A seven-year-old girl presented with complaints of fever and joint pains since one month. She gave history of vague abdominal pain since one month. Since then too, there was a progressively increasing swelling on the vertex of the skull. On examination, this swelling had a firm consistency and was not well defined. The patient's vital parameters were normal.

RADIOLOGICAL FINDINGS:

A radiograph of the abdomen was unremarkable.

Ultrasonography of the abdomen revealed a well-defined, hypoechoic mass measuring 6x4x4 cms in the left adrenal fossa on the upper pole of the left kidney displacing the kidney inferiorly. It was hypo-vascular with fine calcification within.

Fig. 1	Fig. 2

A plain and contrast enhanced CT scan of the abdomen revealed a well defined 6x4x4 cms hypo dense, hypo vascular mass lesion involving the left adrenal gland with displacement of the kidney inferiorly and laterally. No surrounding infiltration was noted. Fine calcification was seen within the mass.

Fig. 3	Fig. 4 .

The skull film showed a destructive lesion with a sunray periosteal reaction over the left parietal bone.

Fig. 5	Fig. 6

Sonography of the skull confirmed the sunray periosteal reaction and showed a subgaleal hypoechoic soft tissue mass measuring 11x9x 8 cm.

Fig.7

A CT scan of the skull revealed lytic lesions in the left parietal bone with sunray periosteal reaction with subgaleal mass with underlying dural involvement suggestive of a metastatic involvement of the calvarium and dura.

Fig. 8	Fig. 9

A bone marrow smear was performed which showed atypical round cells suggestive of a neural crest tumour metastases.

MIBG scan showed increased I-131 MIBG concentration within marrow cavity of the skull and spine indicating metastases.

Bone scan revealed metastases to the skull and spine.

Final diagnosis - Left neuroblastoma with metastases. Stage 4 INSS staging.

DISCUSSION:

Neuroblastomas are the commonest extracranial tumour in children and account for 6-8 % of pediatric malignancies. They originate from the cells of the neural crest origin which give rise to the sympathetic nervous system and adrenal medulla. The median age of patient at the time of diagnosis is two years but the tumour can present at any pediatric age.

Two thirds of patients with neuroblastoma have metastasis (Stage IV disease) at the time of presentation. Metastasis is commonly to the liver, spine, skull and lymph nodes. Staging is done based on the International Neuroblastoma Staging System (INSS) from stage 1 to 4S based on radiological findings, surgical resectability, lymph node involvement and bone marrow involvement.

Technetium 99m methylene diphosphonate whole body bone scintigraphy is a must for detection of metastases. MIBG scan, though less sensitive than MDP should also be done as it can detect both the primary and metastases. FDG PET is likely to play a larger role in neuroblastoma imaging in future.

All patients older than one year with stage IV tumors are considered to be in the high-risk group. These patients seem to require treatment with multi-agent chemotherapy, surgery, and radiotherapy followed by consolidation with high-dose chemotherapy and peripheral blood stem cell rescue.

The five year survival rate from diagnosis is approximately 83% for infants, 55% for children aged 1-5 years, and 40% for children older than 5 years.