ABSTRACT
BACKGROUND: Glioblastoma with primitive neuronal components (GB/PNC) is an extremely rare type of glioblastoma characterized by presenting histological and cytogenetic features of both entities. The mixed nature of these tumors limits the imaging diagnosis and supposes a therapeutic dilemma. CASE DESCRIPTION: We present the case of a 77-year-old female with a GB/PNC who is treated with surgery and adjuvant radiochemotherapy according to the STUPP protocol, where an abnormal uptake of 5-aminolevulinic acid (5-ALA) is evident during surgery in probable relation to the mixed nature of GB/PNC. CONCLUSION: GB/PNC is extremely rare tumors. Given its low prevalence, there are no studies that refer to the macroscopic characteristics of the tumor as well as evidence of the effectiveness of adjuvant treatment. Fluorescence-guided resection with 5-ALA is the surgical treatment of choice in surgery for high-grade gliomas; however, in GB/PNC, it may not be as useful since PNC may have less fluorescent marker uptake and be more dimly visualized when excited by light using the surgical microscope.
ABSTRACT
Presentamos el caso de una mujer de 56 años de edad, que acude al Servicio de Urgencias de nuestro centro por un cuadro de distensión y dolor abdominal difuso con edemas en extremidades inferiores. En los estudios de imagen realizados (ecografía y TC) se demostró la existencia de ocupación intraluminal de la vena cava inferior, por una masa que se extendía desde el drenaje de las venas renales hasta su confluencia en la aurícula derecha, con signos de obstrucción de las venas suprahepáticas. El diagnóstico anatomopatológico final fue de leiomiosarcoma con síndrome de Budd-Chiari asociado. El leiomiosarcoma de vena cava inferior es una patología poco frecuente y su asociación con síndrome de Budd-Chiari es aún más excepcional.
We report the case of a 56-year-old woman who presented at our Emergency Department with symptoms ofdiffuse abdominal pain and distention with lower-extremity edema. Imaging studies (ultrasound and computed tomography) showed an intraluminar inferior vena cava mass extending from the renal veins drain to their confluence at the right atrium, with signs of obstruction of the suprahepatic veins. The final pathology diagnosis was leiomyosarcoma with Budd-Chiari syndrome. The leiomyosarcoma of the inferior vena cava is an infrequent pathology and its association with Budd-Chiari syndrome is even rarer.
