Tanycytic Ependymoma

Tanycytic ependymoma is an uncommon fibrillar variant of ependymoma characterized by streams of piloid, or hair-like, cells having “ependymal” nuclei. True ependymal rosettes are absent, and perivascular rosettes are inconspicuous. Misinterpretation as schwannoma or astrocytoma is a diagnostic problem and well-documented cases are scarce. The purpose of this report is to document the ependymal features of the neoplasm and to increase awareness of the entity's existence. Biopsy tissues from three patients with tanycytic ependymoma were examined. All tumors consisted of sheets of spindle cells that were positive for glial fibrillary acidic and S-100 proteins. Ultrastructural examination showed characteristic ependymal features, including intracytoplasmic intermediate filaments, prominent intercellular junctions, numerous slender surface microvilli, and microvilli-lined lumina. Accurate recognition of the ependymal nature of this spindle neoplasm requires a high index of suspicion. Because the spindle cells are immu-noreactive with antibodies to both glial fibrillary acidic and S-100 proteins, ultrastructural confirmation of ependymal features is necessary.     

Paraganglioma of the Cauda Equina: An Ultrastructural and Immunohistochemical Study of Two Cases

The ultrastructural and immunohistochemical features of 2 paragangliomas arising in the cauda equina are described. In both cases the tumor cells were arranged in small nests or cords and contained characteristic neurosecretory granules, lamellar stacks of rough endoplasmic reticulum (RER), and some well-developed Golgi apparatuses in their cytoplasm. The cells varied in electron density; the darker cells, occasionally resembling sustentacular cells, were probably dehydrated light cells because they contained a few neurosecretory granules. Sustentacular cells were difficult to identify by electron microscopy, but irregularly distributed S-100 protein and glial fibrillary acidic protein (GFAP) were found in these cells by immunostaining. Many tumor cells contained abundant neurofilaments. Curiously, a few cytokeratin-positive cells were found in 1 case. On microscopic examination, a small area of ganglioneuroma was found associated with the paraganglioma in 1 case. Ganglionic differentiation was concluded to be frequent in paragangliomas of the cauda equina region as in duodenal paragangliomas.     

Paraganglioma of the Cauda Equina: An Ultrastructural and Immunohistochemical Study of Two Cases

The ultrastructural and immunohistochemical features of 2 paragangliomas arising in the cauda equina are described. In both cases the tumor cells were arranged in small nests or cords and contained characteristic neurosecretory granules, lamellar stacks of rough endoplasmic reticulum (RER), and some well-developed Golgi apparatuses in their cytoplasm. The cells varied in electron density; the darker cells, occasionally resembling sustentacular cells, were probably dehydrated light cells because they contained a few neurosecretory granules. Sustentacular cells were difficult to identify by electron microscopy, but irregularly distributed S-100 protein and glial fibrillary acidic protein (GFAP) were found in these cells by immunostaining. Many tumor cells contained abundant neurofilaments. Curiously, a few cytokeratin-positive cells were found in 1 case. On microscopic examination, a small area of ganglioneuroma was found associated with the paraganglioma in 1 case. Ganglionic differentiation was concluded to be frequent in paragangliomas of the cauda equina region as in duodenal paragangliomas.     

Paraganglioma of the cauda equina. A case report and review of the literature

A case of paraganglioma of the cauda equina is reported. The patient was a 55-year-old Japanese woman who complained of lower back pain and gradual weakening of the left lower extremity, she was diagnosed as having a spinal cord tumor, and the tumor was removed surgically. Histologically, the tumor was encapsulated, and consisted of solid nests of large, polyhedral epithelioid cells with abundant eosinophilic cytoplasm. The nests were separated from each other by a thin fibrovascular stroma. Grimelius staining revealed neurosecretory granules in the cytoplasm of the tumor cells. This was further confirmed by electron microscopic observation. The tumor cells were immunohistochemically positive for neuron-specific enolase (NSE), but negative for glial fibrillary acidic protein (GFAP). On the basis of the histologic, immunohistochemical and electron microscopic features of the tumor cells, the tumor was diagnosed as paraganglioma. The cauda equina is a rare location for this type of tumor, and only a limited number of cases have been reported.     

