Neuroblastoma in Adults: A Systematic Review and Case Presentation

Bader H. Alsaikhan, Basmah Alwahhabi, Abdullah Alshalan, Alex Koziarz, Mohammad Alghafees*, Abdullah M. Alkhayal and Khalid Alrabeeah

1College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Saudi Arabia 2King Abdullah International Medical Research Centre (KAIMARC), Saudi Arabia 3Division of Urology, Department of Surgery, King Abdulaziz Medical City, National Guard Health Affairs (MNGHA), Saudi Arabia 4Department of Medicine, King Abdullah bin Abdulaziz University Hospital, Saudi Arabia 5Department of Surgery, Division of Urology, King Abdulla bin Abdulaziz University Hospital, Saudi Arabia

^*Correspondance to: Mohammad Alghafees 

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Abstract:

Introduction: Neuroblastoma is a heterogeneous disease rarely diagnosed in adults. The aim of this study is to summarize all published reports to inform clinicians of the available evidence of this heterogeneous disease. Methods: We performed a systematic review of four databases to identify adults with neuroblastoma. Cohort studies, case series, and case reports were synthesized qualitatively. Presentations, genetic abnormalities, and therapies were detailed. Progression-free and overall survival were compared amongst cohort studies. We also describe a case of a 24-year-old male presenting with an L1 Neuroblastoma. Results: Of 2287 unique records, 136 studies published in 141 articles were included. A total of 679 patients were included. When totalling case series, the adrenal gland and retroperitoneum were the most common primary sites (47.3%), which was similar in single cohort studies reporting adrenal gland primary site occurrence as high as 59%. MYCN was rarely amplified: two studies reported a single patient with MYCN amplification and five studies reported none. Adult patients appear to show a high frequency of somatic mutations, specifically ALK and ATRX. Registry data of included studies showed the 5-year overall survival to be 36.3% in adults aged ≥20 years. Our presented case underwent surgical resection alone, and was disease-free at 3 months follow-up. Conclusion: We provide a comprehensive overview of the variable presentations, management strategies, and outcomes of 679 adult patients with neuroblastoma. Adult neuroblastoma is a rare disease entity that may present at any age, and appears to show considerable rates of somatic mutations. Future studies evaluating targeted therapies in larger samples are needed.

Keywords:

Adult neuroblastoma; Genetic mutations in neuroblastoma; Adrenal gland tumors; Genitourinary Tumors

Cite the Article:

Alsaikhan BH, Alwahhabi B, Alshalan A, Koziarz A, Alghafees M, Alkhayal AM. Neuroblastoma in Adults: A Systematic Review and Case Presentation. Ann Clin Case Rep. 2024; 9: 2708..