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ABSTRACT: Nevi of specialized sites (NOSS) occur on the scalp, ears, flexural, acral, and genital areas and display atypical clinical and histologic features. We assessed NOSS recurrence and progression to melanoma, management patterns, and associations between histologic features and treatment recommendations. We queried all histologic diagnoses of NOSS (n = 275) from 2012 to 2017 from a large U.S. academic medical center with reference dermatopathology laboratory and matched these to clinical records. A blinded panel of dermatopathologists re-evaluated lesions, catalogued histologic findings, and gave management recommendation. Associations with dermatopathologist decision and concordance between new and original recommendations were assessed. Of 117 cases with follow-up, 2 locally recurred (1.46%) and none eventuated in melanoma. Clinical features were not associated with original treatment recommendations. After histopathologic review, large melanocytes [odds ratio ratio (ORR) = 8.00, 95% CI, 1.35-47.4] and junctional mitotic figures (ORR = 65.0, 6.5-650) predicted excision recommendation. Likewise, accumulation of many (>9) high-risk features was associated with excision recommendation. Panel review changed treatment recommendation in 27% of cases. Fair concordance existed between original and panel recommendations (κ = 0.29, 0.15-0.44). The low rate of recurrence and lack of melanoma occurrence suggest that despite an atypical clinical and histopathologic appearance, these nevi have limited potential for malignant transformation. Histopathologic findings seem to be principal drivers behind the recommendation for excision in this analysis. Variability existed in treatment recommendations; the panel's consensus recommendation tended to downgrade treatment. This highlights the importance of further outcomes-based studies to identify true high-risk features and refine management guidelines.
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Melanoma , Nevo , Neoplasias Cutâneas , Humanos , Neoplasias Cutâneas/patologia , Estudos de Coortes , Melanoma/patologia , Nevo/terapia , Nevo/patologia , Melanócitos/patologiaRESUMO
BACKGROUND: Studies characterizing clinical and pathologic details of pretibial pruritic papular dermatitis (PPPD) are scarce. Several cases of PPPD at our institution have displayed lymphocyte atypia and CD30 positivity, resembling lymphomatoid papulosis (LyP). We explore the clinical and histological spectrum of PPPD, with emphasis on lymphocyte atypia. METHODS: Retrospective observational study of 40 archived pathological specimens (hematoxylin/eosin, CD3, CD20, and CD30 immunohistochemistry) from 38 PPPD patients in an academic center. Clinical photographs were available in 22 cases. RESULTS: Microscopic epidermal changes were focal, but common (spongiosis 75%, parakeratosis 90%, interface changes 43%, Langerhans cell microgranulomas 25%, multinucleated keratinocytes 55%, Civatte bodies 55%, erosion 20%, and more than focal irregular psoriasiform hyperplasia 37%) and certain dermal changes were universal (papillary dermal fibrosis 100%, stellate fibroblasts 100%, and multinucleated fibroblasts 93%). At least focal lymphocyte atypia was present in all cases. Lymphocytes were almost exclusively CD3 T cells with rare CD20 B cells. Up to 30% of lymphocytes exhibited weak CD30 staining. Clinically, all cases exhibited discrete papules, but plaques and erosions were not uncommon. LIMITATIONS: As a retrospective series, clinical images were not available for all cases. CONCLUSION: This study suggests a broader histological and clinical spectrum of PPPD than previously described. Epidermal changes are common in PPPD, as are atypical lymphocytes and focal CD30 positivity. Although the papular clinical appearance, lymphocyte atypia, and focal CD30 positivity may resemble LyP, the relatively low number of atypical lymphocytes, low intensity of CD30 staining, and absence of spontaneous resolution help to distinguish PPPD from LyP.
