Fibrous hamartoma of infancy

Case contributed by Bao Nguyen
Diagnosis certain

Presentation

The patient presented with a rapidly enlarging left arm mass.

Patient Data

Age: 1 year
Gender: Male

Left arm

mri
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Axial
T1
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Axial
T1 C+
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Axial T1
C+ fat sat
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Axial
T2
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Coronal T1
C+ fat sat
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Coronal
T1
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Coronal
STIR
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Sagittal
STIR
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Sagittal T1
C+ fat sat
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Info

There is a well-defined, homogeneously enhancing soft tissue mass in the subcutaneous fat in the left anteromedial upper arm. The mass abuts the anterior abdominal musculature with no intervening fat plane and has no definite intramuscular extension.

ultrasound
Transverse
Doppler
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Info

There is a diffusely hypoechoic mass with interspaced hyperechoic and isoechoic foci which appear to represent areas of microcalcifications and interspersed subcutaneous fat. The mass is hypovascular overall, with a vascular appearance similar to adjacent musculature.

Case Discussion

The patient underwent a core needle biopsy.

Histology

Gross: multiple core needle specimens measuring from 1.4 to 2.5 cm in length.

Microscopy: characteristic triphasic growth pattern with bland fibromatosis-like fibroblastic zones, hyalinized zones with crackling artifacts, and small foci of more primitive mesenchyme, showing myxoid stromal change.

Immunohistochemical stains: ALK1 negative, desmin negative, myogenin negative, Factor XIII A negative, EBV negative, CD34 variable staining non-contributory, beta-catenin non-contributory, S100 highlights fat, smooth muscle actin highlights smooth muscle.

Final diagnosis: fibrous hamartoma of infancy.

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