PMC5476998_01
First patient. MRI brain at time of diagnosis, 1.5 T. Images are T1-weighted and Gd-enhanced. (A) Axial: heterogeneous extra-axial enhancement is seen in the left cerebello-pontine angle, with some local compression. (B) Coronal: enhancement is seen along the tentorium cerebelli on the right, which is characteristic of leptomeningeal spread. The left cerebellar lesion appears intraparenchymal.
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F1
Her MRIs at this time are shown in Figures 1 and 2.
First patient. MRIs of spine, 1.5 T, sagittal. (A) Gd-enhanced: diffuse, nodular enhancement is seen throughout. This is striking dorsally and at the conus medullaris. (B) T2-weighted: the thoracic cord is swollen with intra-medullary edema. This appears to be a reaction to the surrounding leptomeningeal tumor; there may also be an element of cord compression in the thoracic cord. (C) Gd-enhanced: disease in the upper thoracic cord is much improved following radiotherapy. Progression has occurred around the untreated cord caudally. (D) T2-weighted: similarly, intra-medullary edema has improved in the treated areas but worsened elsewhere.
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F2
Her MRIs at this time are shown in Figures 1 and 2. Her MRI confirmed progression (Figure 2).
PMC5476998_02
Second patient. MRIs of brain. Upper row (A-C): T1-weighted, Gd-enhanced (magnetization-prepared rapid gradient-echo (MPRAGE)). A suprasellar and interpeduncular lesion is seen with mixed solid and cystic (lobulated) elements. There is heterogeneous enhancement of intermediate intensity. Lower row (A,B): T1-weighted, Gd-enhanced. There is no evidence of tumor recurrence. Smooth linear enhancement is present at the resection site, which is consistent with the long-term changes seen following surgery.
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F3
His MRI at this time, showing a suprasellar mass, is shown in Figure 3.
Second patient. Immunohistochemistry. (A) Hematoxylin and eosin (H&E) stain, 200x magnification. The tumor comprises two main elements: sheets of large cells, many with a rhabdoid appearance (top) and small, more primitive-appearing cells (bottom). (B) (H&E) stain, 400x. This shows large cells, many with rhabdoid features and central mitoses. (C) INI1 stain, 400x. There is no nuclear staining in the tumor cells. A positive control is provided by the endothelial cells in the vessel at the center, and in other scattered glial and inflammatory cells.
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F4
The pathology is shown in Figure 4.