Choroid plexus papilloma

Introduction

Choroid plexus papillomas (CPPs) are rare tumors that account for less than 1% of intracranial neoplasms, but account for up to 20% of the tumors in the first year of life. Most of these tumors arise within the ventricles, with the lateral ventricles being the most common; however, these tumors can also involve the extraventricular space. The typical approach for these lesions is complete resection, and in some instances chemotherapy and radiation therapy. In a single institutional experience, the 5-year local control was 84%, distal brain control was 92%, and overall survival was 97%, in which gross total resection was associated with improved local control but not overall survival. Even after subtotal resection, only 50% of patients had recurrence requiring additional therapy. Although these tumors are associated with favorable outcomes following complete resection, they tend to recur both locally and distally with disseminated disease along the neuraxis. In this chapter, we present a case of a patient with a recurrent left cervicomedullary CPP.

Example case

Chief complaint: headaches, nausea, vomiting

History of present illness

A 36-year-old, right-handed woman with multiple CPPs status post temporal intraventicular gross total resection 4 years prior, and fourth ventricular subtotal resection 2 years prior, with concern of recurrence. All the prior surgeries were done at an outside hospital, and 2 years prior she underwent subtotal resection of the fourth ventricular lesion followed by radiation therapy. On serial imaging, this lesion has increased in size with increasing hydrocephalus and headaches, nausea, and vomiting, as well as imbalance ( Fig. 76.1 ).

Medications : None.
Allergies : No known drug allergies.
Past medical and surgical history : Temporal horn CPP resection 4 years prior, fourth ventricular CPP 2 years prior with postoperative radiation therapy.
Family history : No history of intracranial malignancies.
Social history : Teacher, no smoking, no alcohol.
Physical examination : Awake, alert, oriented to person, place, time; Cranial nerves II to XII intact, except left House-Brackmann 2/6; No drift, moves all extremities with good strength; Left greater than right finger-to-nose dysmetria.
Spinal imaging : No evidence of drop metastases.

	Lucas Alverne Freitas de Albuquerque, MD, Hospital Geral de Fortaleza, Ceara, Brazil	Franco DeMonte, MD, MD Anderson Cancer Center, Houston, TX, United States	Vicent Quilis-Quesada, MD, PhD, University of Valencia, Valencia, Spain	Isaac Yang, MD, University of California at Los Angeles, Long Angeles, CA, United States
Preoperative
Additional tests requested	Cerebral angiogram Audiogram Anesthesiology evaluation Ophthalmology evaluationGenetic evaluation	Spine MRI Neurooncology evaluation Radiation oncology evaluation CSF analysis Speech pathology for swallowing function	CT angiography Spinal MRI	Cerebral angiogram CT head MRI with CISS protocol
Surgical approach selected	Left extreme lateral craniotomy	Right frontal ventriculoperitoneal shunt	Left far lateral craniotomy	Left far lateral craniotomy
Anatomic corridor	Left extreme lateral	Right frontal transcortical	Left far lateral	Left far lateral
Goal of surgery	Maximal safe resection	Relief of hydrocephalus	GTR	Debulking of tumor, relief of hydrocephalus
Perioperative
Positioning	Park bench	Right supine	Semisitting	Left lateral
Surgical equipment	IOM (SSEP, MEP, cranial nerves VII–XII EMG) Doppler Surgical microscope Ultrasonic aspirator	Shunt system with programmable valve and possible intrathecal chemotherapy	IOM (SSEP, MEP, cranial nerves EMG VII–XII) Surgical microscope Ultrasonic aspirator Echocardiogram	IOM (SSEP, BAERs, cranial nerve EMG V/VII–VIII/XI–XII) Surgical navigation
Medications	Steroids Hypertonic saline	None	Steroids	Steroids Mannitol Antiepileptics
Anatomic considerations	Vertebral artery, lower cranial nerves	Kocher point	Vertebral/basilar artery and branches, lower cranial nerves, brainstem	Left vertebral, PICA, AICA, basilar artery
Complications feared with approach chosen	Injury to lower cranial nerves, vascular injury, hemiparesis	Shunt malposition or malfunction	Vascular and cranial nerve injuries	Posterior circulation vessel injury
Intraoperative
Anesthesia	General	General	General	General
Skin incision	Left hockey stick	Right frontal curvilinear	Fishhook shape from 5 cm below mastoid tip to superior nuchal line and 5 cm below EOP	Left hockey stick from midline C2 to inion to mastoid
Bone opening	Left suboccipital with foramen magnum, C1 hemilaminectomy, occipital condyle	Right frontal burr hole	Left suboccipital with foramen magnum, C1 hemilaminectomy, occipital condylectomy	Left suboccipital with foramen magnum, C1 hemilaminectomy, 1/3 left occipital condyle
Brain exposure	Left lateral cerebellar surface	Right frontal	Left cerebellopontine angle	Left cerebellopontine angle
Method of resection	Park bench position, convert midline linear incision into left hockey stick, muscle layers above suboccipital triangle retracted as a single flap, leave myofascial pad attached to the superior nuchal line, expose suboccipital triangle and identify vertebral artery and C1 root, lateral suboccipital craniotomy, left C1 hemilaminectomy, extradural transcondylar resection to expose anterior craniocervical junction, resection 2/3 to 1/2 of occipital condyle, dural opening, identify cranial nerves, vascular inspection with Doppler, resection tumor, watertight dural closure, subcutaneous drain	Right frontal burr hole, tunneling of catheter in subcutaneous space, insertion into frontal horn of lateral ventricle, placement of distal catheter into peritoneum	Dissection of suboccipital triangle with vertebral artery exposure, left suboccipital craniotomy from midline to sigmoid sinus, opening of foramen magnum with C1 hemilaminectomy, dural opening following sigmoid sinus, cisterna magna opening and release of CSF, posterior fossa relaxation, exposure of tumor and dissection of lower cranial nerves/vertebral artery/PICA, total resection of cisternal part of tumor prior to fourth ventricle	EVD before surgery, position in left lateral, C1 laminectomy, suboccipital craniotomy, drill 1/3 of left occipital condyle, open dura toward left, debulk tumor and decompress brainstem and fourth ventricle, watertight dural closure
Complication avoidance	Extreme lateral transcondylar approach, resect less than 1/2 of occipital condyle	Avoiding surgery for disseminated disease	IOM, dissection of suboccipital triangle for identification of vertebral artery, cisterna magna opening, dissection of critical neurovascular structures from tumor	EVD prior to surgery, IOM, far lateral craniotomy, debulk tumor
Postoperative
Admission	ICU	Floor	ICU	ICU
Postoperative complications feared	Dysphagia, facial paresis, vascular injury, CSF leak, hydrocephalus	Shunt malfunction or malposition, infection	Vascular and cranial nerve injury	Posterior circulation injury, inadequate decompression of brainstem, hydrocephalus
Follow-up testing	MRI within 48 hours after surgery Speech therapy	CT and shunt survey after surgery	MRI within 48 hours after surgery	MRI within 48 hours after surgery
Follow-up visits	14 days, 1 and 3 months after surgery	7–10 days after surgery	4–6 weeks after surgery	3–4 weeks after surgery
Adjuvant therapies recommended	STR–radiosurgery GTR–observation	Chemotherapy +/– reirradiation	STR–+/– radiation GTR–observation	STR–fractionated radiotherapy GTR–observation