Updated: 6/22/2021

Neuroblastoma

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  • 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

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

QID: 3438
1

Leukemia

2%

(94/3918)

2

Lymphoma

11%

(421/3918)

3

Neuroblastoma

66%

(2597/3918)

4

Ewing's Sarcoma

18%

(704/3918)

5

Eosinophilic granuloma

2%

(94/3918)

L 3 C

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