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Title: Rapid neurologic recovery following partial surgical resection of spinal

glioblastoma multiforme in a pediatric patient presenting with complete

paraplegia

Authors: Gabriel N. Friedman, .A.,!," en#amin $. Grannan, %.&.,!," 'i#ay

(anamadala, %.&.,!," Ganesh %. )han*ar, %.&. +h.&.,!," ohn -. &ewitt, %.&.

+h.&., 'idya +uthenpura, %.&.,!Robert /offie, %.&. +h.&.,!,"&avid 0. 1bb,

%.&.,",2%atthew +. Frosch, %.&. +h.&.,",Ann3-hristine &uhaime, %.&.!,"

Affiliations:

!&epartment of Neurosurgery, %assachusetts General 0ospital, 44 Fruit )treet,

oston, %assachusetts 5"!!2, 6)A

"0arvard %edical )chool, "75 $ongwood Avenue, oston, %assachusetts 5"!!4,

6)A

&epartment of +athology, %assachusetts General 0ospital, 44 Fruit )treet,

oston, %assachusetts 5"!!2, 6)A

2&epartment of +ediatrics, %assachusetts General 0ospital, 44 Fruit )treet,

oston, %assachusetts, 5"!!2, 6)A

-orresponding Author8s name and complete mailing address:

en#amin $. Grannan, %.&.

%assachusetts General 0ospital

&epartment of Neurosurgery

9ang uilding, )uite 5"

44 Fruit )treet

oston, %A "5!!2

+hone: ;7!< "73!55"

1mail: bgrannan=partners.org

/ey 9ords: glioblastoma multiforme> resection> pediatric> spinal> oncology

Running Title: Rapid neurologic recovery following resection of spinal G%

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Abstract

)pinal cord glioblastoma multiforme ;G%< is a rare entity with a poor

prognosis that often presents with lower e?tremity wea*ness or paralysis.

)tandard treatment consists of surgical resection followed by radiation and

chemotherapy. +revious literature suggests that aggressive surgical resection may

provide overall survival benefit> however, there is limited concurrent analysis of

the impact of surgical resection on neurologic recovery. 0ere, we present the case

of a @3year3old boy who presented with complete loss of lower e?tremity motor

and sensory function and successfully regained the ability to ambulate

independently following subtotal surgical resection, proton beam radiation, and

temoolomide treatment. The case demonstrates the need for consideration of

both meaningful neurologic recovery in addition to overall survival when

deciding on the e?tent of surgical resection in cases of spinal G%, especially

when patients present with severe deficits soon after symptom onset.

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Bntroduction

)pinal cord glioblastoma multiforme ;G%< is an e?tremely rare tumor of

the central nervous system ;-N)

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iliopsoas level, no reliable sensation to pain and light touch below T7 dermatome,

bilateral positive abins*i sign, and loss of rectal tone ;A)BA A e?am at the T7

level

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movement or sensation in the lower e?tremities

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straight catheteriation for inability to completely empty his bladder and has

intact voluntary bowel function.

&iscussion

Bn this case, we describe a @3year3old boy who presented with complete,

rapidly progressive paraplegia due to a spinal cord G% and underwent urgent

surgical decompression and debul*ing, proton beam therapy, and temoolomide

chemotherapy, subseDuently e?periencing near complete motor and sensory

recovery in the lower e?tremities. To our *nowledge, this is the first case in which

rapid and mar*ed recovery of function from complete paraplegia in spinal cord

G% is reported. The overall survival in spinal G% is around !5 months, and

the ma#or cause of death is thought to be from meningeal seeding and cerebral

metastases.!4There appear to be few differences between pediatric and adult

spinal G%, as both are treated similarly and carry a comparable life e?pectancy.

