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Home , Craniopharyngioma, Siderosis, Superficial siderosis

pISSN 2384-1095 iMRI 2018;22:249-253 https://doi.org/10.13104/imri.2018.22.4.249 eISSN 2384-1109

Craniopharyngioma with Intratumoral Hemorrhage and Superficial

Jeong Hee Oh, Sung-Tae Park, Hyun Kyung Lim Department of , Seoul Hospital, Soonchunhyang University College of , Seoul, Korea

Superficial siderosis of the (CNS) is a progressive and debilitating neurological disease manifesting sensorineural hearing loss, cerebellar , and pyramidal tract signs. Chronic extravasation of blood into the subarachnoid space results in the accumulation of derivate in the subpial layer of the CNS, which is toxic to the neural tissues. Craniopharyngioma is a benign Case Report third ventricle tumor, which rarely presents with tumor bleeding. We report a rare case of associated with craniopharyngioma with intratumoral hemorrhage in a patient with no history of prior trauma or CNS .

Received: September 12, 2018 Keywords: Superficial siderosis; Craniopharyngioma; Chronic ; Revised: September 19, 2018 Accepted: October 8, 2018 Magnetic resonance imaging

Correspondence to: Sung-Tae Park, M.D. Department of Radiology, INTRODUCTION Soonchunhyang University Hospital, 59 Daesagwan-ro, Yongsan-gu, Seoul 04401, Korea. Superficial siderosis of the central nervous system (CNS) is a rare condition caused by Tel. +82-2-709-9396 deposition of in the brain and . The typical clinical manifestation Fax. +82-2-709-9066 of superficial siderosis includes sensorineural hearing loss, gait ataxia and pyramidal E-mail: [email protected] signs (1). The chronic existence of bleeding source in the subarachnoid space results in the deposition of hemosiderin in the subpial layer of the CNS (2). The source of bleeding is variable, including vascular malformations, trauma, CNS tumors, and CNS surgery (1, 3). Craniopharyngioma is a rare and intratumoral hemorrhage is one of the uncommon manifestations of craniopharyngioma (4). Also, superficial siderosis

This is an Open Access article distributed associated with craniopharyngioma is rarely reported in previous studies (3). We report under the terms of the Creative Commons a rare case superficial siderosis associated with craniopharyngioma with intratumoral Attribution Non-Commercial License (http://creativecommons.org/licenses/ hemorrhage. by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. CASE REPORT

A 50-year-old man presented to the emergency department with dizziness and . He had intermittent dizziness and headache for the past three years, and the Copyright © 2018 Korean Society symptoms aggravated two weeks back. The patient had medications for hypertension of Magnetic Resonance in and panic disorder. He had no history of the traumatic event. Neurologic examination Medicine (KSMRM) revealed no significant neurologic deficit. Computed tomography (CT) revealed the presence of about 2.3 cm sized isodense lesion with an internal hyperdense component in suprasellar cistern, and the lesion showed heterogenous contrast enhancement on www.i-mri.org 249 Craniopharyngioma Presenting Hemorrhage and Superficial Siderosis | Jeong Hee Oh, et al. following contrast-enhanced brain CT (Fig. 1). Brain MRI operation field, the tumor was hard and gray in color and revealed the location of the mass in the anterior aspect located in the third ventricle. Arachnoid membrane showed of the third ventricle involving the and dark brown color. The pathologic diagnosis of the tumor medial aspect of bilateral thalami. There was no evidence was papillary type craniopharyngioma. Also, hemosiderin of . The mass showed heterogeneous pigment was reported on a pathologic report of . high signal intensity (SI) on T2-weighted images (T2WI) (CSF) study revealed no evidence of the with strong and heterogeneous contrast enhancement. presence of malignant cells. After 14 days of the surgery, Peritumoral T2 high SI involved optic chiasm but spared the patient showed a decrease in mentality and CT revealed pituitary fossa. Focal T2WI dark SI lesions with blooming diffuse brain swelling with subfalcine brain herniation. After artifact on gradient-echo T2*-weighted images (GRE two times of decompressive craniectomy, brain swelling and T2*WI) were noted in the tumor, suggesting intratumoral brain herniation improved gradually. However, during the hemorrhage. The preoperative diagnosis was hypothalamic- postoperative care period, the patient developed 38 degrees chiasmatic multiforme. The result of single- of fever with an elevation of C-reactive protein. After voxel spectroscopy supported this diagnosis, as prominent application of vancomycin and cefepime for coverage of lactate peak presented, although there was noise in hospital-acquired and operation site infection basal spectroscopy due to intratumoral hemorrhage. for 15 days, a rapid decrease in glomerular filtration rate However, the tumor did not show diffusion restriction was observed. Therefore, the patient underwent continuous or extensive peritumoral T2WI high SI, and the findings renal replacement for acute kidney injury. Also, the were not consistent with the findings of glioblastoma patient underwent follow-up CT for evaluation of decreased multiforme. On T2WI and GRE T2*WI, diffuse linear low SI mental status, which revealed the marked progression was noted along bilateral Sylvian fissures, frontotemporal of brain swelling. On the subsequent day (42nd day of and cerebellar sulci, suggesting superficial admission), the patient expired as the blood pressure was (Fig. 2). A small amount of intraventricular hemorrhage not maintained even after application of vasopressin. was noted in both the lateral ventricles (Fig. 2). The third ventricle tumor was considered as the bleeding focus, as there was no other evidence of focus of intracranial DISCUSSION hemorrhage. No significant diffusion restriction was noted in the tumor. The endocrinologic blood test revealed Recurrent and prolonged hemorrhage in subarachnoid subclinical , but no other significant finding space leads to superficial siderosis of CNS. CNS tissues was reported. Serum electrolyte levels were within the convert heme to free , ferritin, and hemosiderin in CSF normal range. The patient underwent tumor resection with and accumulation of these hemoglobin derivates in the pterional-transsylvian and subfrontal approach. On the leptomeninges results in destruction and demyelination

