We report a case of myxopapillary ependymoma presenting as a primary tumor of the subcutaneous tissue in the sacrococcygeal region. However, surgery is typically less curative in tumors that are large, multifocal or extend. Spinal myxopapillary ependymoma in an adult male presenting. Nov 17, 2015 myxopapillary ependymoma mpe is a rare subtype of ependymoma that develops almost exclusively within the spinal cord. Three years later, she presented with back pain and hypoesthesia of the left upper limb. Pfb15 posterior fossa group b ependymoma, subtypes 15. Mr characteristics of histopathologic subtypes of spinal ependymoma article pdf available in american journal of neuroradiology 171.
Pdf mr characteristics of histopathologic subtypes of. Longterm outcome of patients with spinal myxopapillary. Although variables influencing the prognosis, such as age, the extent of surgery and radiotherapy, have been widely discussed, no definitive standard has been established. Despite the benign histology and slowgrowing nature of most. Spinal ependymoma s make up 2440% of all spinal tumours depending on the age at diagnosis, are the most common spinal glial tumour and occur especially in adults oh 2014, engelhard 2010. Multiple primary and histology coding rules table of contents. Blood vessels medicine india heart vessels medical. Myxopapillary ependymoma genetic and rare diseases. Spinal tuberculoma in a patient with spinal myxopapillary. A rare case of an intramedullary metastasis of a myxopapillary. Myxopapillary ependymoma is a subtype of ependymoma that occurs almost exclusively at the cauda equina and the filum terminale of the spinal cord in middle. Despite its benign biological nature, mpe has a propensity to recur locally or distantly. Myxopapillary ependymoma is characterized by papillary formations with a mucinous core and arises most commonly in the lumbosacral spinal cord and sometimes in the soft tissues of the lumbosacral region. Less than 5% of mpes occur in sites outside the lumbar thecal sac.
Cytokeratin positivity in myxopapillary ependymoma a. Identification of relevant prognostic histopathologic features in 69. Lumbar myxopapillary ependymoma mimicking neurofibroma. The term is synonymous due to the vast majority of filum terminale neoplasms are myxopapillary. This tumor type may be cured if all of the tumor is removed during surgery, which is referred to as total resection, and there is usually a favorable outlook in these cases. Clinicopathological features of myxopapillary ependymoma. This pathology is also referred to myxopapillary ependymoma of the filum terminale. A clinicopathologic investigation was performed on 32 cases of myxopapillary ependymoma occurring as a primary tumor of the skin and subcutaneous tissue of the sacrococcygeal area. Mib1 myxopapillary ependymomas are a variant type of ependymoma that occurs predominantly in the filum terminale andor conus medullaris.
Subcutaneous myxopapillary ependymoma histopathology section1 histopathology path quiz case4. The clinicopathologic features for 14 cases of anaplastic myxopapillary ependymoma. Primary extraneural myxopapillary ependymoma of the broad. They tend to occur in the lower part of the spinal column and are usually considered to be benign, lowgrade or grade i tumors. The mass was large, wellencapsulated, lobulated, and multiseptated, with varying signal intensity on t1 and t2weighted mr images caused by hemorrhagic necrosis, blood degradation products, and. Myxopapillary ependymoma with pleuropulmonary metastases and. Apr, 2015 myxopapillary ependymoma mepn is a slowgrowing ependymoma a type of glioma, which is a tumor that arises from the supportive tissue of the brain and spinal cord. Subcutaneous myxopapillary ependymoma histopathology. The histologic variants of ependymoma are classified according to the world health organization who grading system into the following ependymal tumor types.
Ependymoma can occur at any age, but most often occurs in young children. Apr 01, 2005 metastasizing myxopapillary ependymoma metastasizing myxopapillary ependymoma al. Mar 06, 2020 webpathology is a free educational resource with 10197 high quality pathology images of benign and malignant neoplasms and related entities. Apr, 2015 standard treatment of myxopapillary ependymoma is surgery with the aim of removing as much of the tumor as possible. Youll have lots of questions about how to treat the disease and manage the. Cases are ordered by age of presentation eight pediatric cases followed by six adult cases. Myxopapillary ependymomas mpes are world health organization who grade i tumors that arise from the ependyma of the filum terminale located in. Ependymoma is a type of tumor that can form in the brain or spinal cord. A unique variant of ependymoma, myxopapillary ependymoma, occurs in the distal spinal cord filum terminale and has a distinctive morphologic appearance and excellent prognosis. In most of the published series, the lumbosacral ependymomas have been classified as a distinct subgroup and regarded as a tumor of lowgrade malignancy who grade 1. Lowgrade areas of tumor figure 2a have classic histology of ependymal cells surrounding blood vessels producing a somewhat. Risk factors for intracranial ependymoma are not well defined. Tumors with pseudopapillary histology matched to the methylation class of myxopapillary ependymoma 46 cases, whereas the trabecular case was anatomically and molecularly a spinal ependymoma.
