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Management of Pediatric Cervicomedullary Astrocytoma

April 2021
Journal of Radiation and Cancer Research 12(2)

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Cervicomedullary astrocytomas are low grade gliomas of childhood and young age which are typically centered at the junction of brainstem and cervical spine and present with a long duration of symptoms. The diagnosis of cervicomedullary astrocytomas is normally delayed as these tumours are slow growing and patients present with a long duration of symptoms. The symptoms are typical and can be correlated to the location of the tumour. Histopathology and Immunohistochemistry(IHC) guides us proving the diagnosis. Management of cervicomedullary astrocytomas includes surgery, radiotherapy and chemotherapy. A 13 years old female child presented with complaints of headache, vomiting, neck pain and progressive neck tilt. MRI of brain and cervical spine revealed moderate to significant expansile lesion in the dorsal aspect of lower medulla, cervicomedullary junction and the upper cervical cord upto C4-5 disc level.She underwent subtotal resection of the tumour. Histopathology and IHC confirmed her diagnosis as Astrocytoma WHO Grade-II. Patient underwent adjuvant radiation treatment to a radiation dose of 54 Grey in 27 fractions by Volumetric modulated arc therapy(VMAT) technique over a period of 6 weeks to the gross residual tumour and post-op tumour bed. Patient tolerated the treatment well. Patient experienced mild symptoms like nausea and vomiting during the course of treatment but well managed with supportive medications.

MRI -T2 W(Axial) showing hyperintense lesion in the cervical spine Figure 2: MRI -T2 W (Axial) showing hyperintense lesion in the brainstem

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Planning CT scan(Axial) showing dose distribution including residual tumour and post op tumour bed Figure 6: Planning CT scan(Sagittal) showing dose distribution including residual tumour and post op tumour bed

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Cervicomedullary astrocytomas are low grade gliomas of childhood and

young age which are typically centered at the junction of brainstem and

cervical spine and present with a long duration of symptoms. The diagnosis of

cervicomedullary astrocytomas is normally delayed as these tumours are slow

growing and patients present with a long duration of symptoms. The symptoms

are typical and can be correlated to the location of the tumour. Histopathology

and Immunohistochemistry(IHC) guides us proving the diagnosis. Management of

cervicomedullary astrocytomas includes surgery, radiotherapy and chemotherapy.

A 13 years old female child presented with complaints of headache, vomiting,

neck pain and progressive neck tilt. MRI of brain and cervical spine revealed

moderate to signicant expansile lesion in the dorsal aspect of lower medulla,

cervicomedullary junction and the upper cervical cord upto C4-5 disc level.She

underwent subtotal resection of the tumour. Histopathology and IHC conrmed

her diagnosis as Astrocytoma WHO Grade-II. Patient underwent adjuvant radiation

treatment to a radiation dose of 54 Grey in 27 fractions by Volumetric modulated

arc therapy(VMAT) technique over a period of 6 weeks to the gross residual tumour

and post-op tumour bed. Patient tolerated the treatment well. Patient experienced

mild symptoms like nausea and vomiting during the course of treatment but well

managed with supportive medications.

Keywords: Cervicomedullary astrocytomas, positron‑emission tomography

computed tomography, radiation treatment

Management of Pediatric Cervicomedullary Astrocytoma

Gopal Pemmaraju, Anand Parab, Anuradha Singh, Shubhangi Barsing

Address for correspondence: Dr. Gopal Pemmaraju,

E‑mail: drgpsubha@gmail.com

The symptoms are typical and can be correlated to the

location of the tumor. Nausea, vomiting, lower cranial

dysfunction, sleep apnea, and chronic aspiration are seen

in patients with a focus within the medulla, whereas

cervical lesions present with pain, head tilt, progressive

weakness, and motor dysfunction. Magnetic resonance

imaging (MRI) is the gold standard in the radiological

identication of cervicomedullary astrocytomas.

Histopathology and immunohistochemistry (IHC)

guide us in proving the diagnosis. Management of

cervicomedullary astrocytomas includes surgery,

radiotherapy, and chemotherapy. Gross total

resection (GTR) or subtotal resection is the surgery

Case Report

Introduction

Cervicomedullary astrocytomas are low-grade gliomas

of childhood and young age which are typically

centered at the junction of brainstem and cervical

spine and present with a long duration of symptoms.

