Surgical Outcome of Trigeminal Schwannomas

(1) Background: As resection of trigeminal schwannomas is challenging, due to anatomical involvement of the anterior, middle and posterior fossa, the appropriate approach is important. We report our experience with surgical resection of trigeminal schwannomas by simple and classic skull-base approaches. (2) Methods: We performed a retrospective single-center study including patients who underwent surgery for trigeminal schwannoma tumors between June 2007 and May 2020, concentrating on surgical technique, extent of resection, postoperative outcome and complications. (3) Results: We included 13 patients (median age 57.5 with range of 36-83 years, 53.8% (7/13) female. The most common preoperative clinical presentations were facial pain in six (46.2%), hypoacusis in four (30.8%), trigeminal nerve hypesthesia in 11 (V1 46.2% (6/13), V2 (61.5% (8/13), V3 46.2% (6/13)) and headache in three (23.1%) patients. In three cases, the tumor was resected in a two-stage technique. The intradural subtemporal approach was performed in six cases, the extradural technique in two cases, the retrosigmoid approach in five cases, a Kawase approach in two cases and the transnasal endoscopic approach in one case. The gross total resection rate was 76.9% (10/13) and the median follow-up time 24.0 (0–136) months. Three (23.1%) patients developed postoperative anesthesia in at least one branch of the trigeminal nerve. Trigeminal motor function was preserved in 11 (84.6%) patients. Ten of the 11 patients (76.9%) who reported decreased gustation, cerebellar ataxia, visual impairment, or headache improved postoperatively. Two (15.4%) patients exhibited minimal facial palsy (House and Brackmann II–III), which resolved during the follow-up. The total adverse event rate requiring surgical intervention during follow-up was 7.7%. Surgery-related mortality was 0%. (4) Conclusions: Trigeminal schwannomas are rare benign lesions with intra- and extracranial extension. Considering the low operative morbidity and satisfying functional outcome, gross total resection of trigeminal schwannomas is achievable by classic, but also individually tailored approaches. More invasive or combined techniques were not needed with meticulous surgical planning.

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Presigmoid Approach to Dumbbell Trigeminal Schwannoma

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0038-1669977 ◽

2018 ◽

Vol 79 (S 05) ◽

pp. S391-S392

Author(s):

Katherine Kunigelis ◽

Daniel Craig ◽

Alexander Yang ◽

Samuel Gubbels ◽

A. Youssef

Keyword(s):

Trigeminal Nerve ◽

Treatment Options ◽

Extent Of Resection ◽

Direct Access ◽

Petrous Apex ◽

Total Resection ◽

Middle Fossa Approach ◽

Nerve Preservation ◽

Presigmoid Approach ◽

Trigeminal Schwannoma

This case is a 15-year-old male, presenting with headaches, right face, and arm numbness, and ataxia. MRI (magnetic resonance imaging) revealed a large right sided dumbbell shaped lesion, extending into the middle and posterior fossa with compression of the brainstem consistent with a trigeminal schwannoma. Treatment options here would be a retrosigmoid suprameatal approach or a lateral presigmoid approach. Given the tumor extension into multiple compartments, a presigmoid craniotomy, combining a middle fossa approach with anterior petrosectomy, and retrolabyrinthine approach with posterior petrosectomy were used to maximize the direct access corridor for resection. The petrous apex was already expanded and remodeled by the tumor. Nerve fascicles preservation technique is paramount to the functional preservation of the trigeminal nerve. The extent of resection should be weighed against the anatomical functional integrity of the nerve. Near total resection is considered if that means more nerve preservation. Postoperatively, the patient had a slight (House–Brackman grade II) facial droop, which resolved over days and developed right trigeminal hypesthesia at several weeks. This case is presented to demonstrate a combined petrosectomy technique for resection of lesions extending into both the middle and posterior cranial fossa with near total resection and trigeminal nerve preservation.The link to the video can be found at: https://youtu.be/kA9GyFhL1dg.

