1 year 9 month old male complaining for progressively worsening right leg pain for 3 weeks and has been unable to weight-bear for the last week.
Radiographs – Metaphyseal permeative bony destruction in the appendicular skeleton extending across the physeal plate into the epiphysis.
I-131 MIBG scan – Multiple MIBG-avid lesions are seen in the axial and appendicular skeleton, including the upper and lower limbs bilaterally, suggestive of bone metastases. MIBG uptake in the axillae, abdomen and pelvis corresponds to lymphadenopathy. Increased MIBG uptake is seen in the the lower paraspinal regions. Physiological tracer activity is noted elsewhere (salivary gland, endocardium, liver, bladder)
Multifocal osteomyelitis, Langerhans histiocytosis, leukaemia/lymphoma, rhabdomyosarcoma, Ewing’s sarcoma, metastatic neuroblastoma
Neuroblastoma is the 3rd most common pediatric malignancy behind leukemia/lymphoma and CNS tumors. Metastasis is the leading cause of presentation in up to 60% of patients: the common sites being bones, regional lymph nodes and the liver. The presence of extensive skeletal metastases from a primary adrenal neuroblastoma leading to bone pain (poor weight bearing in this case) is also known as Hutchinson’s syndrome. Pepper’s syndrome is when there is massive hepatomegaly caused by metastatic neuroblastoma.
Radiographically, skeletal metastases can present as: (a) periosteal reaction; (b) multifocal lytic foci; (c) lucent horizontal or vertical metaphyseal lines; (d) vertebral collapse; (e) widening of the cranial sutures due to dural involvement. These lesions often become sclerotic with healing and can often mimic other round cell tumors, such as Ewing’s sarcoma, leukemia/ lymphoma and rhabdomyosarcoma.
In regards to MIBG scintigraphy, Metaiodobenzylguanidine uptake is related to catecholamine production. It is excellent for following the extent of disease in MIBG-avid tumors but note that approximately 30% of neuroblastomas are not MIBG avid. If the primary tumour is MIBG-avid, scintigraphy may better pick up early osseous involvement preceding cortical destruction. Any osseous uptake is abnormal. In the image above, uptake within the physeal plates should not be confused for a physiological process.
- Lonergan GJ, Schwab CM, Suarez ES et-al. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics. 2002;22 (4): 911-34.
- David, Ruppert, et al. “The many faces of neuroblastoma.” Radiographics 9.5 (1989): 859-882.