Final results of the Choroid Plexus Tumor study CPT-SIOP-2000

Introduction Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established. Methods CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled. Results For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators. Conclusions Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.

Management of Choroid Plexus Papillomas

Introduction: Choroid plexus papillomas are rare neuroepithelial tumors found primarily in children. It represents less than 1% of all central nervous system tumors. Materials and methods: A retrospective study including 14 patients with choroid plexus papilloma tumors were performed at the Neurosurgery Department in Ait IDDIR Health Hospital Establishment between January 2010 and December 2017. In each case, diagnosis was made clinically and confirmed radiologically and histo-pathologically. All patients were operated. Results and discussion: The mean age was 26 years (ranged 3 months –48 years) .In our department, we grouped together 14 cases of choroid plexus papilloma tumors. For mortality we had one case who died during surgery, survival rate for 04 years is 100% .We had not recurrence during the study period.All patients had intracranial hypertension (HIC) without neurological deficit and benefited from brain CT, MRI and an Angiography. The location of the tumor was: Lateral ventricle, Fourth ventricle, Third ventricle. All patients underwent surgical excision with or without ventriculo-peritoneal shunt. Conclusion: Choroid plexus papillomas are rare neuroepithelial tumors, typically considered benign lesions, derived from the choroid plexus and appear like cauliflower.

Rare Brain Tumors with Infrequent Clinical Manifestations: Illustrative Cases

This chapter describes the epidemiology, clinical and neuroimaging features, histological characteristics, surgical approach, outcomes, and prognostic factors of different cases of very rare intracranial tumors, associated with complex clinical syndromes. Highlighting the important aspects in the diagnosis and management that were considered relevant through the experience of our center. Here we included an intracranial Rosai-Dorfman disease manifested as an apparent multiple meningiomatosis, a choroid plexus papilloma clinically manifested as a hemifacial spasm originated by a compression of the facial colliculus, and a neuroenteric cyst associated with Klippel-Feil syndrome. This type of tumor presents a challenge to the neurosurgeon, originating various questions about its management. In this chapter, we present the experience we had with these pathologies to establish the most appropriate management decisions.

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.

An unexpected sudden death due to a choroid plexus papilloma: an autopsy case report

Background Choroid plexus papillomas (CPPs) are histopathologically benign and rare central nervous system tumors. These tumors remain more frequent in children than adults. It is infrequent for these tumors to cause a sudden unexpected death. We aim in this case to discuss the unusual and fatal presentation of choroid plexus papilloma and the mechanism of death. Case presentation we report the case of a 61-year-old man with no medical history, diagnosed at autopsy with a previously unknown CPP. Initial complaints were chronic headache occurring in the last month and acute chest pain for two days. The forensic autopsy including the histopathologic examination showed a tumoral mass of the choroid plexus in the fourth ventricle diagnosed as a psammomatous CPP. The cause of death in this case was attributed to a massive cerebral edema caused by the tumoral mass. Conclusions Through this case report, we stress the importance of an early and a vigorous investigation of every headache and an early detection of this tumor and we highlight as well the role of the post mortem examination to detect such a fatal complication.

Choroid plexus papilloma in a free ranging eared dove (Zenaida auriculata)

Choroid plexus tumors (CPT) are rare neoplasms and histologically classified as choroid plexus papilloma (CPP), atypical choroid plexus papilloma (APP), and choroid plexus carcinoma (CPC). These neoplasms have been described in humans, domestic animals (canine, feline, equine, caprine) and some wild animals (cetaceans and Psittacidae birds). To our best knowledge, herein we report the first CCP in a free-ranging eared dove (Columbiformes; Zenaida auriculata, de Murs 1847). Histologically, the ventricle was dilated, with a papillary proliferation (arboriform pattern) in topography of CP. The neoplasm was well-differentiated, composed by a single layer of cuboidal cells, anchored in a delicate fibrovascular stroma. The neoplastic cells exhibited moderate stroma, with well-defined borders and round nuclei, with vesicular chromatin and inconspicuous nucleoli. Mitotic activity was low (<1 mitosis per 10 high-power fields). Immunohistochemistry for cytokeratin markers (AE1/AE3 antibody) were implemented, however, both neoplastic cells and normal epithelial tissues do not show immunoreactivity.

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.