Myxopapillary ependymoma with anaplastic features

A case of myxopapillary ependymoma with anaplastic features in 15-year-old boy is reported. The tumor was located in the intradural space extending to the 12th thoracic to 2nd lumbar vertebral level. It was excised with the accompanying spinal arch of the T12 to L2 vertebra. At operation, the tumor was not attached to the surrounding soft and bony tissues. The tumor, measuring 49 x 19 x 15 mm, was brownish-yellow in color and involved the conus medullaris and filum terminale. Histologically, the tumor was composed of biphasic features of a hypercellular papillary growth area and a hypocellular myxoid area. In the papillary growth area, ependymal rosettes and perivascular pseudorosettes were observed. These findings were consistent with those of a myxopapillary ependymoma, although multiple foci of punctate necrosis within the tumor and proliferation of endothelial cells showing glomeruloid structures were observed. Many mitotic figures were also observed. In addition, the Ki-67 labeling index of tumor cells was 10.1%. These findings are unusual for myxopapillary ependymoma, and therefore, it appeared that the diagnosis of myxopapillary ependymoma with anaplastic features was appropriate.     

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).     

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.     

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.     

Fine-needle aspiration of metastatic sacrococcygeal myxopapillary ependymoma

A 45-yr-old white woman with a 24-yr history of sacrococcygeal myxopapillary ependymoma developed a large metastasis of scalp and skull diagnosed as metastatic ependymoma on fine-needle aspiration, based on cytologic features, histologic pattern in cell block fragments, and a positive reaction with the glial fibrillary acidic protein immunoperoxidase study. The fine-needle study obviated the need for biopsy in this case, for which a surgical approach was considered to be inappropriate due to the extent of the process demonstrated by various imaging techniques.     

Myxopapillary ependymoma of the sacrococcygeal region. Report of a case

A case of subcutaneous myxopapillary ependymoma arising in the sacrococcygeal region of a 42-year-old woman is reported. Less than 60 cases of such tumors have so far been described in locations outside the central nervous system. Sacrococcygeal ependymomas are locally aggressive tumors that require a complete and wide surgical excision to prevent recurrences. They tend to metastasize in about 17% of cases.     

MYXOPAPILLARY EPENDYMOMA OF THE LATERAL VENTRICLE

Reported was the first case of myxopapillary ependymoma arising from the right lateral ventricle of a 29-year-old Japanese male. The histological and ultrastructural findings were identical to those from the filum terminale.
It was suggested that insudation of plasma proteins found within hyaline-thickened blood vessels in the tumor stroma represented the presence of previous long-standing anoxia and thus, circulatory disturbance would be closely related to the formation of the stromal myxoid change.     

Myxopapillary ependymoma of the lateral ventricle. A study on the mechanism of its stromal myxoid change

Reported was the first case of myxopapillary ependymoma arising from the right lateral ventricle of a 29-year-old Japanese male. The histological and ultrastructural findings were identical to those from the filum terminale. It was suggested that insudation of plasma proteins found within hyaline-thickened blood vessels in the tumor stroma represented the presence of previous long-standing anoxia and thus, circulatory disturbance would be closely related to the formation of the stromal myxoid change.     

Extradural sacrococcygeal myxopapillary ependymoma

Histological and electron microscopic evidence has been obtained on a rare tumor--extradural sacrococcygeal myxopapillary ependymoma which was detected in two boys. A 5-year-old had a malignant recurrent tumor localized presacrally and metastatic to the lungs. The other boy aged 9 developed a benign subcutaneous tumor.     

Subcutaneous myxopapillary ependymoma presenting as a childhood sacrococcygeal tumor: a case report

Subcutaneous myxopapillary ependymoma in a sacrococcygeal location is an uncommon lesion. We report such a case in a 16-mo-old female child, who presented with a sacrococcygeal mass since birth. The cytological picture was that of a malignant small round cell tumor and the diagnosis was missed on cytology, which was retrospectively confirmed on comparison with histology. Although rare, this lesion can be a potential diagnostic pitfall and needs to be distinguished from other malignant tumors occurring at this age and at a similar location, like sacrococcygeal teratoma with immature elements, primitive neuroectodermal tumor (PNET), and PNET with ependymal differentiation.     