Paraganglioma of the Cauda Equina

A case of paraganglioma of the cauda equina is reported. The patient was a 55 year old Japanese woman who complained of lower back pain and gradual weakening of the left lower extremity, she was diagnosed as having a spinal cord tumor, and the tumor was removed surgically. Histologically, the tumor was encapsulated, and consisted of solid nests of large, polyhedral epithelioid cells with abundant eosinophilic cytoplasm. The nests were separated from each other by a thin fibrovascular stroma. Grimelius staining revealed neurosecretory granules in the cytoplasm of the tumor cells. This was further confirmed by electron microscopic observation. The tumor cells were immunohistochemically positive for neuron-specific enolase (NSE), but negative for glial fibrillary acidic protein (GFAP). On the basis of the histologic, immunohisto-chemical and electron microscopic features of the tumor cells, the tumor was diagnosed as paraganglioma. The cauda equina is a rare location for this type of tumor, and only a limited number of cases have been reported.     

An immunohistological study of 66 ependymomas

Sixty-six ependymomas were examined immunohistologically to determine their distribution of glial fibrillary acidic proteins, S-100 protein and vimentin. The neoplasms were subdivided into four groups: (1) ependymomas from the cauda equina, predominantly of the myxopapillary type; (2) benign ependymomas; (3) malignant ependymomas; and (4) ependymoblastomas. Marked differences in antigen reactivity were observed between each group. The intensity of the reaction with the three antibodies was strongest in malignant ependymomas. Ependymomas from the cauda equina showed a patchy distribution of positivity for the three antigens in cells surrounding blood vessels but there was no staining of collagenous septa or the myxoid areas. In ependymoblastomas, the cells of the rosettes were negative for glial fibrillary acidic protein, but there was focal positivity for vimentin and S-100. Other areas showed tumour cells containing moderate amounts of vimentin and small amounts of S-100, and a few bands of filaments positive for glial fibrillary acidic protein. The cytogenetic and biological implications of these findings are discussed.     

Subcutaneous sacrococcygeal myxopapillary ependymoma. A case report and review of the literatures

A case of myxopapillary ependymoma originating in the soft tissue is described. The tumor was located subcutaneously over the coccyx of an 11-year-old girl but was connected neither to the filum terminale nor cauda equina. Clinically, the tumor was locally resected with a diagnosis of pilonidal cyst. Histological and electron microscopic findings were identical to myxopapillary ependymoma. The tumor cells showed a positive reaction by immunoperoxidase method (PAP method) of glial fibrillary acidic protein (GFAP).     

Infratentorial giant cell ependymoma: a rare variant of ependymoma

We describe a giant cell ependymoma occurring in a 50-year-old man. The mass was located in the posterior aspect of the foramen magnum, extending from the cerebellar tonsil to the upper cervical spine. The tumor was a highly cellular neoplasm showing biphasic histology. Diffuse sheets of non-cohesive atypical giant cells, having eccentrically located single or multiple nuclei and plump eosinophilic cytoplasm, partly infiltrated the desmoplastic inflammatory stroma. Parts of perivascular pseudorosette-forming or pseudopapillary areas were composed of atypically elongated cells, which looked like conventional anaplastic ependymoma. There was a transitional area between two patterns. Numerous mitoses and focal necrosis were observed. Immunohistochemically, the tumor cells were immunoreactive for glial fibrillary acidic protein, vimentin, S-100 protein, and CD99. None of the tumor cells showed immunoreactivity for epithelial membrane antigen except for the intracytoplasmic lumen of a few vacuolated cells. Ultrastructurally, tumor cells were ependymal in nature; we noted cytoplasmic intermediate filaments and intercellular microrosettes with microvilli, cilia, and long zonula adherens. The features of this tumor, e.g. its superficial location, mixed giant cells, perivascular pseudorosettes or papillaries, complicated its differentiation from rhabdoid/papillary meningioma. However, immunohistochemistry and electron microscopy confirmed the diagnosis of ependymoma. The giant cell variant should be included in the subclassification of the ependymoma.     

SUBCUTANEOUS SACROCOCCYGEAL MYXOPAPILLARY EPENDYMOMA A Case Report and Review of the Literatures

A case of myxopapillary ependymoma originating in the soft tissue is described. The tumor was located subcutaneously over the coccyx of an 11-year-old girl but was connected neither to the filum terminale nor cauda equina. Clinically, the tumor was locally resected with a diagnosis of pilonidal cyst. Histological and electron microscopic findings were identical to myxopapillary ependymoma. The tumor cells showed a positive reaction by immunoperoxidase method (PAP method) of glial fibrillary acidic protein (GFAP). ACTA PATHOL. JPN. 35 : 925–931, 1985.     

Clear-cell Ependymoma of the Cerebellum: A Case Report

A case of clear-cell ependymoma occurring in the cerebellum of a 3-year-old girl is reported. Light-microscopically, the tumor consisted mainly of clear cells with a perinuclear halo and showed some vague perivascular pseudorosettes, not true rosettes. In addition, the histological features of anaplasia, characterized by increased mitosis and focal pseudopalisading necrosis, were also observed. Immunohistochemically, the tumor cells were focally positive for glial fibrillary acidic protein and weakly positive for epithelial membrane antigen. Ultrastructurally, the intermediate junctions and rudimentary cilia confirmed the ependymal differentiation. Fifteen cases of infratentorial clear-cell ependymoma have been reported to date, and this case is the second childhood tumor among them, to the best of the authors' knowledge.     