Fig. 1. (a) Coronal non-enhanced computed tomography (CT) scan shows about 2.3 × 1.8 × 2.0 cm sized isodense suprasellar mass with the focal internal high attenuated area. (b) Contrast- enhanced CT scan demonstrates heterogenous contrast enhancement of the lesion. a b

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a b c

Fig. 2. (a) On coronal T2-weighted image (T2WI), the mass is in the third ventricle, without obstruction of foramen of Monro. No evidence of hydrocephalus is seen. The mass shows high signal intensity (SI) on T2WI and (b) strong, heterogeneous contrast enhancement. (c) On sagittal contrast- enhanced T1-weighted image, the mass is in the anterior aspect of the third ventricle, without involvement of pituitary fossa. (a) Marked T2 low SI foci is noted within the mass, which d e can be correlated with high density lesion in non-enhanced CT scan, and this finding suggests acute hemorrhage in the mass. (d, e) On axial gradient-echo T2*- weighted images, diffuse linear low SI is noted along bilateral Sylvian fissures and cerebellar sulci, suggesting superficial hemosiderosis. Small amount of intraventricular hemorrhage is noted in bilateral lateral ventricles. of CNS tissues (2). Patients usually manifest sensorineural additional information, such as the presence of a bony hearing loss, gait ataxia, , and myelopathy, but defect associated with trauma a or prior surgery and dural the number of presymptomatic cases has increased with defect associated with pseudomeningocele (6). Cerebral the widespread use of MRI (5, 6). The source of chronic angiography and spinal angiogram are rarely beneficial bleeding is quite variable. Head or back trauma and prior for the detection of an occult bleeding focus, which might intradural surgery are well-known risk factors (6). Other be due to the slow flow and intermittent character of the etiologies include CNS tumor, nerve root injury, vascular bleeding source (1). malformation and amyloid angiopathy (5, 6). MRI is the For differential diagnosis of anterior third ventricle best imaging modality for the diagnosis of superficial masses in adults, hypothalamic-chiasmatic masses should siderosis and detection of bleeding focus (1, 6). T2WI low SI be considered, including craniopharyngioma, hypothalamic- along with the brain sulcus, spinal cord, and cranial nerves chiasmatic , chordoid glioma, lymphoma, and is the pathognomonic finding of superficial siderosis. GRE metastases (7). Papillary type craniopharyngioma is an T2*WI exhibits a higher sensitivity for the detection of uncommon subtype of craniopharyngioma, which usually hemosiderin deposition, because of magnetic susceptibility occurs in middle-aged adults. Most of the lesions are effects of blood-degradation products (1). Often, superficial suprasellar, spherical and predominantly solid mass and siderosis is accompanied by cerebellar atrophy and rarely show calcification (8). Spontaneous tumor bleeding spinal cord atrophy. CT and CT myelography may provide is a very uncommon finding of craniopharyngioma (9). www.i-mri.org 251 Craniopharyngioma Presenting Hemorrhage and Superficial Siderosis | Jeong Hee Oh, et al.