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Clinical, surgical and postoperative outcome analysis of tumors occupying both posterior and middle cranial fossa

Nepal Journal of Neuroscience ◽

10.3126/njn.v18i2.33783 ◽

2021 ◽

Vol 18 (2) ◽

pp. 36-44

Author(s):

Rahul Singh ◽

Ravi Shankar Prasad ◽

Ramit Chandra Singh ◽

Kulwant Singh ◽

Anurag Sahu

Keyword(s):

Postoperative Outcome ◽

Middle Cranial Fossa ◽

Posterior Cranial Fossa ◽

Gross Total Resection ◽

Outcome Analysis ◽

Subtotal Resection ◽

Combined Approach ◽

Total Resection ◽

Trigeminal Schwannoma ◽

Cranial Fossa

Objectives: To analyse clinical, surgical and postoperative outcome perspectives of tumors occupying both middle and posterior cranial fossa. Materials and Methods: This retrospective cohort study includes 23 patients operated for tumors involving both middle and posterior cranial fossa in our department between August 2016 and August 2020. Each patient was evaluated for age, sex, co morbidities, tumour histopathology, clinical presentation, radiological characteristics, surgical and outcome characteristics. Unpaired t- test and chi-square test was used for statistical analysis. P < 0.05 was considered statistically significant. Results: The mean age was 46 years (range 40–57 years), with the majority of patients being female (56.5%). Most tumors (65.3%) were trigeminal schwannoma, with the remaining being vestibular schwannoma (21.7%), facial schwannoma (8.7%) and epidermoid (4.3%). The most commonly used surgical approach was the retrosigmoid approach (30.4%) and combined approach (30.4%). Gross total resection (GTR) was done in 14 patients while subtotal resection (STR) was done in 9 patients. STR was significantly associated (p<0.05) with persisting cranial nerve deficit and tumour progression. Midbrain compression, internal carotid artery and cavernous sinus involvement were significantly (p<0.05) associated with STR. Conclusions: Trigeminal schwannoma is the most common tumour involving both middle and posterior cranial fossa. Combined approach for such tumours is required in tumours having significant size in both middle cranial fossa and posterior cranial fossa. The intent of surgery is to achieve a gross total resection (GTR) but adhesions and involvement of critical brain structures results in STR.

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SURG-10. PROGNOSTIC IMPORTANCE OF THE EXTENT OF RESECTION IN IDH-WILD TYPE GRADE II ASTROCYTOMAS ACCORDING TO PTERT MUTATION

Neuro-Oncology ◽

10.1093/neuonc/noaa215.857 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii205-ii205

Author(s):

Alessia Pellerino ◽

Francesco Bruno ◽

Antonio Silvani ◽

Tamara Ius ◽

Lorenzo Bello ◽

...

Keyword(s):

Extent Of Resection ◽

Gross Total Resection ◽

National Database ◽

Wild Type ◽

Total Resection ◽

Kaplan Meier ◽

Grade Ii ◽

Prognostic Importance ◽

The Impact

Abstract BACKGROUND IDH-wild type diffuse astrocytomas with pTERT mutation have been suggested by cIMPACT-NOW update 3 to share a poor prognosis with glioblastoma (GBM). In a previous series of the Italian Association of Neuro-Oncology, we reported that IDH-wild type grade II astrocytomas benefit from gross total resection. However, the impact of surgery in the pTERT-mutated subgroup has not been addressed so far. Here, we present our preliminary data about the impact of the extent surgery according to pTERT status. MATERIAL AND METHODS We re-analysed a national database of 122 patients with grade II IDH-wild type astrocytoma. P-TERT mutation was evaluated by gene sequencing. Kaplan-Meier curves were used for the analysis of progression-free and overall survival (PFS and OS). RESULTS Median follow-up was 33.0 months. P-TERT status was available in 40 cases and the mutation was found in 27 cases (67.5%). Patients with pTERT mutation had a significantly shorter PFS (9.4 vs 147.7 months, P < 0.001) and OS (NR vs 36.6 months, P = 0.012). Furthermore, the OS of patients with pTERT mutation, who underwent gross total resection, was significantly longer than in patients with subtotal / partial resection (37.0 vs 32.0 months, P = 0.018). Thus far, the OS of patients without pTERT mutation was not reached with either subtotal / partial or gross total resection. CONCLUSIONS IDH-wild type astrocytomas may be stratified into classes with different outcome based on the pTERT mutation. As far as we know, this is the first study that specifically investigated the importance of a gross total resection according to pTERT status in IDH-wild type grade II astrocytomas.

