Gliomatosis cerebri

Introduction

Gliomatosis cerebri is characterized by widespread infiltration of the brain in three or more lobes with bilateral involvement. Gliomatosis cerebri type 1 is when there is no obvious mass, but there is a widespread tumor pattern, whereas gliomatosis cerebri type 2 shows a widespread tumor pattern with a tumor mass. The symptoms of gliomatosis cerebri depend on the tumor location. The management of these lesions is typically limited to surgical biopsy, as there usually is no mass lesion to remove. The tissue obtained through the biopsy will be used to guide possible adjuvant therapy. In this chapter, we present a case with bilateral multifocal disease characteristic of gliomatosis cerebri.

Example case

Chief complaint: seizures

History of present illness

A 57-year-old, right-handed woman with no significant past medical history presented with seizures. She was driving when she had a witnessed acute onset of right facial and arm twitching followed by loss of consciousness with resulting motor vehicle collision. She was brought to the emergency room where imaging revealed multifocal brain lesions ( Fig. 12.1 ).

Medications : None.
Allergies : No known drug allergies.
Past medical and surgical history : None.
Family history : No history of intracranial malignancies.
Social history : Elementary school teacher. No smoking or alcohol.
Physical examination : Awake, alert, oriented to person, place, and time; Language: intact naming and repetition; Cranial nerves II to XII intact; No drift, moves all extremities with full strength.

	Gordon Li, MD, Stanford University, Palo Alto, CA, United States	Nader Sanai, MD, Barrow Neurological Institute, Phoenix, AZ, United States	Shota Tanaka, MD, PhD, The University of Tokyo, Tokyo, Japan	Graeme F. Woodworth, MD, University of Maryland School of Medicine, Baltimore, MD, United States
Preoperative
Additional tests requested	CSF analysis	DTI fMRI	DTI fMRI PET methionine	Neurology evaluation
Surgical approach selected	Left posterior temporal stereotactic needle biopsy	Left frontal stereotactic needle biopsy	Left fronto-temporal craniotomy for temporal lobectomy and partial insular resection	Left temporal craniotomy for open excisional biopsy
Anatomic corridor	Left posterior MTG/ITG	Left frontal	Left temporal	Left anterior ITG
Goal of surgery	Diagnosis	Diagnosis	Diagnosis, debulking of highest-grade potion of lesion based on PET	Diagnosis, molecular testing
Perioperative
Positioning	Left supine	Left supine	Left supine with right rotation	Left supine with rotation
Surgical equipment	Needle biopsy kit Surgical navigation	Needle biopsy kit Surgical navigation	Surgical navigation IOM (MEP) Surgical microscope with 5-ALA Doppler ultrasound	Surgical navigation Surgical microscope
Medications	Antiepileptics	None	Steroids Antiepileptics	Steroids after biopsy Antiepileptics Mannitol
Anatomic considerations	Wernicke area	Sylvian vessels Internal capsule	MCA, lenticulostriate artery	Eloquent language regions
Complications feared with approach chosen	Language deficit with Wernicke superiorly, left insula, and right perirolandic	Aphasia Motor deficit	Motor deficit, semantic paraphasias	Speech dysfunction, nondiagnostic tissue
Intraoperative
Anesthesia	General	General	General	General
Skin incision	Linear	Linear	Curvilinear	Curvilinear
Bone opening	Left temporal burr hole	Left frontal burr hole	Left fronto-temporal	Left anterior temporal
Brain exposure	Left MTG/ITG	Left SFG/MFG (Kocher point)	Left fronto-temporal	Left anterior temporal
Method of resection	Preplan entry point for left MTG/ITG, twist drill burr hole, puncture dura with k-wire, navigation biopsy kit, take four biopsies at the same depth with one biopsy in each quadrant, frozen diagnosis, incision closed if pathology is lesional	Left frontal linear incision, Kocher point identified, and burr hole made, stereotactic needle biopsy passed into left basal ganglia, biopsies taken, frozen pathology, incision closed if pathology is lesional	Left fronto-temporal craniotomy, dural opening, left temporal lobectomy up to 4 cm with microscope, identify and preserve Sylvian fissure vessels including MCA, debulk insular portion through resection cavity	Preplan entry point based on navigation, craniotomy over inferior anterior temporal region, cruciate dural opening, enter temporal region low and anterior based on navigation, open biopsy, microscope as needed for visualization
Complication avoidance	Avoid Wernicke area	Avoid Sylvian vessels and internal capsule	IOM, awareness of Sylvian vessels, debulk insular portion through resection cavity	Low and anterior trajectory, open biopsy with direct visualization
Postoperative
Admission	Floor	ICU	ICU	ICU
Postoperative complications feared	Hemorrhage, language deficit	Hemorrhage	Vessel spasm/stroke	Speech loss, seizures, hemorrhage, cerebral edema
Follow-up testing	None	Head CT immediately after surgery	MRI within 48 hours after surgery	Head CT immediately after surgery
Follow-up visits	10 days after surgery	14 days after surgery	12–31 days after surgery	14 days after surgery Radiation Oncology and Neuro Oncology pending pathology results
Adjuvant therapies recommended
Diffuse astrocytoma (IDH mutant, retain 1p19q)	Radiation/temozolomide	Radiation/temozolomide	Radiation/temozolomide	Radiation, possible chemotherapy
Oligodendroglioma (IDH mutant, 1p19q LOH)	Radiation/PCV or radiation/temozolomide	Radiation/temozolomide	Radiation/PAV	Radiation/PCV
Anaplastic astrocytoma (IDH wild type)	Radiation/temozolomide	Radiation/temozolomide	Radiation/temozolomide	Radiation/temozolomide