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Tanycytic ependymomas mostly occur in the spinal cord and it is the rarest histological subtype of ependymoma. A 29-year-old male was referred from the infertility clinic after serum prolactin levels were found to be elevated. Magnetic resonance imaging (MRI) brain showed an irregular necrotic lesion in the periventricular region of the left parietal lobe which had an intraventricular component and associated perilesional edema. In addition, a sellar mass with suprasellar extension was also found on the MRI. He was started on cabergoline therapy for macroprolactinoma and underwent a left parietal craniotomy, and microsurgical excision of the tumor using intraoperative neurosonographic guidance. Histologically, the tumor showed spindle cytologic features and poorly developed inconspicuous pseudorosettes, with areas of rounded nuclear profiles and perinuclear cytoplasmic clearing. Tumor cells were positive for vimentin, glial fibrillary acidic protein and S100, and negative for epithelial membrane antigen. Ki67 was <7%. He was diagnosed with tanycytic ependymoma and a coexistent prolactinoma. He received 10 cycles of image-guided radiotherapy. Post-operative imaging showed minimal residual tumor the size of which remained stable at 1-year follow-up scan. The pituitary macroadenoma regressed with cabergoline therapy and he clinically improved. This presentation of synchronous macroprolactinoma and tanycytic ependymoma has not been reported in the literature previously. An exhaustive literature review showed only 18 previously reported cases of supratentorial tanycytic ependymoma. [ABSTRACT FROM AUTHOR]

Tanycytic ependymoma is a neuroectodermal tumor that arises from ependymoglial cells or tanycytes. It is highly uncommon. We reported a 34-year-old man who was diagnosed with intradural-intramedullary tanycytic ependymoma, located at the level of C4-5 who had a 9-months history of neck pain and left arm pain, and numbness on fingers. One month prior to presentation, his left arm numbness and paresthesia deteriorated. The lesion was removed totally by C4, C5 hemilaminoplasty. The histologic pattern of this lesion was consisted of fascicles forming nebula-like whorling structures. Because of these structures, tanycytic ependymoma should be taken into consideration in the differential diagnosis of a whorling-sclerosing variant of meningiomas. [ABSTRACT FROM AUTHOR]

Background: Tanycytic ependymoma is a rare variant of ependymoma that commonly affects the cervical and thoracic spinal cord. It usually arises as intramedullary lesions and extramedullary cases are extremely rare. Case Description: We present a 77-year-old woman with the complaints of a 2-year history of progressive paraparesis and sensory loss in her lower extremities. Magnetic resonance imaging revealed a stretched and fusiform intradural extramedullary lesion at T5-T10 level. Gross total removal of the tumor was achieved and a definitive diagnosis of tanycytic ependymoma was established. Conclusion: This case thus represents a rare case of thoracic intradural extramedullary tanycytic ependymoma and, to the best of our knowledge, it represents the longest intradural extramedullary tanycytic ependymoma in craniocaudal direction ever reported in the literature. [ABSTRACT FROM AUTHOR]

Tanycytic ependymoma (TE), a rare subtype of ependymoma, was classified as grade II ependymoma by the World Health Organization in 2000 and 2007. Preoperative diagnosis of TE is challenging due to its similarities to schwannoma and astrocytoma; therefore, differentiation is required. The present study investigated the clinical, imaging and pathological characteristics of TE in the filum terminale. A retrospective analysis was conducted on the clinical, imaging, pathological and immunohistochemical characteristics of 8 patients with TE in the filum terminale and the relevant literature was reviewed. Of the 8 patients 7 were female and 1 was male, with an age range of 24–62 years old. The primary clinical symptom observed was lumbago, accompanied by lower limb pain and numbness. Magnetic resonance imaging predominantly identified isointensity on thoracic (T) 1-weighted images and iso- or hyperintense signal intensity on T2-weighted images, with homogeneous or inhomogeneous enhancement. All patients underwent resection of the tumor through a posterior median approach, and total resection was acighieved in 7 patients. During postoperative follow-up, all patients experienced improvement compared with their preoperative status, and were without tumor recurrence. The present study comprised the largest group of cases with TE in the filum terminale reported so far, to the best of our knowledge, which could foster a better understanding of this disease. Complete surgical resection of the tumor has the greatest effectiveness of any treatment for TE in the filum terminale. Postoperative histological examination, immunohistochemistry and electron microscopy for tumor specimens may assist in its diagnosis and differential diagnosis. [ABSTRACT FROM AUTHOR]

