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Key Features

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Essentials of Diagnosis
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  • Clinical manifestations vary with the primary site of malignant disease and the neuroendocrine function of the tumor

  • Presence of constitutional symptoms such as fever, weight loss, and irritability

  • Bone pain suggests metastatic disease, which is present in 60% of children older than 1 year

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General Considerations
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  • Arises from neural crest tissue of the sympathetic ganglia or adrenal medulla

  • Composed of small, fairly uniform cells with little cytoplasm and hyperchromatic nuclei that may form rosette patterns.

  • Accounts for 7–10% of pediatric malignancies and is the most common solid neoplasm outside the CNS

  • Fifty percent of neuroblastomas are diagnosed before age 2 years and 90% before age 5 years

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Clinical Findings

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Symptoms and Signs
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  • Fever, weight loss, and irritability

  • Firm, fixed, irregularly shaped mass that extends beyond the midline

  • Margins are often poorly defined

  • Most children have an abdominal primary tumor (40% adrenal gland, 25% paraspinal ganglion)

  • Tumor in the posterior mediastinum

    • Usually asymptomatic

    • Often discovered on a chest radiograph obtained for other reasons

  • Cervical neuroblastoma

    • Presents as neck mass, which is often misdiagnosed as infection

    • Horner syndrome (unilateral ptosis, myosis, and anhidrosis) or heterochromia iridis (differently colored irises) may accompany tumor

  • Paraspinous tumors

    • Can extend through the spinal foramina, causing cord compression

    • May present with paresis, paralysis, and bowel or bladder dysfunction

  • Neuroblastoma has a predilection for metastasis to the skull, in particular the sphenoid bone and retrobulbar tissue; causes periorbital ecchymosis and proptosis

  • Liver metastasis, particularly in the newborn, can be massive

  • Neuroblastoma may also be associated with unusual paraneoplastic manifestations, such as opsoclonus-myoclonus, also called dancing eyes/dancing feet syndrome

    • This phenomenon is characterized by

      • Acute onset of rapid and chaotic eye movements

      • Myoclonic jerking of the limbs and trunk

      • Ataxia

      • Behavioral disturbances

    • Often persists after therapy is complete

    • Thought to be secondary to cross-reacting antineural antibodies

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Differential Diagnosis
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  • Ewing sarcoma

  • Rhabdomyosarcoma

  • Peripheral neuroectodermal tumor

  • Lymphoma

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Diagnosis

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Laboratory Findings
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  • Anemia

    • Present in 60% of children with neuroblastoma

    • Can be due to chronic disease or marrow infiltration

  • Occasionally, thrombocytopenia is present

  • However, thrombocytosis is a more common finding, even with metastatic disease in the marrow

  • Urinary catecholamines are elevated in at least 90% of patients at diagnosis and should be measured prior to surgery

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Imaging
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  • Plain radiographs of the primary tumor

    • May show stippled calcifications

    • Metastases to bone appear irregular and lytic

    • Periosteal reaction and pathologic fractures may also be seen

  • CT scanning

    • Provides more information, including the extent of the primary tumor, its effects on surrounding structures, and the presence of liver and lymph node metastases

    • Classically, in tumors originating from the adrenal gland, the kidney is displaced inferolaterally, which helps differentiate neuroblastoma from Wilms tumor

  • MRI is useful in determining ...

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