Adrenal Neuroblastoma with Calvarial involvement Sparing Orbit

Palwasha Gul, Zeenat Gul, Imran Masoud Qasmi


Abstract: Neuroblastoma is the third commonest childhood tumor. It is the most common extracranial pediatric neoplasm. It is a tumour of neuroblastic origin. Intra abdominal occurrence is common with adrenals (35%) being the most common site of origin. Other sites may be retroperitoneum: 30-35%, posterior mediastinum: 20%, neck: 1-5%, pelvis: 2-3%. They have been associated with a number of disorders, such as Hirschsprung disease, fetal alcohol syndrome, DiGeorge syndrome, Von Recklinghausen disease, and Beckwith-Wiedemann syndrome. Approximately 70-80% of patients older than 18 months present with metastatic disease in liver, lymph nodes, bones, and bone marrow. Skull vault metastases are generally more common in adults and infrequent in children. Calvarial metastases are characteristically seen with simultaneous involvement of the orbits and isolated metastasis rarely occurs.1,2,3We report a case of five year old male who was diagnosed as a case of neuroblastoma which initially presented with extensive calvarial metastasis with no involvement of the orbits. CT showed cortical irregularity and thinning of the skull bones and hair on end periosteal reaction which was involving the fronto parieto temporal bones bilaterally. The coronal and sagital sutures were also widened. Intracranial soft tissue component was noted along the fronto-parieto-temporal bones bilaterally. Concave convex extracranial soft tissue component was also noted in both fronto parietal regions. The differentials included lymphoprolifertaive disorders. Patient ultrasound abdomen showed a well-defined hyperechoic mass in right adrenal gland. It showed no internal calcifications. Abdominal lymphadenopathy was also noted. Patient was further referred to oncologist.

Full Text:



  • There are currently no refbacks.

© Copyright PJR 2008-