PMC3433120_01
Swelling present on the forehead measuring 2.5 x 3 cm in dimension.
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fig1
On clinical examination, the infant appeared systemically healthy with a firm, subcutaneous nodule situated in the right frontal bone region of the skull measuring 2.5 x 3 cm in dimension (Figure 1).
(a) T2 weighted image showing a well-defined mass, mildly hyperintense in comparison to brain and (b) T1 weighted image showing well-defined lesion with lytic cortical borders with homogenous enhancement.
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fig2
MRI revealed an expansile soft tissue mass, oval in shape, and 2 x 3 cm in size with well-demarcated cortical borders mildly hyperintense in comparison with the brain matter (Figures 2(a) and 2(b)).
(a) Intraoperative image showing surgical excision of the lesion and (b) surgical site following excision.
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fig3
Involved pericranium was excised and the wound was closed in layers (Figures 3(a) and 3(b)).
Excised specimen showing the periosteal lining (a), Photomicrograph showing tumour cells arranged in a biphasic pattern (H/E, x40) (b), Photomicrograph showing light staining areas composed of plump myoid spindle cells arranged in short fascicles with elongated/cigar-shaped nuclei and dark staining areas composed of round or polygonal cells with slightly pleomorphic hyperchromic nuclei (H/E, x100) (c).
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fig4
Macroscopically, the mass appeared as a grayish-white gelatinous mass measuring 2 x 3 cm in dimension and was sent along with a piece of the periosteum covering the bone defect (Figure 4(a)) for histopathologic examination. Microscopically, there was a multinodular growth pattern that had a biphasic pattern owing to the alternation of light and dark staining areas (Figure 4(b)). The dark staining areas were composed of round cells with slightly pleomorphic hyperchromic nuclei around a distinct hemangiopericytoma-like vascular pattern (Figure 4(c)).
(a) Photomicrograph showing immunopositivity for vimentin (x100) and (b) photomicrograph showing immunopositivity for SMA (x100).
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fig5
Immunopositivity was seen for vimentin, SMA (Figures 5(a) and 5(b)), and negative for desmin, S-100 indicating a myofibroblastic lineage.