Neuroblastoma (craniocerebral metastases)

Changed by Bruno Di Muzio, 30 Aug 2015

Updates to Article Attributes

Title was changed:
Metastatic neuroblastomaNeuroblastoma: craniocerebral metastases
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NeuroblastomaCraniocerebral neuroblastoma metastases  is the 3rd most common malignancy after leukemia and primary CNS tumors, it arises in the adrenal gland or along the sympathetic chain 2.

Metastasis occurs in about 70% of patients at the time of diagnosis 3.

Craniocerebral metastasis usually involve the calvaria, orbits, skull base and the dura.

Please refer on the article neuroblastoma for a general discussion on this entity. 

Clinical presentation

  • proptosis
  • periorbital ecchymosis "raccoon eyes"
  • palpable calvarial masses

Radiographic findingsfeatures

CT
  • spiculated periorbital bone mass, typically involving the roof or lateral wall/sphenoid wings
  • "Hair on end" spiculated periostitis of orbits and skull usually associated with bone destruction
  • sutural separation: non uniform sutural separation with indistinct sutures margins
  • cranial metastasis nearly always extra-dural (may mimic epidural or subdural hematoma)
MRI
  • T1 WI: hypointense to muscles
  • T2 WI: heterogeneous usually hyperintense to muscles
  • T1 WI C+ C+ (Gd): vigorously enhances, may be heterogeneous
  • MRV: may narrow or invade adjacent dural sinuses
Nuclear medicine
  • MIBG (meta-iodobenzylguaidine-iodo benzyl guanidine): avid uptake by neural crest tumors
  • TC-99-m-MDP: shows increased uptake from calcium metabolism of tumor
  • PET: shows high sensitivity and specificity for recurrent tumors

Clinical profileDifferential diagnosis

  • proptosis
  • periorbital ecchymosis "Raccon eyes"
  • leukemia

  • palpable calvarial masses

Differential diagnosis

  • Leukemia:

    dural or calvarial based masses,

  • usually associated with intraparenchymal masses

  • Langerhanslangerhans cell histocytosishistiocytosis:

    • lytic bone lesions without periosteal bone formation

  • spiculated periosteal reaction
  • Extraextra-axial hematomahaematoma
  • Ewingewing sarcoma
  • osteosarcoma
    • -<p><strong><a href="/articles/neuroblastoma">Neuroblastoma</a></strong> is the 3<sup>rd</sup> most common malignancy after leukemia and primary CNS tumors, it arises in the adrenal gland or along the sympathetic chain <sup>2</sup>.</p><p>Metastasis occurs in about 70% of patients at the time of diagnosis <sup>3</sup>.</p><p>Craniocerebral metastasis usually involve the calvaria, orbits, skull base and the dura.</p><h4>Radiographic findings</h4><h5>CT</h5><ul>
    • +<p><strong>Craniocerebral neuroblastoma metastases </strong>usually involve the calvaria, orbits, skull base and the dura.</p><p>Please refer on the article <a title="Neuroblastoma" href="/articles/neuroblastoma">neuroblastoma</a> for a general discussion on this entity. </p><h4>Clinical presentation</h4><ul>
    • +<li>proptosis</li>
    • +<li>periorbital ecchymosis "raccoon eyes"</li>
    • +<li>palpable calvarial masses</li>
    • +</ul><h4>Radiographic features</h4><h5>CT</h5><ul>
    • -<li>"<strong>Hair on end</strong>" spiculated periostitis of orbits and skull usually associated with bone destruction</li>
    • +<li>"<a title="Hair on end sign" href="/articles/hair-on-end-sign">Hair on end</a>" spiculated periostitis of orbits and skull usually associated with bone destruction</li>
    • -<strong>T1 WI</strong>: hypointense to muscles</li>
    • +<strong>T1</strong>: hypointense to muscles</li>
    • -<strong>T2 WI</strong>: heterogeneous usually hyperintense to muscles</li>
    • +<strong>T2</strong>: heterogeneous usually hyperintense to muscles</li>
    • -<strong>T1 WI C+</strong>: vigorously enhances, may be heterogeneous</li>
    • +<strong>T1 C+ (Gd)</strong>: vigorously enhances, may be heterogeneous</li>
    • -<li>
    • -<strong>MIBG </strong>(meta-iodobenzylguaidine): avid uptake by neural crest tumors</li>
    • -<li>
    • -<strong>TC-99-m-MDP</strong>: shows increased uptake from calcium metabolism of tumor</li>
    • -<li>
    • -<strong>PET</strong>: shows high sensitivity and specificity for recurrent tumors</li>
    • -</ul><h4>Clinical profile</h4><ul>
    • -<li>proptosis</li>
    • -<li>periorbital ecchymosis "<strong>Raccon eyes</strong>"</li>
    • -<li>palpable calvarial masses</li>
    • -</ul><h4>Differential diagnosis</h4><ul>
    • -<li>Leukemia: dural or calvarial based masses, usually associated with intraparenchymal masses</li>
    • -<li>
    • -<a href="/articles/langerhans-cell-histocytosis">Langerhans cell histocytosis</a>: lytic bone lesions <em>without</em> periosteal bone formation</li>
    • -<li><a title="Spiculated periosteal reaction" href="/articles/spiculated-periosteal-reaction">Spiculated periosteal reaction</a></li>
    • -<li>Extra-axial hematoma</li>
    • -<li>Ewing sarcoma</li>
    • +<li>MIBG (meta-iodo benzyl guanidine): avid uptake by neural crest tumors</li>
    • +<li>TC-99-m-MDP: shows increased uptake from calcium metabolism of tumor</li>
    • +<li>PET: shows high sensitivity and specificity for recurrent tumors</li>
    • +</ul><h4><span style="font-size:1.5em; line-height:1em">Differential diagnosis</span></h4><ul>
    • +<li>
    • +<p>leukemia</p>
    • +<ul>
    • +<li><p>dural or calvarial based masses</p></li>
    • +<li><p>usually associated with intraparenchymal masses</p></li>
    • +</ul>
    • +</li>
    • +<li>
    • +<p><a href="/articles/langerhans-cell-histocytosis">langerhans cell histiocytosis</a> </p>
    • +<ul><li><p>lytic bone lesions without periosteal bone formation</p></li></ul>
    • +</li>
    • +<li><a href="/articles/spiculated-periosteal-reaction">spiculated periosteal reaction</a></li>
    • +<li>extra-axial haematoma</li>
    • +<li>ewing sarcoma</li>

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