ZFTA (Zinc Finger Translocation Associated) Fusion in Supratentorial Ependymomas: Low Prevalence in South Asians and No Correlation with Survival.

BACKGROUND: Supratentorial ependymomas (STEs) are an aggressive group of ependymomas, topographically distinct from their posterior fossa and spinal counterparts. Zinc finger translocation associated (ZFTA) fusion-positive cases have been reported to account for the majority of STEs, although data on its association with poorer outcomes are inconsistent. MATERIALS AND METHODS: We assessed the prevalence of the ZFTA fusion by reverse-transcription polymerase chain reaction and fluorescence in situ hybridization in a cohort of 61 patients (68 samples) with STE. Our primary outcome was to determine the role of the ZFTA fusion on progression-free and overall survival of patients with STE. Our secondary objectives were to assess the impact of ZFTA fusion on nuclear factor (NF)-kB pathway signaling via surrogate markers of this pathway, namely COX-2, CCND1, and L1 cell adhesion molecule. RESULTS: ZFTA fusion was noted in 21.3% of STEs in our cohort. The presence of this rearrangement did not significantly impact the progression-free or overall survival of patients with STEs and was not associated with upregulation of markers of the NF-kB pathway. Only gross total resection was significantly associated with better progression-free survival. CONCLUSIONS: In contradiction to previous reports from across the world, the ZFTA fusion is far less prevalent among our population. It does not appear to drive NF-kB signaling or significantly affect outcomes. Gross total resection must be attempted in all cases of STE and adjuvant radiation and/or chemotherapy employed when gross total resection is not achieved.

A novel neuromodulation technique for the rehabilitation of balance and gait: A case study.

Cranial-nerve non-invasive neuromodulation (CN-NINM) through the tongue has been proposed as an adjuvant intervention to improve efficacy of rehabilitation. However, CN-NINM effects have only been explored in multiple sclerosis and stroke populations. In this report we used CN-NINM during a 2-week (2 × 1.5 h sessions daily) physiotherapy program for the rehabilitation of a 57 y/o woman presenting with balance and gait impairments after a surgical resection of a fourth ventricular ependymoma. Clinical and instrumented balance and gait assessments showed improved performance in all tests and without adverse effects This study shows the beneficial effects and feasibility of combined physiotherapy and CN-NINM in this patient.

Ependymoma in adults: Local experience with an uncommon tumour.

This study reviews our tertiary hospital experience in an adult population of ependymoma patients. Ependymomas are uncommon tumours of the central nervous system (CNS) and the literature provides little information to guide management and predict prognosis. The prospectively maintained Australian Comprehensive Cancer Outcomes and Research Database of CNS tumours was searched for patients diagnosed with ependymomas at the Royal Melbourne Hospital between January 2008 and December 2013. A total of 39 adult patients with ependymoma were identified, including 13 with spinal myxopapillary ependymoma. The mean age at diagnosis was 44 years. All patients underwent surgical resection, 67% of whom had a gross macroscopic resection. Postoperative adjuvant radiotherapy was administered to 11 patients (30%), two (5%) died from progressive disease and seven (18%) developed recurrent disease. Our findings are consistent with the existing literature for patient demographics and the approach to treatment, whilst our clinical outcomes appear more favourable. This study provides the basis for further and necessary research, including determination of the molecular characterisation of these tumours and the identification of prognostic and predictive biomarkers and treatment targets.

Time-dependent structural changes of the dentatothalamic pathway in children treated for posterior fossa tumor.