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Dermatite/diagnóstico , Dermatite/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Papulose Linfomatoide/diagnóstico , Papulose Linfomatoide/patologia , Masculino , Pessoa de Meia-Idade , Prurido/patologia , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Angioimmunoblastic T-cell lymphoma (AITL) is a systemic peripheral T-cell lymphoma with a follicular helper T-cell (TFH ) immunophenotype that frequently involves the skin. However, the histopathology of cutaneous involvement by AITL has not been fully established. METHODS: We reviewed the clinicopathological features of 19 patients seen at our institution with AITL involving the skin. Pan-T-cell and TFH marker expression was evaluated by immunohistochemistry. Epstein-Barr virus (EBV) was detected using in situ hybridization (ISH) for Epstein-Barr virus-encoded small RNA (EBER). T-cell receptor (TCR) gene rearrangement was evaluated by PCR. RESULTS: AITL affected both trunk and extremities in 15/19 cases (79%). Perivascular infiltration by small and/or medium-sized lymphocytes was seen in 18/19 (95%). Granulomatous inflammation was identified in 4/19 (21%). Aberrant loss of CD2, CD5, or CD7 was identified in 1/18 (6%), 2/18 (11%), or 7/19 (37%) cases, respectively. Seventeen of eighteen evaluable cases (95%) expressed 2 to 3 TFH markers: PD-1 in 19/19 (100%), BCL6 in 94% (17/18), and CD10 in 37% (7/19). EBV-positive cells were detected in 3/18 (17%) with varying density. Clonal TCR gene rearrangement was identified in 9/11 (82%). CONCLUSIONS: Cutaneous involvement by AITL shows relatively non-specific histopathological features. However, an immunohistochemical panel including TFH markers and EBER ISH is useful in differential diagnosis.
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Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4/imunologia , Linfoma Cutâneo de Células T , Linfoma de Células T Periférico , Neoplasias Cutâneas , Linfócitos T Auxiliares-Indutores , Adulto , Idoso , Idoso de 80 Anos ou mais , Infecções por Vírus Epstein-Barr/imunologia , Infecções por Vírus Epstein-Barr/patologia , Infecções por Vírus Epstein-Barr/virologia , Feminino , Humanos , Imunofenotipagem , Linfoma Cutâneo de Células T/imunologia , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/virologia , Linfoma de Células T Periférico/patologia , Linfoma de Células T Periférico/virologia , Masculino , Pessoa de Meia-Idade , Neoplasias Cutâneas/imunologia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/virologia , Linfócitos T Auxiliares-Indutores/imunologia , Linfócitos T Auxiliares-Indutores/patologia , Linfócitos T Auxiliares-Indutores/virologiaAssuntos
Janus Quinases/metabolismo , Linfoma de Células T/metabolismo , Fatores de Transcrição STAT/metabolismo , Transdução de Sinais , Antineoplásicos/farmacologia , Antineoplásicos/uso terapêutico , Humanos , Janus Quinases/genética , Linfoma de Células T/tratamento farmacológico , Linfoma de Células T/genética , Terapia de Alvo Molecular , Mutação/efeitos dos fármacos , Fatores de Transcrição STAT/genética , Transdução de Sinais/efeitos dos fármacosRESUMO
BACKGROUND: Cutaneous carcinosarcoma is a rare tumor with distinct malignant epithelial and mesenchymal cell populations. The histologic subtypes of epithelial and mesenchymal components in cutaneous carcinosarcoma are variable, as an assortment of carcinomatous and sarcomatous patterns have been described in the literature. METHODS: Clinical information was obtained from patient charts and archival slides were retrieved and reviewed. RESULTS: We present a novel series of six distinct cases of cutaneous carcinosarcoma and review the literature. Our cases consisted of basal cell, pilomatrical, squamous cell, and trichoblastic variants. These cases occurred in elderly men on sun exposed skin with treatment and follow up was available for 4 of 6 cases. The four cases were treated with Mohs micrographic surgery with mean follow up of nine months. CONCLUSION: We report six cases of cutaneous carcinosarcoma with distinctive clinical and histologic characterization not previously described in a single series.
