PMC10242979_01
Brain magnetic resonance imaging on the first admission. Brain magnetic resonance imaging revealed a space-occupying lesion in the sellar region with right parasellar and suprasellar extension. The lesion (indicated by asterisks and outlined by dashed lines) appeared isointense on T1-weighted imaging [(A), sagittal; (B) coronal] and heterogenous signals on T2-weighted imaging [(C), sagittal; (D) coronal]. Contrast-enhanced T1-weighted images demonstrated a remarkable homogeneous enhancement [(E) sagittal; (F), coronal].
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Brain magnetic resonance imaging (MRI) was requested, yielding a space-occupying lesion in the sellar region with right parasellar and suprasellar extension ( Figure 1 ).
Brain magnetic resonance imaging after a 7-year conservative observation. Brain magnetic resonance imaging showed the lesion (indicated by asterisks and outlined by dashed lines) significantly progressed [(A, C, E) sagittal; (B, D, F) coronal]. On cranial computed tomography, the mass was hyperdense (G). Computed tomographic angiography identified no significant vascular aberrance (H).
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The latest brain MRI after a 7-year conservative observation demonstrated the lesion significantly progressed ( Figure 2 ), and the patient was readmitted. Cranial computed tomography (CT) and MRI demonstrated a giant lobulated sellar mass with parasellar and suprasellar extension, and the optic chiasm was remarkably displaced; the lesion was isointense on T1-weighted imaging and heterogeneously hyperintense on T2-weighted imaging, with remarkable enhancement after administration of contrast medium ( Figures 2A-G ). CT angiography did not identify any significant vascular aberrance ( Figure 2H ).
Ophthalmologic examinations. Visual field examination showed bilateral tubular contraction of visual fields [(A) OS; (B) OD]. Fundus examination demonstrated posterior staphyloma and tigroid fundus [(C), OS; (D) OD].
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Ophthalmologic examination showed bilateral visual field defects and pathologic myopia with posterior staphyloma and tigroid fundus ( Figure 3 ).
Histopathological and immunohistochemical examinations. (A) Hematoxylin-eosin staining showed a lobulated pattern and cellular nests surrounded by vascular fibrous septa, which were consistent with the morphological characteristics of paraganglioma (Zellballen pattern) (Original magnification x200). Immunohistochemical stains showed the tumor cells were negative for glial fibrillary acidic protein (B) and S100 protein (C), but strongly positive for synaptophysin (D), microtubule-associated protein 2 (E), and chromogranin A (F). Additionally, the tumor showed no immunoreactivity against epithelial membrane antigen (G) or cytokeratin (H). The Ki-67 proliferation index was <5% (I).
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Histopathological sections revealed a tumor with a lobulated pattern and cellular nests surrounded by vascular fibrous septa ( Figure 4 ).
Postoperative and follow-up computed tomography examinations. (A) Postoperative computed tomography confirmed the tumor was subtotally removed, and the tumor cavity was filled with hemostatic material (arrowhead). (B) Repeated computed tomography showed ventricle dilation with interstitial edema. (C) Computed tomography showed a ventriculoperitoneal shunting was performed. (D) A week postoperatively, computed tomography showed the ventricles shrank. (E, F) Eight months postoperatively, cranial CT showed no recurrence of the tumor residue and the hemostatic material had been absorbed (E), and the hydrocephalus had been relieved (F).
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Postoperative CT confirmed the tumor was subtotally removed, and the tumor cavity was filled with hemostatic material ( Figure 5A ). Repeated cranial CT showed ventricle dilation with interstitial edema ( Figure 5B ). A secondary hydrocephalus was diagnosed and a ventriculoperitoneal shunting was performed ( Figure 5C ). A week postoperatively, the neurological deficiencies were partially improved, and CT showed the ventricles shrank ( Figure 5D ). Eight months postoperatively, cranial CT showed no recurrence of the residual tumor, and the hydrocephalus had been relieved ( Figures 5E, F ).