Hypothalamic-chiasmatic glioma also appears as third of symptoms (6). In cases of patients with non-specific ventricle tumor, which shows a more aggressive clinical symptoms and underlying disease such as brain tumor, course in adults (8). Larger tumors tend to be heterogeneous like our case, it is important to inform the clinicians with both cystic and solid components (7). Chordoid that existence of superficial siderosis might have caused glioma is another anterior third ventricle the symptom, as the symptom may persist even after containing both glial and chordoid histologic component. treatment of the underlying disease. In our patient, he Chordoid glioma appears as a well-defined, T1 iso SI, ovoid had an intermittent headache and dizziness without suprasellar mass with intense contrast enhancement, which neurologic deficit, and the symptoms were not specific for displaces infundibulum posteriorly (7). It is often difficult to superficial siderosis. As the patient expired after the tumor differentiate between these disease entities. Therefore, the removal surgery, we could not evaluate the response of the final diagnosis usually depends on the pathologic diagnosis. symptoms after the surgery. In conclusion, we report an In our case, diffuse hemosiderin deposition along bilateral extremely rare case of superficial siderosis with hemorrhagic Sylvian fissures and were compatible with typical craniopharyngioma in a 50-year-old man. As the patient findings of superficial siderosis. The patient did not have had no history of head trauma or operation history on a history of trauma and intradural surgery and no definite brain and spine, craniopharyngioma with intratumoral evidences of occult head trauma were noted on brain CT hemorrhage in the third ventricle was considered as and MRI. There was a solid mass in the third ventricle with the bleeding focus, which is a rare manifestation for acute hemorrhage on CT. Therefore, the tumor bleeding was craniopharyngioma. Our case report is worthwhile to review a highly suspected bleeding focus of superficial siderosis. similar cases of hemorrhagic craniopharyngiomas and Our case report is the second reported case of superficial differential diagnoses for the third ventricle tumors. Also, siderosis due to preoperative intratumoral hemorrhage of our case demonstrated superficial siderosis of the CNS as craniopharyngioma detected in a living patient worldwide. a rare complication of hemorrhagic craniopharyngioma. The first case was reported in Japan by Tosaka et al. (3) In Although superficial siderosis of the CNS is a very rare both the cases, patients were healthy males in their 6th-7th condition, it is associated with pathognomonic findings on decades. The pathologic type of craniopharyngiomas was brain MRI. Detection of the bleeding source is critical for papillary type in both the patients, and this result is similar treatment of superficial siderosis of the CNS. Therefore, to previously reported craniopharyngiomas with hemorrhage radiologists should be aware of this rare condition and (9). Although the pathogenesis of intratumoral hemorrhage be able to suggest appropriate imaging modality for the in craniopharyngioma is unclear, several hypotheses were evaluation of patients with superficial siderosis of the CNS. suggested, such as degenerative changes and rupture of blood vessels walls in the craniopharyngioma and connective tissue, and the presence of numerous immature Conflict of Interest blood vessels (10). In the present case, it was not possible The authors declare that there are no conflicts of interest to detect pathologic findings associated with intratumoral regarding the publication of this paper. hemorrhage due to the limitation of the specimen. In There are no financial or other relations that could lead to contrary to the patient in the first case report, our patient a conflict of interest. did not show hydrocephalus on images. Hydrocephalus is one of the complications of superficial siderosis, which is probably due to adhesion in subarachnoid space (5). REFERENCES The mainstay of treatment for superficial siderosis is identification and ablation of the bleeding focus for 1. Kumar N. Neuroimaging in superficial siderosis: an in- prevention of progression of symptoms (6). Once symptoms depth look. AJNR Am J Neuroradiol 2010;31:5-14 occur, it is difficult to reverse CNS damage caused by 2. Koeppen AH, Michael SC, Li D, et al. The of hemosiderin deposition (3). Therefore, early diagnosis and superficial siderosis of the central nervous system. Acta timely treatment are important for the better outcome. Neuropathol 2008;116:371-382 Until now, several cases reported that chelation , 3. Tosaka M, Sato K, Amanuma M, et al. 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