, Ann Arbor or tumor, node, metastasis staging specific to CTCL other than MF or SS are used. For many subtypes, there is no standard treatment to date. Available recommended treatments range widely, from no active or minimal intervention with skin-directed therapy to aggressive systemic therapies that include multi-agent chemotherapy with consideration for hematopoietic stem cell transplant. Emerging targeted therapies, such as brentuximab, a chimeric antibody targeting CD30, show promise in altering the disease course of non-MF/SS CTCLs. [ABSTRACT FROM AUTHOR]

Primary cutaneous T-cell lymphomas (CTCLs) are defined as lymphomas with a T-cell phenotype that present in the skin without evidence of systemic or extracutaneous disease at initial presentation. CTCLs other than mycosis fungoides and Sézary syndrome (SS) account for approximately one third of CTCLs and encompass a heterogenous group of non-Hodgkin lymphomas, ranging from indolent lymphoproliferative disorders to aggressive malignancies with a poor prognosis. The spectrum of CTCLs continues to broaden as new provisional entities are classified. Given the morphologic and histologic overlap among CTCLs and other diagnoses, a thorough clinical history, physical evaluation, and clinicopathologic correlation are essential in the work up and diagnosis of these rare entities. This article will summarize the epidemiologic, clinical, pathologic, and diagnostic features of CTCLs other than mycosis fungoides and SS. [ABSTRACT FROM AUTHOR]

Aims: Patients with aggressive CD8+ cutaneous T‐cell lymphomas (CTCLs) progress rapidly and respond poorly to therapy. Confounding treatment planning, there is clinicopathological overlap between aggressive CD8+ CTCLs and other lymphoproliferative disorders (LPDs). Hence, improved diagnostic methods and therapeutic options are needed. The aim of this study was to examine C‐C chemokine receptor 4 (CCR4) expression as a diagnostic and therapeutic biomarker in CD8+ CTCLs/LPDs. Methods and results: Forty‐nine cases (41 patients) with CD8+ CTCLs/LPDs were examined, including CD8+ mycosis fungoides (MF) (n = 14), aggressive epidermotropic CD8+ cytotoxic T‐cell lymphoma (AETCL) (n = 8), subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) (n = 7), CD30+ LPDs (n = 6), primary cutaneous γδ T‐cell lymphoma (GDTCL) (n = 6), and others (n = 8). Immunohistochemical tissue staining was performed with a CCR4 monoclonal antibody on formalin‐fixed paraffin‐embedded tissue sections. CCR4 immunostaining was graded as percentage infiltrate, i.e. high (>25%) and low (≤25%), and the results were correlated with clinicopathological diagnoses. CCR4 expression was seen in 69% of the studied cases. Any CCR4 positivity was seen in all CD8+ MF cases, in 83% of CD30+ LPD cases, in 75% of AETCL cases, in 33% of GDTCL cases, and in none of the SPTCL cases. High CCR4 expression was seen in 79% of CD8+ MF cases versus 33% of CD30+ LPD cases, in 17% of GDTCL cases, and in 12.5% of AETCL cases. Patients with more advanced MF stage had higher CCR4 expression. Conclusions: CCR4 immunohistochemistry may be an adjunct in distinguishing advanced CD8+ MF from other CD8+ CTCLs/LPDs. Although CCR4 expression may justify therapeutic targeting of this receptor in CD8+ MF, the role of such therapies in other CD8+ CTCLs/LPDs is not yet clear. [ABSTRACT FROM AUTHOR]

Follicular mucinosis (FM), which is defined by mucin accumulation within follicular epithelium, may occur in mycosis fungoides (MF). FM without MF is occasionally reported in systemic hematologic malignancies and may be diagnostically challenging. To describe clinicopathologic characteristics of FM in patients with hematologic malignancies other than MF. Clinical data and histopathology features were analyzed in patients with FM and hematologic malignancies diagnosed between 1994 and 2017. A total of 18 patients with FM and systemic hematologic malignancies without cutaneous T-cell lymphoma (CTCL) were identified; 9 of them were discovered after hematopoietic stem cell transplantation. No patients with non–CTCL-associated FM (n = 46 [37 biopsy specimens]) developed CTCL during a mean follow-up of 4.3 years. Of the cases of CTCL associated with FM (n = 44 [31 biopsy specimens]), MF was the most common subtype (n = 38), although other CTCLs were identified. FM in patients with non-CTCL hematologic malignancies differed clinically from those with MF-associated FM, presenting most frequently with erythematous papules (P <.0001), without plaques (P <.0001), without alopecia (P =.001), and without histopathologically identified epidermal exocytosis (P =.013). A retrospective study in a single cancer center. FM can present in systemic hematologic malignancies, including after hematopoietic stem cell transplantation. Papular lesional morphologic and histopathologic features may help to distinguish these cases from MF. [ABSTRACT FROM AUTHOR]

