Two cases of desmoplastic non⁃infantile astrocytoma with review of the literature

Abstract

Objective To investigate clinical pathological characteristics, diagnosis and differentiation of desmoplastic non ⁃ infantile astrocytoma (DNIA). Methods & Results Two female patients with DNIA aged 5 and 10 years old, respectively. The patient of case 1 had repeated unconsciousness with limbs twitch, MR image showed a mass in the inner part of the left temporal horn, the tumor was located in the underside of cortex of temporal lobe, about 3.00 cm × 3.50 cm × 3.50 cm in size with yellow in color and medium blood supply, the boundaries of the tumor were fairly clear and the mass was completely dissected. The patient of case 2 had recurrent headache with vomiting, MR image showed a large cystic solid mass in the right frontal temporal lobe⁃lateral ventricle. The tumor was located at 1 cm under the subcortex and drew off yellow cyst fluid about 80 ml. The tumor was about 11.00 cm × 10.00 cm × 10.00 cm in size with yellow in color and brittle texture and abundant blood supply, adhesion to the brain tissue and was completely dissected. Histology showed obvious interstitial fibrosis associated with neoplastic astrocytes with primary neuroepithelium. Immunohistochemical staining indicated that the expression of both tumor cells was positive for vimentin, GFAP, S⁃100, Nestin, and negative for CK, EMA, NeuN, the expression of primary cells was positive for Syn; Ki ⁃ 67 labeling index was 6% and 10% , respectively. Histochemical staining showed that the tumor cells were surrounded by reticular fibers. Patients were followed up for 18 and 52 months, respectively, both had no recurrence and metastasis. Conclusion DNIA is a rare supratentorial neuroepithelial tumor. The prognosis is generally good. It needs to be differentiated from other tumors, especially malignant glioma. DOI：10.3969/j.issn.1672⁃6731.2012.03.015