Poster - 63
Clinical insights into congenital neuroblastoma in early infancy
Boukhrissa Nouha 1, Sfar Sami 2, Toumi Afef 1, Bel Hssan Samia 2, Mani Salma 2, Ksia Amine 2, Mekki Mongi 2, Belghith Mohssen 2, Sahnoun Lassaad 2
1 Department of Pediatric Surgery, Monastir University Hospital, Tunisia
2 department of pediatric surgery , University Hospital of Monastir
Introduction:
Congenital neuroblastoma represents approximately 5% of all neuroblastoma cases and is typically diagnosed within the first two months of life. Despite its rarity, the prognosis is generally favorable, underscoring the importance of early diagnosis and timely intervention.
Objective:
To describe the clinical characteristics and therapeutic management of congenital neuroblastoma.
Materials and Methods:
We conducted a retrospective study of all patients diagnosed with neuroblastoma before the age of two months, between 2016 and 2023.
Results:
Seven infants (4 boys, 3 girls) with a mean age of 1.6 months (range: 1 day to 2 months) were included. Abdominal distension was the presenting symptom in all cases, with hepatomegaly observed in three. One patient was diagnosed prenatally with an abdominal mass. Imaging (abdominal ultrasound and thoraco-abdomino-pelvic CT scan) revealed tumor localization as follows: unilateral adrenal in 4 cases, bilateral adrenal in 1, mediastinal in 1, and midline in 1. Three patients presented with Pepper syndrome, characterized by diffuse micronodular hepatic involvement. Diagnosis was confirmed by CT-guided tumor biopsy in all cases, with hepatic biopsy confirming liver metastasis in three. N-Myc amplification was detected in five patients, and bone marrow infiltration was present in three. All patients received neoadjuvant chemotherapy followed by complete surgical excision of the tumor. Only one patient required adjuvant chemotherapy. Postoperative follow-up showed no clinical or radiological recurrence in any case.
Conclusion:
Although rare, congenital neuroblastoma should be considered in any infant under two months presenting with an abdominal mass. Early diagnosis and appropriate multidisciplinary management are essential to ensure a favorable outcome