Mediastinal myxopapillary ependymoma primary or late metastases of paracoccygeal ependymoma: a case report

A 25-year-old woman presented in 2002 with progressive shortness of breath and weight loss. A computed tomographic scan of the chest showed a huge anterior mediastinal mass, and pathological examination of a mediastinoscopic needle biopsy revealed typical myxopapillary ependymoma, an extremely unusual diagnosis at this site. Further workup and questioning of the patient revealed that she had opted not to disclose a history of surgery for right gluteal fold mass in 1993, which was primarily treated with surgery followed by radiotherapy for relapse. Review of the histology of the excised mass showed a myxopapillary ependymoma, similar to current histology. Clinical examination of the local gluteal and paracoccygeal site, computed tomographic imaging, and magnetic resonance imaging failed to demonstrate any evidence of recurrent disease in soft tissue or bone. The case is presented, and this very unusual presentation is discussed.     

Metastatic myxopapillary ependymoma: Report of a case with fine-needle aspiration findings

A case of recurrent and metastasizing myxopapillary ependymoma of the sacral region in a 35-yr-old man is reported. Fifteen years after the original diagnosis, he presented with an abdominal mass, subcutaneous nodules in the previous surgical excirion line, and bilateral inguinal lymph node enlargement. FNA cytology of the inguinal lymph nodes showed a poorly cohesive, highly cellular smear pattern exhibiting papillary formations and rosette-like structures composed of slim columnar cells having cytoplasmic processes without evidence of atypia. Histologic and ultrastructural findings confirmed the diagnosis. Chromosomal analysis was also done. © 1994 Wiley-Liss, Inc.     

Clonal Telomeric Fusions and Chromosome Instability in a Subcutaneous Sacrococcygeal Myxopapillary Ependymoma

Subcutaneous sacrococcygeal myxopapillary ependymoma (SSME) is a very rare neurologic tumor with no demonstrable connection to the spinal column. Little is known of its etiology, clinical characteristics, or cytogenetics. Giemsa-band analysis revealed a stemline karyotype showing 62 chromosomes. Sidelines within the tumor showed clonal telomeric fusions resulting in dicentric chromosomes involving the fusion of numerous chromosomes. Recurrent telomeric fusions resulted in the progressive deletion of chromosome bands 11q25 and 11q23 and subsequently the entire long arm. This is the first case of a SSME to show clonal cytogenetic aberrations. However, of greater interest is the demonstration of the clonal progression of telomeric fusions resulting in dicentric chromosomes and the subsequent loss of chromosome arms. The observation of clonal telomeric breakage/fusion cycles as progenitor lesions to subsequent deletions provides evidence for telomeric association as an intermediate step in the progression of chromosomal instability.     

Ependymoma of the mediastinum

A case of primary ependymoma of the mediastinum is reported. The tumor was adherent to the lung and metastasized to adjacent mediastinal lymph nodes. An autopsy showed no evidence of tumor in the central nervous system. The diagnosis of ependymoma was confirmed by the immunohistochemical positivity for glialfibrillary acidic protein. To the best of our knowledge, this is the first reported example of an ependymoma in this location.     

Tanycytic ependymoma in a 76-year-old Puerto Rican male

Ependymoma is a slowly growing tumor in children and young adults originating from the wall of the ventricles or from the spinal canal that is composed of neoplastic ependymal cells. Tanycytic ependymoma is a rare variant of ependymoma usually arising in the intra medullary spine. The World Health Organization classifies the tanycytic ependymoma as a grade II tumor. The diagnosis of tanycytic ependymoma is challenging since the morphology of the lesions resemble those found in schwannoma and astrocytomas. In the present study, we show a case of a 76 years old male with a progressive paraparesis for 8 years, due to a spinal tumor. Radiological and histological studies were used to classify the tumor as tanycytic ependymoma. Therefore, it is important to be aware of tanycytic ependymoma and its immunohistochemistry profile in older patients, especially within the Caribbean Hispanic population. To our knowledge this is the oldest patient known to have this rare tumor and the first case reported in Puerto Rico.     

Fine-needle aspiration diagnosis of metastatic anaplastic sacrococcygeal ependymoma to the lungs

Ectopic ependymomas are uncommon neoplasms, and most of them occur in the sacrococcygeal area. They usually present as subcutaneous sacral masses. The most common histological subtype is the myxopapillary. We describe a case of anaplastic sacrococcygeal ependymoma metastatic to the lungs diagnosed by fine-needle aspiration biopsy. Diagn Cytopathol 1996;15:228–230. © 1996 Wiley-Liss, Inc.     