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Carcinossarcoma/patologia , Neoplasias Cutâneas/patologia , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Feminino , Humanos , Imuno-Histoquímica , MasculinoRESUMO
The cell of origin and the tumor microenvironment's role remain elusive for the most common peripheral T-cell lymphomas (PTCLs). As macrophages promote the growth and survival of malignant T cells and are abundant constituents of the tumor microenvironment, their functional polarization was examined in T-cell lymphoproliferative disorders. Cytokines that are abundant within the tumor microenvironment, particularly interleukin (IL)-10, were observed to promote alternative macrophage polarization. Macrophage polarization was signal transducer and activator of transcription 3 dependent and was impaired by the Janus kinase inhibitor ruxolitinib. In conventional T cells, the production of T helper (Th)2-associated cytokines and IL-10, both of which promote alternative macrophage polarization, is regulated by the T-cell transcription factor GATA-binding protein 3 (GATA-3). Therefore, its role in the T-cell lymphomas was examined. GATA-3 expression was observed in 45% of PTCLs, not otherwise specified (PTCL, NOS) and was associated with distinct molecular features, including the production of Th2-associated cytokines. In addition, GATA-3 expression identified a subset of PTCL, NOS with distinct clinical features, including inferior progression-free and overall survival. Collectively, these data suggest that further understanding the cell of origin and lymphocyte ontogeny among the T-cell lymphomas may improve our understanding of the tumor microenvironment's pathogenic role in these aggressive lymphomas.
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Fator de Transcrição GATA3/genética , Interleucina-10/genética , Linfoma de Células T Periférico/genética , Microambiente Tumoral/genética , Western Blotting , Linhagem Celular Tumoral , Citocinas/genética , Citocinas/metabolismo , Fator de Transcrição GATA3/metabolismo , Regulação Neoplásica da Expressão Gênica , Humanos , Imuno-Histoquímica , Interleucina-10/metabolismo , Estimativa de Kaplan-Meier , Linfoma de Células T Periférico/metabolismo , Linfoma de Células T Periférico/patologia , Macrófagos/efeitos dos fármacos , Macrófagos/metabolismo , Macrófagos/patologia , Nitrilas , Pirazóis/farmacologia , Pirimidinas , Interferência de RNA , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Fatores de Risco , Linfócitos T/metabolismo , Linfócitos T/patologia , Células Th2/metabolismo , Células Th2/patologiaRESUMO
BACKGROUND: In patients with malignancy-associated Sweet syndrome, a thorough evaluation for leukemia cutis should be considered. OBJECTIVE: We sought to describe the clinicopathologic characteristics of histiocytoid Sweet syndrome. METHODS: We retrospectively identified patients with histiocytoid Sweet syndrome at our institution from January 1992 through December 2010. We evaluated the underlying cutaneous infiltrate using immunohistochemistry and fluorescence in situ hybridization. RESULTS: We re-evaluated all 22 patients with hematologic malignancy-associated Sweet syndrome. Six patients had a monocytoid infiltrate that was consistent with histiocytoid Sweet syndrome; subsequent evaluation of these patients demonstrated cytogenetic abnormalities on prior bone-marrow biopsy specimens. Fluorescence in situ hybridization analysis was feasible in cutaneous specimens from 5 of the 6 patients and demonstrated the same cytogenetic abnormalities that were identified on prior bone-marrow biopsy specimens in 4 patients. Therefore, these 4 patients may have had a form of leukemia cutis. LIMITATIONS: This was a retrospective study. CONCLUSION: For patients with histiocytoid Sweet syndrome, an underlying hematologic malignancy, and a monocytoid infiltrate on biopsy specimen, fluorescence in situ hybridization of the cutaneous infiltrate may be beneficial to identify cytogenetic abnormalities that may indicate leukemia cutis.
