Rare association of secondary superficial siderosis caused by a fourth ventricle hemorrhagic ependymoma mimicking a cavernoma: Case report and literature review

Abstract

Background:The association of a hemorrhagic tumor with secondary superficial siderosis (SS) is a relatively rare although well described phenomenon.Case Description:We present the case report of a 35-year-old male with a history of drowsiness, hypoacusia, drop attacks, and multidirectional nystagmus during the last 2 months, who presented with acute obstructive hydrocephalus caused by a fourth ventricle mass displaying radiological signs of repeated intra and extratumoral hemorrhage with SS. He underwent gross surgical removal of the solid component of the tumor. Microscopic examination revealed an ependymoma with atypical features, including prominent angiomatous formations and internal chronic hemorrhages with hemosiderin deposits, resembling a cavernoma. The scarce tumoral component, which extended around these cavernous vessels, lacked the gross typical features of fibrillary stroma or perivascular pseudorosettes.Conclusion:To our knowledge, including the present case, there are 45 published reports of tumors associating secondary SS. Besides ependymoma, no other hemorrhagic lesion, tumoral or vascular, has been previously published associating a fourth ventricle location with secondary SS. The present case represents the fifth with this finding, and we strongly suggest ependymoma as a presumptive diagnosis when this rare association is encountered. In addition, this appears to be the first case reported in the scientific literature of a hemorrhagic fourth ventricle ependymoma mimicking both, radiologically and histologically, a cavernous malformation.

Classical infratentorial superficial siderosis of the central nervous system: pathophysiology, clinical features and management

“…Vascular tumours—particularly those related to the ependymal surface—can also generate chronic subarachnoid haemorrhage and thus classical iSS (table 2). 10 Classical iSS typically does not result from ‘macrovascular’ lesions that cause symptomatic arterial bleeding, for example, aneurysms, arteriovenous malformations, cavernomas or dural fistulae. Thus, cerebral or spinal angiography—previously traditionally widely used to investigate iSS—is not usually helpful and is not indicated in the routine investigation of iSS.…”

“…Given the widespread distribution of iSS in both infratentorial and supratentorial regions (see below), it is unsurprising that other neurological symptoms such as epilepsy (focal-onset seizures), dysphagia may occur in iSS 1 4 6 10. Severe persistent back pain may be a feature of arachnoiditis associated with iSS 4…”

“…Vascular tumours -particularly those related to the ependymal surface -can also generate chronic SAH and thus classical siderosis (Table 2) 10 . Classical iSS typically does not result from "macrovascular" lesions that cause symptomatic arterial bleeding, for example aneurysms, arteriovenous malformations, cavernomas, or dural fistulae.…”

Erratum: Rare association of secondary superficial siderosis caused by a fourth ventricle hemorrhagic ependymoma mimicking a cavernoma: Case report and literature review

“…8 of Surgical Neurology International,[1] the name of the second author is written incorrectly in the “How to cite this article” section as “Moro RC” instead of “Carrasco-Moro R”. The “How to cite this article” section should be read correctly as follows: Espinosa Rodríguez EE, Carrasco-Moro R, Martínez San Millán JS, Pian Arias HG.…”

“…In the article titled, “Rare association of secondary superficial siderosis caused by a fourth ventricle hemorrhagic ependymoma mimicking a cavernoma: Case report and literature review”, published as elocation ID 14, in vol. 8 of Surgical Neurology International,[ 1 ] the name of the second author is written incorrectly in the “How to cite this article” section as “Moro RC” instead of “Carrasco-Moro R”. The “How to cite this article” section should be read correctly as follows: Espinosa Rodríguez EE, Carrasco-Moro R, Martínez San Millán JS, Pian Arias HG.…”