Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children
Background: To study the diagnosis, management, and outcome of abdominal neuroblastoma (NBL) in infants and children and also the behavior of some rare types. Patients and Methods: This is a retrospective review of 46 infants and children having abdominal NBL at King Abdulaziz University Hospital, J...
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doaj-f4055c78f8e549878b81c3160e12b1352020-11-25T00:19:34ZengWolters Kluwer Medknow PublicationsAfrican Journal of Paediatric Surgery0189-67250974-59982013-01-0110210010710.4103/0189-6725.115032Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in childrenSaleh M AldaqalAli M TurkiBackground: To study the diagnosis, management, and outcome of abdominal neuroblastoma (NBL) in infants and children and also the behavior of some rare types. Patients and Methods: This is a retrospective review of 46 infants and children having abdominal NBL at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia and Tanta University Hospital, Tanta, Egypt (a joint study) between February 2008 and January 2012. The patients′ medical records were reviewed for demographic details, clinical presentations, histopathology, radiological diagnosis, management, and outcome. Results: Of the 46 patients, 5 (10.7%), three males and two females, aged 1.5-6.5 years (mean: 4.5 years) had rare types of NBL (one bilateral NBL, one pelvic NBL, one pelvic-abdominal, and there were two cases of ganglioneuroblastoma). Three patients (two ganglioneuroblastoma and one pelvic) underwent complete surgical removal of the tumor with a good disease survival, whereas the other two patients (two bilateral and one pelviabdominal) had advanced disease and received palliative chemoradiotherapy. The remaining 41 patients, 23 males (56.1%) and 18 females (43.9%), with mean age 3.9 years (range: 1-7 years), had unilateral abdominal NBL. Twelve of them had resectable tumor and underwent primary surgical removal of the tumor, whereas the remaining 29 patients had unresectable tumor and received pre-operative chemotherapy with good response of the tumor in seven patients and no response in the remaining 22 patients. Conclusion: Primary complete surgical removal of tumor is advisable in localized NBL with a good outcome, whereas in advanced cases, it is better to start with pre-operative chemotherapy to downsize the tumour mass and safe delayed surgical excision. An increase in patient′s age is associated with advanced stage of NBL and poor prognosis except in ganglioneuroblastoma cases due to maturation of tumor cells.http://www.afrjpaedsurg.org/article.asp?issn=0189-6725;year=2013;volume=10;issue=2;spage=100;epage=107;aulast=AldaqalAbdominal neuroblastomachildhood malignanciesmalignant tumorsolid tumor |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Saleh M Aldaqal Ali M Turki |
spellingShingle |
Saleh M Aldaqal Ali M Turki Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children African Journal of Paediatric Surgery Abdominal neuroblastoma childhood malignancies malignant tumor solid tumor |
author_facet |
Saleh M Aldaqal Ali M Turki |
author_sort |
Saleh M Aldaqal |
title |
Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children |
title_short |
Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children |
title_full |
Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children |
title_fullStr |
Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children |
title_full_unstemmed |
Clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children |
title_sort |
clinico-pathological patterns of a rare presentation of abdominal neuroblastoma in children |
publisher |
Wolters Kluwer Medknow Publications |
series |
African Journal of Paediatric Surgery |
issn |
0189-6725 0974-5998 |
publishDate |
2013-01-01 |
description |
Background: To study the diagnosis, management, and outcome of abdominal neuroblastoma (NBL) in infants and children and also the behavior of some rare types. Patients and Methods: This is a retrospective review of 46 infants and children having abdominal NBL at King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia and Tanta University Hospital, Tanta, Egypt (a joint study) between February 2008 and January 2012. The patients′ medical records were reviewed for demographic details, clinical presentations, histopathology, radiological diagnosis, management, and outcome. Results: Of the 46 patients, 5 (10.7%), three males and two females, aged 1.5-6.5 years (mean: 4.5 years) had rare types of NBL (one bilateral NBL, one pelvic NBL, one pelvic-abdominal, and there were two cases of ganglioneuroblastoma). Three patients (two ganglioneuroblastoma and one pelvic) underwent complete surgical removal of the tumor with a good disease survival, whereas the other two patients (two bilateral and one pelviabdominal) had advanced disease and received palliative chemoradiotherapy. The remaining 41 patients, 23 males (56.1%) and 18 females (43.9%), with mean age 3.9 years (range: 1-7 years), had unilateral abdominal NBL. Twelve of them had resectable tumor and underwent primary surgical removal of the tumor, whereas the remaining 29 patients had unresectable tumor and received pre-operative chemotherapy with good response of the tumor in seven patients and no response in the remaining 22 patients. Conclusion: Primary complete surgical removal of tumor is advisable in localized NBL with a good outcome, whereas in advanced cases, it is better to start with pre-operative chemotherapy to downsize the tumour mass and safe delayed surgical excision. An increase in patient′s age is associated with advanced stage of NBL and poor prognosis except in ganglioneuroblastoma cases due to maturation of tumor cells. |
topic |
Abdominal neuroblastoma childhood malignancies malignant tumor solid tumor |
url |
http://www.afrjpaedsurg.org/article.asp?issn=0189-6725;year=2013;volume=10;issue=2;spage=100;epage=107;aulast=Aldaqal |
work_keys_str_mv |
AT salehmaldaqal clinicopathologicalpatternsofararepresentationofabdominalneuroblastomainchildren AT alimturki clinicopathologicalpatternsofararepresentationofabdominalneuroblastomainchildren |
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