[1] These tumors are thought to arise in the spinal cord

and extend dorsally into the medulla and 4th ventricle.

The direction of growth may be due to resistance at

the  pyramidal decussation area.[2,3] Radiologically,

cervicomedullary astrocytomas come under a subset

of Type IV brainstem gliomas.[4] The World Health

Organization (WHO) classied astrocytomas into

Grades I–IV and majority of brainstem tumors are either

Grade‑I(pilocytic)orGrade‑II(brillary)astrocytomas.

The diagnosis of cervicomedullary astrocytomas is

normally delayed as these tumors are slow growing

and patients present with a long duration of symptoms.

Department of Advanced

Centre for Radiation

Oncology, Dr. Balabhai

Nanavati Hospital, Mumbai,

Maharashtra, India

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How to cite this article: Pemmaraju G, Parab A, Singh A, Barsing S.

Management of pediatric cervicomedullary astrocytoma. J Radiat Cancer

Res 2021;12:85-8.

Access this article online

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Website: www.journalrcr.org

DOI: 10.4103/jrcr.jrcr_40_20

Received: 04-08-2020

Accepted: 13-08-2020

Published: 21-04-2021

Abstract

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Pemmaraju, et al.: Pediatric cervicomedullary astrocytomas

86 Journal of Radiation and Cancer Research ¦ Volume 12 ¦ Issue 2 ¦ April-June 2021

being performed in cervicomedullary astrocytomas.

Radiation treatment has been an excellent treatment

option with long‑term disease‑free survival benet.

Chemotherapy may be used as an adjuvant treatment

along with radiotherapy or in recurrent tumor settings.

Case Report

A  13‑year‑old female  child presented with complaints

of headache, vomiting, neck pain, and progressive

neck tilt. MRI of the brain and cervical spine revealed

moderate‑to‑signicant expansile lesion in the dorsal

aspect of lower medulla, cervicomedullary junction, and

the upper cervical cord up to C4–C5 disc level with a

hyperintense signal on T2-weighted and isointense on

T1-weighted images with prominent central canal/thin

syrinx in the cervicodorsal cord distal to the lesion from

C5 to D1 level [Figures 1-4]. The patient underwent

subtotal resection of the tumor. Microscopically, the

tumor composed of mild pleomorphic glial cells. On

IHC, the tumor cells were positive for glial brillary

acidic protein (GFAP) and S100, retained expression

of ATRX, and Ki-67 proliferation index about 2%–3%

and her diagnosis was conrmed as AstrocytomaWHO

Grade-II.

The patient underwent adjuvant radiation treatment after

proper immobilization and computed tomography (CT)

simulation on Halcyon 2.0 with kV cone-beam

CT (CBCT) Linear accelerator from M/s. Varian, USA,

using 6 MV  Flattening Free Filter (FFF) beam. She

received a radiation dose of 54 Gy in 27 fractions by

volumetric modulated arc therapy (VMAT) technique

over a period of 6 weeks to the gross residual tumor

and postoperative tumor bed. Radiation planning was

done on Eclipse 15.6 and the optimized VMAT plan

was achieved with V95%–99.9% of the planning target

volume [Figures 5 and 6] and sparing all the critical

structures. The same plan was then compared with

Figure 1: MRI - T2 W(Axial) showing hyperintense lesion in the

cervical spine

Figure 2: MRI - T2 W (Axial) showing hyperintense lesion in the

brainstem

Figure 3: MRI - T1 W (Axial) showing isointense lesion in the

brainstem Figure 4: MRI - T2 W (Sagittal) showing cervicomedullary

astrocytoma

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Pemmaraju, et al.: Pediatric cervicomedullary astrocytomas

Journal of Radiation and Cancer Research ¦ Volume 12 ¦ Issue 2 ¦ April-June 2021

intensity-modulated radiotherapy (IMRT) plan [Table 1].

Precise treatment was delivered with image guidance

by daily kV-CBCT prior to treatment and setup error

corrections. The patient tolerated the treatment well.

The patient experienced mild symptoms such as nausea

and vomiting during the course of treatment but well

managed with supportive medications. No major side

eects or neurologic decits were noticed during or

after the completion of treatment.

