RESUMEN
Expression of CD30 in blastoid cutaneous infiltrates typically signifies a CD30 lymphoproliferative disorder, often requiring minimal immunohistochemical workup, if clinically consonant. However, myeloid and other hematologic malignancies often express CD30. We retrospectively examined the prevalence of CD30 expression in 41 patients (median age 59) and 55 biopsies with the diagnosis of leukemia cutis (LC) to determine whether an extensive immunohistochemical workup is warranted in all large, round cell CD30 cutaneous infiltrates. Each patient had refractory or recurrent disease, the histologic presence of a large mononuclear cell infiltrate, and varied cytogenetics. CD30 mononuclear cells within the infiltrate ranged from rare to many in 22 biopsies (22/55). In 18 biopsies, CD30 cells were interpreted as lymphocytic based on morphology, strong cytoplasmic and Golgi staining for CD30, and negative CD34 and CD117 staining. One case showing 3+ staining of lymphocytes was identified as a posttransplant lymphoproliferative disorder. The second 3+ case was favored to represent a subset of CD30-positive acute myeloid leukemia. Three other cases with 1+ membranous and cytoplasmic staining were interpreted as myeloid leukemia. In conclusion, CD30 positivity in myeloid leukemia in the skin is rare and does not often exhibit the strong membranous (2+ or 3+) and/or Golgi staining seen in reactive lymphocytes. Acute myeloid leukemia or myeloid LC may occasionally show 1+ (and rarely 2-3+) cytoplasmic/membranous or nonspecific blush nuclear CD30 labeling. Strong diffuse staining for CD30 should prompt consideration of a reactive lymphoid/lymphoproliferative process, and, when the clinical likelihood of CD30 LC is low, may obviate the need for further immunohistochemistry.
Asunto(s)
Antígeno Ki-1/inmunología , Leucemia Mieloide Aguda/patología , Neoplasias Cutáneas/patología , Adolescente , Adulto , Distribución por Edad , Anciano , Algoritmos , Biomarcadores/metabolismo , Biopsia con Aguja , Niño , Estudios de Cohortes , Femenino , Humanos , Inmunohistoquímica , Incidencia , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/epidemiología , Leucemia Mieloide Aguda/genética , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Medición de Riesgo , Distribución por Sexo , Neoplasias Cutáneas/diagnóstico , Neoplasias Cutáneas/inmunología , Neoplasias Cutáneas/fisiopatología , Adulto JovenRESUMEN
Anaplastic large cell lymphoma (ALCL) either as primary cutaneous or nodal disease is rare in children and difficult to distinguish, which is important both prognostically and for treatment purposes. We present a case of anaplastic lymphoma kinase (ALK)+ skin-limited ALCL that highlights this challenge and draws attention to pitfalls in assessing ALK status. The patient was an 11-year old girl with a twice recurrent nodule on her right shoulder. Each biopsy revealed a deep infiltrate of atypical lymphocytes that expressed CD3, CD4, CD43, CD45RO and CD30. The initial biopsy was epithelial membrane antigen (EMA)+ with vague cytoplasmic ALK-1 positivity by immunohistochemistry, while the second biopsy was EMA+ and nuclear ALK-1+. Fluorescence in situ hybridization analysis for an ALK (2p23) rearrangement of the first specimen was negative, while an ALK gene rearrangement was present in the second specimen. Therefore, this case was treated as nodal ALCL, despite negative bone marrow and radiographic imaging studies. The patient was treated with combination chemotherapy and remains disease-free. Demonstration of nuclear ALK-positivity, ALK (2p23) gene rearrangement is suggestive of systemic ALCL. Without evidence of systemic disease, this case highlights challenges of skin-limited ALCL, whose clinical behavior as either cutaneous ALCL systemic ALCL may not be immediately apparent.
Asunto(s)
Linfoma Anaplásico de Células Grandes/patología , Linfoma Cutáneo de Células T/patología , Proteínas Tirosina Quinasas Receptoras/metabolismo , Neoplasias Cutáneas/patología , Quinasa de Linfoma Anaplásico , Biopsia , Niño , Supervivencia sin Enfermedad , Femenino , Humanos , Linfocitos/patología , Linfoma Anaplásico de Células Grandes/tratamiento farmacológico , Linfoma Anaplásico de Células Grandes/enzimología , Linfoma Anaplásico de Células Grandes/genética , Linfoma Cutáneo de Células T/tratamiento farmacológico , Linfoma Cutáneo de Células T/enzimología , Linfoma Cutáneo de Células T/genética , Mucina-1/genética , Pronóstico , Proteínas Tirosina Quinasas Receptoras/genética , Neoplasias Cutáneas/enzimología , Neoplasias Cutáneas/genética , Translocación GenéticaRESUMEN
Epithelioid sarcoma (ES) represents an aggressive soft tissue tumor with varied morphologic and histopathologic presentations that typically elicits a broad differential diagnosis, including granuloma annulare, necrobiotic granuloma, fibrous histiocytoma, synovial sarcoma, amelanotic melanoma and poorly differentiated primary cutaneous and metastatic adenocarcinoma. ES is characterized microscopically by a nodular arrangement of abundant, deeply eosinophilic, polygonal tumor cells with frequent central necrosis and hemorrhage, rare mitotic figures and minimal pleomorphism. At the periphery, tumor cells are spindle shaped and may exhibit frequent local infiltration along tendons, fascial planes and neurovascular bundles. Immunohistochemistry typically reveals expression of both epithelial and mesenchymal antigens, such as cytokeratin and vimentin, respectively. The absence of a connection between tumor cells and the overlying epidermis, with or without an in situ carcinoma component, typically rules out a primary cutaneous squamous cell carcinoma. We report a case of stage IV proximal-type ES that mimicked molluscum contagiosum clinically and was histopathologically reminiscent of invasive squamous cell carcinoma because of attachment and colonization of the overlying epidermis. The case represents an unusual pathologic presentation of ES and highlights potential pitfalls in establishing the diagnosis.
Asunto(s)
Sarcoma/secundario , Neoplasias Cutáneas/patología , Adulto , Biomarcadores de Tumor/análisis , Diagnóstico Diferencial , Humanos , Inmunohistoquímica , Masculino , Molusco Contagioso/diagnóstico , Metástasis de la Neoplasia/patología , Sarcoma/diagnósticoRESUMEN
KEY CLINICAL MESSAGE: It's important to assess cases both clinically and pathologically for factors potentially predictive of an aggressive clinical course. We concluded that the relative immunosuppressive effects of PD1 may contribute to tumor progression while the lack of staining for cutaneous lymphocyte antigen may be an additional factor facilitating distant extracutaneous migration.
RESUMEN
The localization of atherosclerotic lesions is due, in part, to regional variations in the permeability of arterial endothelium to macromolecules. In turn, endothelial permeability may be influenced by fluid shear stresses. The spatial variation in endothelial permeability is reviewed and evidence for shear stress dependence upon permeability is presented. These results are examined in light of various signaling mechanisms that increase permeability by increasing the transport of water and macromolecules through the junctions separating endothelial cells. Signaling pathways cause a change in the dense peripheral band of actin and actin stress fibers or alter the phosphorylation of junction proteins which affects their ability to localize in junctions. Future directions to clarify the effect of shear stress on permeability are considered.