This tumor, which is a rare occurrence at this level, was observed in a 73yearold woman with no relevant medical history who presented with obstructive hydrocephalus due to compression and blockage of the foramen of monro. Myxopapillary ependymoma mpe is classified as a world health organization who grade i glioma, located almost exclusively in the region of the conus medullaris, cauda equina, and filum terminale of the spinal cord. A minority arise from the filum terminale or conus medullaris, and are histologicallyclassifiedasmpes,accountingfor5%ofall ependymomas 1. More than one size or location is listed for cases with recurrences, separated by commas. Immunohistochemical study of cd99 and ema expression in. In childhood ependymoma, cancer forms in the tissues of the brain and spinal cord. Lino fonseca, marta cicuendez, francisco martinezricarte, elena martinezsaez, esteban cordero, agustin bescos. In some studies, pediatric patients have a less predictable outcome than their adult counterparts, even after gross total resection 21, 31, 35. Dec 6, 20 subcutaneous myxopapillary ependymoma histopathology section2 histopathology path quiz case4. They represent % of all spinal ependymomas and are the most common tumors of the cauda equina region. Mr characteristics of histopathologic subtypes of spinal. Histopathologic features have been welldescribed in the literature whereas cytological findings have been sporadically reported by various authors mainly as case reports. The diagnosis of mpe should be made considering the histology. Hana mrazkova, department of surgery, university hospital motol, v uvalu 84, 150 06 prague, czech.
Myxopapillary ependymoma of the sacrum rsna publications. Myxopapillary ependymoma 93941 choroid glioma 94441 neurofibromas nerve sheath tumors. If you learn that you or your child has a type of spine and brain cancer called an ependymoma, theres a lot to take in. Pathology, hospital universitari vall dhebron, barcelona, spain. The results of surgery, with or without radiotherapy, for primary spinal myxopapillary ependymoma. Case report pathological features of myxopapillary ependymomas.
Myxopapillary ependymoma, a benign special variant of ependymoma that is thought to arise from ependymocytes of the filum terminale, constitutes approximately % of all spinal ependymomas in this region. Review article ependymoma diagnosis and treatment progress xiwei zhang 1, xueyong wu, xiaofang sheng2, yang wang2, hongyuan gao1, li xu, yueming zhu 1department of oncology, jingan district center hospital of shanghai huashan hospital affiliated to fudan. Review article ependymoma diagnosis and treatment progress. It is considered as degenerative phenomenon in otherwise typical myxopapillary lesion. We report the case of a mpe with anaplastic features in a 24yearold female who presented with a dominant lumbar mass along with intracranial and sacral metastases. Subtotal resection, lack of initial radiation treatment and a young age at diagnosis are factors that have been associated with worse prognosis 17, 39. Ependymal tumors are glial neoplasms believed to arise from ependymal cells that line the ventricular system. It is a rare tumor that arises from ependymal cell.
Myxopapillary ependymoma mpe is a rare and distinctive tumor which occurs in the sacrococcygeal area of young adults and children, often intradural in location. Myxopapillary ependymomas mpe occur in the filum terminale of the spinal cord, but also present in extraspinal locations such as subcutaneous tissue and brain. Subependymoma, is most commonly an intraventricular 4th ventricle tumor of adults. To report a case of lumbar myxopapillary ependymoma in whom neuroradiological and surgical findings strongly suggested neurofibroma. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Mr imaging was valuable in identifying the extent of tumors and in defining their relationship to the intraspinal structures. We present morphological findings observed from the study of a myxopapillary ependymoma located in the iii ventricle. About 10% of ependymomas are benign myxopapillary ependymoma mpe. Hussaini department of pathology, jordan university hospital, amman, jordan, department of pathology, royal group of hospitals trust, belfast, northern ireland. Ependymoma prognosis, survival rate, symptoms, anaplasti. Myxopapillary ependymoma mpe with anaplastic features is extremely rare, with only three case reports in the literature. Myxopapillary ependymoma is a benign slowgrowing tumour, arising predominantly in the region of the filum terminale.
Historically, ependymoma was believed to disseminate throughout the neuraxis, and csi became the standard management approach however, more recent retrospective studies have shown that failure is primarily local in 90%. Pilonidal cyst was the most frequent clinical diagnosis. It has been designated histologically as grade i neoplasm according to the. Case courtesy of associate professor renate kalnins. Subcutaneous sacrococcygeal myxopapillary ependymoma. Myxopapillary ependymoma mpe was first described as a distinct subtype of ependymoma by kernohaw in 1932. Childhood ependymoma treatment pdqhealth professional. Find out about the molecular and clinical features, diagnosis, prognosis, treatment, and clinical trials for pediatric ependymoma, subependymoma, myxopapillary ependymoma, anaplastic ependymoma, and rela fusionpositive ependymoma. Radiologic pathology has judged the best cases by organ system, and recognition is given to the winners on the. Ependymomas may occur anywhere along the neuroaxis and grade 1 tumors myxopapillary ependymomas and subependymomas are biologically distinct from grade 2 low grade and grade 3 anaplastic ependymomas. The mass was large, wellencapsulated, lobulated, and multiseptated, with varying signal intensity on t1 and t2weighted mr images caused by hemorrhagic necrosis, blood degradation products, and calcification. Typically in myxopapillary lesions solid, round structures with prickly appearance on reticulin stains are present which are referred as balloons giant cell ependymoma. Standard treatment of myxopapillary ependymoma is surgery with the aim of removing as much of the tumor as possible.
