Malignant peripheral nerve sheath tumour (MPNST)

Case contributed by Ammar Haouimi
Diagnosis almost certain

Presentation

Right gluteal region mass since birth with a large cutaneous lesion suggestive of giant congenital naevus.

Patient Data

Age: 7 months
Gender: Male
This study is a stack
Axial
T1
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Axial
T2
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Coronal
T2
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Axial
STIR
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Axial
Gradient Echo
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Axial T1
fat sat
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Axial T1
C+ fat sat
This study is a stack
Coronal T1
C+ fat sat
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Info

The MRI sequences demonstrate a large well-circumscribed lobulated soft tissue mass arising from the gluteus maximus muscle. It is isointense to striated muscle on T1WI and shows inhomogeneous high signal on T2WI. It contains foci of haemorrhage with haematocrit effect, wherein the nondependent plasma appears hypointense on T1WI and hypointense on T2WI, whereas the RBC sediment (haematocrit) appears hypointense on both sequences. The mass enhances intensely post-contrast.

Case Discussion

The infant went on to have complete resection of the tumour. The histopathological exam and immunohistochemistry were consistent with a malignant peripheral nerve sheath tumour (MPNST) on a pre-existing giant congenital naevus.

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