Mucin-secreting cellular ependymoma: a light and electron microscopy study

Mucin accumulation in ependymomas is thought to be limited to the myxopapillary variant and represents an important diagnostic feature. Similarly, signet-ring cells in ependymomas have been shown by electron microscopy to represent microrosette instead of mucin secretion. This study describes an infratentorial ependymoma largely composed of mucinous areas and signet-ring cells. The ependymal nature of mucin-secreting cells was confirmed by ultrastructural analysis. This case widens the variable spectrum of ependymal morphology. The value of electron microscopy in differentiating central nervous system neoplasms showing mucous secretion is stressed.     

Extraspinal ependymoma of the broad ligament

Extraspinal ependymoma is a rare tumor, occurring most commonly in the sacrococcygeal region, and only a small number of cases have been reported to arise in the uterine ligament. Herein is reported a case of extraspinal ependymoma arising in the broad ligament of a 27‐year‐old woman. The lesion was 14 cm in diameter with an intra‐abdominal implant in the omentum. On cut section the tumor was found to be solid, and demonstrated hemorrhaging, necrosis, myxoid foci, and central cystic spaces. Microscopically the tumor was composed of a proliferation of short spindle or polygonal cells arranged in short fascicles or in a solid sheet‐like fashion with occasional perivascular pseudorosettes, together with myxoid areas and variable histological architectures exhibiting cribriform, pseudopapillary, and variable‐sized cystic patterns. On immunohistochemistry most tumor cells were positively reactive to glial fibrillary acidic protein (GFAP), CD99, estrogen receptor, and progesterone receptor. The patient has remained disease‐free for 6 months after the adjuvant chemoradiotherapy. Extraspinal ependymoma should be considered as a differential diagnosis when examining unusual intrapelvic tumors, especially in young female patients. The identification of characteristic histological features such as perivascular pseudorosettes and immunohistochemical expression of GFAP are helpful for confirming the diagnosis.     

Microtubular aggregates within rough endoplasmic reticulum in myxopapillary ependymoma of the filum terminale

Aggregates of microtubules with-in rough endoplasmic reticulum were found in many neoplastic cells of three cases of myxopapillary ependymoma of the filum terminale studied ultrastructurally. The cytoplasm and cellular processes of some neoplastic cells were distended by the aggregates. In general the involved rough endoplasmic reticulum contained three to six microtubules, but some enclosed more than 10 microtubules. The enclosed microtubules were straight parallel hollow cylindrical structures with fuzzy coats. They had an inner diameter of 12 to 15 nm, an outer diameter of 30 to 35 nm, and a center-to-center distance of 50 to 62 nm. The involved rough endoplasmic reticulum often showed various degrees of loss of surface ribosomes and some appeared totally degranulated. Vacuolar degeneration of involved rough endoplasmic reticulum with fragmentation and disintegration of the enclosed microtubules was frequent. Direct relationship of the enclosed microtubules to the cytoplasmic microtubules and ciliary formation was not found. This unusual microtubular aggregate has not been described in other types of ependymoma, or in other brain tumors. They may represent a characteristic ultrastructural feature of myxopapillary ependymoma.     

cIMPACT‐NOW update 7: advancing the molecular classification of ependymal tumors

Advances in our understanding of the biological basis and molecular characteristics of ependymal tumors since the latest iteration of the World Health Organization (WHO) classification of CNS tumors (2016) have prompted the cIMPACT‐NOW group to recommend a new classification. Separation of ependymal tumors by anatomic site is an important principle of the new classification and was prompted by methylome profiling data to indicate that molecular groups of ependymal tumors in the posterior fossa and supratentorial and spinal compartments are distinct. Common recurrent genetic or epigenetic alterations found in tumors belonging to the main molecular groups have been used to define tumor types at intracranial sites; C11orf95 and YAP1 fusion genes for supratentorial tumors and two types of posterior fossa ependymoma defined by methylation group, PFA and PFB. A recently described type of aggressive spinal ependymoma with MYCN amplification has also been included. Myxopapillary ependymoma and subependymoma have been retained as histopathologically defined tumor types, but the classification has dropped the distinction between classic and anaplastic ependymoma. While the cIMPACT‐NOW group considered that data to inform assignment of grade to molecularly defined ependymomas are insufficiently mature, it recommends assigning WHO grade 2 to myxopapillary ependymoma and allows grade 2 or grade 3 to be assigned to ependymomas not defined by molecular status.