Case Study: Long-Term Effects of Treatment in a Survivor of Stage IV Neuroblastoma

Purpose: Neuroblastoma is a tumor of the sympathetic nervous system and adrenal medulla that most frequently affects the paravertebral area of the abdomen and the posterior mediastinum of children with a median age of 18 months at diagnosis [1]. It accounts for 8% of pediatric cancers, yet it is responsible for 15% of deaths from pediatric cancer [2]. Although great strides have been made in the treatment of this disease over the last several decades, children diagnosed with high-risk neuroblastoma still have an unfavorable 5-year survival rate of 40%[1]. While extensive literature exists on the progression of neuroblastoma, not much can be found on the long-term effects of those who have survived high risk disease. Methods: An 8-year-old female was diagnosed in 2005 with localized stage II neuroblastoma of her upper abdominal paraspinal region. The localized tumor was resected and she appeared to be cancer-free. However, she relapsed 3 months later with metastatic stage IV disease that had progressed to widespread bone marrow involvement. After approximately 3 years of undergoing multiple protocols of chemotherapy along with radiation, I-MIBG (metaiodobenzylguanidine) therapy and hematopoietic stem cell transplantation, this young girl was found to be tumor-free. She has been in continuous remission for 10 years but her survivorship has not been without great obstacles. Results: While long term survival of stage IV neuroblastoma is a remarkable feat, the patient has continued to face obstacles resulting from late effects of treatment. She has had recurring bouts of thrombocytopenia, delayed-onset puberty with amenorrhea, primary ovarian failure and high frequency hearing loss, all of which resulted from her exposure to chemotherapeutic agents and radiation at a young age. She has faced these challenges with courage and a commitment to live as normally as possible; however, she must continue to be monitored closely for any long-term residual effects that could still manifest, in order to manage these effectively. Conclusions: This is a unique case of a child diagnosed at an older than expected age with stage IV neuroblastoma who survived the high-risk cancer and has remained in remission for many years after her treatment. She has encountered and overcome many hardships during her remission. This case highlights the importance of studying the long-term effects of treatment that survivors of childhood cancer face as they enter adulthood.