Paravertebral malignant tumors of childhood: analysis of 28 pediatric patients.
OBJECTIVE
To evaluate the clinical features and treatment results of the primary paravertebral malignant tumors (PMTs) in our department.


METHODS
Medical records of 28 children with primary PMTs treated between 1988-2007 were analyzed retrospectively.


RESULTS
Primary PMTs constituted 4.8% of the cancer cases in our department. Tumor diagnoses were mostly neuroblastoma (46.4%) and soft tissue sarcomas (35.7%). These cases presented with pain (64.3%), motor dysfunction (42.8%), sphincter dysfunction (35.7%), palpable mass (32.1%), and sensory deficits (7.1%). All tumors were extradural. Physical examination revealed motor deficits (53.6%), deep tendon reflex alterations (53.6%), sphincter dysfunction (35.7%), pathologic reflexes (25%), abnormal cutaneous reflexes (25%), and sensory deficits (17.8%). Sixteen had cord compression (CC; 13 clinical, three radiological CC). Eleven of them presented with advanced disease. Seven were managed by surgical departments by primary surgery (three unresponsive). Others were managed by pediatric oncology: five with corticosteroids+/-chemotherapy (one unresponsive), one with radiotherapy (RT), and two with surgery for the clinical CC. Surgery was tumor excision in nine, laminectomy in nine, laminotomy in one, and delayed surgery after chemotherapy in two cases. In chemotherapy and surgery groups, there were neurologic sequela associated with the advanced disease at diagnosis in 38% and 37%, respectively. At 3-year median follow-up, nine patients died, 17 are alive (four with neurologic sequela), and two are lost of follow-up.


CONCLUSIONS
Majority of cases presented with advanced disease. Late referral is the major cause of morbidity and mortality. The CC caused by PMTs should be initially managed with corticosteroids +/- chemotherapy to avoid the adverse late effects of RT and surgery.
Gunes
Dilek
D
Department of Pediatric Oncology, Dokuz Eylul University Institute of Oncology, Mithatpasa Caddesi, No: 1606, Inciralti, 35340, Izmir, Turkey. dilek.gunes@deu.edu.tr
Uysal
Kamer Mutafoglu
KM
Cetinkaya
Hilal
H
Tekin
Hande Gazeteci
HG
Yuceer
Nurullah
N
Sarialioglu
Faik
F
Olgun
Nur
N
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009447:Neuroblastoma / Q000628:therapy*; D020127:Recovery of Function / Q000502:physiology; D012189:Retrospective Studies; D012509:Sarcoma / Q000628:therapy; D013120:Spinal Cord Neoplasms / Q000628:therapy*; D013125:Spinal Neoplasms / Q000628:therapy*; D016896:Treatment Outcome