Premium
Pediatric meningeal sarcoma: Report of a case with long survival
Author(s) -
Chang Howard T,
Schelper Robert L
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a390
Subject(s) - medicine , desmoplasia , vimentin , pathology , sarcoma , immunohistochemistry , stroma
A 10‐month‐old boy presented with a short history of intermittent episodes of fixed eye deviation to the right, drooling and unresponsiveness. MRI studies revealed a heterogeneous 7.5 x 7 x 6 cm mass that appeared to be attached to the dura in the right fronto‐temporal area. A gross‐total resection was performed. Histological examination showed a hypercellular tumor with focal desmoplasia and focal ischemic changes. Several morphologic patterns were seen, including papillary areas with large vessels surrounded by neoplastic cells, areas of loose collections of neoplastic cells that were round to polygonal, as well as solid areas that showed slit‐like spaces with focal ependymal lining. Mitoses, spindle cells, and reticulin depositions were seen. Immunohistochemistry with an extensive panel of antibodies showed that the tumor cells were focally positive for vimentin and S‐100. The Ki‐67 labeling index was up to 10% focally. Two external experts reviewed this case and both considered this tumor as a malignant meningeal sarcoma. The patient was discharged 7 days after surgery with a left hemiparesis that improved significantly with physical and occupational therapy. The parents decided not to pursue adjuvant radiation or chemotherapy. However, the patient remains seizure and symptom free 24 months after surgical resection.