PMC3943380_01
Representative axial MR images with gadolinium, taken on initial admission. (A) T1-weighted MR image (T1WI); (B) Contrast-enhanced T1WI and (C) T2-weighted MR image (T2WI) showing a left occipital tumor with mild contrast enhancing; (D) at one month follow up, T1WI; (E) contrast-enhanced T1WI and (F) T2WI showing no apparent enhanced lesion; (G) 8 months later after the first surgery, T1WI; (H) contrast-enhanced T1WI and (I) T2WI showing marked enhanced mass on the cavity and recurrence; (J) 48 h after the second surgery, T1WI; (K) contrast-enhanced T1WI, and (L) T2WI showing no apparent enhanced lesion; (M) 8 months later after combination radiotherapy and chemotherapy, T1WI; (N) contrast-enhanced T1WI and (O) T2WI showing marked enhanced mass of the wall in the posterior portion of the removal cavity and left temporal areas, and recurrence of the enhanced tumor. (P, Q) 8 months later after combination radiotherapy and chemotherapy, sagittal spinal contrast-enhanced T1WI after gadolinium infusion showing high-intensity mass lesion in (P) T7, T10, and T12 vertebral bodies (arrowheads) and (Q) T12, L2, L3, L5, and S1 vertebral bodies (arrows).
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F1
Magnetic resonance imaging (MRI) showed a left solid occipital tumor with mild contrast enhancing (Figure 1A,B,C). On 15 September 2011 he underwent a left solid occipital craniotomy with gross total resection confirmed by subsequent MRI scans (Figure 1D,E,F). A repeated left occipital tumor resection was performed 8 months later (Figure 1G-L). Regretfully, brain MRI showed evident progression of the intracranial lesion (Figure 1M-O). MR images, and low-intensity signals in the temporal area. The new lesion was thought to be a recurrent tumor with malignant transformation (Figure 1M-O). MR images of the spine showed diffuse patchy areas of increased signal intensity and abnormal enhancement of the T7, T10, T12, L2, L3, L5, and S1 vertebral bodies (Figure 1P,Q).
Representative photomicrographs of the tumor specimens. Higher cell densities (A, B; 100x, 200x, respectively, H & E) with perinuclear haloes (C; 200x, H & E; arrows), and microscopically round-to-oblong cells with hyperchromatism and pleomorphism (D; 400x, H & E), are compatible with AO. (E, F) Clusters of capillary or plexiform capillaries (arrows), and the irregular mitosis densities were higher (100x, 200x, respectively, H & E). (G, H) Obvious false fence structure (thick arrows)-shaped necrosis (slim arrows; 100x, 200x, respectively, H & E). (I, J) After the right iliac bone marrow needle biopsy, cells in the bone marrow specimen from the patient were small and round with a thin rim of eosinophilic cytoplasm (400x, H & E).
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F2
Photomicrographs of the resected tumor showed that there were higher cell densities, densely packed round cells with perinuclear haloes, microscopically round-to-oblong cells with hyperchromatism and pleomorphism (Figure 2A-D), clusters of capillary or plexiform capillaries (Figure 2E,F), and obvious false fence structure-shaped necrosis (Figure 2G,H). An open biopsy of the infiltrated right iliac bone revealed the replacement of the patient's marrow by malignant cells, which exhibited nuclear pleomorphism (Figure 2I,J).
Representative emission computed tomography scans. (A) anterior scans, and (B) posterior scans, showed a hypermetabolically abnormal uptake at the right iliac bone (arrowheads) and T10, and T12 vertebral bodies (arrows).
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F3
A whole body 99mTc-methylene diphosphonate bone scan showed hyper-activity in the right iliac bone and the tenth and twelfth thoracic vertebral bodies (Figure 3).
Representative whole body PET-CT scans. (A) and (B) Multiple foci of increased 18 F-fluoro-2-deoxyglucose (FDG) uptake at the bilateral iliac bones, C4, T7, T11, T10, T12, L2, L3, S1 vertebral bodies and the right acetabulum; (C) Focus of increased 18 F-FDG uptake at the lymph node near the left side of the T11 vertebral body (arrows); (D) Focus of increased 18 F-FDG uptake at the lymph node of the right supraclavicular region (arrows).
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F4
PET-CT scans also showed multifocal invasion of the bilateral iliac bones, the right acetabulum, the right femoral neck and the C4, T7, T10, T11, T12, L2, L3, and S1 vertebral bodies (Figure 4A,B), the lymph nodes at the left side of the eleventh thoracic vertebral body (Figure 4C) and the right supraclavicular region (Figure 4D).
Representative scans of bone marrow smear. (A, B) No plasmocytoma cells were found (100x and 1000x, respectively).
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F5
A bone marrow smear of the right iliac bone showed no plasmacytoma cells (Figure 5).
Representative immunochemical markers in tumor specimens. (A, B) Positive reaction for IDH1 (100x, 200x, respectively). (C) Positive reaction for Ki-67, with proliferating index >80% (200x). (D, E) Tumor cells were positive for GFAP and Oligo-2, respectively (200x). (F, G, H) showed the tumor cells were negative for EMA, MGMT, and vimentin, respectively (200x).
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F6
They were all strongly positive for isocitrate dehydrogenase-1 (IDH1; Figure 6A,B) and Ki-67, with proliferation index >80% (Figure 6C). They were also all positive for the glial fibrillary acidic protein (GFAP) marker (Figure 6D), which is positive in glial, Schwannian, and ependymal tumors (all neural tumors), and for the marker oligodendrocyte transcription factor (Oligo-2; Figure 6E). Further findings were all negative for other pertinent immunohistochemical stains: epithelial membrane antigen (EMA), O6- methylguanine-DNA methyltransferase (MGMT) and vimentin (Figure 6F,G,H, respectively).