- Department of Neurosurgery, “12 Octubre” University Hospital, Madrid, Spain
- Department of Neuropathology, “12 Octubre” University Hospital, Madrid, Spain
Department of Neurosurgery, “12 Octubre” University Hospital, Madrid, Spain
DOI:10.4103/2152-7806.93865Copyright: © 2012 Martinez-Perez R. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Martinez-Perez R, Hernandez-Lain A, Paredes I, Munarriz PM, Ana M. Castaño-Leon, Lagares A. Acute neurological deterioration as a result of two synchronous hemorrhagic spinal ependymomas. Surg Neurol Int 14-Mar-2012;3:33
How to cite this URL: Martinez-Perez R, Hernandez-Lain A, Paredes I, Munarriz PM, Ana M. Castaño-Leon, Lagares A. Acute neurological deterioration as a result of two synchronous hemorrhagic spinal ependymomas. Surg Neurol Int 14-Mar-2012;3:33. Available from: http://sni.wpengine.com/surgicalint_articles/acute-neurological-deterioration-as-a-result-of-two-synchronous-hemorrhagic-spinal-ependymomas/
Background:Ependymomas are the most common intramedullary tumors in adults and are the most common in mid-adult years. The presence of synchronous ependymomas in different sites of the spine is not common and it is even more infrequent to find hemorrhage from a spinal ependymoma as a cause of neurological deterioration.
Case Description:A 32-year-old man presented with back pain and progressive paraparesia. Magnetic resonance (MR) showed two intradural extramedullary lesions on spinal canal with signs of acute hemorrhage. The patient underwent emergent surgical decompression and resection. Pathology revealed myxopapillary ependymomas.
Conclusion:To our knowledge, we report the first case of a patient with acute neurological deterioration as a consequence of synchronous bleeding of two spinal ependymomas located at different levels in the spinal cord. This study illustrates the importance of recognizing the rare, but known occurrence of acute neurological deterioration after spontaneous hemorrhage in spinal ependymomas.
Keywords: Deterioration, ependymoma, hemorrhage, spine, tumor bleeding
Spinal cord tumors can be classified as extradural (55%) or intradural (45%), the latter being intramedullary (5%) or extramedullary (40%).[
We report a patient who presented after spontaneous bleeding from two separate spinal ependymomas.
A 32-year-old man presented with a history of lumbar pain without irradiation and numbness on the right leg. He was a healthy patient without any important bleeding risk factor, as anticoagulation, trauma, or heavy lifting. He was admitted to the neurosurgical department because of acute progressive paraparesia and urinary incontinence. Magnetic resonance (MR) showed two lesions on the spinal canal [
Preoperative MR. (a–c) Dorsal lesion. (a) T1-weighted image showing an isointense extraxial mass. (b) T2-weighted image. Isointense mass with a hyperintense ring surrounded by a better well-defined hypointense area, representing different stages of bleeding. (c) T1-weighted image with contrast administration, where the lesion enhances homogenously. (d–f) Lumbar lesion. (d) Sagittal T1-weighted image. Isointense mass viewed between cauda equina roots with a hyperintense caudal part, as a sign of acute hemorrhage. (e) Sagittal and (f) axial T2-weighted images, where the lesion is heterogeneous suggesting different stages of bleeding
Intraoperative photographs: (a) Dorsal and (b) lumbar lesions were both seen as well-defined extramedullary masses after opening the dura mater, with blood products and clots in both levels. There are signs of acute and chronic hemorrhage surrounding the tumor, as can be seen in a macroscopic image after the resection of the caudal lesion (c)
Microscopy (a, HE 4×; b, HE 10×). Both tumors are formed by papillary structures surrounded by a single layer of columnar cells with round nuclei and delicate chromatin. The cores of the papillae have a central blood vessel surrounded by a mucinous/myxoid matrix. There is extensive thickening and hyalinization of vessel walls. Mitotic activity is absent. Well-defined margins are present. (c, HE 10×) Dorsal and (d, HE 2.5×) lumbar lesions. Histological signs of acute and chronic bleeding can be seen within the tumor, with fresh red blood cells (white arrow) and hemosiderin-laden macrophages (black arrows)
Here, we report a case presenting with two synchronous spinal ependymomas, both with acute hemorrhage and resulting in spinal cord syndrome, which regained motor function after emergency decompression. Ependymoma is a histological benign and slow-growing tumor, with a mean duration of symptoms before diagnoses between 28 and 36 months.[
In our opinion, the most probable hypothesis for the presence of two concomitant ependymomas in the thoracic and cauda equina regions resulted from the implant of neoplastic cells from a primary filum terminale ependymoma, following the physiologic ascending spinal CSF flow. The same mechanism could account for rare cases of intracranial metastasis,[
Nevertheless, it is not possible to be sure if multiplicity of these lesions is due to CSF spread or multicentric foci. Genetic analysis of the tumor samples is the only way to know with certainty the mechanism of multiple ependymomas. Vural et al.[
Few theories have been described to explain the specific predisposition to bleeding in these tumors, related to their peculiar anatomical location and histological features. Relative to the rostral lesion, abnormal mobility in the thoracolumbar region plays an important role in the development of hemorrhage in tumors situated in this location.[
The effect of early surgery on final outcome remains unclear. In eight patients, the timing of surgery was reported. Five of eight cases and our present case underwent emergency surgical descompression and resection. Only three of these and the patient reported here showed improvement in their neurological condition. In three of eight cases, the timing of operation was delayed, and all had good motor and blader function recovery. Though series of surgical management of spinal ependymomas has previously been published, early diagnosis and treatment were associated with a more favorable outcome.[
It has been widely held that myxopapillary ependymoma has a better prognosis than other variants. Nevertheless, new series of cases show that prognosis after surgery for some myxopapillary ependymomas seems worse than generally believed.[
This report shows the importance of recognizing spontaneous bleeding of a spinal ependymoma as a possible cause of rapid neurological deterioration and acute cord compression, even in the absence of bleeding risk factors such as trauma or anticoagulation. It is also the first case description of synchronous bleeding from spinal ependymomas of different levels.
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