Unusual case of inflammatory spinal epidural mass (Castleman syndrome).
Castleman syndrome (giant lymph node hyperplasia) is a rare, heterogeneous lymphoproliferative disorder of unknown etiology and pathogenesis. Most cases occur as mediastinal masses, although extrathoracic involvement including nodal and extranodal locations have been reported. The localized variants (solitary lesions) respond well to surgical excision. We report a 10-year-old boy who presented with headache, intermittent fever, and progressive weakness of his legs. MRI imaging showed an enhancing epidural mass with impingement on the spinal cord at the C6-T2 level. Other laboratory abnormalities included anemia, hypergammaglobulinemia, increased erythrocyte sedimentation rate, and cerebrospinal fluid pleocytosis with slightly increased cerebrospinal protein. The mass was partially resected and the histopathology showed lymphoplasmocytic infiltration compatible with Castleman syndrome. There was no evidence of malignancy. Castleman syndrome is the most likely diagnosis in the presence of the associated systemic findings, although the epidural site for lymphoplasmocytic inflammation is atypical.
Alper
G
G
Division of Pediatric Neurology, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pennsylvania.
Crumrine
P K
PK
Hamilton
R L
RL
Albright
A L
AL
Wald
E R
ER
D005871:Castleman Disease / Q000150:complications / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002648:Child; D003937:Diagnosis, Differential; D004824:Epidural Space; D006261:Headache / Q000209:etiology; D006801:Humans; D007866:Leg / Q000294:innervation; D016131:Lymphocyte Subsets / Q000473:pathology; D008223:Lymphoma / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000209:etiology; D010954:Plasmacytoma / Q000175:diagnosis; D013117:Spinal Cord Compression / Q000209:etiology*