Tanycytic ependymoma is a rare subtybe of ependymoma with a predilection for the spinal cord and intracranial tanycytic ependymoma is thus extremely rare. Most studies on intracranial tanycytic ependymomas included only one or two cases. Here we report nine patients with pathologically confirmed intracranial tanycytic ependymomas. The clinical characteristics, including radiological and histological examination, operative records, and prognoses were reviewed. The case series included six male and three female patients with an average age of 19.3 years. Tumors were located in the lateral ventricle (3/9), the fourth ventricle (2/9), and the supratentorial extraventricle (4/9). Gross total resection (GTR) of the tumor was achieves in seven cases, and subtotal resection (STR) was achieved in the other two cases. One patient died 21 months after discharge. The left eight patients showed improved symptoms after surgery, and no tumor recurrence was found in these cases during the follow-up. It seems that intracranial tanycytic ependymoma has the best long-term prognosis compared to the other two subtypes of ependymoma. According to our experience, we recommend surgery including GTR and STR followed by radiotherapy for patients with intracranial tanycytic ependymomas. [ABSTRACT FROM AUTHOR]

Background: Imaging and histology of clear-cell ependymoma and cerebellum-based hemangioblastoma are similar; distinguishing between them is a diagnostic challenge. Case presentation: A 62-year-old Chinese woman presented with an intermittent headache of 8 years' duration. Computed tomography and magnetic resonance imaging revealed a mass in the cerebellum. Neurological imaging suggested hemangioblastoma (HB). Histologically, the tumor included cellular and paucicellular areas, in which cells were arranged in nests or diffusely distributed; and a highly vascular area, in which tumor cells were arranged in clusters and separated by capillaries. At low magnification, the tumor mimicked cellular HB, but at high magnification, tumor cells showed clear cytoplasm instead of the vacuolated cytoplasm typically observed in HB. Moreover, spindly, bipolar elements resembling tanycytes were observed within the nest structures. Although these features indicated the possibility of ependymoma, neither true ependymal rosettes nor an ependymal-lined profile was observed. The tumor was characterized by prominent vascularity, but glomeruloid formation was absent. We saw pleomorphism in foci of some tumor giant cells, but pathologic mitosis and palisaded necrosis were absent. Most tumor cells were positive for glial fibrillary acidic protein and S100. Epithelial membrane antigen was expressed with a paranuclear dot-like or a ring-like pattern. The Ki-67 index was approximately 2%. Considering the patient's symptom, neurological imaging, and pathological findings, she was diagnosed as cerebellar ependymoma (WHO grade II). Conclusions: Here, we report a case of ependymoma with overlapping clear-cell and tanycytic features, and review the literature to evaluate its real incidence. Pathologists should consider this rare diagnosis when confronted with a similar presentation. [ABSTRACT FROM AUTHOR]

We studied one frontal lobe tumor and multiple spinal cord tumors (one in an extramedullary location) that had been resected from a 24-year-old man. The frontal lobe tumor was well demarcated and non-infiltrating, and consisted of eosinophilic, elongated fibrillary cells arranged in a fascicular pattern. A similar histology was reproduced in the spinal cord tumors, with additional areas showing standard features of ependymoma. Immunohistochemical and ultrastructural observations revealed that all the tumors were ependymal in nature with positivity for GFAP and epithelial membrane antigen and negativity for oligodendrocyte transcription factor 2, showing intra- and intercellular microrosettes, leading us to a diagnosis of tanycytic ependymoma for the frontal lobe tumor and tanycytic ependymoma with ordinary ependymomatous component for the spinal cord tumors. The spinal extramedullary tumor was a schwannoma. Importantly, a heterozygous truncating mutation in the NF2 gene was identified in the blood lymphocytes from the patient. It is known that multiple nervous system tumors can occur in neurofibromatosis type 2 ( NF2), which is caused by mutation in the NF2 gene, and that occurrence of ependymoma, including the tanycytic variant, can be associated with this genetic condition. The present case provides further information about the clinicopathology of tanycytic ependymoma with details of the immunohistochemical, ultrastructural and genetic features. [ABSTRACT FROM AUTHOR]

