ABSTRACT
Introduction:
Neuroblastoma is the most common intra-abdominal malignancy of infancy and the most common extra cranial solid tumor of childhood. Compared to any other tumour, this tumour has varied clinical presentation and has great differences in distribution and response to therapy. Although there have undoubtedly been major advances in therapy over recent decades, there is still room for significant improvements in outcome to be made.
Aim of the work:
To review the clinical behaviors, management options and outcome of pediatric retroperitoneal neuroblastoma, treated at Sohag university hospital and to highlight on some prognostic factors affecting the outcome.
Patients and methods:
Thirteen children with abdominal neuroblastoma were diagnosed and treated between April1997 and April 2004 at surgery department of Sohag university Hospital.
The data included the age, sex, and clinical presentation, duration of symptoms, primary anatomic site, size, stage of the tumour, metastatic site if present, histopathology, and modalities of treatment and prognostic factors. Patients were stratified as stage 1, 2A, 2B, 3, 4,4S according to the post surgical International Neuroblastoma Staging System (INSS).
Results
Thirteen patients were encountered. There were seven males and six females. Their ages ranged from 9 months to 5 years. The mean age was 1.8 years. Clinical presentation included painless abdominal swelling in 7patients (54%), abdominal pain in 5 (38%), vomiting in 5 (38%), fever in 4 (30%), diarrhea in 2 (15%) and loss of weight in 2 (15%). Duration of symptoms was from 2 weeks to 8 months, the mean duration was 11.5 weeks. Primary sites of involvement included: retroperitoneal in8 patients (62%) the pelvis in 3(23 %) and adrenal in 2 cases (15%). site of metastasis was the liver in 3, bone marrow in 4, cortical bone in 2 and distant lymph nodes in 3 patients. According to (INSS), stage 2B in 3, stage 3in 3, stage 4in3and stage4s in 4 patients. Pathological features showed: neuroblastoma in 9 patients (70%), gangiloneuroblastoma in 3 (23%) and Ganglloneuroma in one patient (7%).
All cases were subjected to surgery, where complete excision was done in 7, total nephrectomy in 2, debulking with biopsy in 2 and only biopsy in 2 patients.
All patients were subjected to postoperative chemotherapy, 6 patients to postoperative radiotherapy. Overall survival rates at 3 years at different stages were as followed: Stage 2: 100 %; Stage 3: 33.3%; Stage 4: 0 %; Stage 4S: 100 %. The overall survival rate during 3years follow up was 61.5 % (5 out of 13 patients) died. Mortality rate is 38.5%.
Conclusion:
The tumour growth rates of neuroblastoma in age < two years are quicker than in age > 2 years. Wide local excision with retroperitoneal lymphadenectomy is the treatment of choice in operable cases. Neuroblastoma in pediatrics age group patients is more sensitive to chemotherapy than in older children. Factors of favorable prognosis are infants and children less than two years, stage 1, 2, and 4S, the tumour site at the pelvis or retroperitoneal, low level of N.S.E and favorable histology.
Key words: retro peritoneal neuroblastoma, children, surgery, chemotherapy, radiotherapy.