Posterior fossa ependymoma in childhood: 60 years event-free survival after partial resection—a case report

Lundar, Tryggve; Due-Tønnessen, Bernt Johan; Krossnes, Bård Kronen; Due‐Tønnessen, Paulina; Brandal, Petter

doi:10.1007/s00381-015-2766-7

Cited by 5 publications

(2 citation statements)

References 12 publications

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“…Four of the 6 first patients in this series lived for 49 to 65 years after primary treatment, and 2 of these patients are still in excellent condition at 63 and 78 years of age. 17 The other 2 patients, who experienced extensive meningiomas during late follow-up, presumably secondary to radiotherapy given in childhood, underwent operative treatment before they died at 49 and 55 years, respectively, following primary treatment. Another patient died of breast cancer at the age of 43 years, after 25 years in FTW.…”

Section: Discussionmentioning

confidence: 99%

Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival

Lundar¹,

Due-Tønnessen²,

Frič³

et al. 2020

Journal of Neurosurgery: Pediatrics

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OBJECTIVEEpendymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution.METHODSThe authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years.RESULTSThe authors identified 22 children (median age at the time of surgery 3 years, range 0–18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945–1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987–2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work.CONCLUSIONSPediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.

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Section: Discussionmentioning

confidence: 99%

Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival

Lundar¹,

Due-Tønnessen²,

Frič³

et al. 2020

Journal of Neurosurgery: Pediatrics

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“…Spinal cord may be the site for recurrences. (11) Witt and Colleagues described two distinct subgroups in ependymomas. Group-A of lower age and lateral tumour position with serious prognosis.…”

Section: Discussionmentioning

confidence: 99%

Spectrum of Posterior Cranial Fossa Space Occupying Lesions- Our Experience at a Tertiary Care Centre

Gollapalli¹,

Moula²,

Shriram³

et al. 2018

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BACKGROUND Posterior cranial fossa is located between the Foramen Magnum and Tentorium Cerebelli. It contains cerebellum, pons and medulla oblongata. Tumours of posterior cranial fossa can affect any of the above structures and can lead to pressure symptoms, neurological deficits or sometimes even death. This study has been conducted at our tertiary care centre. Aims and Objectives-To know the usefulness of intraoperative squash cytology and its diagnostic accuracy in differentiating posterior fossa lesions in correlation with clinical, radiological and histopathological diagnosis. Newer diagnostic modalities like MRI help in detecting these tumours early. Squash cytology and histopathological study diagnose these lesions accurately, which may benefit the patient for further treatment. MATERIALS AND METHODS This is a retrospective descriptive study. A total of 28 cases of posterior cranial fossa lesions were analysed with squash cytology and histopathology correlation. RESULTS Among the 28 cases analysed, 27 cases (96.4%) showed squash cytology with histopathologic correlation. A case of ependymoma was misdiagnosed as embryonal tumour/ medulloblastoma on squash cytology. Male preponderance was noted. The commonest age group was first decade in this study. Cerebellopontine angle was the most common site of the tumours in our study. CONCLUSION Analysing the posterior fossa tumours by squash cytology along with radioimaging in tumour localising and patient's demographics helped in correct histopathological diagnosis for further management and follow-up.

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Four-Hand Suction-Irrigation Technique Leads to Gross Total Resection and Long-Term Progression-Free Survival in Fourth Ventricular Ependymoma

et al. 2017

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Posterior fossa ependymoma in childhood: 60 years event-free survival after partial resection—a case report

Cited by 5 publications

References 12 publications

Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival

Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival

Spectrum of Posterior Cranial Fossa Space Occupying Lesions- Our Experience at a Tertiary Care Centre

Four-Hand Suction-Irrigation Technique Leads to Gross Total Resection and Long-Term Progression-Free Survival in Fourth Ventricular Ependymoma

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