PMC4531729_01
(A) Multiple variable sized cafe-au-lait spots and nodular skin lesions over the chest wall and both arms. (B) Chest PA shows a homogeneous mass density at right upper lung field with well demarcated margin and elevation of right side diaphragm. (C) Chest MRI shows a homogeneous, well-enhanced and well-circumscribed 12x10x8 cm mass involved in the mediastinum and right supraclavicular fossa with multiple adjacent lymphadenopathy. The lesion extrinsically compressed the trachea, esophagus, right carotid and subclavian artery, and encased the brachial plexus. (D) Lower power view of MPNST shows nodular growth pattern and conspicious necrosis (H & E stain, x100). (E) The tumor cells show pleomorphism and twisted and irregular-shaped nuclei in high power field (H & E stain, x400). (F) Immunohistochemical stain for S-100 stain shows positive reaction on tumor lesion (x100).
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Physical examination showed a firm, non-tender and fixed mass associated with ptosis of right eyelid, engorgement of right neck vein, facial swelling and multiple cafe-au-lait spots and pigmented nodular skin lesions on the chest wall and both arms (Figure 1. (A)). Chest radiography showed an increased homogeneous mass density at right upper lung field with well-demarcated margin and elevation of right side diaphragm (Figure 1. (B)). T-1 weighted image. The main mass was located in right upper hemithorax and was associated with multiple adjacent lymphadenopathy. The lesion compressed the trachea, esophagus, right carotid and subclavian artery, and encased the brachial plexus (Figure 1. (C)). Densely cellular fascicles alternated with hypocellular zones where the parallel orientation of the cell was lacking (Figure 1. (D)). The nuclei were wavy, buckled, irregular-shaped or occasionally comma-shaped, and the cytoplasm was lightly stained and usually indistinct (Figure 1. (E)). Immunohistochemical stain for s-100 protein showed positive reaction on the cytoplasm of tumor cells (Figure 1.(F)).