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Histiócitos/patologia , Hibridização in Situ Fluorescente/métodos , Leucemia/diagnóstico , Neoplasias Cutâneas/diagnóstico , Síndrome de Sweet/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Biópsia por Agulha , Aberrações Cromossômicas , Estudos de Coortes , Comorbidade , Feminino , Seguimentos , Humanos , Imuno-Histoquímica , Incidência , Leucemia/epidemiologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Medição de Risco , Neoplasias Cutâneas/epidemiologia , Síndrome de Sweet/epidemiologiaRESUMO
BACKGROUND: Sweet syndrome is a neutrophilic dermatosis with cutaneous tender lesions that can be associated with malignancies, infections, systemic inflammatory disorders, and medications. Although numerous studies have described Sweet syndrome, few studies have systematically investigated Sweet syndrome. OBJECTIVE: We sought to describe characteristics and treatments of patients with Sweet syndrome and evaluate clinical differences depending on the underlying cause. METHODS: A retrospective study was conducted to identify patients with Sweet syndrome evaluated at Mayo Clinic from 1992 to 2010. RESULTS: Of 77 patients with Sweet syndrome (mean age of onset 57 years), 43 (56%) were male. Eighteen patients (23%) reported a preceding infection. A total of 41 (53%) patients were classified as having classic Sweet syndrome, 27 (35%) patients had malignancy-associated Sweet syndrome, and in 9 (12%) patients drug-induced Sweet syndrome was considered. In all, 21 patients had a hematologic malignancy or myeloproliferative/myelodysplastic disorder, whereas 6 patients had solid tumors. The mean hemoglobin level, in both male and female patients (P < .0443 and P < .0035, respectively), was significantly lower in malignancy-associated versus classic and drug-induced Sweet syndrome. Systemic corticosteroids were the most frequently used treatment (70%). LIMITATIONS: This is a retrospective study and represents patients from a single academic center. CONCLUSIONS: Sweet syndrome is a distinctive disorder with certain clinical and histologic characteristics, which usually has a complete response to systemic corticosteroids. It is important to evaluate Sweet syndrome patients who have laboratory evidence of anemia for an underlying malignancy.
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Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Neoplasias/epidemiologia , Síndrome de Sweet/tratamento farmacológico , Síndrome de Sweet/epidemiologia , Centros Médicos Acadêmicos , Adjuvantes Imunológicos/uso terapêutico , Corticosteroides/uso terapêutico , Adulto , Fatores Etários , Idade de Início , Idoso , Idoso de 80 Anos ou mais , Antibacterianos/uso terapêutico , Antineoplásicos/uso terapêutico , Estudos de Coortes , Comorbidade , Fármacos Dermatológicos/uso terapêutico , Quimioterapia Combinada , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Neoplasias/patologia , Estudos Retrospectivos , Medição de Risco , Índice de Gravidade de Doença , Fatores Sexuais , Síndrome de Sweet/etiologia , Síndrome de Sweet/fisiopatologia , Resultado do TratamentoRESUMO
Background: Cutaneous hypersensitivity eruptions in chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) are a clinically and histologically heterogeneous group that can either precede, occur with, or follow the development of a hematologic malignancy. Therefore, establishing the diagnosis requires careful clinical and pathologic correlation and an understanding of the broad spectrum of presentations. Data is lacking on the correlation of skin disease with molecular/cytogenetic risk profiling of the tumor. Objectives: The aims of this study were to characterize the clinical, histological, and genetic aberrations in recurrent cutaneous hypersensitivity reactions in patients with CLL/SLL. Methods: A single site academic retrospective chart review of medical records, histopathology, molecular and cytogenetic data in CLL/SLL patients who developed biopsy-proven cutaneous hypersensitivity reactions. Results: Five hundred one new diagnoses of CLL/SLL with 73 patients requiring cutaneous biopsies for skin lesions or rashes were identified. With exclusion criteria, 20 biopsies were identified from 17 patients (mean age, 69.6 years, females = 9) with unexplained cutaneous eruptions. These were commonly pruritic, erythematous papules above the waist. Most biopsies had a prominent superficial, deep dermal eosinophilic infiltrate (85%), with a robust T-cell predominant dermal infiltrate in 40%. Five out of 17 patients (29%) had a predominately folliculocentric CD4+ T-cell infiltrate; all occurring on the head and neck. Overall, the prevalence of cutaneous hypersensitivity eruptions requiring biopsy was 3.4% (n = 17), and the prevalence of folliculocentric CD4+ T-cell infiltrate was 1% (n = 5). Conclusion: Cutaneous hypersensitivity reactions in CLL/SLL are heterogeneous; however, folliculotropic CD4+ T-cell infiltrates may be seen in a small but distinct clinical subset of patients. Commonly tested cytogenetic aberrations in CLL/SLL do not appear to be correlated with the presence of cutaneous hypersensitivity reactions.
