No abstract

Two cases of intracranial gliomas with extraneural metastases are described. Case 1, studied with biopsy material only, was a left malignant astrocytoma from the area of the rolandic fissure with right cervical lymph nodes metastases in a 43-year-old man. Case 2 was a left temporal malignant astrocytoma in a 21-year-old woman. Fifteen days after craniotomy, a left submandibular lymph node metastasis appeared. Forty days after surgery, a ventriculoperitoneal shunt was performed. Fifty-four days after surgery, the patient died. Autopsy revealed three liver metastases. Our review of the literature consists of 72 autopsy cases with extraneural deposits. Thos metastases occurred mainly in adults (63/72) and among men (46/72). The primary glioma was supratentorial in 67 cases. Metastases were mainly pulmonary and pleural. The majority of patients (82.8%) died within 2 years after onset of symptoms. In 8 of the cases, metastasis developed without any craniotomy and in 8 other cases, through a shunt.

Desmoplastic small round cell tumors (DSRCTs) present a reciprocal chromosomal translocation, t(11;22)(p13;q12), that results in fusion of Ewing's sarcoma and Wilms' tumor (WT1) genes. The authors evaluated 15 DSRCTs and 71 other tumors often considered in the differential diagnosis for immunoreactivity using a polyclonal antibody directed against the WT1 part of the chimeric protein resulting from this translocation. WT1 immunostaining was performed on paraffin material using the WT(C-19) antibody after heat-antigen retrieval. All the DSRCTs (15 of 15, 100%) demonstrated strong WT1 nuclear immunoreactivity. Ten of 14 nephroblastomas (71%) disclosed WT1-positive nuclei in accordance with the staining reported by others, and rare and focal nuclear positivity was detected in two of 17 rhabdomyosarcomas. WT1 immunoreactivity was not observed in Ewing's sarcoma/primitive neuroectodermal tumors (zero of 21, 0%), neuroblastomas (zero of 17, 0%), or rhabdoid tumors of the kidney (zero of two, 0%). In nephroblastoma, differential diagnosis with DSRCT was not difficult: Clinical and morphologic data are not similar for these two entities. The current study validates WT1 immunoreactivity as a useful marker to separate DSRCT from other small round cell tumors.