Clear-cell ependymoma of the cerebellum: a case report

A case of clear-cell ependymoma occurring in the cerebellum of a 3-year-old girl is reported. Light-microscopically, the tumor consisted mainly of clear cells with a perinuclear halo and showed some vague perivascular pseudorosettes, not true rosettes. In addition, the histological features of anaplasia, characterized by increased mitosis and focal pseudopalisading necrosis, were also observed. Immunohistochemically, the tumor cells were focally positive for glial fibrillary acidic protein and weakly positive for epithelial membrane antigen. Ultrastructurally, the intermediate junctions and rudimentary cilia confirmed the ependymal differentiation. Fifteen cases of infratentorial clear-cell ependymoma have been reported to date, and this case is the second childhood tumor among them, to the best of the authors' knowledge.     

November 2004: intradural mass of the cauda equina in a woman in her early 60s

November 2004. A 63-year-old woman presented with slowly aggravating lower back pain and recent urinary urge incontinence. MRI revealed a sharply-delineated, partly cystic intradural mass with inhomogenous contrast-enhancement and ectatic vessels at the upper pole. An ependymoma was suspected, and the tumor was resected in toto. Histologically, at first glance, the tumor strongly resembled an ependymoma, showing a monomorphic cellular pattern, perivascular pseudorosettes and ependymal canal-like structures. However, the finding of a delicate collagen capsule, compartmentation of tumor cells into zellballen and the presence of ganglionic cells were untypical. These features were indicative of a paraganglioma with a gangliocytic component. Immunoreactivity of the tumor cells for neuroendocrine antigens, the detection of GFAP-positive sustentacular cells and the ultrastructural confirmation of neurosecretory granules substantiated this diagnosis. The clinical, radiological and morphological similarity between ependymomas, which are far more common in the cauda equina region than paragangliomas, has led to substantial diagnostic confusion in the past.     

Aggressive oncocytic neuroendocrine tumour (''oncocytic paraganglioma'') of the cauda equina

An oncocytic neuroendocrine tumour ('oncocytic paraganglioma') of the cauda equina is reported. The tumour was predominantly intradural, with extension into and destruction of surrounding vertebral bone. The tumour had an organoid pattern, and the tumour cells had abundant non-argyrophilic eosinophilic cytoplasm. Immunocytochemical stains for neurone-specific enolase, S-100 protein, keratin and carcinoembryonic antigen were positive, but stains for glial fibrillary acidic protein were negative. On ultrastructural examination, there were numerous mitochondria and scattered 200 nm dense-core membrane-bound granules, that rarely clustered in small aggregates. Intermediate filaments were focally arranged in long compact bundles. The histogenesis of tumours reported as cauda equina paragangliomas is discussed.     

Case of clear cell ependymoma of medulla oblongata: clinicopathological and immunohistochemical study with literature review

Clear cell ependymoma has been included in the WHO classification of the central nervous system in 1993, after the first report by Kawano et al. Since then, only a few cases have been reported. Most clear cell ependymoma cases reported in the literature so far were located in the supra-tentorial compartment and/or cerebellum, and one case was in the cervical spinal cord. We report a case of clear cell ependymoma whose histological features were sufficient for the diagnosis and was unusually located in the fourth ventricle originating from the medulla oblongata. The tumor showed uniform tumor cells with perinuclear halo, nuclei being centrally located. Most of the tumor cells were arranged as perivascular pseudorosettes, and no ependymal canals or rosettes were evident. Mitotic figures were not frequent. Immunohistochemically, the tumor cells were strongly reactive for glial fibrillary acidic protein and vimentin, and weak and dot-like positive for epithelial membrane antigen. Clear cell change of the tumor cells appeared to be fixation artifact because this feature was not evident in the frozen section.     

Primary primitive neuroectodermal tumor of the cauda equina

Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification has been controversial, PNETs are well recognized primary tumors of both central and peripheral nervous systems. PNETs must be distinguished from other round-cell tumors, including Ewing's sarcoma, lymphoma, rhabdomyosarcoma, and small cell carcinoma. Intraspinal PNETs are rare neoplasms that are usually metastatic in origin. We describe the eighth reported primary PNET of the cauda equina that developed in a 52-year-old man with no significant medical history. The tumor was characterized by Homer-Wright rosettes and immunoreactivity for CD99, glial fibrillary acidic protein, neuron-specific enolase S100, and synaptophysin. The anatomic location of primary intrathecal PNETs is important as those arising in the spinal cord develop in the central nervous system, whereas those arising in the cauda equina develop in the peripheral nervous system. The histogenesis of intrathecal PNETs may be multifactorial.     

