Choroid plexus papilloma and atypical choroid plexus papilloma in children: treatment results

Choroid plexus tumors (CPTs) are rare intraventricular tumors, accounting 2–5% of brain tumors in children. Choroid plexus papilloma (CPP) classified by the World Health Organization (WHO) on the basis of histological criteria as benign tumor (WHO grade I), CPPs accounts for 10–20% of brain tumors during the first year of life, the median age of patients with CPP at the time of diagnosis – 3.5 years. Atypical choroid plexus papilloma (APP) is an intermediate (WHO grade II) tumor which was officially included in the WHO classification in 2007. Median age 0,63 years. In this work, for the first time in the Russian literature, we present the outcomes of CPP and APP therapy in a large cohort of patients. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. In our study from January 1, 2009 to December 31, 2019, 83 CPP and 37 APP were histologically verified. For all patients, surgical removal of the tumor was the first stage of treatment. In 79 (95.2%) patients with CPP and in 32 (86.5%) children with APP, radical resection was performed at the first stage. In 73.5% of children with CPP and 62.2% of children with APP there was no intra- and postoperative complications. In both CPP and APP, the 5-year EFS in patients with complete resection was statistically significantly higher compared to subtotal tumor resection (100% and 33 ± 27% in CPP, 94 ± 4% and 25 ± 22% for APP) (p < 0.001). Chemoradiation therapy was given to 1 patient with CPP, 4 patients with APP with tumor recurrence/progression.

The use of exoscope combined with tubular retractor system for minimally invasive transsulcal resection of an ventricular atrium atypical choroid plexus papilloma: Three-dimensional operative video

Background: Choroid plexus papilloma represents 1–4% of pediatric brain tumors, mostly located in the ventricular atrium.[1] Intraventricular tumors represent a challenge due to the poor visualization of the surgical field and damage to surrounding structures.[2] Use of tubular retraction reduces cerebrovascular trauma to the surrounding parenchyma by distributing pressure uniformly, allowing less invasive corticotomy, and more stability on surgical corridors that allow the surgeon to use both hands and external visualization devices.[2-5] Case Description: We present the case of a 3-year-old boy with progressive headache, vomiting, and loss of control in the left hand for 3 months, with a history of ventricular shunt placement for acute obstructive hydrocephalus. The MRI revealed large lobulated lesion, which was hypointense on T1, hyperintense on T2, marked enhancement on T1 C+ (Gd) within the atrium of the right lateral ventricle, and spectroscopy with a peak of choline. Written consent for the use of photos and videos on this work was obtained from the patient’s mother. A high-definition two-dimensional exoscope (VITOM® Karl Storz, Tuttlingen) was used during the surgical approach and throughout tumor removal, which was aided by ViewSite Brain Access System (VBAS®; Vycor Medical Inc.).[3] We performed a transparietal minimally invasive transsulcal parafascicular approach through the Frazier point for direct access to the ventricular atrium. Histological examination confirmed atypical choroid plexus papilloma. Postoperative imaging shows no residual tumor. The postoperative course was satisfactory with improvement of the headache and control of the left hand, leading to discharge home 1 week after surgery. Conclusion: The tubular transparietal minimally invasive approach obviates the need for traditional approaches to the atrium. This technique is safe and effective for the treatment of intraventricular and periventricular lesions, thus making this challenging target in more accessible to neurosurgeons, avoiding structure damage and any associated morbidity or mortality.