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Child's Nervous System : ChNS :... Jun 2023Exceedingly refractory, pediatric anaplastic ependymoma in many cases requires multisurgical removal. The high risk of poor wound healing and CSF leakage especially at...
OBJECTIVE
Exceedingly refractory, pediatric anaplastic ependymoma in many cases requires multisurgical removal. The high risk of poor wound healing and CSF leakage especially at the posterior fossa make this tumor difficult to treat.
CASE
A 9-year-old girl has had 4th ventricular anaplastic ependymoma since the age of 3. She experienced tumor removal 8 times including 4 posterior fossa craniotomies because tumors were disseminated not only to the posterior fossa but also to the cerebral hemispheres. She also underwent a dermal graft using a free flap. She experienced CSF leaks and meningitis frequently because the wound healing was poor. We performed a dermal flap closure using a pedicle trapezius muscle flap with a plastic surgeon when we performed the 5th tumor removal.
RESULT
We achieved complete wound closure in spite of broad deficiencies in subcutaneous and epidermal tissues. After that, recurrences of posterior fossa tumors presented within a short term, and tumor removal via an incision of a pedicle trapezius muscle flap was performed without recurrence of CSF leaks and meningitis.
DISCUSSION AND CONCLUSION
For the first time, we are able to report on the efficacy of using the pedicle trapezius muscle flap for multisurgical removal of pediatric posterior fossa anaplastic ependymoma. The muscle flap was found to be effective because of the multiple surgeries expected, and the pedicle trapezius muscle flap was found to be resilient to multiple surgical procedures. Although advantageous, the dorsal scapular artery which is required for flap creation is actually difficult to harvest. Compared to a flee flap, the pedicle trapezius muscle flap maintains vascular supply. Furthermore, this technique has the possibility of being applied to defective dura mater closure that cannot be watertight due to multiple surgeries. However, it is very important to inform the patient's family not only about the improved efficacy of surgery, but also to raise awareness on consequential cosmetic issues.
Topics: Female; Humans; Child; Superficial Back Muscles; Surgical Flaps; Plastic Surgery Procedures; Meningitis; Ependymoma
PubMed: 37016090
DOI: 10.1007/s00381-023-05941-0 -
Journal of Neurosurgical Sciences Aug 2017
Topics: Breast Neoplasms; Ependymoma; Fatal Outcome; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Quadriplegia; Radiotherapy; Spinal Cord Neoplasms
PubMed: 28555490
DOI: 10.23736/S0390-5616.16.03399-3 -
The Neuroradiology Journal Jun 2017Ependymal tumours in adults are rare, accounting for less than 4% of primary tumours of the central nervous system, and exceptionally metastasise outside the nervous... (Review)
Review
Ependymal tumours in adults are rare, accounting for less than 4% of primary tumours of the central nervous system, and exceptionally metastasise outside the nervous system. In this study, we present a case of anaplastic ependymoma, which developed metastases outside the nervous system less than a year after its clinical onset. A healthy 65-year-old woman suddenly presented with drowsiness of unknown origin, accompanied by ingravescent fatigue, inability to maintain the upright posture, headache, nausea and vomiting. Computed tomography study performed in the emergency department showed the presence of an inhomogeneously hypodense area, with temporoparietal extension and median line deviation. After surgical excision, performed after two days, the bioptic examination demonstrated an anaplastic ependymoma with multiple areas of necrosis. The patient was submitted to adjuvant radiation therapy. At magnetic resonance imaging follow-up, performed three months after surgery, neither local recurrences nor typical 'drop metastases' to the spinal area were observed. Three months later, magnetic resonance imaging control revealed bone metastases and recurrences in the left insula and at the ipsilateral cerebellar hemisphere. Total body computed tomography examination showed metastases in the liver, vertebrae and pelvic bones, and involvement of paratracheal lymph nodes.