Histological characterization of an ependymoma in the fourth ventricle of a cat

A tumour occupying the fourth ventricle in a 3-year-old cat was removed surgically and characterized as a tanycytic ependymoma on the basis of histological features of low cellularity, inconspicuous perivascular pseudorosettes and fascicular architecture. Immunohistochemical analysis of sections revealed that the neoplastic cells were immunoreactive for glial fibrillary acidic protein (GFAP), vimentin and S-100. The histological and immunohistochemical findings were similar to those of human tanycytic ependymoma, a subclassification of ependymoma not previously described in domestic species.     

Primary extraneural myxopapillary ependymoma of the broad ligament

Primary extraneural ependymomas are rare tumors that arise in ectopic sites, including pulmonary, sacrococcygeal region, ovarian, and paraovarian tissues. Four such ependymomas reported in the literature involve the paraovarian tissues, including 2 broad ligament ependymomas. Here we describe a myxopapillary ependymoma of the broad ligament in a 22-year-old woman, which may be the first tumor of this type to be reported in this location. Cytology, histology, cytochemistry, immunohistochemistry, and flow cytometry ploidy analysis are studied and described. Identification of perivascular ependymal rosettes, ependymal canals, vimentin and glial fibrillary acidic protein immunoreactivity, cytochemical staining of blepharoplasts or terminal bars by phosphotungstic acid hematoxylin, and presence of multiple foci of myxoid degeneration among the ependymal rosettes characterized a myxopapillary ependymoma.     

Gliosarcoma arising from an anaplastic ependymoma: a case report of a rare entity

We report the first case of a gliosarcoma arising from an anaplastic ependymoma and the second case of gliosarcoma arising from any type of ependymal neoplasm. The patient was a 48-year-old woman with a solid and cystic, peripherally enhancing, 7-cm right frontal mass lesion. Histologically, the lesion displayed characteristics of anaplastic ependymoma (grade III, World Health Organization scale). The tumor recurred despite multiple cycles of postoperative radiation and chemotherapy. After the fourth recurrence, the tumor displayed a biphasic pattern of differentiation. The first pattern was similar to the original anaplastic ependymoma, whereas there was a second new sarcomatous pattern resembling fibrosarcoma that was admixed with and overrunning the ependymal component. The spectrum of gliosarcoma is therefore expanded to include not only astrocytic and oligodendroglial components but ependymal components as well.     

Perivascular pseudorosettes in childhood brain tumours: an ultrastructural and immunohistochemical study

Perivascular pseudorosettes (PP) in childhood central nervous system tumours were examined with light and electron microscopy and immunohistochemistry for glial fibrillary acidic protein, S-100 protein and albumin. One kind of PP at light microscopy comprised a central thin-walled vessel surrounded by a thick mantle of eosinophilic fibrillary material and rings of usually regular nuclei. Adjacent tumour tissue was compact. This type was correlated closely with ultrastructural evidence of ependymal differentiation. The central vessel showed continuous endothelium in all. Another type of PP comprised a central vessel of varying thickness surrounded by hyaline material, clearly defined tapering processes, and rings of often irregular nuclei. Adjacent tissue showed extensive edema and microcystic change. Ultrastructurally, this type showed no ependymal differentiation except in one myxopapillary ependymoma. Fenestrated vessels were seen in half of the tumours associated with this kind of PP. It is proposed that variation in vascular permeability, rather than in the structure of tumour cells, is the main cause for the difference in histological appearance of the two types of PP. Fenestrated vessels may also be responsible for the "myxoid" change in myxopapillary ependymomas. The amount of extracellular albumin showed no consistent correlation with the presence of fenestrations in vessels. A variable degree of positivity to GFAP and S-100 protein was seen in the tumours associated with both types of PPs with no clear difference in pattern.     

Primitive neuroectodermal tumor of the testis. Report of a case

A 30-year-old man died of a testicular tumor with widespread metastases. Both the right testis and the adjoining epididymis were replaced by a mass that consisted mainly of small cells, but differentiated focally into medullary tubules, ependymal rosettes, and glia in which glial fibrillary acidic protein was demonstrated; rare foci of cartilage and cellular mesenchyme established the teratomatous nature of the neoplasm. We believe this is the first reported case of a primitive neuroectodermal tumor of the testis.