Management of Choroid Plexus Tumors and the Benefit of Preoperative Embolization in Pediatric Patients: Report of 46 Cases from a Single Institution.

OBJECTIVE: Optimal choroid plexus tumor (CPT) treatment involves gross total resection; however, intraoperative hemorrhage risk remains significant given tumor vascularity. This study describes pediatric CPT management and identifies patients most likely to benefit from preoperative embolization. METHODS: CPTs resected from 1997 to 2021 were included. The characteristics of embolized patients were compared to nonembolized patients; nonembolized patients were further stratified based on open vascular control-pedicle feeder ligation versus no pedicle ligation prior to tumor debulking. Statistical analyses identified factors associated with estimated blood loss (EBL), transfusion, length of stay, and complications. RESULTS: Among the 46 CPT cases identified, 98% achieved gross total resection, and 15% received embolization. Embolized patients were younger, smaller, and had larger tumors compared to nonembolized patients (median: 0.8 vs. 2.1 years; 9.3 vs. 14.4 kg; 91.08 vs. 5.5 cm3). Transfused patients were similarly younger and smaller (P < 0.05) than nontransfused patients. Among nonembolized patients, open vascular control was achieved in smaller tumors (<13 cm3) with significantly lower EBL (P = 0.002). Higher EBL was observed in patients with larger tumors, hydrocephalus, transependymal edema, vomiting, lethargy, and developmental regression (all P < 0.05). Patients with lethargy had longer hospital stays and a higher likelihood of postoperative complications (P < 0.05). There were no significant differences in complication rates between the embolization and nonembolization groups. CONCLUSIONS: Despite higher surgical risk profiles, embolized patients had similar complication rates and postoperative hydrocephalus management as nonembolized patients. Embolization was particularly beneficial in patients at high risk for surgical morbidity, such as those <2 years, weighing <10 kg, and with a tumor volume >15 cm3.

Extracerebral choroid plexus papilloma in a newborn: a rare presentation.

Choroid plexus papillomas (CPPs) are extremely rare lesions that originate in the central nervous system. Still rarely, these can occur in heterotopic locations. We report a case of aneonate who presented at fourth week of life with a small swelling in the left side of the oropharynx. There were no other symptoms. MRI scan revealed a cystic lesion with the possibility of lymphatic malformation or teratoma. Swelling which was gradually increasing in size was electively excised. Histopathological examination revealed it to be an extracerebral CPP. Postoperative period was uneventful, and baby had no further complications. CPP is a rare lesion and, almost always, cannot be anticipated before surgery. Imaging only helps in surgical planning. Complete excision is curative.

Tractography Navigated Endoscopic En-Bloc Excision of Pediatric Giant Choroid Plexus Tumor.

Giant choroid plexus (CP) tumors in children pose a formidable surgical challenge due to extensive vascularity/blood loss, tumor size impeding early visualization of the pedicle, hydrocephalus/mass effect distorting cerebral localization, considerable prevalence of atypical tumors and carcinoma demanding excision without tumor spillage, and retraction-associated morbidity. However, total resection of CP papilloma has excellent potential for cure. This is probably the first report in the literature of diffusion tensor imaging navigation-guided tumor pedicle targeting, endoscopic devascularization and division of pedicle followed by en bloc delivery in optimally tackling most of these challenges in a 6-year-old girl presenting with a giant lateral ventricular CP tumor. Giant CP tumors pose a formidable challenge. Extensive vascularity can cause life-threatening blood loss in children.1 Large tumor size makes it impractical during microsurgery to achieve early visualization of pedicle.2 Hydrocephalus and mass effect can distort sulcal anatomy, with potentially devastating deficits.3 Still, prevalence of atypical tumors and carcinoma warrants excision without tumor spillage.4 In Video 1, we demonstrate our "10-D" steps of en-bloc excision, exploiting panoramic visualization of endoscope5: 1. Diagnosis, 2. Diffusion tensor imaging guided pedicle targeting, 3. Design position & exposure, 4. Durotomy, 5. Dissection of sulcus, 6. Delineation of pedicle, 7. Devascularization, 8. Division of pedicle, 9. Delivery of tumor, and 10. Dural & skin closure. The conventional superior parietal lobule approach to get the tumor en-bloc would have been from the posterosuperior direction, where the tumor is likely to conceal the pedicle. The trajectory to first get to the pedicle must be from an anterosuperior direction but will violate corticospinal fibers. Hence entry point was chosen in between, just posterior to the post-central sulcus. To accommodate the 'en-bloc' excision avoiding ventricular seedlings, a 5 cm mini-craniotomy was fashioned centered on the entry point planned in the navigation system. Ventricle was entered perpendicular to the sulcus through the roof of the atrium, with least cortical transgression and avoiding injury to laterally placed optic radiation and speech areas.6 A 30-degree, 4-mm endoscope was inserted anterolateral to the tumor and fixed. The wide-angled vision offered by endoscopes enhancing meticulous dissection is the likely cause of better neurological outcomes, as noted in other ventricular lesions.7 Pedicular attachment of the tumor is coagulated thoroughly and cut, ensuring initial sparing of venous drainage. The draining vein is then coagulated and divided. 'En-bloc' excision is also known in other vascular lesions to decrease the risk of bleeding.8 The angled optics & panoramic visualization helps to identify any possible tumor seedlings.9 This is probably the first report of endoscopic en-bloc excision of a giant choroid plexus tumor in literature.