Myxopapillary ependymomas must be distinguished from chordomas tumors of notochord remnants, which also occur at this location. Myxoid material is present within the pseudorosettes, in many places forming microcysts. Clinicopathologic features of anaplastic myxopapillary. Although the mr findings in myxopapillary ependymoma are nonspecific, the diagnosis can be suggested by a large, intensely enhancing, intradural extramedullary thoracolumbar mass that extends for several vertebral levels. Ependymomas are derived from the ependymal cells in the central nervous system, and are centrally located in the pia mater and spinal fluid. Grade ii ependymomas are low grade tumors and can occur in either the brain or the spine. It usually appears as an oval or sausageshaped contrastenhancing mass, like the one pictured in this mri image sagittal postcontrast t1 fatsat. It should be remembered that these symptoms also are prevalent in many other illnesses not associated with ependymoma. Cronicon open access ec neurology case report intraspinal extradural myxopapillary ependymoma in upper thoracic location jf martinezcanca and pablo martinezsoler department of neurosurgery, chip advanced hospital grouptm avda. Ependymoma prognosis, survival rate, symptoms, anaplastic, myxopapillary, causes, symptoms, definition. Areas of solid growth pattern with aggregates of cells with epithelioid morphology seen in mpe can mimic metastatic carcinoma. Radiation oncologycnsependymoma wikibooks, open books for. Multiple primary and histology coding rules table of contents i. The subtypes include subependymoma and myxopapillary ependymoma.
The presence of occasional cells with clear cytoplasm and. The presurgical differentiation between ependymomas and astrocytomas is beneficial. Pathological features of myxopapillary ependymomas in lumbar spinal canal. Spinal ependymomas are rare in children and more common in adults, with a median age at diagnosis of 30 to 40 years. A rare case of an intramedullary metastasis of a myxopapillary ependymoma. There is an increased incidence of intramedullary spinal cord ependymoma in patients with neurofibromatosis type 2 nf2. Myxopapillary ependymoma mepn is a slowgrowing ependymoma a type of glioma, which is a tumor that arises from the supportive tissue of the brain and spinal cord.
Methods the mr images from 26 cases of pathologically proved spinal ependymomas were evaluated with respect to seven criteria. Tumor cells are arranged in diffuse sheets between pseudorosettes. Myxopapillary ependymoma radiology reference article. The designation cellular ependymoma is depreceated. Mib1 oct 09, 2014 myxopapillary ependymoma mpe is classified as a world health organization who grade i glioma, located almost exclusively in the region of the conus medullaris, cauda equina, and filum terminale of the spinal cord. Ependymomas are common spinal tumours, with an average age of 35years and a male predilection 1, 2. Myxopapillary ependymomas are a variant type of ependymoma that occurs predominantly in the filum terminale andor conus medullaris. Of the 8 patients with positive andor suspicious diagnoses, 5 ependymomas exhibited anaplastic features and 1 tumor was a myxopapillary ependymoma.
Head and neck multiple primary 277 head and neck histology 279. Please wash your hands and practise social distancing. Myxopapillary ependymoma is a slowgrowing tumour, arising predominantly in the region of the filum terminale. Cerebrospinal fluid cytology in patients with ependymoma. Spinal myxopapillary ependymomas tend to manifest in adulthood mean age, 35 years and is more commonly seen in male patients. The tumor usually presented as an asymptomatic mass at the base of the spine in young individuals. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at m. Metastasizing myxopapillary ependymoma, histopathology 10. Myxopapillary ependymoma arises in the lumbar region of the spinal cord and typically produces symptoms associated with impingement of the spinal nerve roots of the cauda equina. There are 2 who grade i special variants of ependymoma. Distribution of age groups in site and histology categories for all primary brain and cns ependymal tumours, cbtrus analytic file, 20042009. Final diagnosis mixopapillary ependymoma, who grade 1.
Oct 19, 2008 myxopapillary ependymomas mpe occur in the filum terminale of the spinal cord, but also present in extraspinal locations such as subcutaneous tissue and brain. A 19yearold female was diagnosed and treated for lumbosacral myxopapllary ependy moma mpe. Myxopapillary ependymoma mpe was first described as a distinct subtype. Histopathologic features have been welldescribed in the literature whereas cytological findings have been sporadically reported by various authors mainly as. Myxopapillary ependymoma with pleuropulmonary metastases and high plasma glial fibrillary acidic protein levels introduction myxopapillary ependymoma is a rare glial. Purpose to examine mr characteristics and enhancement patterns of spinal ependymomas and compare these data with histopathologic subtypes.
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