The small number of such cases has limited the potential for prospective trials and

so much of the treatment strategy has been e?trapolated from clinical trials

involving intracranial G%.7

Extent o! "esection

Ef the three treatment modalities available, both radiation therapy and

chemotherapy are considered the standard of care based on modest improvements

in progression free and overall survival. The role of that e?tent of surgical

resection on overall survival and its tradeoff with potential for neurologic

recovery is less clearly established. Gross total resection is often challenging due

to the lac* of clear tumor margins as well as the ris* of causing devastating

neurological damage to ad#acent viable nervous tissue where tumor bul* is less

obvious but where tumor invasion is li*ely.","!The general surgical approach

previously suggested in the literature for spinal G% involves performing limited

resection of only grossly abnormal tissue in order to preserve remaining

neurological function given that e?tent of resection had not been shown to

increase survival.!5,!2,"5%ore recent studies still demonstrate conflicting evidence.

)pecifically, a study investigating the prognosis of infiltrative spinal cord

astrocytoma ;90E grade BB3B'< in 7 patients ; with G%< found a survival

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benefit trend of !! months ;5 vs. !@ months< for patients who underwent only

biopsy versus surgical resection.!!Bn contrast, in an 3patient series of pediatric

spinal G% patients, average survival for patients who underwent a gross total

resection was !@." months compared with !".7 months for those who received

subtotal resection, with 2 patients e?periencing unspecified improvements in

neurological function ;reportedly independent of the e?tent of resection

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considerable role in his eventual neurologic recovery. 9hile minimiing time to

intervention has been advocated strongly in the setting of traumatic spinal cord

in#ury, this has been less thoroughly evaluated for spinal cord compression due to

malignancy. 2,4,!,""

Functional "ecovery in #pinal Cord $umors Presenting with Paraplegia

9hile e?ceedingly rare, isolated case reports have described patients with

other primary spinal cord tumors ;e.g., schwannoma, hemangiopericytoma< who

presented with acute paraplegia and subseDuently regained near complete motor

ability following resection.,1ven with thorough multimodal treatment,

functional recovery with spinal glioblastoma is still uncommon, though

occasional instances where treatment has allowed for some improvement in

function have been previously reported. For instance, a recent case highlighted a

""3year3old patient with spinal G% who presented with absent sensation and

complete paraplegia in both legs and went on to regain a moderate degree of

motor function and complete sensation after subtotal resection and five wee*s of

conventional radiation therapy.")imilarly, a !3year3old who presented with

Duadriparesis from a cervical spinal G% achieved sustained functional recovery

after multimodal treatment with radiation therapy and temoolomide."7The case

that we have presented here also serves as a star* cautionary note that even in a

patient who presents with complete loss of lower e?tremity function, meaningful

neurologic recovery including independent ambulation is possible following

subtotal resection. This report cannot advocate one strategy over another in

treatment of spinal cord glioblastoma, as it is an isolated case with short follow3

up, but it does demonstrate that good neurologic recovery is possible, even in the

setting of paraplegia.

-onclusion

Bn this report, we described a @3year3old boy who presented with T7 paraplegia

with spinal G% who subseDuently recovered both lower e?tremity sensation and

ability to ambulate independently following early decompression with subtotal

resection, proton beam therapy, and temoolomide therapy. +ediatric spinal G%

is an e?tremely rare tumor that carries a dismal prognosis. There have been few

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reports of such e?tensive functional improvement following surgical debul*ing

and this case serves to demonstrate that even with complete loss of motor

function, significant meaningful neurologic recovery is possible in setting of early

surgical decompression and multimodal therapy. )pecifically with regard to

surgical approach, recent data may demonstrate that gross total resection provides

an overall survival benefit, but the interplay between e?tent of surgical resection,

survival, and meaningful neurologic recovery needs to be further studied to better

understand the role that surgery plays in optimiing short3 and long3term

outcomes in patients with spinal G%.

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&isclosure:

The authors have no conflicts of interest, sources of financial support, or industry

affiliations to disclose relevant to this investigation.

Ac*nowledgments:

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Figure $egend

/igure 1 %RB imaging of thoracic G% ;T" to T@ levels shown