Presentation
Presentation to ED in extremis. Intubated.
Patient Data
Large right-sided chest mass with mediastinal shift to the left. ET tube position is appropriate. No bony abnormality. Left lung and right upper lobe (compressed) appear normal.
Huge right-sided tumor within the chest. There is internal calcification.
The tumor extends through neural foramina into the spinal canal although the spinal cord is not compressed.
Small right-sided pleural effusion, sub-pulmonic effusion and trace fluid around the liver.
CT confirms internal calcification. No bone destruction (bone windows not uploaded).
MIBG images. High uptake in the tumor. No metastatic disease. The remainder of uptake is physiological.
Reduction in volume of disease following three rounds of chemotherapy.
Pathology
The morphological appearances are those of neuroblastoma which shows evidence of ganglion cell differentiation (estimated as >5% of the cells) and therefore is classified as neuroblastoma differentiating. This morphological diagnosis is confirmed on immunohistochemistry with strong positivity with antibodies to CD56 and synaptophysin. Only very occasional cells exhibit positivity with chromogranin. Desmin is negative.
Case Discussion
Appearances here are of neuroblastoma. Neuroblastoma in the chest is unusual, but there are numerous hallmarks of the disease here including a paraspinal location, calcification, neural-foraminal extension and a lobulated appearance. The MIBG update confirms the diagnosis and no metastatic disease.
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