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Neuropathology 2014; 34, 243–252
doi:10.1111/neup.12087
O rig i na l Ar t i cl e
Supratentorial cortical ependymoma: Case series and review of the literature Zhiguo Liu,1 Jing Li,4 Zhiyan Liu,2 Qian Wang,3 Peter Famer,5 Ashesh Mehta,6 David Chalif,6 Yunyan Wang1 and Jian Yi Li5 1
Department of Neurosurgery, Brain Science Research Institute of Shandong University, Departments of 2Pathology, Radiology, Qilu Hospital of Shandong University, Jinan, Shandong Province, 4Department of Pathology, Fan Yu District Central Hospital, Guangzhou, Guangdong Province, China, 5Department of Pathology and Laboratory Medicine, North Shore-Long Island Jewish Health System, Hofstra North Shore-LIJ School of Medicine, Lake Success and 6Department of Neurosurgery, North Shore University Hospital, Manhasset, New York, USA
3
Supratentorial cortical ependymoma (CE), a rare type of ependymoma, is located in the superficial cortex. We reported 11 patients (six female and five male) with CE. The age of the patients ranged from 2 to 63 years old with a median age of 47 years at the time of diagnosis. On MRI, enhancement was noted in all cases with solid appearance in six cases, and solid and cystic appearance in five cases. The frontal and parietal regions were the most common locations for CE. On histology, two were low-grade (WHO grade II) and nine were WHO grade III anaplastic ependymomas. Some tumors exhibited clear cell, spindle (tanycytic) and giant cell morphologies, as well as the classic ependymoma morphology. Dura-based tumor nodules and even tumor dissemination along the dura can be seen in CEs. Low grade CEs have a higher likelihood to present with seizures, a lower likelihood to cause brain edema, tumor recurrence and lower mortality than anaplastic ependymomas. While difficult, anaplastic CEs may be distinguished from glioblastoma by a clear interface between tumor and adjacent brain tissue, relative uniformity of tumor cell nuclei and immunopositivity for epithelial membrane antigen and/or CD99. As is the case for ependymomas in general, gross total resection is still the treatment of choice for CEs. Key words: anaplastic ependymoma, cortical ependymoma, recurrence, seizures, supratentorial ependymoma.
Correspondence: Jian Yi Li, MD, PhD, Department of Pathology and Laboratory Medicine, North Shore-Long Island Jewish Health System, 6 Ohio Drive, Suite 202, Lake Success, NY 11042, USA. Email:
[email protected] Received 10 July 2013; revised 24 October 2013 and accepted 7 November 2013; published online 20 December 2013.
© 2013 Japanese Society of Neuropathology
INTRODUCTION Ependymomas account for 2–9% of all neuroepithelial tumors and predominantly involve the spinal cord and the fourth ventricle.1 They commonly occur in children and young adults.2 According to the WHO 2007 classification of CNS tumors, ependymomas were graded as Grade II (low grade) and Grade III (anaplastic) ependymomas.1,3 Anaplastic ependymomas are characterized by increased cellularity, cytological atypia, increased mitotic activity, microvascular proliferation and/or pseudopalisading tumor necrosis. Cortical ependymomas (CEs) are designated as supratentorial ependymomas that are primarily located in the cerebral cortex. There are only 34 cases of CEs in the literature.4–24 The majority of publications are case reports. In this study, 11 cases of CEs are reported and characterized. To our knowledge, this is one of the largest case series. Finally, clinical characteristics, locations, neuroimaging features and outcomes for published cases of WHO grade II CE and anaplastic CE in the literature are discussed.
MATERIALS AND METHODS Medical records of 11 patients with cortical ependymomas were reviewed, according to the Institutional Review Board regulation in US and the regulation of the National Research Ethics Committee for Neurology and Neurosurgery in China. The demographic data, presenting symptoms, neuroimaging features, pathological findings, treatment and outcome were documented (Tables 1 and 2). Pathological diagnoses were rendered based on morphologic features and immunohistochemical (IHC) profiles in all cases. The specimens were fixed in formalin and embedded in paraffin, and 4-μm-thick
63/F
54/F
7
11
47/F
6
20/M
2/M
5
10
50/F
4
15/F
50/M
3
9
30/M
2
52/F
24/M
1
8
Age (years)/sex
Case
A well-defined solid and cystic lesion (4.3 × 4.1 × 3.5 cm) in the left temporal lobe with enhancement in the wall of cyst. No surrounding vasogenic edema. A 7 mm solid enhancing lesion within left temporal cortex without surrounding vasogenic edema. A solid and focally cystic enhancing cortical mass (4.0 × 2.7 cm) in left parietal lobe with perilesional vasogenic edema.
