Superficial Siderosis of the Central Nervous System Caused by Hemorrhagic Intraventricular Craniopharyngioma: Case Report and Literature Review

Abstract

Superficial siderosis is a rare condition caused by hemosiderin deposits in the central nervous system (CNS) due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). CNS tumor could be one of the sources of bleeding, both pre- and postoperatively. We report an extremely rare case of superficial siderosis associated with purely third ventricle craniopharyngioma, and review previously reported cases of superficial siderosis associated with CNS tumor. A 69-year-old man presented with headache, unsteady gait, blurred vision, and progressive hearing loss. Brain magnetic resonance (MR) imaging with gadolinium revealed a well enhanced, intraventricular mass in the anterior part of the third ventricle. T2*-weighted gradient echo (GE) MR imaging revealed a hypointense rim around the brain particularly marked within the depth of the sulci. Superficial siderosis was diagnosed based on these findings. The tumor was diffusely hypointense on T2*-weighted GE imaging, indicating intratumoral hemorrhage. The lateral ventricles were dilated, suggesting hydrocephalus. [18F]fluorodeoxyglucose positron emission tomography revealed increased uptake in the tumor. The whole brain surface appeared dark ocher at surgery. Histological examination showed the hemorrhagic tumor was papillary craniopharyngioma. His hearing loss progressed after removal of the tumor. T2*-weighted GE MR imaging demonstrated not only superficial siderosis but also diffuse intratumoral hemorrhage in the tumor. Superficial siderosis and its related symptoms, including hearing loss, should be considered in patients with hemorrhagic tumor related to the CSF space. Purely third ventricle craniopharyngioma rarely has hemorrhagic character, which could cause superficial siderosis and progressive hearing loss.

Continuous Minor Bleeding from Tumor Surface in Patients with Craniopharyngiomas: Case Series of Nonobstructive Hydrocephalus

“…Generally, superficial siderosis results from idiopathic bleeding from a vascular abnormality, trauma, brain tumor, or surgery of the central nervous system. 11,[18][19][20][21] Several cases associated with hydrocephalus were also reported, and in such patients, including ours, the impairment of transparenchymal CSF absorption might be associated with nonobstructive hydrocephalus development. 18,22,23 Despite the accumulation of case reports, the relationship between hemorrhage into the CSF and nonobstructive hydrocephalus development has not been well investigated.…”

“…We conducted a review of the literature and found several reports of craniopharyngioma associated with hydrocephalus or hemorrhage (►Table 2). 1,5,[8][9][10][11][12] Obstructive hydrocephalus was identified at diagnosis in 49 (45.4%) of 108 patients with craniopharyngioma, but none had nonobstructive hydrocephalus. 12 In our series, obstructive hydrocephalus was identified in 22 patients at diagnosis, and 8 of them (36.4%) required a VP shunt even after tumor removal.…”

Craniopharyngioma with Intratumoral Hemorrhage and Superficial Siderosis

“…Craniopharyngioma is a rare brain tumor and intratumoral hemorrhage is one of the uncommon manifestations of craniopharyngioma (4). Also, superficial siderosis associated with craniopharyngioma is rarely reported in previous studies (3). We report a rare case superficial siderosis associated with craniopharyngioma with intratumoral hemorrhage.…”

“…The mainstay of treatment for superficial siderosis is identification and ablation of the bleeding focus for prevention of progression of symptoms (6). Once symptoms occur, it is difficult to reverse CNS damage caused by hemosiderin deposition (3). Therefore, early diagnosis and timely treatment are important for the better outcome.…”

“…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).…”