Peripheral-type primitive neuroectodermal tumor arising in the tentorium. Case report.
The authors report the case of a peripheral primitive neuroectodermal tumor (PNET) arising in the tentorium in a 5-year-old boy who presented with frequent vomiting and mild palsy of the left abducent nerve. Following complete surgical excision of the tumor via a transpetrosal approach, the patient has thus far been disease free for 7 years. The tumor tissue was composed of small cells with uniform round nuclei and minimal identifiable cytoplasm. Homer-Wright rosettes were frequently observed. Immunohistochemical studies demonstrated a positive reaction to HBA-71, which recognizes the cell surface glycoprotein p30/32, a product of the MIC2 gene. Both the clinical and immunohistochemical characteristics of this tumor are consistent with a diagnosis of peripheral PNET, which is genetically distinct from the more common intracranial PNET.
Department of Neurological Surgery, Nihon University School of Medicine, Tokyo, Japan.
Y
Y
Katayama
S
S
Kimura
T
T
Watanabe
A
A
Yoshino
M
M
Koshinaga
D000071443:12E7 Antigen; D000010:Abducens Nerve; D015703:Antigens, CD / Q000032:analysis; D001932:Brain Neoplasms / Q000473:pathology* / Q000601:surgery; D015815:Cell Adhesion Molecules / Q000032:analysis; D002467:Cell Nucleus / Q000648:ultrastructure; D002675:Child, Preschool; D003389:Cranial Nerve Diseases / Q000175:diagnosis; D003593:Cytoplasm / Q000648:ultrastructure; D018572:Disease-Free Survival; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000032:analysis; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology* / Q000601:surgery; D010243:Paralysis / Q000175:diagnosis; D014839:Vomiting / Q000175:diagnosis
D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D008562:Membrane Glycoproteins