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The objective of this study was to evaluate the feasibility of developing personalized, tumor-informed assays for patients with high-risk resectable melanoma and examine circulating tumor DNA (ctDNA) levels in relation to clinical status. Pilot prospective study of clinical stage IIB/C and resectable stage III melanoma patients. Tumor tissue was used to design bespoke somatic assays for interrogating ctDNA in patients' plasma using a multiplex PCR (mPCR) next-generation sequencing (NGS)-based approach. Plasma samples for ctDNA analysis were collected pre-/post-surgery and during surveillance. Out of 28 patients (mean 65â years, 50% male), 13 (46%) had detectable ctDNA prior to definitive surgery and 96% (27/28) tested ctDNA-negative within 4â weeks post-surgery. Pre-surgical detection of ctDNA was significantly associated with the later-stage ( P â =â 0.02) and clinically evident stage III disease ( P â =â 0.007). Twenty patients continue in surveillance with serial ctDNA testing every 3-6â months. With a median follow-up of 443â days, six out of 20 (30%) patients developed detectable ctDNA levels during surveillance. All six of these patients recurred with a mean time to recurrence of 280â days. Detection of ctDNA in surveillance preceded the diagnosis of clinical recurrence in three patients, was detected concurrent with clinical recurrence in two patients and followed clinical recurrence in one patient. One additional patient developed brain metastases without detection of ctDNA during surveillance but had positive pre-surgical ctDNA. Our results demonstrate the feasibility of obtaining a personalized, tumor-informed mPCR NGS-based ctDNA assay for patients with melanoma, particularly in resectable stage III disease.
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DNA Tumoral Circulante , Melanoma , Neoplasias Cutâneas , Humanos , Masculino , Feminino , Melanoma/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/genética , Estudos Prospectivos , Estudos de Viabilidade , Biomarcadores Tumorais/genética , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/patologia , MutaçãoRESUMO
BACKGROUND: Although rare, cutaneous small-vessel vasculitis (CSVV) secondary to solid organ malignancy has been documented. OBJECTIVE: We sought to better understand the frequency, clinical course, therapeutic response, and outcome of CSVV associated with solid organ malignancy. METHODS: We conducted a retrospective chart review of patients seen between 1996 and 2009 with diagnoses of biopsy-proven cutaneous leukocytoclastic vasculitis and solid organ malignancy separated by less than 12 months. RESULTS: Of 17 patients (mean age, 66.5 years), 10 patients (59%) were male. CSVV occurred before (3 patients; 18%), concurrent with (3 patients; 18%), and after (11 patients; 65%) diagnosis of solid organ malignancy. The most common solid organ malignancy was of the lung (n = 4; 24%). Other associated cancers were breast (n = 3); prostate (n = 2); colon (n = 2); renal (n = 2); thyroid (n = 1); bladder (n = 1); gallbladder (n = 1); and peritoneal (n = 1). Three patients had cutaneous vasculitis in association with malignancy recurrence despite having no cutaneous vasculitis associated with their primary malignancy. Vasculitis remission with use of immunosuppressive agents alone occurred in 9 patients (53%). Eleven patients (65%) were alive at last follow-up (mean follow-up duration, 27 months). LIMITATIONS: This was a retrospective study with a relatively small number of patients. CONCLUSION: Solid organ malignancy should be considered as a possible cause of CSVV of unknown origin. In contrast to previous reports, our patients were more likely to respond to immunosuppressive therapies without treatment of the associated malignancy and to be alive at last follow-up.
