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Neuroradiology Case of the Week

Case 294

Ashwani K Sharma, MD and P-L Westesson, MD, PhD, DDS

Clinical Presentation: Patient is a 4-year-old female already diagnosed with neuroblastoma of thorax and imaging of head performed for staging purposes.

Imaging Findings: Metastases of neuroblastoma occurs along the external surface of the dura and causes separation of the brain parenchyma from the bony calvarium. The dural metastases are continuous with subcutaneous deposits via the separated sutures. Sutural separation is nonuniform, and the sutural margins are indistinct due to bony involvement. The lytic bony metastases may produce localized calvarial thickening that may have a sunburst calcification.

Diagnosis: Calvarial metastasis from neuroblastoma with spiculated calcification

Discussion: Neuroblastoma constitutes 10% of all pediatric malignancies and 75% of them present in children below 4 years of age [1]. Seventy-five percent of neuroblastomas arise in the abdomen and pelvis, 20% in the thorax and 5% in the neck [2]. Metastases in neuroblastoma characteristically occurs in a disseminated fashion the common sites being liver, lymph nodes, bones and bone marrow [1]. Calvarial metastases however, do occur in neuroblastoma and are characteristically multiple lesions with simultaneous involvement of the orbits [3].
     The characteristic radiographic pattern of skeletal metastases in neuroblastoma is of permeative destruction [4,5]. Calvarial involvement in neuroblastoma is also classically of multiple lucencies, other patterns being diastasis of cranial sutures and "sunray spiculation" or a "hair brush appearance" [1,5]. The latter is a rare presentation, known to have poor prognosis with early death [1]. The differential diagnosis in a child with multiple calvarial mass lesions with spiculated new bone formation is a feature usually characteristic of metastases from leukemia, lymphoma, osteosarcoma or Ewing's sarcoma, rarely neuroblastoma.

References:

1. Egelhoff JC, Zalles C. Unusual CNS presentation of neuroblastoma. Pediatr Radiol. 1996;26(1):51-4. [Medline]
2. Alexander F. Neuroblastoma. Urol Clin North Am. 2000 Aug;27(3):383-92. [Medline]
3. Shaw PJ, Eden T. Neuroblastoma with intracranial involvement: an ENSG study. Med Pediatr Oncol. 1992;20(2):149-55. [Medline]
4. Haddad A, Azouz EM, al-Jedher S, Baruchel S, Bernstein ML, Cotton RT, Tewfik TL. Metastatic neuroblastoma presenting as a mandibular mass. Can Assoc Radiol J. 1992 Dec;43(6):436-40. [Medline]
5. Pascaul-Cartroviejo I, Lopez-Martin V, Rodriguez-Costa, Pascaul-Pascual JI. Radiological and anatomical aspects of cranial metastases of neuroblastoma. Neuroradiology 1975;9:33-8.