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Facial nerve outcome and extent of resection in cystic versus solid vestibular schwannomas in radiosurgery era

Neurosurgical FOCUS ◽

10.3171/2017.12.focus17667 ◽

2018 ◽

Vol 44 (3) ◽

pp. E3 ◽

Cited By ~ 11

Author(s):

Pinar Eser Ocak ◽

Ihsan Dogan ◽

Umut Ocak ◽

Cem Dinc ◽

Mustafa K. Başkaya

Keyword(s):

Facial Nerve ◽

Cyst Formation ◽

Extent Of Resection ◽

Gross Total Resection ◽

Vestibular Schwannomas ◽

Total Resection ◽

Mean Size ◽

Microsurgical Resection ◽

Poor Outcomes

OBJECTIVECystic vestibular schwannomas (CVSs) are a subgroup of vestibular schwannomas (VSs) that are reported to be associated with unpredictable clinical behavior and unfavorable postoperative outcomes. The authors aimed to review their experience with microsurgical treatment of CVSs in terms of extent of resection and postoperative facial nerve (FN) function and compare these outcomes with those of their solid counterparts.METHODSTwo hundred-eleven VS patients were treated surgically between 2006 and 2017. Tumors were defined as cystic when preoperative neuroimaging demonstrated cyst formation that was confirmed by intraoperative findings. Solid VS (SVSs) with similar classes were used for comparison. Clinical data of the patients were reviewed retrospectively, including clinical notes and images, as well as operative, pathology, and neuroradiology reports.RESULTSThirty-two patients (20 males and 12 females) with a mean age of 52.2 years (range 17–77 years) underwent microsurgical resection of 33 CVSs (mean size 3.6 cm, range 1.5–5 cm). Forty-nine patients (26 males and 23 females) with a mean age of 49.9 years (range 21–75 years) underwent microsurgical resection of 49 SVSs (mean size 3 cm, range 2–4.5 cm). All operations were performed via either a retrosigmoid or a translabyrinthine approach. Gross-total resection was achieved in 30 cases in the CVS group (90.9%) and 37 in the SVS group (75.5%). The main reason for subtotal and near-total resection was adherence of the tumor to the brainstem and/or FN in both groups. None of the patients with subtotal or near-total resection in the CVS group demonstrated symptomatic regrowth of the tumor during the mean follow-up period of 41.6 months (range 18–82 months). The FN was anatomically preserved in all patients in both groups. Good FN outcomes were achieved in 15 of CVS (grade I–II; 45.5%) and 35 of SVS (71.4%) surgeries at discharge. Good and fair FN functions were noted in 22 (grade I–II; 81.5%) and 5 (grade III only; 18.5%) of the CVS patients, respectively, at the 1-year follow-up; none of the patients showed poor FN function.CONCLUSIONSSurgery of CVSs does not necessarily result in poor outcomes in terms of the extent of resection and FN function. Special care should be exercised to preserve anatomical continuity of the FN during surgery, since long-term FN function outcomes are much more satisfactory than short-term results. High rates of gross-total resection and good FN outcomes in our study may also suggest that microsurgery stands as the treatment of choice in select cases of large CVSs and SVSs in the era of radiosurgery.

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Transcavernous Approach for Gross Total Resection of a Dumbbell-Shaped Giant Trigeminal Schwannoma

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0041-1729995 ◽

2021 ◽

Author(s):

Sima Sayyahmelli ◽

Emel Avci ◽

Burak Ozaydin ◽

Mustafa K. Başkaya

Keyword(s):

Skull Base ◽

Cavernous Sinus ◽

Tumor Resection ◽

Mass Effect ◽

World Health ◽

Gross Total Resection ◽

Petrous Apex ◽

Total Resection ◽

Who Grade ◽

Trigeminal Schwannoma

AbstractTrigeminal schwannomas are rare nerve sheet tumors that represent the second most common intracranial site of occurrence after vestibular nerve origins. Microsurgical resection of giant dumbbell-shaped trigeminal schwannomas often requires complex skull base approaches. The extradural transcavernous approach is effective for the resection of these giant tumors involving the cavernous sinus.The patient is a 72-year-old man with headache, dizziness, imbalance, and cognitive decline. Neurological examination revealed left-sided sixth nerve palsy, a diminished corneal reflex, and wasting of temporalis muscle. Magnetic resonance imaging (MRI) showed a giant homogeneously enhancing dumbbell-shaped extra-axial mass centered within the left cavernous sinus, Meckel's cave, and the petrous apex, with extension to the cerebellopontine angle. There was a significant mass effect on the brain stem causing hydrocephalus. Computed tomography (CT) scan showed erosion of the petrous apex resulting in partial anterior autopetrosectomy (Figs. 1 and 2).The decision was made to proceed with tumor resection using a transcavernous approach. Gross total resection was achieved. The surgery and postoperative course were uneventful, and the patient woke up the same as in the preoperative period. MRI confirmed gross total resection of the tumor. The histopathology was a trigeminal schwannoma, World Health Organization (WHO) grade I. The patient continues to do well without any recurrence at 15-month follow-up.This video demonstrates important steps of the microsurgical skull base techniques for resection of these challenging tumors.The link to the video can be found at https://youtu.be/TMK5363836M

