ABSTRACT
Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver malignancy in adolescents and young adults. Surgery is the mainstay of therapy for primary and metastatic disease. Most patients relapse, with development of both local and distant metastases. Brain metastases from solid tumors are rare in the pediatric and young adult population. Here, we document three patients with brain metastases from FLHCC, confirmed by histology and molecular characterization of the chimeric fusion DNAJB1-PRKACA, each necessitating neurosurgical intervention. These observations highlight the ability of FLHCC to metastasize to the brain and suggest the need for surveillance neuroimaging for patients with advanced-stage disease.
Subject(s)
Brain Neoplasms , Carcinoma, Hepatocellular , Liver Neoplasms , Neuroimaging , Neurosurgical Procedures , Adolescent , Brain Neoplasms/diagnostic imaging , Brain Neoplasms/genetics , Brain Neoplasms/secondary , Brain Neoplasms/surgery , Carcinoma, Hepatocellular/diagnostic imaging , Carcinoma, Hepatocellular/genetics , Carcinoma, Hepatocellular/metabolism , Carcinoma, Hepatocellular/surgery , Cyclic AMP-Dependent Protein Kinase Catalytic Subunits/genetics , Cyclic AMP-Dependent Protein Kinase Catalytic Subunits/metabolism , Female , HSP40 Heat-Shock Proteins/genetics , HSP40 Heat-Shock Proteins/metabolism , Humans , Liver Neoplasms/diagnostic imaging , Liver Neoplasms/genetics , Liver Neoplasms/metabolism , Liver Neoplasms/surgery , Neoplasm Metastasis , Oncogene Proteins, Fusion/genetics , Oncogene Proteins, Fusion/metabolismABSTRACT
El liposarcoma es un tumor maligno de origen mesenquimal. Comparado con otros tipos de neoplasias malignas, los sarcomas de partes blandas son tumores relativamente raros. Representan el 1% de todas las neoplasias y supone del 9 al 18% del total de sarcomas de partes blandas. Es una neoplasia propia de pacientes adultos, con mayor incidencia entre los 40 y los 60 años, con ligera preferencia del sexo masculino. Hasta hace tres décadas, la tendencia ante la presencia de este tipo de lesiones en las extremidades era la cirugía radical. Presentamos el caso de paciente femenino de 37 años quien presenta una tumoración en el muslo izquierdo. La biopsia incisional reportó una neoplasia maligna poco diferenciada. Ante la sospecha clínica y radiológica de la presencia de un liposarcoma fue llevada a quirófano donde se realizó compartamentectomía medial del muslo izquierdo, con colocación intraoperatoria de 12 catéteres para braquiterapia y linfadenectomía inguinofemoral izquierda. La braquiterapia es una forma de radioterapia adyuvante interna y una alternativa útil y atractiva(AU)
Liposarcoma is a malignant tumor of mesenchymal origin. Compared with other types of malignancies, soft tissue sarcomas are relatively rare tumors. Represent 1% of all malignancies and is from 9 to 18% of soft tissue sarcomas. Its an adult patients tumor, with the highest incidence between 40 and 60 years and with a slight prevalence of males. Until three decades ago, the trend in the presence of these lesions in the extremities was radical surgery. We report the case of a 37 years old female patient who has a growth on the thigh. Incisional biopsy reported a poorly differentiated malignant neoplasm. Suspecting a liposarcoma was taken to the operating room where left medial thigh compartamentectomy was made and intraoperative placement of 12 brachytherapy catheters and left inguinofemoral lymphadenectomy. Brachytherapy is a form of internal radiation and an alternative, useful and attractive adjuvant therapy(AU)
