ABSTRACT
Objetivos: Describimos y documentamos el primer caso de PEComa publicado en España según la base de datos Pub Med. Y revisamos la bibliografía existente sobre este tipo de tumores. Métodos/Resultados: Se Trata de una paciente de 39 años con un tumor pélvico de 9 cms. descubierto en una revisión ginecológica rutinaria. El TAC muestra múltiples adenopatías periaórticas que se extienden hasta vena renal izquierda El diagnóstico de PEComa se obtiene por punción con aguja gruesa. Se realiza exéresis del tumor junto a anexo izquierdo y linfadenectomía aortoiliaca. El resultado patológico confirma el diagnóstico tras la inmunohistoquímica con actina de músculo liso y HMB-45. No recibió tratamiento adyuvante. Tras un año de seguimiento la paciente no muestra en el TAC signos de recidiva. Se realiza una amplia búsqueda bibliográfica en Pub Med encontrando 73 referencias a este tipo de tumor cuyas conclusiones se exponen en este articulo. Conclusiones: El tumor de celulas epitelioides perivasculares, PEComa, es una neoplasia mesenquimal de muy baja incidencia y con malignidad incierta. Tumores muy raros: el angiomiolipoma epitelioide, la linfangioleiomiomatosis, el linfangiomioma, el tumor miomelanocitico del ligamento falciforme, el tumor pulmonar de celulas claras «de azucar» y su variante extrapulmonar; son antiguas descripciones de lo que ahora es una única entidad tumoral denominada PEComa. Aun no de ha descrito la célula que en tejidos normales da origen a este tumor. Afectando principalmente a mujeres en edad media, pueden encontrarse en cualquier lugar anatómico. Su tratamiento es quirúrgico aunque se desconoce el papel de la linfadenectomía y del tratamiento adyuvantes (AU)
Objective: We describe and document the first case of PEcoma published in Spain following the PubMed database. We review the bibliography about these tumors. Methods/Results: 39-year-old female patient with a 9 cm pelvic tumor discovered in a routine gynecologic review. CT scan showed multiple periaortic adenopathies extending up to the left renal vein. The diagnosis of PEcoma was obtained by needle biopsy. Tumor excision including left annex and aortoiliac lymphadenectomy were performed. Pathology confirmed the diagnosis after immunohistochemical study with smooth muscle actin and HMB-45. No adjuvant treatment was given. After one year of follow-up the patient does not show signs of recurrence of the CT scan. A PubMed search was performed finding 73 references about this kind of tumor the conclusions of which are exposed in this article. Conclusions: Perivascular epithelioid cell tumor, PEcoma, is a very low incidence mesenchymal neoplasia with uncertain malignancy. Very rare tumors such as epithelioid angiomyolipoma, lymphangioleiomyomatosis, lymphangiomyoma, myelomelanocytic tumor of the falciform ligament, «sugar» clear cell lung tumor and its extrapulmonary variant are all descriptions of what is now a unique tumoral entity named PEcoma. It has not been described yet the normal tissue cell giving origin to this tumor. It affects mainly middle age women, and may appear in any anatomical site. Treatment is surgical, although the role of lymphadenectomy and adjuvant treatment is unknown (AU)
Subject(s)
Humans , Female , Adult , Pelvic Neoplasms/complications , Pelvic Neoplasms/diagnosis , Lymph Node Excision/methods , Immunohistochemistry/methods , Lymphangioleiomyomatosis/complications , Lymphangioleiomyomatosis/diagnosis , Laparoscopy/methods , Diagnosis, Differential , Immunohistochemistry/trends , Chondrosarcoma, Mesenchymal/complications , Sarcoma/complications , Sarcoma/diagnosisABSTRACT
BCL6 is a transcriptional repressor whose deregulated expression plays a key role in diffuse large B-cell lymphomas (DLBCLs). BCL6 expression characterizes one of the two main subtypes (GC type) of DLBCL, while the other (ABC type) is recognized by increased NFkappaB activation. The mechanistic basis of this distinction remains unclear and the BCL6 targets have been only partially explored. Here we describe how NFkappaB activity is increased after BCL6 silencing by shRNA in DLBCL cells, leading us to propose that BCL6 represses NFkappaB activity. We also demonstrate that this repression is brought about by a mechanism involving protein-protein interaction between BCL6 and NFkappaB members, both in vitro and in vivo. Analysis of a series of DLBCLs shows a negative correlation between the expression of NFkappaB target genes and BCL6. This combined approach using silenced cells and a series of human DLBCL samples leads us to a better understanding of the role of BCL6 as an NFkappaB regulator in B-cells.
Subject(s)
Lymphoma, Large B-Cell, Diffuse/metabolism , NF-kappa B/metabolism , Proto-Oncogene Proteins c-bcl-6/physiology , Gene Expression Regulation, Neoplastic , Gene Silencing , HeLa Cells , Humans , Lymphoma, Large B-Cell, Diffuse/genetics , NF-kappa B/genetics , Proto-Oncogene Proteins c-bcl-6/genetics , Proto-Oncogene Proteins c-bcl-6/metabolism , Tumor Cells, Cultured , Up-Regulation , Zinc Fingers/geneticsSubject(s)
Leukemia, Prolymphocytic, T-Cell/diagnosis , Lymphocytosis/etiology , T-Lymphocytes/pathology , Thymoma/diagnosis , Thymus Neoplasms/diagnosis , Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Diagnosis, Differential , Fatal Outcome , Humans , Leukemia, Prolymphocytic, T-Cell/complications , Lymphocytosis/pathology , Male , Thymoma/complications , Thymoma/drug therapy , Thymoma/radiotherapy , Thymus Neoplasms/complications , Thymus Neoplasms/drug therapy , Thymus Neoplasms/radiotherapyABSTRACT
Describimos clínicopatológicamente un caso de linfoma de Burkitt no endémico, excepcional por la edad de aparición (21 años) y por su origen anatómico (el estómago). Presentamos y discutimos los resultados de su estudio inmunohistoquímico y el estadiaje, tumoral, que consideramos una importante aportación a la. literatura, ya que hasta la fecha no existen estudios estadísticamente concluyentes que definan fenotípicamente el linfoma de Burkitt no endémico y que correlacionen su origen anatómico, su estadiaje -tumoral y su pronóstico (AU)
