Surgical treatment of malignant mediastinal neurogenic tumors in children.
BACKGROUND
The aim of this study was to identify the role of surgical resection in the treatment of malignant mediastinal neurogenic tumors in children.


METHODS
Thirty-eight consecutive children, who underwent surgical resection of a malignant mediastinal neurogenic tumor between 1986 and 2004, were included in this study. The tumor cell types were neuroblastoma in 23 patients (60.5%), ganglioneuroblastoma in 14 (36.8%), and malignant neuroepithelioma in 1 (2.6%). Surgery was performed for curative resection in localized tumors and salvage resection of residual mediastinal masses after chemotherapy in stage IV tumors. Of the 16 patients (42.1%) who underwent salvage resection, 14 had neuroblastoma and 2 ganglioneuroblastoma.


RESULTS
Mean patient age was 3.4+/-3.0 years (1 month-13 years) and 26 patients (68.4%) were symptomatic at presentation. Adjacent structure invasion was found in eight patients (21.1%), invasion of chest wall in four, heart and vena cava in two, lung in one, and chest wall and lung in one. Complete gross resection was possible in 30 patients (78.9%) and there was no surgical mortality. Surgical morbidity occurred in 10 patients (26.3%) and Horner's syndrome was the most frequent complication (n=7). The 5-year survival was 95.2% for a localized tumor and 52.5% for a stage IV tumor (p=0.004). The significant risk factors of long-term survival were adjacent structure invasion (p=0.002) and a stage IV tumor (p=0.002) by multivariate Cox regression analysis.


CONCLUSIONS
Surgical resection of localized malignant mediastinal neurogenic tumor in children showed good long-term survival, and salvage operations after chemotherapy showed acceptable long-term survival.
Kang
Chang Hyun
CH
Department of Thoracic and Cardiovascular Surgery, Cancer Research Institute, Xenotransplantation Research Center, Clinical Research Institute, Seoul National University College of Medicine, Seoul, South Korea. chkang@snu.ac.kr
Kim
Young Tae
YT
Jeon
Sang-Hoon
SH
Sung
Sook-Whan
SW
Kim
Joo Hyun
JH
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D018305:Ganglioneuroblastoma / Q000401:mortality / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008479:Mediastinal Neoplasms / Q000401:mortality / Q000601:surgery*; D009361:Neoplasm Invasiveness; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000401:mortality / Q000601:surgery; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000401:mortality / Q000601:surgery*; D011183:Postoperative Complications; D011300:Preoperative Care / Q000379:methods; D012189:Retrospective Studies; D012307:Risk Factors; D016896:Treatment Outcome