Neuroblastoma in an Adult: A Case Report

Introduction Neuroblastoma (NB) typically arises in the adrenal glands, sharing a similar developmental origin with nerve cells [1]. NB is exceptionally rare in adults, with an incidence of 1 in 10 million annually and a 5-year survival rate of 36%, compared to 85% in infants [2, 3]. Case Presentation In September 2021, a routine examination in a 34-year-old male revealed a left kidney mass with symptoms of hot flushes, sweating, dizziness, and blood pressure fluctuations. CT showed an 8.1 cm left adrenal mass without distant metastases. A biopsy revealed a highly malignant neuroendocrine tumor. Surgery involved adrenal tumor resection, left nephrectomy, and lymphadenectomy. Immunohistochemistry confirmed undifferentiated neuroblastoma, highrisk NB staged L2 (INRGSS) and 2B (INSS). Adjuvant chemotherapy (COG-regimen) was administered from January 4 to September 19, 2022. The first recurrence in November 2023 was treated with VIP chemotherapy from January 3 to September 9, 2024. By November 9, 2024, the disease progressed with ascites, pain, lymphadenopathy, and rising chromogranin A (from 670 to 2467 µg/L). Third-line chemotherapy (irinotecan, temozolomide) was started on December 1, 2024, resulting in symptom regression, including resolution of ascites and pain. Chromogranin A decreased to 250 µg/L, with partial radiologic response confirmed. Treatment with dinutuximab (anti-GD2) and interleukin-2 (IL-2) is planned, aiming for disease control and improved survival. Discussion Adult NB is rare, leading to limited research and standard treatments [4, 5]. Due to adults' poorer tolerance of polychemotherapy, treatment is often personalized, which may reduce effectiveness [2]. Optimizing chemotherapy is essential, and anti-GD2 immunotherapy like dinutuximab may also be beneficial when used as maintenance therapy following a partial or complete radiologic response [6, 7]. In children, dinutuximab has significantly improved survival rates, with a clinical trial reporting five-year event-free and overall survival rates of 61% and 72%, respectively [8]. Its effectiveness in children suggests potential benefits for adults. Conclusions In our case, the patient has been living with high-risk NB for 4 years and starting dinutuximab is expected to further improve outcomes.