PMC4199185_01
Clinical and pathological images of Case 1. (a): MRI and gadolinium-enhanced T1-weighted MRI at the initial examination. A highly enhanced mural nodule type tumor was shown in the right cerebellar hemisphere. (b): Cerebral angiography showed obvious tumor stain and early venous drainage. (c): Tumor consisted of capillary proliferation and proliferation of interstitial foamy stromal cells.(H and E, x200) (d): Nodular recurrence findings were shown by contrast-enhanced MRI at 38 months after the initial surgery. (e): The tumor markedly shrank 2 years after stereotactic radiosurgery at 20 Gy. (f): Development of three spinal cord tumors 91 months after the initial surgery. A large lesion at Th12 accompanied by flow void was resected. (g): Irradiation to the whole brain and whole spine was performed, but the tumor was disseminated to the subarachnoid space surrounding the brain stem. Respiratory failure developed due to compression of the medulla oblongata. (h): The entire spinal cavity was filled with the tumor tissues. (i): Autopsy of the brain showed that nodular tumor tissues sporadically presented around the brain stem. (j): The tumor tissues were sporadically observed around the spinal cord. (k): Spinal subarachnoid space was filled with the tumor tissues (H and E, x40). (l): The tissues after radiosurgery for the initial recurrence. The tissues were highly fibrillated, and no viable tumor cells were observed (H and E, x40)
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F1
Head computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a highly contrasted cystic tumor with a mural nodule in the right cerebellar hemisphere [Figure 1a]. The cerebral angiography showed an intense tumor stain from the anteroinferior cerebellar artery and draining into the petrosal vein [Figure 1b]. Total tumor resection by craniotomy was conducted, and a pathological diagnosis of hemangioblastoma was made [Figure 1c]. Thereafter, recurrence of a nodular tumor developed at the site of the initial tumor 38 months after the initial surgery [Figure 1d]. Figure 1E]. Th7, and L2 [Figure 1f]. Ventriculo-peritoneal shunt (VP shunt) and irradiation at 36 Gy to the whole brain and whole spine were performed, but no improvement was obtained [Figure 1g,h]. There was multicentric nodular dissemination of hemangioblastoma in the cerebrospinal subarachnoid space, and compression of the medulla oblongata was considered to be the cause of death [Figure 1i-k]. The initial tumor treated by SRS became fibrosis foci accompanied by hyalinization with a maximum diameter of 15 mm, and no viable tumor tissues were observed [Figure 1l].
PMC4199185_02
Clinical and pathological images of Case 2. (a): A contrast-enhanced CT scan image at the initial examination. A homogenously enhanced solid type tumor was observed in the left cerebellar hemisphere. (b): Cerebral angiography showed pronounced tumor stain and venous drainage. (c): The tumor consisted of capillary proliferation and proliferation of interstitial foamy stromal cells (H and E, x200). (d): Total resection of tumor was performed at the initial surgery. (e): Gadolinium-enhanced T1-weighted MRI showed enhanced diffused dissemination at the basal cistern and ambient cistern of the brainstem 53 months after the initial surgery. (f): Dissemination image in the ambient cistern of the brainstem and in the suprasellar cistern with a complication of severe hydrocephalus. (g): Neuroendoscopic examination of the third ventricle during ventriculo-peritoneal shunt showed a clear ventricular wall and a yellowish brown colored nodular tumor at the ventricle floor. The presence of an obvious draining vein was confirmed. (h): A small number of large tumor cells with foamy cytoplasm were observed in the cerebrospinal fluid. (Giemsa stain, original magnification x100) (i): VP shunts improved hydrocephalus, but there was a gradual increase in tumor tissues contrasted in the subarachnoid space. (j): The entire subarachnoid space of spinal cord was filled with tumor tissues
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F2
CT showed a solid tumor highly enhanced in the left cerebellar hemisphere [Figure 2a]. Cerebral angiography revealed tumor stain from the postero-inferior cerebellar artery and draining to the cerebellar vein [Figure 2b]. Total resection of the brain tumor by craniotomy was performed, and a pathological diagnosis of hemangioblastoma was made [Figure 2c, d]. Head CT and MRI showed no tumor recurrence at the site of the initial surgery, but diffuse disseminated foci were observed in the subarachnoid space, accompanied by hydrocephalus [Figure 2e, f]. A neuroendoscopy examination showed a yellowish orange colored tumor attached to the third ventricle floor with a definite draining vein [Figure 2g]. The presence of hemangioblastoma cells was confirmed by a cytological examination of the cerebrospinal fluid [Figure 2h]. The disseminated foci gradually proliferated during the 90 months after the initial surgery, and the patient remains bedridden due to quadriplegia [Figure 2i, j].