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Neoplasias/complicações , Vasculite Leucocitoclástica Cutânea/etiologia , Idoso , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Vasculite Leucocitoclástica Cutânea/complicações , Vasculite Leucocitoclástica Cutânea/tratamento farmacológicoRESUMO
BACKGROUND: Lymphomatoid papulosis (LyP) is a benign recurrent papulonodular skin eruption with histologically malignant features that sometimes (10%-20%) progresses to lymphoma. OBJECTIVE: We retrospectively evaluated the clinical course of patients with LyP and identify prognostic factors possibly indicating a malignant course. METHODS: Clinical, histopathologic, and immunologic features and molecular genetics were examined and correlated with clinical course and outcomes. Immunophenotyping and chemokine profiling were performed in select skin biopsy samples. A follow-up questionnaire was sent to patients. Clinical course and association with neoplastic disorders were correlated with LyP subtypes, molecular genetics, and immunophenotyping studies. RESULTS: Of 123 patients with LyP (1991-2008) followed up a mean of 4 years (range, 2 months to 14 years), 17 (14%) had an associated hematologic malignancy, 8 of which were mycosis fungoides. Histopathologic analyses demonstrated classic LyP type A (n = 69), B (n = 13), or C (n = 6), and a slight predominance of T-cell CD8 marker expression for type A. More than one type of lesion was present in 9 patients with a higher incidence of hematologic malignancies. T-cell receptor gene rearrangement positivity was about two times higher, with LyP associated with hematologic malignancy (82% vs 44%; odds ratio 5.7; P = .02). Chemokine studies in a subset of 25 patients showed chemokine receptor (CCR) CCR4(+) and thymus and activation-related chemokine (TARC(+)) in all LyP types and CCR3(+) and chemokine-related receptor (CXCR) CXCR3(+) in types B and C. LIMITATIONS: Retrospective study design is a limitation. CONCLUSIONS: Positive T-cell receptor gene rearrangement or diagnosis of mixed-type LyP may be a prognostic indicator of disease more prone to progress to lymphoma.
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Transformação Celular Neoplásica/genética , Rearranjo Gênico do Linfócito T , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Transformação Celular Neoplásica/imunologia , Transformação Celular Neoplásica/patologia , Criança , Pré-Escolar , Progressão da Doença , Feminino , Perfilação da Expressão Gênica , Humanos , Papulose Linfomatoide/genética , Papulose Linfomatoide/imunologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Receptores de Antígenos de Linfócitos T/genética , Estudos Retrospectivos , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/imunologia , Adulto JovemRESUMO
BACKGROUND: T cells expressing the γδ T-cell receptor (TCR) (γδ T cells) are found in normal epithelial tissues such as the skin. However, the proportions of γδ T cells that may be observed in commonly encountered cutaneous diseases with a prominent lymphocytic infiltrate have not been elucidated. METHODS: Our pathology database was searched for cases of mycosis fungoides, erythema multiforme, graft-versus-host disease, lichen planus, lupus panniculitis and spongiotic dermatitis. Immunostaining for CD3, ßF1 and the TCR γ chain was performed on formalin-fixed paraffin-embedded specimens retrieved from these cases to determine the normal range of γδ T cells in these diseases. RESULTS: In 100 of the 101 cases studied (99.0%), γδ T cells accounted for less than 10% of the T-cell infiltrate. Furthermore, γδ T cells were essentially absent in 74 cases (73.3%). CONCLUSIONS: These results suggest that γδ T cells very rarely account for more than 10% of the lymphocytic infiltrate in common inflammatory or infiltrative processes of the skin. Any case of suspected mycosis fungoides or interface dermatitis that possesses more than 10% γδ T cells should raise consideration for further investigation.
