Esplenectomía por tumor hematopoyético extramedular esclerosante del bazo / Splenectomy for sclerosing extramedullary hematopoietic tumor of the spleen

RESUMEN

RESUMEN Los tumores hematopoyéticos extramedulares son infrecuentes; se caracterizan por la presencia de elementos formes de la sangre en distintas etapas madurativas, con megacariocitos atípicos y proliferación fibroblástica. Se comunica el caso clínico de un tumor hematopoyético extramedular esclerosante del bazo en un varón de 71 años, con antecedentes patológicos de hipertensión, diabetes, portador de virus hepatitis C. En estudio por malestar abdominal se identificaron mediante imágenes lesiones esplénicas múltiples. Se planteó origen vascular, sin poder descartar otro tipo de lesiones, por lo que se decidió esplenectomía laparoscópica. El estudio histopatológico e inmunohistoquímico diagnosticó un tumor hematopoyético extramedular esclerosante del bazo. Son neoplasias de baja incidencia y escasa evidencia en cuanto a etiopatogenia, diagnóstico y tratamiento. Se presentan como lesiones únicas o múltiples, y pueden afectar diferentes órganos. Están asociados a síndromes mieloproliferativos crónicos. Solo su confirmación histopatológica permite diferenciarlos de otros tumores malignos.

ABSTRACT

ABSTRACT Extramedullary hematopoietic tumors are rare and characterized by the presence of hematopoietic elements at various stages of maturity, atypical megakaryocytes, and fibroblastic proliferation. We report the case of a patient with sclerosing extramedullary hematopoietic tumor of the spleen.The patient was 71-year-old man with a history of hypertension, diabetes, and hepatitis C virus infection. Multiple spleen lesions were identified in imaging tests during workup due to abdominal discomfort. Although a vascular tumor was suspected, laparoscopic splenectomy was decided after considering other possible causes. The pathology examination revealed a sclerosing extramedullary hematopoietic tumor of the spleen, which was confirmed by immunohistochemical tests. These tumors are usually single or, less frequently, multiple lesions affecting different organs and are associated with chronic myeloproliferative syndromes. The histologic confirmation is mandatory due to their similarity to malignant tumors. Extramedullary hematopoietic tumors are rare, and there is little scientific clinical evidence regarding their diagnosis and management. The histological confirmation is mandatory due to their similarity to malignant tumors.