Neuroblastoma

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CHAPTER 34 Neuroblastoma

Naira Baregamian, Dai H. Chung

Step 1: Surgical Anatomy

♦ Neuroblastoma is the most common extracranial solid tumor in infants and children, accounting for 6% to 10% of all childhood cancers.

♦ As a neural crest cell–derived tumor arising from neuroblasts of the sympathetic nervous system, neuroblastoma can occur anywhere along sympathetic chain, ranging from head and neck, thorax to abdomen, and pelvic regions.

♦ The adrenal gland is the most common site of origin.

♦ Neuroblastoma is an aggressive tumor with a tendency to invade adjacent tissues, such as major vascular structures or spinal canal.

♦ Metastasis is usually present at the time of diagnosis (about 65% of cases).

♦ A special category of patients with disseminated disease exists (stage 4S) in which prognosis is surprisingly favorable and includes infants under 1 year of age with primary mass and metastases to the bone marrow, liver, and skin.

Step 2: Preoperative Considerations

♦ Careful multidisciplinary presurgical planning and evaluation of biologic markers are critical for the management.

♦ Features associated with a better prognosis include age at diagnosis younger than 1 year, histologic grade (Shimada system), single N-myc copy, stage 4S, and thoracic primary tumor.

♦ Poor prognostic biologic markers include neuron-specific enolase (>100 ng/mL), lactate dehydrogenase (>1500 U/mL), ferritin (>142 ng/mL), vanillymandelic acid (VMA)/homovanillic acid (HVA) ratio less than 1, N-myc amplification, chromosome 1 short arm (p) deletion, DNA ploidy, decreased CD44 expression, increased tyrosine kinase A expression, increased multidrug resistance–associated protein, and unbalanced 11q loss of heterozygosity.

♦ Screening markers include urinary catecholamine metabolites, HVA, and VMA.

Staging

♦ Computed tomography (CT) scan of chest, abdomen, and pelvis is typically performed at initial workup.

♦ The occult areas of metastasis can be assessed by technetium 99m (Tc^99m MDP) scan.

♦ Tumor viability, disease extent, recurrence, and metastatic disease can also be determined with the use of the radiolabeled metaiodobenzylguanidine (MIBG) scintigraphy.

♦ Plain radiographs of areas of bony tenderness are obtained to evaluate for cortical bone metastasis.

♦ Bilateral posterior iliac crest bone marrow aspiration is performed.

♦ Tissue biopsy should be obtained via methods of needle, incisional, or excisional approach of the primary or metastatic lesions to assess for tumor behavior and diagnosis before the initiation of therapy.

♦ Paraneoplastic syndromes and associated abnormalities include opsoclonus-myoclonus syndrome, vasoactive intestinal peptide–mediated severe secretory syndrome, central hypoventilation syndrome (Ondine’s curse), DiGeorge syndrome, and congenital heart disease.

Imaging

♦ Routine imaging studies include chest radiographs, ultrasonography, CT, and magnetic resonance imaging (MRI).

♦ The preferred diagnostic tool for initial imaging, staging, and the detection of metastases is CT (Fig. 34-1).

♦ Calcifications are noted in about 85% of cases.

♦ In patients with thoracic tumors or tumors with intraspinal extensions, MRI is thought to be more helpful.

Figure 34-1

Differential Diagnoses

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