Two cases of tanycytic ependymoma are reported, one in a woman aged 33 years and one in a man aged 36 years. The woman presented with lesions at L2 and L3 in the spinal cord and clinical symptoms suggested spinal cord compression, such as movement and sensory dysfunction. The man had a lesion located in the lateral ventricle and presented with headache, vomiting, and impaired vision. Both of these patients developed gradual increases in the intensity of their symptoms over a few months prior to admission. A spinal cord MRI of the woman showed a well-defined, solid cystic mass measuring 1.0 × 6.5 cm at L2 and L3 of the spinal cord. The mass showed low signal intensity on T2-weighted images in the intervertebral disc with equal signal intensity T1-/T2-weighted images. The signal was uniform and enhanced scanning showed a light strengthening of signal. A brain MRI of the man showed a well-defined mass measuring 4 cm in diameter invading the right ventricle. The mass showed low signal intensity on right T1-weighted images and high signal intensity on T2-weighted images. Histopathologically, the tumors were rich with significant and uniform proliferation of long spindle cells, which were arranged in fasciculate and knitted patterns with bipolar and spindled processes focally forming perivascular pseudorosettes. The cell nuclei showed minor pleomorphism. A few tumor cells centered on the vessels resulted in a nuclear-free zone, which was characteristic of ependymoma. Immunohistochemically, the tumor cells were positive for GFAP, EMA, and CD99, but negative for S-100. A review of the cases reported in the literature shows that tanycytic ependymoma occurs more often in the spinal cord. As it can resemble pilocytic astrocytoma and schwannoma, tanycytic ependymoma should be differentially diagnosed to exclude some of the benign spindle cell tumors of the central nervous system. [ABSTRACT FROM AUTHOR]

A tanycytic ependymoma measuring 1.5 cm in maximal dimension, which involved the filum terminale and conus medullaris of a 55-year-old woman, is reported. The tumor consisted of a compact fascicular proliferation of spindle cells having long bipolar cytoplasmic processes, and immunohistochemical and ultrastructural studies demonstrated the ependymal features of neoplastic cells. The most prominent finding was an appearance of many atypical and pleomorphic, often monstrous, giant cells, which was not associated with an increase in proliferative activity. Remarkable nuclear atypism and pleomorphism with formation of giant cells have not been documented previously in tanycytic ependymoma. These nuclear changes are considered essentially degenerative in nature and probably do not portend a worse prognosis despite their worrisome appearance. This tumor could be appropriately termed “giant cell tanycytic ependymoma.” [ABSTRACT FROM AUTHOR]

In a 43-year-old man, an intramedullary spinal cord tumor spreading from the level of the T2 to T5 vertebrae was subtotally resected. The tumor predominantly consisted of a fascicular proliferation of spindle cells having bland nuclei and bipolar, long cytoplasmic processes, and a few perivascular pseudo-rosettes were found. Although there were no true ependymal rosettes, intracytoplasmic dot-like immunoreactivity for epithelial membrane antigen (EMA) was found in a few cells. In some areas, a dense and diffuse proliferation of anaplastic, short-spindled cells having hyperchromatic nuclei and scant cytoplasm was noted, and the Ki-67 labeling index was remarkably higher (18.2%) in these areas. Neither microvascular proliferation nor necrosis was observed. In the boundary region, these two areas showed gradual transition from one to the other. The patient has remained free from recurrence for 10 months postoperatively. This is the first documentation of tanycytic ependymoma in which tumor cells showed anaplastic cytological features. [ABSTRACT FROM AUTHOR]

Tanycytic tumors of the cauda equina region are being increasingly reported. Such tumors can occur among pediatric patients and adults. An unusual case of tanycytic ependymoma located at the region of the cauda equina is reported in a girl. The clinical presentation, radiological features, intraoperative findings, and histopathologic features are described. The prognostic significance of differentiating such a neoplasm from other intramedullary tumors is noted. A heightened awareness of this pathologic entity among neurosurgeons and pathologists will help in diagnosing this neoplasm and in enabling better patient management. [ABSTRACT FROM AUTHOR]

We report a rare case of tanycytic ependymoma arising from the cerebral hemisphere. A 59-year-old man was admitted to our hospital because of the incidental detection by MRI of a tumor lesion in the right temporooccipital paratrigonal region. The mass showed low-to iso-intensity on T1-weighted images and high intensity on T2/proton-weighted images. Partial resection was performed using a transsulcal approach to avoid compromising the visual field. Most of the tumor cells showed elongated spindle shapes arranged in dense fascicles. A few true ependymal rosettes and perivascular pseudorosettes were visible. The tumor cells were positive for GFAP, S-100, and vimentin, but negative for synaptophysin, EMA, and keratin. The MIB-1 labeling index was approximately 1%. Ultrastructurally, the tumor cells had ependymal cell features, such as microvilli and cilia. From these findings, a pathological diagnosis of tanycytic ependymoma was made. [ABSTRACT FROM AUTHOR]

