CASE 13144 Published on 10.02.2016

Metastatic involvement of the skull in neuroblastoma : a case report

Section

Paediatric radiology

Case Type

Clinical Cases

Authors

AMMOR HICHAM, BOUJARNIJA HAJAR

Ibn Baja Hospital, Taza, Morocco.
Personal adress: Rue Termidi Appt 6 30000 Fes, Morocco; Email: ammor_hicham@hotmail.com
Patient

2 years, female

Categories
Area of Interest Head and neck, Adrenals, Thorax, Thoracic wall ; Imaging Technique CT
Clinical History
A 2-year-old girl presented with a history of gradually increasing scalp swelling across the right temporal region for 3 to 4 weeks.
There was no history of trauma, convulsions, vomiting, limb weakness, abnormal bleeding or bladder/bowel disturbances.
Imaging Findings
Contrast CT of the head revealed an enhancing extracranial tumor in the right temporal region, extending intracranially. The intracranial component had lobulated margins convex to the brain surface. There was no edema; a midline shift was present. Bone and soft tissue windows showed "sunray spiculation" of skull vault under the extracranial mass.
there was also, an other lesion in the left frontal region extending intracranially.
abdominal ultrasound was performed and showed a heterogeneous mass with internal vascularity and large areas of necrosis appearing as regions of low echogenicity.
A thoraco-abdominopelvic CT scan followed and showed a large heterogeneously enhancing soft-tissue adrenal mass with intratumoural calcifications in the right side abdomen, this right adrenal mass lesion is predominantly of low attenuation, which would suggest tissue necrosis. The right kidney is displaced inferiorly. A left pleural thickening was also shown associated with a right chest wall mass with no involvement of the ribs
Discussion
Neuroblastoma (NB) is one of the most common solid tumors in infancy and childhood.
Prenatal diagnosis of NB is possible and is normally diagnosed during the third trimester.
This tumor originates from neural crest, may occur in the adrenal medulla and along the sympathetic ganglion chain from the neck to the pelvis[1].
75% of NBs are located in the retroperitoneum, in either the adrenal medulla (50%) or the paraspinal ganglia (25%). Less than 5% arise in the neck or pelvis.
50-75% of cases present with an abdominal mass. This pathology could also be revealed by metastasis to skull (as the case of our patient), orbit, liver(Pepper's syndrome)or other sites[1].
50-60% of patients with NB present with disseminated disease, this finding is more expected in patients over 1 year of age[2].
Metastases to bone marrow and bones have been described in disseminated disease[3].
We can observe two types of marrow disease: Diffuse type and nodular type[3].
Bone marrow metastases arise initially in the sinusoids where tumor cells adhere and extravasate to parenchyma, forming nodular lesions in the medullary cavity which sometime progress to diffuse lesions and progressively grow to erode trabecular bone and invade the bone cortex, engendering bone metastases[4].
Skull metastasis has been found in up to 25% of patients with NB. NB is the most frequent malignant metastasis to the skull in children[5].These calvarial lesions frequently enlarge to produce epidural deposits.
The staging evaluation of patients with NB consists of computed tomography or magnetic resonance imaging of the primary tumor, a skeletal survey and either bone scintigraphy or metaiodobenzylguanidine scintigraphy for skeletal metastases.
Whole-body MRI is an emerging imaging method that appears useful in staging and monitoring NB; It is helpful in detecting distant metastasis, assessing initial treatment responses, and identifying tumor recurrence.
Metastatic involvement of the skull has various possible radiographic findings: thickened bone,
the “hair-on-end” periosteal reaction, lytic defects, and sutural separation[6,7].
The sutural separation caused by direct involvement of NB differs from that found with generalized increased intracranial pressure. In NB, the separation of sutures is not uniform and the margins of the sutures are partially indistinct[5,8].
The hair-on-end appearance is a periosteal response to tumor cells spreading from a calvarial metastasis. The inner aspect of the periosteum resists notably to tumor cells’ penetration. So the tumor, as it breaks out of the bone, lifts the periosteum, producing plaquelike epidural deposits of tumor[6].
Skull metastases are often very subtle.These calvarial metastases are frequently associated with Scalp lesions[7, 9].
Differential Diagnosis List
skull metastasis of neuroblastoma
Langerhans cell histiocytosis
leukemia
lymphoma
sarcoma metastases
Final Diagnosis
skull metastasis of neuroblastoma
Case information
URL: https://eurorad.org/case/13144
DOI: 10.1594/EURORAD/CASE.13144
ISSN: 1563-4086
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