Discussion

Cervicomedullary astrocytomas represent a subset of

low‑grade brainstem gliomas and, as per denition, are

intramedullary tumors that span the cervicomedullary

junction. These tumors generally grow into the medulla

in a dorsal exophytic fashion and compress the cranial

nerves which produce signs and symptoms related to

the same. As these tumors have good prognosis, timely

diagnosis is essential though dicult and frequently

delayed.

Robertson et al. demonstrated that longer the

preoperative duration of symptoms (>15 weeks), longer

is the progression-free survival.[5]

MRI is a better tool to identify cervicomedullary

astrocytomas. On MRI, they show heterogeneous

enhancement with contrast and appear hypo- or isointense

on T1-weighted images and hyperintense on T2-weighted

images. As ependymomas also appear the same on MRI,

biopsy, histopathological examination, and IHC are

crucial in diagnosis.[6] Histopathologically, WHO Grades

II–IVarecategorizedasdiuseinltrativeastrocytomas.

WHO Grade-II astrocytomas are composed of atypical

brillary or gemistocytic astrocytes.[7] These tumors

express GFAP, low proliferation index (MIB-1), and no

detectable mitosis. Molecular genetic studies showed

activation of ERK/MAPK pathway, KIAA1549-BRAF

gene fusions, and loss of NF1 gene with hyperactivation

of oncogene KRAS.[8]

GTR is an optimal surgical procedure which is rarely

possible. Any damage to densely packed neural

pathways can have disastrous clinical outcomes. Most

surgical interventions are hence limited to biopsy or

partial excision of spinal tumor. Radiation therapy very

often is the only option of treatment. Radiation therapy

is also considered following inadequate surgery or for

salvage of recurrent gliomas.

Klimo et al. have unequivocally demonstrated the

eectiveness of radiation therapy even after complete

resection of the tumor. Radiotherapy is also indicated

as an adjuvant treatment in cases with residual tumors

after surgery or in recurrent tumors. Radiation to

a dose of 45–54 Gy has been the standard proven

schedule with improved outcomes.[9] With the advent

of modern radiotherapy techniques such as VMAT and

IMRT and with image guidance, precise treatment can

be delivered sparing organs at risk with less toxicities

to the patient. Experiences from the past have shown

Figure 5: Planning CT scan(Axial) showing dose distribution

including residual tumour and post op tumour bed

Figure 6: Planning CT scan(Sagittal) showing dose distribution

including residual tumour and post op tumour bed

Table 1: Comparision between IMRT and VMAT plans

regarding dose coverage and dosage to Organs at risk

(OAR)

Structure/Target volume VMAT IMRT

V 95% 99.9% 99.9%

SPINAL CORD (Max) 28.6 Gy 34 Gy

RT EYE (Max) 1.42 Gy 2.52Gy

LT EYE (Max) 2.59Gy 3.11Gy

RT LENS (Max) 1.11Gy 2.12Gy

LT LENS (Max) 1.48Gy 2.7Gy

ESOPHAGUS (Mean) 6.59Gy 8.24Gy

PHARYNGEAL

CONSTRICTORS (Mean)

35.9Gy 36.2Gy

RT LUNG V30

V20

0.08%

0.95%

0.14%

0.96%

LT LUNGV30

V20

0.09%

1.04%

0.31%

1.98%

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Pemmaraju, et al.: Pediatric cervicomedullary astrocytomas

88 Journal of Radiation and Cancer Research ¦ Volume 12 ¦ Issue 2 ¦ April-June 2021

that VMAT is comparable to IMRT in the treatment of

cervicomedullary and spinal tumors. Chemotherapy may

be considered as an adjuvant treatment option concurrent

with radiation or as salvage treatment in recurrent and

progressive lesions.

Conclusion

Cervicomedullary astrocytomas are rare, low-grade

tumors of pediatric and young age group which

present with typical symptoms of long duration and are

associated with good prognosis. MRI, histopathology,

and IHC guide us in diagnosis. Surgical resection (gross

total or subtotal) plays an important role in the

treatment. The role of radiation has traditionally been

the standard and cannot be replaced. Newer techniques

such as VMAT and IMRT can deliver the desired

radiation dose to the target sparing all the critical

structures.