Tanycytic ependymomas are a subtype of ependymomas that were formally recognized as a new pathological entity in the latest World Health Organization (WHO) classification of 2000. They occur mostly in the spinal cord. Only a few reports have analyzed the proliferative potentials of these tumors; however, it has been reported that the MIB-1 labeling index of tanycytic ependymoma is lower than that of other subtypes of WHO grade II ependymomas. We report a rare case of cervicomedullary junction tanycytic ependymoma associated with marked cyst formation. A 62-year-old man had a history of progressive gait disturbance, diplopia, and swallowing disturbance over a one-month period prior to admission. Magnetic resonance imaging (MRI) showed a cystic mass with a mural nodule at the cervicomedullary junction with Gd-DTPA enhancement. Cyst-subarachnoid shunt was performed using a far lateral approach. After 6 years, however, the man was readmitted to the hospital because of reaccumulation of the cyst. Partial removal of a mural nodule and a cyst-subarachnoid shunt were performed simultaneously by a midline suboccipital approach. The pathological diagnosis was tanycytic ependymoma. Postoperatively, the patient recovered well and was discharged from the hospital without further treatment. Most of the tumor cells had small, round nuclei; pleomorphism was minimal. The cytoplasm was dilated. The tumor cells were positive for EMA and s-100, and negative for CD-34. GFAP was not determined due to difficulty caused by background glial processes. The MIB-1 labeling index was less than 1%. Ultrastructurally, the tumor cells had ependymal cell features, such as desmosomes and microvilli. Based on these findings, the pathological diagnosis was tanycytic ependymoma. [ABSTRACT FROM AUTHOR]

We report a case of extraventricular ependymoma arising in a 50-year-old woman that took an aggressive clinical course with recurrence three times. The initial tumor was a well-circumscribed nodule in the right temporal white matter measuring 2 cm in diameter. It showed variegated histological findings mimicking metastatic adenocarcinoma: an epithelioid arrangement of highly pleomorphic cells with pseudopapillary structures and perivascular pseudorosettes, and bizarre multinucleated giant cells with occasional emperiporesis surrounded by abundant mononuclear inflammatory cells, as well as a focal small area of conventional ependymoma. Emperiporesis and abundant mononuclear cell infiltration were not previously described in an ependymoma. The recurrent tumors predominantly showed an epithelioid pattern with frequent formation of astroblastoma-like pseudopapillary structures. Neoplastic cells were markedly atypical and had characteristic intracytoplasmic eosinophilic inclusion bodies. Much of the cells in both the initial and recurrent tumors showed a positive immunostaining for glial fibrillary acidic protein (GFAP) with accentuation to the cytoplasmic processes of the pseudorosettes and pseudopapillary structures. Epithelial membrane antigen (EMA) highlighted the epithelial differentiation of the tumor cells, while cytokeratin was completely negative. Although this tumor might be classified to at least WHO grade III from the histology and aggressive behavior, the exact grading is still controversial because of the rarity of such cases. [ABSTRACT FROM AUTHOR]

Three cases of spinal tanycytic ependymoma are reported, a man aged 45 years and two women aged 36 and 55 years. Each patient developed gradual paraparesis over a few months prior to admission. Magnetic resonance imaging showed an enhancing, well-circumscribed tumor in the spinal cord in each case. Histologically, the tumors consisted of monotonous proliferation of long spindle cells with markedly eosinophilic cell processes; focally forming perivascular pseudorosettes. The tumor cells were strongly immunopositive for glial fibrillary acidic protein, S-100 protein and vimentin. Ultrastructurally, in addition to massive intermediate filaments, many tumor cells showed abundant microtubules. Well-developed desmosomes and microvilli/cilia-lined microlumina were occasionally observed. The tumors were grossly totally removed and the patients remain recurrence free at 9, 9, and 2 years postoperatively. Reviewing reported cases including our three cases, tanycytic ependymoma may occur frequently in spinal cord, especially in the cervical region of the spinal cord. Since histologically it resembles pilocytic astrocytoma and schwannoma, tanycytic ependymoma should be included in the differential diagnosis of benign spindle cell tumors of the central nervous system. [ABSTRACT FROM AUTHOR]