Acral angiokeratoma-like pseudolymphoma is a rare type of pseudolymphoma presenting as dark-red papules on the hand or foot. We describe a 59-year-old woman who presented with an unusual unilateral, clustered aggregate of scaly violaceous papules on the toe with an indolent course. Skin biopsy showed a prominent vascular proliferation associated with a dermal infiltrate of monoclonally rearranged T-follicular helper phenotype T-cells, in keeping with CD4+ small/medium T-cell lymphoproliferative disorder ( SMPTC-LPD). Based on the unique clinical morphology, distribution of the lesions and dermoscopic appearance, a clinicopathologic diagnosis of acral angiokeratoma-like pseudolymphoma was favored. This case demonstrates the importance of clinicopathological correlation in such diagnostically challenging patients who present with overlapping features on the spectrum of pseudolymphoma and cutaneous T-cell lymphoma. [ABSTRACT FROM AUTHOR]

Background Primary cutaneous CD4+ small-medium pleomorphic T cell lymphoma ( SMPTCL) is a low-grade cutaneous T cell lymphoma. Its clinical and histopathologic features are comparable with those of CD8+ lymphoid proliferations ( LPs) of the ear and acral sites. Objectives We performed a retrospective analysis of patients with CD4+ SMPTCL or CD8+ LP to elucidate the clinical course, prognosis, and outcomes. Methods Demographic, clinical, and treatment data were reviewed. Histopathologic data based on architectural, cytomorphologic, and immunohistochemical features were assessed. Immunohistochemical staining for T and B cell markers was evaluated. Results A total of 25 patients including 22 with CD4+ SMPTCL and three with CD8+ LP were identified. All patients presented with a single lesion, predominantly on the head, neck, or upper trunk (84%). No patients showed extracutaneous disease at any evaluation. The most common histopathologic changes showed a dense nodular infiltrate of small cells with hyperchromatic nuclei without significant follicular or adnexal involvement. Patients were treated with excision (48%), local radiation (28%), or topical or intralesional steroids (24%). All patients achieved complete resolution of disease. Five patients demonstrated cutaneous relapse at new sites. Conclusions The CD4+ SMPTCL/ CD8+ LP subgroup usually presents with solitary lesions and demonstrates an indolent clinical course. Typical presentation, classic histopathology, widespread expression of follicular T helper cell markers, and loss of a T cell antigen are diagnostic features of CD4+ SMPTCL, whereas monomorphous CD8+ infiltrate without follicular T helper cell markers is consistent with CD8+ LP. Local skin-directed therapy is appropriate in these patients. [ABSTRACT FROM AUTHOR]

Conventional presentations of mycosis fungoides may be diagnostically challenging, particularly in light of the controversial boundaries defining the disease. Variant presentations of this cutaneous T-cell lymphoma add a further layer of complexity, requiring a sophisticated and informed perspective when evaluating lymphoid infiltrates in the skin. Herein we discuss well-defined (WHO-EORTC) variants pagetoid reticulosis, granulomatous slack skin and folliculotropic mycosis fungoides as well as less well-defined morphologic/architectural variants, and divergent immunohistochemical presentations of this typically indolent T-cell lymphoproliferative disease. [ABSTRACT FROM AUTHOR]

Background: Granulomatous mycosis fungoides (GMF) is an uncommon variant of mycosis fungoides (MF). Objective: We sought to analyze the relative frequency, clinicopathologic characteristics, prognosis, and therapeutic responses of GMF. Methods: We conducted a retrospective case-control study of patients with GMF and age- and stage-matched patients with classic MF between 1981 and 2012. Results: A total of 27 patients with GMF were identified, representing 6.3% of all patients with MF at our center. Skin manifestations were similar to classic MF having an atypical lichenoid CD4+ CD8− lymphocytic infiltrate with interstitial histiocytes and/or perivascular granulomas with giant cells. Fewer patients with GMF achieved a partial response or complete response with topical (57% vs 83%; P = .002) or ultraviolet light (62% vs 90%; P = .006) therapy. The 5- and 10-year progression-free survival rates were significantly lower in patients with GMF (59% and 33%) compared with patients with classic MF (84% and 56%; P = .02), but overall survival was similar between groups (86% and 72% vs 85% and 85%; P = .54). Limitations: The retrospective methodology may underestimate the frequency of GMF. The median follow-up time may be too short to detect possible differences in overall survival. Conclusion: More frequent disease progression and poorer response to skin-directed therapies were observed in patients with GMF. Our findings may be helpful in selecting the most appropriate treatment for these patients. [Copyright &y& Elsevier]