J Korean Neurosurg Soc.  2012 Jul;52(1):55-57. 10.3340/jkns.2012.52.1.55.

Alveolar Soft Part Sarcoma Metastasized to Both the Skull and the Brain

• Shin TH
• Jung YJ
• Kim OL
• Kim MS

• KMID: 2190525
• DOI: http://doi.org/10.3340/jkns.2012.52.1.55

Abstract

Alveolar soft part sarcoma (ASPS) with skull and brain metastases is extremely rare. A 53-year-old patient diagnosed as skull metastasis of ASPS visited our clinic complaining of an outgrowing scalp mass in spite of radiation therapy. Past medical history revealed that the patient had been diagnosed and treated for ASPS of the thigh 4 years ago. Magnetic resonance imaging revealed a hyperintense ovoid mass on the T2-weighted image, an isointense on the T1-weighted image, and a homogeneous enhanced mass with gadolinium. Another small-sized enhanced mass with mild peritumoral swelling was found at the deep white matter of the left frontal lobe. A gross total resection of the skull lesion with cranioplasty was performed for the surgical defect. A histologic examination of the specimens revealed metastatic ASPS involving the skull. Surgery with a total removal of the lesions may be effective for improving a patient's symptoms especially from neurological dysfunction.

Keyword

Alveolar soft part sarcoma; Brain metastasis; Sarcoma; Surgery

MeSH Terms

Brain
Frontal Lobe
Gadolinium
Humans
Magnetic Resonance Imaging
Middle Aged
Neoplasm Metastasis
Sarcoma
Sarcoma, Alveolar Soft Part
Scalp
Skull
Thigh
Viperidae
Gadolinium

Figure

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  Fig. 1 Magnetic resonance images of the patient with ASPS metastases to both the skull and the brain. A and B: The T1-weighted-enhanced sagittal and axial images reveal an osteolytic mass lesion on the frontal bone. C: The T1-weighted-enhanced axial image reveals a well-enhanced lesion in deep white matter on the left frontal lobe. ASPS: alveolar soft part sarcoma.

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  Fig. 2 Intra-operative photographs. Following subgaleal dissection, a well-circumscribed mass is found on the frontal bone.

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  Fig. 3 A: The photograph shows the resected tumor mass with surrounding skull bone. B: The histological finding shows large, round to polygonal tumor cells growing in the classical alveolar pattern (H&E stain, ×100). C: An ASPS showing uniform, strong nuclear positivity with an antibody directed against the C-terminus of transcription factor 3 (TFE3), confirming the presence of an ASPL-TFE3 fusion protein (×100). ASPS: alveolar soft part sarcoma.

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