Topics: Aged; Brain Neoplasms; Ependymoma; Female; Humans; Magnetic Resonance Imaging; Necrosis; Neoplasm Grading; Neoplasm Metastasis; Tomography, X-Ray Computed; Whole Body Imaging
PubMed: 28627990
DOI: 10.1177/1971400916689371 -
Obstetrics and Gynecology Feb 2014Histopathologic diagnosis and treatment of ovarian anaplastic ependymoma are challenging.
BACKGROUND
Histopathologic diagnosis and treatment of ovarian anaplastic ependymoma are challenging.
CASE
A 61-year-old-woman presented with a 10-cm right adnexal tumor associated with peritoneal carcinomatosis extending to the right diaphragm and liver surface. After initial diagnosis of a papillary serous carcinoma, we performed extensive but nonoptimal cytoreductive surgery including hysterectomy with bilateral oophorectomy. Histology revealed some axially arranged cells with a prominent fibrillary cytoplasm, suggesting an ependymoma. Diagnosis was confirmed by immunophenotype showing strong positivity to glial fibrillary acidic protein. Given the strong tumoral expression of estrogen and progesterone receptors, an aromatase inhibitor was initiated. One year later, computed tomography scan showed stability of the residual peritoneal nodules.
CONCLUSION
Aromatase inhibitor treatment could be effective in cases of extraaxial ependymoma with prominent estrogen receptor expression.
Topics: Anastrozole; Aromatase Inhibitors; Ependymoma; Female; Humans; Middle Aged; Nitriles; Ovarian Neoplasms; Triazoles
PubMed: 24413241
DOI: 10.1097/AOG.0000000000000077 -
Child's Nervous System : ChNS :... Mar 2011
Topics: Brain Neoplasms; Child; Ependymoma; Female; Humans
PubMed: 21110032
DOI: 10.1007/s00381-010-1346-0 -
Surgical Neurology Jun 1999Anaplastic ependymomas are considered to be uncommon cerebral tumors by most authors. We have had the opportunity to study 34 cases of such lesions in 13 years.
BACKGROUND
Anaplastic ependymomas are considered to be uncommon cerebral tumors by most authors. We have had the opportunity to study 34 cases of such lesions in 13 years.
METHODS
34 cases of anaplastic ependymoma operated in different hospitals of Maracaibo, Venezuela, during the period of 1983-1995 were analyzed. Cases of ependymoblastoma were excluded.
RESULTS
Adult patients made up most of the present series. All patients harbored supratentorial growths in locations distant from the ventricular system. The microscopic pattern was of limited value to establish prognosis, for there was no constant correlation between the histologic features and tumor relapse; only in sporadic cases in which high cell density and conspicuous mitotic activity were maximally expressed, did tumor relapse occur shortly after removal of the lesion.
CONCLUSION
This type of paradoxical behavior being the rule makes all attempts at predicting prognosis in these entities a disappointing task.
Topics: Adolescent; Adult; Aged; Brain Neoplasms; Chemotherapy, Adjuvant; Child; Child, Preschool; Ependymoma; Female; Humans; Male; Middle Aged; Radiotherapy, Adjuvant; Retrospective Studies; Treatment Outcome
PubMed: 10369231
DOI: 10.1016/s0090-3019(98)00118-9 -
Ultrastructural Pathology Feb 2015Ependymoma tumors likely derive from the ependymal cells lining the CNS ventricular system. In grade II ependymomas, tumor cells resemble typical ependymocytes, while...
Ependymoma tumors likely derive from the ependymal cells lining the CNS ventricular system. In grade II ependymomas, tumor cells resemble typical ependymocytes, while anaplastic ependymomas are poorly differentiated. We studied three grade II and one anaplastic ependymoma, focusing on the ciliary structures. To unambiguously characterize the ultrastructure and number of cilia, we performed electron microscopy serial section analysis of individual cells. Differentiated ependymomas contained large basal bodies and up to three cilia, and lacked centrioles. Anaplastic ependymoma cells showed instead two perpendicularly oriented centrioles and lacked cilia or basal bodies. These findings could contribute to understand the mechanisms of ependymoma aggressiveness.
