RESUMO
Malignant fibrous histiocytoma(MFH) is a rare primary neoplasm that constitutes less than 1% of the malignant tumors of bone, and involvement of the skull is very rare. We present a case of malignant fibrous histiocytoma of the skull, presenting an intraosseous lesion in a 43-yr-old woman. She had a rapidly growing, tender mass in the right parietal region. A plain radiograph showed an osteolytic lesion of the right parietal bone. Magnetic resonance imaging revealed that the lesion showed heterogeneous low signal intensity on T1-weighted images and slightly high signal intensity on T2-weighted images. No evidence of an extraosseous extension to the adjacent dura and soft tissue was found, and a wide excision of the parietal bone was performed. Histologically, the tumor was a typical MFH displaying pleomorphic spindle cells in a storiform pattern. The results of immunohistochemical stainings revealed that the tumor cells were positive for vimentin, alpha-1-antitryp-sin, and p53, and negative for smooth muscle actin, S100 protein, desmin, and MyoD1. Three months later, a mainly cystic, recurrent mass was developed at the previously operated site. Before the resection, we first performed the percutaneous aspiration cytology, revealing diagnostic multinucleated pleomorphic cells. There-after, she had to receive repetitive resections of recurrent or residual lesions, and she died of postoperative meningoencephalitis two years after the first operation.
Assuntos
Adulto , Feminino , Humanos , Actinas/biossíntese , Encéfalo/patologia , Desmina/biossíntese , Células Gigantes/metabolismo , Histiocitoma Fibroso Benigno/diagnóstico , Imuno-Histoquímica , Imageamento por Ressonância Magnética , Mitose , Músculo Liso/metabolismo , Proteína MyoD/biossíntese , Proteína Supressora de Tumor p53/biossíntese , Proteínas S100/biossíntese , Neoplasias Cranianas/diagnóstico , Tomografia Computadorizada por Raios X , Vimentina/biossíntese , alfa 1-Antitripsina/biossínteseRESUMO
Se informa el caso de un paciente de 65 años de edad quien presentaba un hepatocarcinoma y cirrosis asociados a deficiencia de alfa-1antitripsina (A1-AT). Cuatro de sus seis hermanos, dos hombres y dos mujeres, tenían valor de esta glicoproteína inferiores a los normales, sin manifestación aparente de compromiso clínico hepático ni pulmonar; ninguno tenía antecedentes de hepatitis o colestasis neonatal. La descripción de este primer caso en nuestro medio se propone estimular el interés por esta entidad como causa de hepatopatía crónica, carcinoma hepático primario y enfisema pulmonar, sin factores de riesgo diferentes asociados