J Neurol Surg B Skull Base 2020; 81(S 01): S1-S272
DOI: 10.1055/s-0040-1702326
Oral Presentations
Georg Thieme Verlag KG Stuttgart · New York

Choroid Plexus Papillomas of the Cerebellopontine Angle and Fourth Ventricle: Cohort Study

Amanda M. Casabella
1   Mayo Clinic, Rochester, Minnesota, United States
,
Avital Perry
1   Mayo Clinic, Rochester, Minnesota, United States
,
Christopher S. Graffeo
1   Mayo Clinic, Rochester, Minnesota, United States
,
Lucas P. Carlstrom
1   Mayo Clinic, Rochester, Minnesota, United States
,
Michael J. Link
1   Mayo Clinic, Rochester, Minnesota, United States
› Author Affiliations
Further Information

Publication History

Publication Date:
05 February 2020 (online)

 

Introduction: Choroid plexus papilloma (CPP) is a benign WHO grade-I lesion, which accounts for 1% of adult and 2.5% of pediatric intracranial tumors. CPP predominantly arise in the lateral ventricle, while posterior fossa CPP involving the cerebellopontine angle (CPA) or fourth ventricle are rare, with incompletely understood natural history and optimal treatment.

Methods: Single-institution cohort study of CPP arising within the CPA or fourth ventricle, 1990 to 2018.

Results: A total of 11 CPP were identified in the CPA, and 28 within the fourth ventricle. The most common presentations were headache (89%), imbalance (67%), and dizziness (44%). Cystic components were observed exclusively in CPA tumors (57 vs. 0%), while calcification was comparably prevalent in both groups (27 vs. 25%). Gross total resection (GTR) was achieved in 73% overall, 72.7% in the CPA and 73.1% in the fourth ventricle. Recurrence/progression was observed in five CPA tumors (46%) and nine intraventricular CPP (32%), which were treated with various combinations of radiosurgery (n = 14), radiotherapy (n = 4), repeated surgery (n = 3), and chemotherapy (n = 9). Multiple instances of recurrence/progression were observed in six patients (15%). Atypical (WHO grade II) CPP were diagnosed in one CPA tumors (9%) and three fourth ventricle CPP (11%), while metastatic lesions were subsequently identified in three patients, only one of which was classified as atypical on initial histopathologic diagnosis.

Conclusion: CPP are benign tumors that are rarely encountered in the posterior fossa, where they typically present with characteristic signs of mass effect. Although generally amenable to resection, CPP are frequently adherent to cranial nerves, potentially predisposing to increased risk of recurrence in the CPA. Atypical CPP are exceedingly uncommon, but require aggressive, multi-modality oncologic therapy, and metastatic tumors