ABSTRACT
Kikuchi's disease, also known as histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limited disorder of unknown cause that is usually characterized by cervical lymphadenopathy and fever. The etiology and pathogenesis remain unknown, but the clinical presentation, course, and histologic changes suggest an immune response of T cells and histiocytes to an infectious agent. Numerous inciting agents have been proposed. However, the association between human herpesvirus 7 and Kikuchi's disease has been rarely reported as a possible etiologic agent of Kikuchi's disease. We report the case of a 24-year-old Caucasian female patient with cervical lymphadenopathy and isolated pruriginous maculo-papular lesions who was diagnosed of Kikuchi's disease in whom the presence of human herpesvirus 7 DNA was documented in the affected lymph node specimen in the absent of other viruses. Therefore, a possible etiologic relation between the Kikuchi's disease of this patient and human herpesvirus 7 was established, supporting a role for human herpesvirus 7 involvement in the pathogenesis.
Subject(s)
Herpesvirus 7, Human/physiology , Histiocytic Necrotizing Lymphadenitis/virology , Roseolovirus Infections/complications , Apoptosis , DNA, Viral/genetics , Female , Herpesvirus 7, Human/genetics , Histiocytes/pathology , Histiocytic Necrotizing Lymphadenitis/pathology , Histiocytic Necrotizing Lymphadenitis/physiopathology , Humans , Roseolovirus Infections/pathology , Roseolovirus Infections/physiopathology , Young AdultABSTRACT
Los carcinomas mioepiteliales de partes blandas (STMM), afectan a las extremidades o cinturas de pacientes jóvenes y evolucionan agresivamente. Muestran una marcada atipia y bordes infiltrantes y su pronóstico empeora con el reordenamiento del gen EWSR1, presente en el 45% de los casos. Presentamos el caso de un varón de 26 años al que se le extirpó un STMM situado en la cadera derecha, compuesto por cordones y nidos sólidos de células atípicas rodeadas de un estroma mixoide-hialino, sin diferenciación ductal. Expresaban citoqueratinas, EMA, S100 y calponina. La hibridación in situ fluorescente (FISH) no reveló reordenamientos del gen EWSR1. Tras recibir cuidados paliativos, el paciente murió por múltiples metástasis a los 18 meses del diagnóstico. Se trata de un caso de STMM sin reordenamiento del gen EWRS1 pero inusualmente agresivo(AU)
Soft tissue myoepithelial carcinomas (STMM) are clinically aggressive tumors that arise in the extremities and limb girdles of young patients. Histologically, they show moderate to severe atypia and infiltrative margins. EWSR1-gene rearrangements are present in 45% of cases and are associated with increased malignancy. A large mass in the right hip of a 26-year-old male that had been surgically removed showed strands of cells embedded in a hyalin-myxoid matrix without ductal differentiation. Cells expressed cytokeratins, EMA, S-100 protein and calponin. STMM was diagnosed. Although the tumor did not have an EWSR1 gene rearrangement, it behaved aggressively and the patient died from multiple metastases 18 months after diagnosis(AU)
Subject(s)
Humans , Male , Adult , Myoepithelioma/genetics , Myoepithelioma/pathology , Prognosis , Soft Tissue Neoplasms/complications , Soft Tissue Neoplasms/pathology , Magnetic Resonance Imaging , Immunohistochemistry/methods , Immunohistochemistry , Diagnosis, DifferentialABSTRACT
Presentamos en nuestro trabajo, los hallazgos morfológicos e inmunohistoquímicos de carácter diagnóstico observados en un meningioma de células claras localizado a nivel de la cauda equina en un paciente de 25 años. El tumor mostró un patrón morfológico de predominio nodular en el que destacaban las células de citoplasma claro, positivas para la tinción de PAS. El hallazgo de áreas de aspecto meningoteliomatoso dentro del mismo tumor así como la inmunohistoquímica (positividad de las células tumorales para vimentina y EMA y negatividad para citoqueratinas, CD10, HMB-45 y proteína glial fibrilar ácida) permitió establecer un diagnóstico diferencial con otras neoplasias principalmente con las metástasis de carcinoma renal. De igual modo se comentan con brevedad las características de esta variante de meningioma de grado intermedio de malignidad (meningioma grado ii de la OMS)(AU)
The morphological and immunohistochemical findings of a clear cell meningioma found in the cauda equina of a 25 year old patient are reported. The tumour was predominately nodular with a marked presence of cells with clear cytoplasm which stained positively for PAS. The presence of meningoteliomatous areas in the tumour, together with the immunohistochemistry of the tumour cells (positivity for vimentin and EMA and negativity for cytokeratins, CD10, HMB45 and PGFA) were the diagnostic features that differentiated the tumour from other possible diagnoses, principally metastases from a renal carcinoma. The characteristics of this type of meningioma, which has an intermediate grade of malignancy (WHO grade ii meningioma) are discussed(AU)