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Regulação Neoplásica da Expressão Gênica , Micose Fungoide/metabolismo , Receptores de Antígenos de Linfócitos T alfa-beta/biossíntese , Dermatopatias/metabolismo , Neoplasias Cutâneas/metabolismo , Pele/metabolismo , Linfócitos T/metabolismo , Feminino , Humanos , Masculino , Micose Fungoide/patologia , Pele/patologia , Dermatopatias/patologia , Neoplasias Cutâneas/mortalidade , Linfócitos T/patologiaRESUMO
Adult Henoch-Schönlein purpura (HSP) is rarely associated with solid-organ malignancies. We describe here three adult patients with HSP diagnosed within 3 months of the diagnosis of associated solid-organ malignancies, including pulmonary, prostate, and renal carcinomas. Two patients had complete remission with a combination of immunosuppressive therapies and treatment of the associated malignancy. The third patient had partial remission with immunosuppressive therapies, but never received treatment for the associated malignancy and did not achieve complete remission before his death 10 months after diagnosis of HSP. These cases suggest that HSP associated with solid-organ malignancies may be resistant to immunosuppressive therapies without treatment of the associated malignancy. Therefore, evaluation for solid-organ malignancies should be considered in adult patients without an identifiable cause of HSP, especially if the disease is not self-limited or does not respond appropriately to treatment.
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Vasculite por IgA/complicações , Neoplasias Renais/complicações , Neoplasias Pulmonares/complicações , Neoplasias da Próstata/complicações , Idoso , Feminino , Humanos , Vasculite por IgA/diagnóstico , Vasculite por IgA/tratamento farmacológico , Imunossupressores/uso terapêutico , Neoplasias Renais/diagnóstico , Neoplasias Renais/terapia , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/terapia , Masculino , Pessoa de Meia-Idade , Neoplasias da Próstata/diagnóstico , Neoplasias da Próstata/terapia , Indução de Remissão , Pele/efeitos dos fármacos , Pele/patologia , Resultado do TratamentoRESUMO
Angiosarcoma is an uncommon but aggressive tumor of endothelial origin that may occur in the upper extremities of patients with postmastectomy lymphedema (Stewart-Treves syndrome) as well as in other regions. We present an unusual case of angiosarcoma associated with congenital nonhereditary lymphedema in an 18-year-old man. Our case underscores the need for a careful clinical examination and shows the importance of appropriate sampling and thorough pathologic examination of suspicious areas to exclude the presence of a malignant process.
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Doenças do Pé/patologia , Hemangiossarcoma/complicações , Linfedema/congênito , Linfedema/complicações , Neoplasias Cutâneas/complicações , Adolescente , Amputação Cirúrgica , Doenças do Pé/cirurgia , Hemangiossarcoma/patologia , Hemangiossarcoma/cirurgia , Humanos , Perna (Membro)/cirurgia , Linfedema/patologia , Masculino , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/cirurgiaRESUMO
Mycosis Fungoides (MF) is a rare T-cell lymphoma and evidence on treatment practices, and outcomes are limited. We evaluated changes in practice patterns and corresponding effects on overall survival (OS) in MF using a cross-sectional study of patients diagnosed with MF from 2004 to 2016 in the National Cancer Database. Outcomes evaluated included patterns of care and OS across treatment eras. We found factors associated with chemotherapy use included male gender, treatment from 2004 to 2010 and stage III-IV disease. Factors associated with radiotherapy receipt included stage I-II disease, nonacademic treatment centers, male gender, non-black race, and Medicare status. Immunotherapy use was associated with treatment from 2004 to 2010 and stage III-IV disease. After propensity score matching, there was no OS difference among patients with stage I-II disease between 2004-2010 and 2011-2016. However, amongst patients with stage III-IV disease, OS was significantly improved in those treated from 2011 to 2016.