A large mass (9.6 × 7.4 × 7.3 cm) in left frontal lobe with irregular peripheral enhancement. No surrounding vasogenic edema is seen. A 5.8 × 5.7 × 5.2 cm heterogeneous enhancing cystic and solid mass lesion within right frontal lobe with marked surrounding vasogenic edema. A large partially cystic mass (5 × 4.5 × 5.7 cm) with 2.6 cm diameter enhancing nodule contained within the cystic mass in right posterior parietal lobe. There is minimal surrounding vasogenic edema. A well-defined enhancing lesion (5.6 × 5.1 × 3.8 cm) touching the left side of the falx cerebri in left frontal lobe with significant perilesional edema.
A 5 mm ring-enhancing lesion within right posterior frontal cortex without surrounding vasogenic edema. Extensive calcification on CT scan. An enhancing nodular lesion (2.0 × 1.9 × 1.9 cm) in right parietal lobe with marked surrounding vasogenic edema. There are multiple nodular dura-based enhancing lesions at the last recurrence. A large mass with cystic (5.0 × 5.0 cm) and solid (3.5 × 2.0 cm) components in left frontal lobe. Solid area peripherally located in the lateral aspect of the lesion demonstrates heterogeneous enhancement. The cystic cavity demonstrates smooth ring enhancement. Surrounding vasogenic edema and mass effect are noted. Calcification is seen on CT scan. An enhancing mass (2.1 × 2.0 m) in left frontal lobe with moderate surrounding vasogenic edema.
Neuroimaging findings
Table 1 Clinical findings of cortical ependymoma
Headaches and speech difficulty
Seizures
Right side weakness
Mild confusion, dizziness, and right side weakness
Headache, visual field change, and left side weakness
Headache
Right side weakness
Seizures
Seizures
Seizures
Seizures
Clinical presentation
Not recur in 6 months
Not recur in 11 months
Recur at 9 months after 1st surgery
NA
Recur twice at 3 years and 4.5 years after 1st surgery Recur at 3 years after 1st surgery
Recur 3 times at 14 years, 20 years, 22 years after 1st surgery Recur 3 times at 19 months, 4 years, 6 years after 1st surgery
No
Recurrence
Gross total resection and radiation
Gross total resection
Gross total resection, followed by radiation and chemotherapy after the second surgery Gross total resection, chemotherapy and radiation
Gross total resection
Partial resection
Gross total resection
Gross total resection
Gross total resection, radiation and chemotherapy
Gross total resection, radiation and chemotherapy
Gross total resection
Treatment
Died at 4 months
Alive at 6 months
Alive at 11 months
Alive at 3 years
Died during post-surgery recovery Died at 4 years
Alive at 4 years
Alive at 12.5 years
Died at 7 years
Alive at 22 years
Alive at 2 years
Survival
244 Z Liu et al.
© 2013 Japanese Society of Neuropathology
Ependymoma (WHO grade II) with clear cell change and extensive calcification. Clear interface between tumor and brain parenchyma is seen. Anaplastic ependymoma (WHO grade III) with microvascular proliferation and tumor necrosis, occasional perivascular pseudorosettes, clear cell change and scattered giant cells. Clear interface between tumor and brain parenchyma is seen. Anaplastic ependymoma (WHO grade III) with numerous true ependymal rosettes, rare epndymal surface, spindle cell component, and single cell in myxoid background. Calcification and ossification are noted. Anaplastic ependymoma (WHO grade III) with pseduopilasiding tumor necrosis and focal clear cell. Spindle cells with elongated atypical nuclei and giant cells are noted at multiple foci. Clear interface between tumor and brain parenchyma and an isolated nodule in the brain parenchyma away from the tumor are seen. Anaplastic ependymoma (WHO grade III) with occasional ependymomal surface. Tumor nodules in dura are noted. Microcalcification is present. Anaplastic ependymoma (WHO grade III) with pseduopilasiding tumor necrosis and occasional giant cells. Clear interface between tumor and brain parenchyma is seen. Anaplastic ependymoma (WHO grade III) with PNET-like component. Tumor nodules in dura are noted. Anaplastic ependymoma (WHO grade III) with pseduopilasiding tumor necrosis and spindle cells. Clear interface between tumor and brain parenchyma is seen. Anaplastic ependymoma (WHO grade III) with focal clear cell morphology. Clear interface between tumor and brain parenchyma is seen. Ependymoma (WHO grade II). Anaplastic ependymoma (WHO grade III) with pseduopilasiding tumor necrosis.
1
© 2013 Japanese Society of Neuropathology
PNET, primitive neuroectodermal tumor.
10 11
9
8
7
6
5
4
3
2
Histology
Case
Table 2 Pathologic findings of cortical ependymoma
+ +
+
+ in epedymoma – in PNET-like component +
+
+
+
+
+
+
GFAP
+ +
+
+
+ in both components
+
+
−
NA
+
+
EMA
NA NA
–
NA
NA
NA
NA
+
NA
+
+
CD99
NA NA
40%
Up to 20%
30% in ependymoma 60–70% in PNET
10%
30–40%
NA
20–30%
10% initially 30–40% in recurrence
