TY - JOUR
T1 - The clinico-surgico-pathological spectrum of myxopapillary ependymomas - Report of four unusal cases and review of the literature
AU - Schittenhelm, J.
AU - Becker, R.
AU - Capper, D.
AU - Meyermann, R.
AU - Iglesias-Rozas, J. R.
AU - Kaminsky, J.
AU - Mittelbronn, M.
N1 - Funding Information:
Our research was supported by the Telethon Foundation , Italy (grant GGP19007 ), ERC Advanced grant FP7-3222424 , and a grant from the NRJ-Institut de France (to M.Z.); Associazione Luigi Comini ONLUS ; and a core grant from the Medical Research Council , UK (grant MC_UU_00015/5 ). We are grateful to the MRC-Laboratory of Molecular Biology Electron Microscopy Facility for access to their resources. We thank Ana B. Cortés at UPO for support in CoQ measurements and Dr. Erika Fernandez-Vizarra, Dr. Aurora Gomez-Duran, and Pedro Pinheiro for their help and advice. We are grateful to Dr. Silvia Armelloni, the Renal Research Laboratory, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy for sharing the protocol to isolate podocytes.
PY - 2008
Y1 - 2008
N2 - According to the WHO grading system, myxopapillary ependymomas are assigned to WHO Grade I. However, the clinico-pathological spectrum might be very heterogenous. Herein, we report 4 cases exhibiting lumbar tumor masses, 1 causing muscular atrophy over a 30-year period, 3 displaying clinical history of persisting lumbar pain for only several weeks. All tumors were crooked with dura and spinal roots resulting in incomplete resection in three cases. On histological examination, two tumors were almost acellular and showed polycyclic hyaline and fibrotic extracellular matrix leading to differential diagnoses of chordoma, meningioma, fibrolipoma and ependymoma. Finally, together with the immunohistochemical investigations, electron microscopy led to the diagnosis of myxopapillary ependymoma, WHO Grade I, with massive degenerative changes. The other 2 cases presented with the typical neuropathology of myxopapillary ependymomas but showed local recurrence within 1 and 13 years throughout the whole neuraxis, and in 1 case additional metastases of the 3rd ventricle. Although the morphological feature of these myxopapillary ependymomas was benign, the presented cases showed that the biological behavior of myxopapillary tumors might differ greatly and that these tumors present a serious operative and diagnostic challenge. Myxopapillary ependymomas occur most often in the lumbosacral region. Due to the anatomic complexity of the cauda equina, a complete resection can be technically challenging in this region. However, a gross total resection at the primary surgery is the most predictive factor for the outcome.
AB - According to the WHO grading system, myxopapillary ependymomas are assigned to WHO Grade I. However, the clinico-pathological spectrum might be very heterogenous. Herein, we report 4 cases exhibiting lumbar tumor masses, 1 causing muscular atrophy over a 30-year period, 3 displaying clinical history of persisting lumbar pain for only several weeks. All tumors were crooked with dura and spinal roots resulting in incomplete resection in three cases. On histological examination, two tumors were almost acellular and showed polycyclic hyaline and fibrotic extracellular matrix leading to differential diagnoses of chordoma, meningioma, fibrolipoma and ependymoma. Finally, together with the immunohistochemical investigations, electron microscopy led to the diagnosis of myxopapillary ependymoma, WHO Grade I, with massive degenerative changes. The other 2 cases presented with the typical neuropathology of myxopapillary ependymomas but showed local recurrence within 1 and 13 years throughout the whole neuraxis, and in 1 case additional metastases of the 3rd ventricle. Although the morphological feature of these myxopapillary ependymomas was benign, the presented cases showed that the biological behavior of myxopapillary tumors might differ greatly and that these tumors present a serious operative and diagnostic challenge. Myxopapillary ependymomas occur most often in the lumbosacral region. Due to the anatomic complexity of the cauda equina, a complete resection can be technically challenging in this region. However, a gross total resection at the primary surgery is the most predictive factor for the outcome.
KW - Cauda equina
KW - Lumbar tumor
KW - Myxopapillary ependymoma
UR - http://www.scopus.com/inward/record.url?scp=38549180617&partnerID=8YFLogxK
U2 - 10.5414/NPP27021
DO - 10.5414/NPP27021
M3 - Article
C2 - 18257471
AN - SCOPUS:38549180617
SN - 0722-5091
VL - 27
SP - 21
EP - 28
JO - Clinical Neuropathology
JF - Clinical Neuropathology
IS - 1
ER -