BACKGROUND AND PURPOSE: Injury to the dentatothalamic pathway that originates in the cerebellum has been suggested as a mechanism for neurologic complications in children treated for posterior fossa tumors. We hypothesized that time-dependent changes occur in the dentatothalamic pathway. MATERIALS AND METHODS: Diffusion tensor evaluation was performed in 14 children (median age, 4.1 years; age range, 1-20 years) who underwent serial MR imaging at 3T as part of routine follow-up after posterior fossa tumor resection with or without adjuvant therapy. Tensor metrics were obtained in the acute (≤1 week), subacute (1 to <6 months), and chronic (≥6 months) periods after surgery. We evaluated the following dentatothalamic constituents: bilateral dentate nuclei, cerebellar white matter, and superior cerebellar peduncles. Serial dentate nuclei volumes were also obtained and compared with the patient's baseline. RESULTS: The most significant tensor changes to the superior cerebellar peduncles and cerebellar white matter occurred in the subacute period, regardless of the tumor pathology or therapy regimen, with signs of recovery in the chronic period. However, chronic volume loss and reduced mean diffusivity were observed in the dentate nuclei and did not reverse. This atrophy was associated with radiation therapy and symptoms of ataxia. CONCLUSIONS: Longitudinal diffusion MR imaging in children treated for posterior fossa tumors showed time-dependent tensor changes in components of the dentatothalamic pathway that suggest evolution of structural damage with inflammation and recovery of tissue directionality. However, the dentate nuclei did not show tensor or volumetric recovery, suggesting that the injury may be chronic.

Cervical ependymoma in a male adolescent with neck and back pain.

OBJECTIVE: This case study addresses the clinical presentation, imaging manifestations, and management of an intramedullary ependymoma in an adolescent who presented for chiropractic evaluation with severe neck and back pain. The atypical manifestations of this disorder are emphasized. CLINICAL FEATURES: A 16-year-old male adolescent presented with severe neck and back pain and diffuse paresthesia extending into the dorsum of the forearm and wrist bilaterally. Magnetic resonance imaging revealed an intramedullary mass extending from C1 to C7. Biopsy of this lesion indicated a grade III intramedullary ependymoma. INTERVENTION AND OUTCOME: The patient underwent a successful resection of the tumor with minimal neurological deficit. At 4 months after resection, the follow-up examination yielded minimal discomfort in the neck and upper back, however there was severe cervical kyphosis. Postoperative magnetic resonance imaging revealed no evidence of intramedullary lesion. CONCLUSION: Although it is a rare and slow growing neoplasm, early detection is critical for optimal postoperative functional outcome that is directly related to the preoperative functional status.

TENS and FES for sensory impairment and gait dysfunction following removal of spinal cord ependymoma--a case report.

BACKGROUND: Sensory deficits are commonly reported following the resection of spinal cord tumours. The use of transcutaneous electrical nerve stimulation (TENS) as augmented sensory input is described in the research literature but rarely in the clinical literature. Functional electrical stimulation (FES) is used for people with motor impairments rather than sensory impairments. METHOD AND RESULTS: This case report describes the use of TENS and FES for a patient with severe sensory loss and mild weakness in the right leg following the removal of an intramedullary spinal cord tumour. The patient was able to walk more quickly and more confidently when using TENS and FES in combination. She consistently reported greater benefits from TENS alone compared to FES alone and continued to use TENS delivered via a sock electrode at six months after surgery. CONCLUSION: The use of TENS as a sensory stimulus was an invaluable component of this patient's treatment, allowing her to engage in a more challenging balance and gait programme at an earlier stage in her rehabilitation. Combining FES with TENS was also useful and allowed treatment to address motor and sensory impairments concurrently during functional activity.

[Intracranial ependymomas in adult patients. Prognostic factors, place of surgery and complementary treatment]. / Ependymomes intracrâniens de l'adulte. Facteurs pronostiques, place de la chirurgie et du traitement complémentaire.

Prognostic factors and optimal therapy for adult intracranial ependymoma are still debated. Available data has been generally collected from retrospective and heterogeneous series including pediatric and spinal cases in particular. The goal of this article is to review and discuss the prognostic factors and the various therapeutic strategies suggested in the literature in light of prognostic and decision making features delineated from a multicentric study conducted in France.

Ependymoma of the spinal cord presenting in a chiropractic practice: 2 case studies.

