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Clinical Presentation

A previously healthy 3-year-old female presented with intermittent sharp abdominal pain.

Radiology:

• CT chest: a retroperitoneal mass, measuring 12.9 x 11.1 x 8.2 cm, arising in the right adrenal gland and extending into the hilum of the right kidney.
• MIBG scan showed MIBG accumulation at the site of the intra-abdominal mass.

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Light Microscopic Findings

Immunohistochemical Staining

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IHC panel for SRBCTs

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Immunohistochemical Staining

• Negative: myogenin, desmin, WT1, NKX2.2, CD99

Diagnosis?

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