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Profound hearing loss following surgery in pediatric patients with posterior fossa low-grade glioma

Neuro-Oncology Practice ◽

10.1093/nop/npx025 ◽

2017 ◽

Vol 5 (2) ◽

pp. 96-103 ◽

Cited By ~ 1

Author(s):

Yahya Ghazwani ◽

Ibrahim Qaddoumi ◽

Johnnie K Bass ◽

Shengjie Wu ◽

Jason Chiang ◽

...

Keyword(s):

Hearing Loss ◽

Posterior Fossa ◽

Extent Of Resection ◽

Tumor Location ◽

Low Grade Glioma ◽

Gross Total Resection ◽

Subtotal Resection ◽

Low Grade ◽

Total Resection ◽

Profound Hearing Loss

Abstract Background Hearing loss may occur in patients with posterior fossa low-grade glioma who undergo surgery. Methods We retrospectively reviewed 217 patients with posterior fossa low-grade glioma, including 115 for whom results of hearing tests performed after surgery and before chemotherapy or radiation therapy were available. We explored the association of UHL with age at diagnosis, sex, race, tumor location, extent of resection, posterior fossa syndrome, ventriculoperitoneal shunt placement, and histology. Results Of the 115 patients, 15 (13.0%: 11 male, 6 black, 8 white, 1 multiracial; median age 7 years [range, 1.3–17.2 years]) had profound UHL after surgery alone or before receiving ototoxic therapy. Median age at tumor diagnosis was 6.8 years (range, 0.7–14.1 years), and median age at surgery was 6.8 years (range, 0.7–14.1 years). Patients with UHL had pathology characteristic of pilocytic astrocytoma (n = 10), ganglioglioma (n = 4), or low-grade astrocytoma (n = 1). Of these 15 patients, 4 underwent biopsy, 1 underwent gross total resection, 1 underwent near-total resection, and 9 underwent subtotal resection. UHL was more frequent in black patients than in white patients (OR 7.3, P = .007) and less frequent in patients who underwent gross total resection or near-total resection than in those who underwent subtotal resection (OR 0.11, P = .02). Conclusions Children undergoing surgery for posterior fossa low-grade glioma are at risk for UHL, which may be related to race or extent of resection. These patients should receive postoperative audiologic testing, as earlier intervention may improve outcomes.

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Extent of resection in glioblastoma: a 10-year local survival analysis

Neuro-Oncology ◽

10.1093/neuonc/noab195.049 ◽

2021 ◽

Vol 23 (Supplement_4) ◽

pp. iv19-iv19

Author(s):

Theodore Hirst ◽

Patrick McAleavey ◽

Tom Flannery

Keyword(s):

