Updated: 10/6/2016

Neuroblastoma

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Introduction
  • Neuroblastoma is a malignant tumor which develops from sympathetic neural tissue
    • it is the most common solid tumor of childhood 
    • most cases of neuroblastoma arise in the adrenal gland or near the spinal cord 
  • Epidemiology
    • occurs in approximately 1 out of 100,000 children
    • slight male predominance
    • the majority of cases occur in children younger than 2 years of age
  • Metastasis
    • metastasis to the bone are common and a poor prognostic sign
  • 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
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
 

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Questions (1)

(OBQ11.15) What is the most common malignant solid tumor of childhood? Tested Concept

QID: 3438
1

Leukemia

2%

(94/3815)

2

Lymphoma

11%

(404/3815)

3

Neuroblastoma

66%

(2532/3815)

4

Ewing's Sarcoma

18%

(685/3815)

5

Eosinophilic granuloma

2%

(92/3815)

L 3 C

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