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Micose Fungoide , Neoplasias Cutâneas , Idoso , Estudos Transversais , Humanos , Masculino , Medicare , Micose Fungoide/diagnóstico , Micose Fungoide/terapia , Estadiamento de Neoplasias , Prognóstico , Estudos Retrospectivos , Neoplasias Cutâneas/patologia , Taxa de Sobrevida , Estados Unidos/epidemiologiaRESUMO
Current pathologic criteria cannot reliably distinguish cutaneous anaplastic large cell lymphoma from other CD30-positive T-cell lymphoproliferative disorders (lymphomatoid papulosis, systemic anaplastic large cell lymphoma with skin involvement, and transformed mycosis fungoides). We previously reported IRF4 (interferon regulatory factor-4) translocations in cutaneous anaplastic large cell lymphomas. Here, we investigated the clinical utility of detecting IRF4 translocations in skin biopsies. We performed fluorescence in situ hybridization (FISH) for IRF4 in 204 biopsies involved by T-cell lymphoproliferative disorders from 182 patients at three institutions. In all, 9 of 45 (20%) cutaneous anaplastic large cell lymphomas and 1 of 32 (3%) cases of lymphomatoid papulosis with informative results demonstrated an IRF4 translocation. Remaining informative cases were negative for a translocation (7 systemic anaplastic large cell lymphomas; 44 cases of mycosis fungoides/Sézary syndrome (13 transformed); 24 peripheral T-cell lymphomas, not otherwise specified; 12 CD4-positive small/medium-sized pleomorphic T-cell lymphomas; 5 extranodal NK/T-cell lymphomas, nasal type; 4 gamma-delta T-cell lymphomas; and 5 other uncommon T-cell lymphoproliferative disorders). Among all cutaneous T-cell lymphoproliferative disorders, FISH for IRF4 had a specificity and positive predictive value for cutaneous anaplastic large cell lymphoma of 99 and 90%, respectively (P=0.00002, Fisher's exact test). Among anaplastic large cell lymphomas, lymphomatoid papulosis, and transformed mycosis fungoides, specificity and positive predictive value were 98 and 90%, respectively (P=0.005). FISH abnormalities other than translocations and IRF4 protein expression were seen in 13 and 65% of cases, respectively, but were nonspecific with regard to T-cell lymphoproliferative disorder subtype. Our findings support the clinical utility of FISH for IRF4 in the differential diagnosis of T-cell lymphoproliferative disorders in skin biopsies, with detection of a translocation favoring cutaneous anaplastic large cell lymphoma. Like all FISH studies, IRF4 testing must be interpreted in the context of morphology, phenotype, and clinical features.
Assuntos
Biomarcadores Tumorais/genética , Fatores Reguladores de Interferon/genética , Linfoma Anaplásico Cutâneo Primário de Células Grandes/genética , Transtornos Linfoproliferativos/genética , Neoplasias Cutâneas/genética , Translocação Genética , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Biópsia , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Hibridização in Situ Fluorescente , Linfoma Anaplásico de Células Grandes/genética , Linfoma Anaplásico Cutâneo Primário de Células Grandes/química , Linfoma Anaplásico Cutâneo Primário de Células Grandes/patologia , Papulose Linfomatoide/genética , Transtornos Linfoproliferativos/metabolismo , Transtornos Linfoproliferativos/patologia , Masculino , Pessoa de Meia-Idade , Micose Fungoide/genética , Valor Preditivo dos Testes , Sensibilidade e Especificidade , Neoplasias Cutâneas/química , Neoplasias Cutâneas/patologia , Estados Unidos , Adulto JovemRESUMO
Stromal elements present within the tumor microenvironment may suppress host immunity and promote the growth of malignant lymphocytes in B cell-derived non-Hodgkin lymphoma (NHL). In contrast, little is known about the microenvironment's role in T cell-derived NHL. B7-H1 (PD-L1, CD274), a member of the B7 family of costimulatory/co-inhibitory ligands expressed by both malignant cells and stromal cells within the tumor microenvironment, has emerged as an important immune modulator capable of suppressing host immunity. Therefore, B7-H1 expression and function were analyzed in cutaneous and peripheral T-cell NHL. B7-H1 was expressed by tumor cells, monocytes, and monocyte-derived cells within the tumor microenvironment in T-cell NHL and was found to inhibit T-cell proliferation and promote the induction of FoxP3(+) regulatory T cells. Collectively, the data presented provide the first evidence implicating B7-H1 in the suppression of host immunity in T-cell lymphoproliferative disorders and suggest that the targeting of B7-H1 may represent a novel therapeutic approach.