Declaration of patient consent

The authors certify that they have obtained all

appropriate patient consent forms. In the form the patient

(s) has/have given his/her/their consent for his/her/their

images and other clinical information to be reported in

the journal. The patients understand that their names

andinitialswill not be published anddueeorts will be

made to conceal their identity, but anonymity cannot be

guaranteed.

Financial support and sponsorship

Nil.

Conicts of interest

Therearenoconictsofinterest.

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Intramedullary spinal cord tumors: A review of current and future treatment strategies

Article

Full-text available

Aug 2015
Neurosurg Focus

Intramedullary spinal cord tumors have low incidence rates but are associated with difficult treatment options. The majority of patients with these tumors can be initially treated with an attempted resection. Unfortunately, those patients who cannot undergo gross-total resection or have subtotal resection are left with few treatment options, such as radiotherapy and chemotherapy. These adjuvant treatments, however, are associated with the potential for significant adverse side effects and still leave patients with a poor prognosis. To successfully manage these patients and improve both their quality of life and prognosis, novel treatment options must be developed to supplement subtotal resection. New research is underway investigating alternative therapeutic approaches for these patients, including directed, localized drug delivery and nanomedicine techniques. These and other future investigations will hopefully lead to promising new therapies for these devastating diseases.

Cervicomedullary tumors in children

Article

Full-text available

Jun 2015
J NEUROSURG-PEDIATR

OBJECT Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms. METHODS The charts of all pediatric patients with CMTs who received treatment at St. Jude Children’s Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed. RESULTS Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%. CONCLUSIONS Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.

Management and outcome of focal low-grade brainstem tumors in pediatric patients: The St. Jude experience

Article

Full-text available

Jan 2013
J NEUROSURG-PEDIATR

Object: Whereas diffuse intrinsic pontine gliomas generally have a short symptom duration and more cranial nerve involvement, focal brainstem gliomas are commonly low grade, with fewer cranial neuropathies. Although these phenotypic distinctions are not absolute predictors of outcome, they do demonstrate correlation in most cases. Because there is a limited literature on focal brainstem gliomas in pediatric patients, the objective of this paper was to report the management and outcome of these tumors. Methods: The authors reviewed the records of all children diagnosed with radiographically confirmed low-grade focal brainstem gliomas from 1986 to 2010. Each patient underwent biopsy or resection for tissue diagnosis. Event-free survival (EFS) and overall survival were evaluated. Univariate analysis was conducted to identify demographic and treatment variables that may affect EFS. Results: Fifty-two patients (20 girls, 32 boys) with follow-up data were identified. Median follow-up was 10.0 years, and the median age at diagnosis was 6.5 years (range 1-17 years). The tumor locations were midbrain (n = 22, 42%), pons (n = 15, 29%), and medulla (n = 15, 29%). Surgical extirpation was the primary treatment in 25 patients (48%). The 5- and 10-year EFS and overall survival were 59%/98% and 52%/90%, respectively. An event or treatment failure occurred in 24 patients (46%), including 5 deaths. Median time to treatment failure was 3.4 years. Disease progression in the other 19 patients transpired within 25.1 months of diagnosis. Thirteen of these patients received radiation, including 11 within 2 months of primary treatment failure. Although children with intrinsic tumors had slightly better EFS at 5 years compared with those with exophytic tumors (p = 0.054), this difference was not significant at 10 years (p = 0.147). No other variables were predictive of EFS. Conclusions: Surgery suffices in many children with low-grade focal brainstem gliomas. Radiation treatment is often reserved for disease progression but offers comparable disease control following biopsy. In the authors' experience, combining an assessment of clinical course, imaging, and tumor biopsy yields a reasonable model for managing children with focal brainstem tumors.