Topics: Brain Neoplasms; Child; Cilia; Ependymoma; Female; Humans; Male; Microscopy, Electron, Transmission; Middle Aged; Neoplasm Grading; Young Adult
PubMed: 24830503
DOI: 10.3109/01913123.2014.906526 -
Oncology (Williston Park, N.Y.) May 2002Radiation therapy has long been a mainstay in the treatment of ependymoma. Concerns about the long-term effects of radiation therapy have made many parents and... (Review)
Review
Radiation therapy has long been a mainstay in the treatment of ependymoma. Concerns about the long-term effects of radiation therapy have made many parents and caregivers wary of this treatment modality. However, with the advent of conformal radiation and evidence supporting its use in younger children (ie, < 3 years old), the standard of care for childhood ependymoma is rapidly evolving to include immediate postoperative radiation therapy for all pediatric patients. The role of chemotherapy in the treatment of ependymoma has diminished recently because (1) chemotherapy fails to delay radiation therapy for a meaningful period of time; (2) tumors that progress during chemotherapy do not respond as well to subsequent irradiation; and (3) the combination of chemotherapy and irradiation does not improve overall survival. However, chemotherapy may make residual tumor more amenable to a second resection. Fewer than 50% of pediatric patients with ependymoma undergo complete resection before receiving radiation therapy. Because the extent of resection is one of the most important prognostic factors in the treatment of this disease, increasing the rate of complete resections is a significant means of increasing long-term survival. By incorporating current concepts of ependymoma, a more uniform approach to the treatment of this disease can be developed. In addition, by combining the best available means of detecting and managing side effects, the future for pediatric patients with ependymoma remains optimistic. This review presents historical and current practices used to treat ependymoma, and is intended to provide an information framework for caregivers so that they can assist parents in the decision-making process.
Topics: Adolescent; Adult; Antineoplastic Agents; Central Nervous System Neoplasms; Child; Child, Preschool; Combined Modality Therapy; Ependymoma; Humans; Magnetic Resonance Imaging; Neurosurgical Procedures; Radiotherapy; Reoperation; Treatment Outcome
PubMed: 12108890
DOI: No ID Found -
Brain Tumor Pathology Jan 2021C11orf95-RELA fusion or, less frequently, YAP1 fusion is recurrently detected in most cases of supratentorial ependymoma. Other fusions have rarely been reported in some...
C11orf95-RELA fusion or, less frequently, YAP1 fusion is recurrently detected in most cases of supratentorial ependymoma. Other fusions have rarely been reported in some cases of supratentorial ependymoma, and little is known about their pathological or clinical features. Here, we present a case of supratentorial ependymoma with unusual pathological findings and C11orf95-MAML2 fusion. A 23-year-old man was admitted to our hospital because of headache and vomiting. Magnetic resonance imaging revealed a cystic lesion in the right frontal lobe, and gross total resection of the tumor was performed. Pathologically, the tumor was mainly composed of typical ependymal lesions with perivascular pseudorosettes and contained some atypical lesions, with granular and ganglion cell features. The tumor was diagnosed as anaplastic ependymoma, which was classified as grade III on the World Health Organization scale, and found to be RELA fusion-positive in the DNA methylation analysis. However, the tumor was negative for C11orf95-RELA fusion, and RNA sequencing detected C11orf95-MAML2 fusion. The patient has not received adjuvant therapy and has remained alive without any evidence of disease for 30 months, suggesting that the prognosis might be better than that of typical C11orf95-RELA fusion-positive ependymoma.
Topics: Adult; DNA Methylation; Ependymoma; Gene Fusion; Humans; Male; Neoplasm Staging; Prognosis; Proteins; Supratentorial Neoplasms; Trans-Activators; Young Adult
PubMed: 33221956
DOI: 10.1007/s10014-020-00388-6 -
Neurology India 2012
Topics: Adult; Ependymoma; Glial Fibrillary Acidic Protein; Humans; Magnetic Resonance Imaging; Male; Meningeal Neoplasms; Meningioma; Supratentorial Neoplasms; Tomography, X-Ray Computed
PubMed: 22406799
DOI: 10.4103/0028-3886.93608