summary Neuroblastomas are malignant tumors that develop from sympathetic neural tissue with most cases arising near the adrenal gland or the spinal cord. The condition usually presents in children younger than age 2 with fever, weight loss, and diarrhea. Diagnosis is made with a biopsy showing small round blue cells forming rosette patterns. Treatment may be observation or a combination of chemotherapy, stem cell transplantation, and surgical excision depending on the histological grade and location of the tumor. Epidemiology Incidence occurs in approximately 1 out of 100,000 children it is the most common solid tumor of childhood Demographics slight male predominance the majority of cases occur in children younger than 2 years of age Etiology Metastasis metastasis to the bone are common and a poor prognostic sign Presentation Symptoms fever, malaise, weight loss, diarrhea if the adrenal glands are affected, the child may present with significant tachycardia abdominal mass Exam may reveal abdominal mass or hepatosplenomegaly Biopsy needle or open incisional biopsy is required to obtain lesional tissue and make the histological definitive diagnosis Imaging Radiographs may show bone lesions in cases of metastatic disease bone lesions are often permeative and lytic in nature may occur anywhere in the skeleton CT chest/abd/pelvis is the most common initial imaging sequence for evaluation of the extent of disease helpful in determining the extent of visceral metastasis MRI MRI scan of the chest/abdomen may be useful to delineate anatomic structures Bone scan helpful in determining the extent of metastatic bone disease Histology Small round blue cells forming rosette patterns other small round blue cell tumors of childhood include rhabdomyosarcoma non-Hodgkin's lymphoma Ewing's sarcoma/PNET blastemic component of Wilms’ tumor Treatment Nonoperative observation indications very young infants with low risk tumors a significant portion of neuroblastomas can spontaneously regress chemotherapy and stem cell transplant alone indications children with high grade or metastatic neuroblastomas in a location not amenable to surgical resection technique treat with chemotherapeutics like platinum (cisplatin) or alkylating agents (ifosfamide) followed by stem cell transplant Operative surgical excision indications low grade tumors which can be easily excised with a wide surgical margin while not damaging critical neurovascular structures chemotherapy, stem cell transplant, and surgical excision indications high grade tumors which arise in close proximity to critical neurovascular structures and therefore cannot be easily excised with a wide surgical margin Prognosis The majority of children are cured Metastatic disease is a poor prognostic factor In very young children, spontaneous regression of the tumor without treatment is known to occur