Management of posterior fossa gliomas in children

Article

Full-text available

Oct 2011

Brain tumours form the most common type of solid tumour in children and more that 50% of these are infratentorial. Cerebellar astrocytomas and brain stem gliomas are the commonest posterior fossa glial tumours in children. Cerebellar astrocytomas represent up to 10% of all primary brain tumours and up to 25% of posterior fossa tumors in children, with Low grade gliomas forming the commonest of the cerebellar gliomas. They commonly present with symptoms and signs of raised intracranial pressure due to obstructive hydrocephalus. Radiologically they may be solid or cystic with or without a mural nodule. Surgical excision is the mainstay of treatment and forms the most consistent factor influencing progression free and long term survival. While majority of the tumours are pilocytic astrocytomas, they may also be fibrillary astrocytomas or even high grade tumours. Tumour histology does not appear to be an independent factor in the prognosis of these children, and therefore no palliative treatment after surgery is advocated. Brain stem gliomas account for approximately 10% of all pediatric brain tumours. Cranial nerve signs, ataxia and cerebellar signs with or without symptoms and signs of raised intracranial pressure are classically described symptoms and signs. Radiographic findings and clinical correlates can be used to categorize brain stem tumours into four types: diffuse, focal, exophytic and cervicomedullary. Histologically most brain stem gliomas are fibrillary astrocytomas. Diffuse brain stem gliomas are the most commonly seen tumour in the brain stem. These lesions are malignant high grade fibrillary astrocytomas. Focal tumours of the brain stem are demarcated lesions generally less than 2 cms in size, without associated edema. Most commonly seen in the midbrain or medulla, they form a heterogeneous pathological group, showing indolent growth except when the lesion is a PNET. Dorsally exophytic tumours lie in the fourth ventricle, while cervicomedullary lesions are similar to spinal intramedullary tumours. Expanding lesions are the only lesions amenable for excision while infiltrative and ventral lesions are not.

Spectrum of histopathology in spinal lesions

Article

Jan 2016

Cervicomedullary astrocytomas of childhood: Clinical and imaging follow-up

Article

Aug 1999

Unlabelled: BACKGROUND. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management. Materials and methods: A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2-11 years; mean = 5.2 years). Results: Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5-7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2-11 years). There has been one death. The majority of tumors were pilocytic astrocytomas. Conclusion: Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas.

Intra-Axial tumors of the cervicomedulary junction

Article

Nov 1987
J NEUROSURG

The authors present their experience with the operative management of 20 intra-axial tumors of the cervicomedullary junction. There were two distinct modes of clinical presentation: lower cranial nerve dysfunction and spinal cord dysfunction. Both groups of patients had indolent courses: in 75% of the patients the symptoms had been present for 6 months to 2 years. Radical excision was carried out in all patients. There was no surgical mortality. Postoperative neurological recovery was directly related to the preoperative status; one patient had a significant new deficit. The authors conclude that intrinsic gliomas of the cervicomedullary junction are amenable to radical excision and that an aggressive surgical approach offers the potential for both neurological recovery and long-term survival. The neuroradiological evaluation and operative technique are discussed.

Cervicomedullary tumors in children: A distinct subset of brainstem gliomas

Article

Nov 1994

We reviewed the clinical course of 17 children who underwent surgical resection of an intra-axial cervicomedullary tumor between 1980 and 1992. The clinical symptoms, which reflected medullary dysfunction in nine children and cervical cord deficits in eight, were present for a mean of 2.1 years before diagnosis (range, 2 months to 7.5 years), and for at least 1 year in 80% of the patients. Neurodiagnostic imaging (MRI in 14, CT in 3) showed the tumor epicenter in the medulla in 11 and in the upper cervical cord in six. Surgery was performed for newly diagnosed tumor in 11 children, and for progressive disease in six who had received prior radiotherapy. The surgical resection was gross total in two and partial (60 to 95%) in fifteen. Fifteen patients had low-grade glial tumors (10 astrocytomas, four gangliogliomas, and one mixed glioma), and two had anaplastic gangliogliomas. Four-year progression-free and total survival rates after surgery for patients who had surgery as initial therapy were 70 and 100%; for those who had surgery at the time of progression, these were 41 and 62%. Postsurgical neurologic complications occurred in five children. Four of these children had received prior radiotherapy. Two of them already had severe preoperative deficits and three had moderate deficits that worsened after surgery. Twelve patients with mild deficits were unchanged or improved postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

Cervicomedullary astrocytomas of childhood: Clinical and imaging follow-up

Jan 1999
PEDIATR RADIOL
662-669

Young Poussaint
T Yousuf
N Barnes
P D Antony
D C Zurakowski
D Scott

Young Poussaint T, Yousuf N, Barnes PD, Antony DC, Zurakowski D, Scott RM. Cervicomedullary astrocytomas of childhood: Clinical and imaging follow-up. Pediatr Radiol 1999;29:662-9.

Cervico medullary tumors in children