Neurological Deficit ◽

Survival Benefit ◽

Extent Of Resection ◽

Cox Proportional Hazards ◽

Partial Resection ◽

Gross Total Resection ◽

Molecular Profile ◽

Total Resection ◽

Local Survival ◽

The Impact

Abstract Aims The impact on extent of resection (EOR) in glioblastoma has been well documented. It is clear that gross-total resection (GTR) confers best overall survival (OS), however the minimum EOR required to confer a survival benefit over biopsy is debated. Recent studies favour partial resection (PR) over biopsy for IDH-wildtype, MGMT-unmethylated tumours. We describe our experiences locally with these principles in mind. Method Retrospective evaluation of a single surgeon cohort. All patients over 18 years old, undergoing a surgical treatment for histologically confirmed GBM in the stated period were included. We collected information on demographics, tumour volume, EOR, complications, adjuvant therapies, molecular profile, and OS. We used log rank tests and Cox Proportional Hazards Models to identify factors associated with OS. Results The patient and tumour characteristics of our cohort were similar to those documented in the literature. The mean age was 56.6 years. 72 patients underwent biopsy and 202 had debulking surgery. Median OS was 11 months. Of those debulked, gross-total resection was achieved in 41 patients (20%); associated median OS was 29 months. Patients receiving partial resection (defined as EOR <80%) had no clear survival benefit over patients undergoing biopsy (median OS 6 vs 5 months) but had a higher rate of post-op neurological deficit (3% vs 12%). Tumour molecular profile appeared to influence survival outcome in a manner comparable to worldwide experience. Conclusion In our experience, partial resection is not a justifiable surgical aim in the typical glioblastoma cohort. The limited benefit that it may confer over biopsy appears to be outweighed by the risk of neurological deficit that affects quality and probably quantity of life. This finding applies to our glioblastoma population in general as well as those specifically with an MGM-unmethylated tumour.

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Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children

Journal of Neurosurgery Spine ◽

10.3171/2013.6.spine12927 ◽

2013 ◽

Vol 19 (4) ◽

pp. 471-476 ◽

Cited By ~ 46

Author(s):

William B. Feldman ◽

Aaron J. Clark ◽

Michael Safaee ◽

Christopher P. Ames ◽

Andrew T. Parsa

Keyword(s):

Recurrence Rate ◽

Adjuvant Radiotherapy ◽

Pediatric Patients ◽

Cauda Equina ◽

Extent Of Resection ◽

Gross Total Resection ◽

Tumor Control ◽

Recurrence Rates ◽

Total Resection ◽

Who Grade

Object Myxopapillary ependymomas (MPEs) are rare WHO Grade I tumors found in the conus medullaris, cauda equina, and filum terminale. Treatment generally consists of resection with or without adjuvant radiotherapy. Evidence-based guidelines for surgical management are lacking due to the rarity of this tumor. Methods An English-language PubMed search was performed using the key words “myxopapillary” and “ependymoma.” Reports describing fewer than 3 patients or those lacking data on the extent of resection or radiotherapy were excluded. A total of 28 articles describing 475 patients met the authors' inclusion criteria. Patients were grouped by extent of resection and whether or not they underwent adjuvant radiotherapy. Differences in recurrence rates were assessed by chi-square test. Results The overall recurrence rate was 15.5% in patients treated by gross-total resection (GTR) and 32.6% in patients treated by subtotal resection (STR), irrespective of whether they underwent adjuvant therapy (p < 0.001). Regardless of the extent of resection, adjuvant radiotherapy was not associated with a decrease in recurrence rates. The overall recurrence rate was 15.6% in patients who underwent GTR and radiotherapy compared with 15.9% in patients who underwent GTR alone (p = 0.58), and it was 29.3% in patients who underwent STR and radiotherapy compared with 35.1% in those who underwent STR alone (p = 0.53). The difference between recurrence rates for patients who underwent GTR alone versus STR and radiotherapy was statistically significant (p = 0.02). Subgroup analysis demonstrated significantly higher recurrence rates in pediatric patients compared with adults (40.5% vs 23.4%, respectively; p = 0.02). Even in the setting of GTR alone, recurrence rates were higher in pediatric patients (65% vs 7.6%; p < 0.001). Conclusions Gross-total resection alone is associated with decreased recurrence rates compared with STR with or without radiotherapy. The authors' results suggest that treatment goals should include attempted GTR whenever possible. The observation that children benefitted from radiation therapy to a greater extent than did adults suggests that biological differences between tumors in these patient populations warrants more rigorous scientific studies.

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The impact of adjuvant stereotactic radiosurgery on atypical meningioma recurrence following aggressive microsurgical resection

Journal of Neurosurgery ◽

10.3171/2012.12.jns12414 ◽

2013 ◽

Vol 119 (2) ◽

pp. 475-481 ◽

Cited By ~ 72

Author(s):

Douglas A. Hardesty ◽

Andrew B. Wolf ◽

David G. Brachman ◽

Heyoung L. McBride ◽

Emad Youssef ◽

...

