Adil Abdelhamed Abbas1* and Khalid Abdalla Khalid2
1Department of Pediatrics, Princess Nourah Oncology Centre, King Saud bin Abdulaziz University for Health Sciences, Saudi Arabia
2Department of Pediatric Hematology and Oncology, Princess Nourah Oncology Centre, King Abdulaziz Medical City, Saudi Arabia
Neuroblastoma (NBL) is the most common intra abdominal childhood malignant tumor. NBL is a malignancy of structures derived from the embryonic neural crest. The hallmarks of its enigmatic character include its propensity for spontaneous regression under certain circumstances. Metastatic, High Risk (HR) NBL however is a serious disease at almost any age. Successful treatment requires intensive chemo/radio/surgery approach and biologic therapy using cis-retinoic acid and anti GD2 monoclonal antibodies. The Overall Survival (OS) and Event Free Survival (EFS) figures have improved in the recent years. Spontaneous regression or even cessation of tumor growth is not known in metastatic HR-NBL, and disease progression is inevitable if left untreated. Here we report a child who was initially diagnosed with HR metastatic NBL at the age of 10 months. The tumor involved his suprarenal gland, Bone Marrow (BM), pelvis and skull causing large tumor masses. He initially received 4 cycles of Chemotherapy (CTR) and then stopped for toxicity. He only had minimal response to CTR. However, he remained well thereafter for several years, well-adjusted with his tumors which were growing in parallel to his general growth. He presented to our hospital at the age of six years aiming to improve his appearance. In fact, he was not bothered by the disease and continued to lead normal life. His main problem came after entering primary school where he was continuously teased and bullied by his colleagues. Re-investigations confirmed active metastatic NBL. We believe that some undiscovered feature s of his tumor biology may have played a role in that unusual tumor behavior. We aimed at improving his facial appearance with cosmetic surgery. We initially treated him with CTR but he showed no response. His disease suddenly flared up a year later and rapidly progressed. Unfortunately, he died within few weeks as a result of disease progression. In this case report we discuss the cytogenetic and the molecular genetics features of NBL that could be behind the unique presentation and clinical course.
Chemotherapy; Dinutuximab; High-risk; MYCN gene; Neuroblastoma; Spontaneous regression
Abbas AA, Khalid KA. Chronic Persistent Neuroblastoma, a Unique Presentation and Clinical Course in a Six-Year-Old Child: A Case Report. Clin Oncol. 2019;4:1677.