OBJECTIVE: The purpose of this study is to discuss the cases of 2 patients with previously undiagnosed primary spinal cord tumors presenting in a private chiropractic clinical setting. An overview of treatment and outcome for an ependymoma at T12-L1 and L1-L2 is discussed. CLINICAL FEATURES: One patient was a 46-year-old Hispanic woman with 3 to 4 years of intermittent backache that usually resolved with conservative care but failed to do so during an acute episode. Lower motor neuron signs, including bowel and bladder dysfunction, were revealed upon clinical assessment. The second patient, a 38-year-old white man under routine treatment, had no lower motor neuron signs or symptoms. INTERVENTION AND OUTCOME: Both patients were referred, one to a local hospital emergency department and the other directly to a neurosurgeon. Both underwent surgery. Upon returning home, the first patient received follow-up treatment primarily consisting of radiation therapy. Follow-up telephone interviews (3, 6, 12, 24, and 40 months) revealed the patient doing well. The second case did not require radiation therapy and was doing well at 4, 10, 12, and 18 months; the patient returned for unrelated treatment 1 year after the surgery. CONCLUSION: These cases show that with a careful history and patient examination, enough clinical data may be gathered to make an accurate health care determination under various conditions. It also illustrates the importance of interprofessional cooperation for various disciplines of health care providers regardless of training or specialty.

[Integrated (biopsychosocial) treatment in the family practice: a pregnant patient with a sacral tumor and severe posttraumatic stress disorder]. / Integrale (biopsychosociale) behandeling in de praktijk: een zwangere patiënte met een sacrumtumor en een ernstige posttraumatische stressstoornis.

In a 25-year-old woman pregnant for the second time after a successful first pregnancy, a locally aggressive, invasive sacrum tumour was diagnosed. The execution of the necessary but potentially mutilating surgical procedures was seriously hampered even during the preparative phase, in spite of the conscious wish of the patient to comply, by her severe psychiatric problems (posttraumatic stress disorder with dissociative symptoms). The Psychiatric Consultation Service took over the case management and an integrated (biopsychosocial) diagnostic investigation was carried out, involving analysis of the problems on four system levels: the biological, the psychological, the social and the health care level. An integrated treatment plan was drafted. By collaboration of the entire multidisciplinary treatment team conditions were secured under which patient would let herself be treated. In this way she was enabled to undergo the necessary procedures, with good results.

Pure ovarian ependymoma: report of a case treated with surgery, chemotherapy, irradiation and hyperthermotherapy.

Ependymomas usually develop from neuroectodermal organs. Pure ovarian ependymoma is an extremely rare tumor. We report a patient with ovarian ependymoma who died at the age of 28, 9 years after initial surgery and subsequent intensive combination therapy (chemotherapy, irradiation and hyperthermotherapy) for repeated relapses and metastatic tumors. The diagnosis was confirmed by histopathological and immunohistochemical studies. For recurrent and persistent ependymoma, a combination of the treatment modalities described above is suggested to be beneficial in attenuating the rapid progress and spread of this disease.

Results of a multicenter retrospective study on pediatric brain tumors in Italy.

This retrospective study was undertaken to evaluate the clinical characteristics, course and treatment of children (0-14 years of age) diagnosed with a primary CNS tumor during the period 1976-1982 in Italy. Four hundred and sixty-two patients (263 males and 199 females) were followed by 18 various neurosurgical and pediatric oncology centers. The histologic types most frequently reported were: medulloblastoma (23%), astrocytoma (16%), ependymoma (11%) and spongioblastoma (11%). Of the 388 patients who underwent surgery, radical excision was reported in 42%, partial excision in 32%, biopsy only in 6%, and unqualified surgery in 4%; 19% had no surgery. Radiotherapy and chemotherapy combined were administered in 61% of the 143 patients followed at pediatric oncology centers; 19% received radiotherapy alone, 3% chemotherapy alone, and 17% neither treatment. Forty-six percent of the patients were reported alive, 40% dead, and 14% lost to follow-up. Performance status was identified for 62 patients. The investigation revealed marked differences in the therapeutic treatment administered, thus precluding valid data analysis. This emphasizes the need to coordinate efforts among the institutions and the disciplines involved in the treatment of this form of childhood cancer.