Keyword(s):

Radiation Therapy ◽

Stereotactic Radiosurgery ◽

Recurrence Rate ◽

Tumor Recurrence ◽

Gross Total Resection ◽

Adjuvant Radiation ◽

Total Resection ◽

Microsurgical Resection ◽

Atypical Meningiomas

Object Patients with atypical meningioma often undergo gross-total resection (GTR) at initial presentation, but the role of adjuvant radiation therapy remains unclear. The increasing prevalence of stereotactic radiosurgery (SRS) in the modern neurosurgical era has led to the use of routine postoperative radiation therapy in the absence of evidence-based guidelines. This study sought to define the long-term recurrence rate of atypical meningiomas and identify the value of SRS in affecting outcome. Methods The authors identified 228 patients with microsurgically treated atypical meningiomas who underwent a total of 257 resections at the Barrow Neurological Institute over the last 20 years. Atypical meningiomas were diagnosed according to current WHO criteria. Clinical and radiographic data were collected retrospectively. Results Median clinical and radiographic follow-up was 52 months. Gross-total resection, defined as Simpson Grade I or II resection, was achieved in 149 patients (58%). The median proliferative index was 6.9% (range 0.4%–20.6%). Overall 51 patients (22%) demonstrated tumor recurrence at a median of 20.2 months postoperatively. Seventy-one patients (31%) underwent adjuvant radiation postoperatively, with 32 patients (14%) receiving adjuvant SRS and 39 patients (17%) receiving adjuvant intensity modulated radiation therapy (IMRT). The recurrence rate for patients receiving SRS was 25% (8/32) and for IMRT was 18% (7/39), which was not significantly different from the overall group. Gross-total resection was predictive of progression-free survival (PFS; relative risk 0.255, p < 0.0001), but postoperative SRS was not associated with improved PFS in all patients or in only those with subtotal resections. Conclusions Atypical meningiomas are increasingly irradiated, even after complete or near-complete microsurgical resection. This analysis of the largest patient series to date suggests that close observation remains reasonable in the setting of aggressive microsurgical resection. Although postoperative adjuvant SRS did not significantly affect tumor recurrence rates in this experience, a larger cohort study with longer follow-up may reveal a therapeutic benefit in the future.

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Ependymomas in infancy: underlying genetic alterations, histological features, and clinical outcome

Child s Nervous System ◽

10.1007/s00381-020-04655-x ◽

2020 ◽

Vol 36 (11) ◽

pp. 2693-2700

Author(s):

Stephanie T. Jünger ◽

Felipe Andreiuolo ◽

Martin Mynarek ◽

Evelyn Dörner ◽

Anja zur Mühlen ◽

...

Keyword(s):

Posterior Fossa ◽

Genetic Alterations ◽

Gross Total Resection ◽

Older Children ◽

Total Resection ◽

Who Grade ◽

Chromosome 1Q ◽

Stable Genome ◽

1Q Gain

Abstract Introduction Young age is an adverse prognostic factor in children with ependymomas. Treatment of these infants is challenging since beneficial therapeutic options are limited. As ependymomas are considered a biologically heterogeneous group, we aimed to characterize infant ependymomas with regard to their histological and genetic features. Materials and methods We analyzed 28 ependymomas occurring in children younger than 18 months at diagnosis enrolled into the HIT2000-E protocols with the aim to postpone irradiation until the age of 18 months if possible. All cases underwent neuropathological review, including immunohistochemical characterization. Genome-wide copy number alterations (CNA) were assessed by molecular inversion probe assays, and RELA and YAP1 fusions were detected by RT-PCR and sequencing. Results All infant ependymomas were anaplastic (WHO grade III). Twenty-one (75%) cases were located in the posterior fossa. Gross total resection was accomplished in 12 (57%) of these cases. All posterior fossa tumors showed loss of H3-K27me3 characteristic of PFA ependymomas. CNA analysis showed a stable genome in all cases with lack of chromosome 1q gain, an adverse prognostic marker in PFA ependymomas of older children. However, after a median follow-up of 5.4 years, 15 (71%) relapsed, and 9 (43%) died. Seven ependymomas (25%) occurred in the supratentorial region. Gross total resection could be achieved in only two of these cases. Four tumors carried C11orf95-RELA fusions, and two cases had typical YAP1-MAMLD1 fusions (one case was not analyzable). The RELA-fused cases did not display CDKN2A loss as an adverse indicator of prognosis in this disease entity. Although three infants (43%) with supratentorial ependymomas relapsed, all patients survived (median follow-up, 8.0 years). Conclusion Infant ependymomas seem to fall into three biological entities, with supratentorial tumors carrying RELA or YAP fusions and PFA posterior fossa ependymomas. The latter showed a poor outcome even though chromosome 1q gain was absent.

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