Abstract for presentation at 38th Annual Scientific Meeting of the Australian and New Zealand Society of Nuclear Medicine 2008

FDG-PET Scan of a Retroperitoneal Adrenocortical Carcinoma in a Child with NF1

  • Ms Nelly Keeka, Department of Nuclear Medicine, The Children's Hospital, Westmead, Australia
  • Dr Kevin London, Department of Nuclear Medicine, The Children's Hospital, Westmead, Australia
  • Dr Nicole Graf, Department of Histopathology, The Children's Hospital, Westmead, Australia
  • Prof Robert Howman-Giles, Department of Nuclear Medicine, The Children's Hospital, Westmead and Discipline of Imaging, University of Sydney, Australia

    • Adrenocortical carcinoma (ACC) is a rare paediatric tumour that has a known link to the familial cancer syndromes Beckwith-Wiedemann Syndrome and Li-Fraumeni Syndrome, but has no proven association with Neurofibromatosis type 1 (NF1). It typically presents with virilization or Cushingoid features. ACC has been previously reported in only 2 children with NF1. Methods: This case report presents a rare case of ACC in a child with NF1. Clinical history and radiological imaging are summarised, along with the initial FDG-PET scan and follow up scan after surgical excision.Results: A 10 year old male known to have NF1, presented with a large right sided abdominal mass. Initial US and CT scans showed a large heterogeneous solid mass with calcification in the right upper abdomen posterior and inferior to the liver and displacing the right kidney. A FDG-PET scan showed a large FDG avid tumour. There was a peripheral rim of intense tracer uptake with a central photopenic area consistent with central necrosis but no evidence of metastatic disease. The mass was removed surgically and was separate from the right adrenal gland. Histopathology revealed ACC arising from an extra-adrenal cortical rest. No FDG avid lesions were seen elsewhere. Follow up scan after surgery showed no residual tumour remaining.Conclusions: ACC in a child with NF1 has been shown to be FDG avid and can be used to assess treatment response and monitor for disease recurrence in this rare tumour.

    Conference Organiser - ICMS Pty Ltd