Rare Case of Hemorrhagic Atypical Choroid Plexus Papilloma of Fourth Ventricle in Adult: A Diagnostic Dilemma.

Choroid plexus papillomas are highly vascular tumors, and such tumors causing subarachnoid hemorrhage have been reported in literature. Similarly, few articles have reported atypical fourth ventricular choroid plexus tumors in adults. However, such an atypical tumor presenting with grossly hemorrhagic transformation without any acute symptoms could not be found in the literature.

Infantile Choroid Plexus Papilloma with Multiple Peritumoral Cysts.

Infantile choroid plexus papilloma (CPP) associated with multiple peritumoral cysts is a rare variant of CPP, and clinical course and optimal management are largely unknown. A 9-month-old boy presented with a large solid tumor in the left lateral ventricle associated with multiple peritumoral cysts, arachnoid cysts, and hydrocephalus containing xanthochromic fluid with high protein content. Shrinkage of these cysts and resolution of hydrocephalus were achieved after total resection of the hypervascular solid part of the tumor. Histological examination confirmed the solid part of the tumor as CPP and showed that the wall of the peritumoral cysts consisted of reactive gliosis without neoplastic cells. Follow-up magnetic resonance imaging 12 months after surgery revealed that these cysts remained stable. CPP with nonenhancing peritumoral cysts can be managed by resection of only the solid part of the tumor without permanent cerebrospinal fluid diversion.

Case Report: Detailed Clinical Course and Management Plan for Status Epilepticus Pediatric Patient with Resected Choroid Plexus Papilloma: A Case Report and a Single Center Experience.

Choroid plexus papilloma (CPP) is a benign but rare central nervous system (CNS) neoplasm of the choroid plexus. The onset of symptoms is usually in the first decade and may occur at birth (i.e., congenital). It accounts for 0.4-0.6% of all brain tumors. Usually seen in patients who are young children. The object of this clinical case to highlight early surgical intervention, intensive and multidisciplinary care, and pharmaceutical prescriptions can enhance the patient's condition and quality of life. We herein report a rare presentation of CPP in a 6-year-old Sudanese female child with seizures. Who suffered from obstructive hydrocephalus with lateral ventricular choroid plexus papilloma. The patient underwent resection at the age of 6 months in our center's neurosurgery department. Intensive and multidisciplinary follow-up managed to maintain positive outcome and better quality of life in a relatively benign neoplasm. In spite of a wide range of therapeutic options for the management of CPP described in the literature, studies demonstrated that patients with CPP alone and underwent a surgical procedure can live independently as adults and work full-time with uncommon recurrences.

CSF Rhinorrhea: A Rare Clinical Presentation of Choroid Plexus Papilloma.

Choroid plexus papilloma (CPP) is a rare brain tumour occurring mostly in infants and children. Most CPPs are intraventricular and present with symptoms and signs of increased intracranial pressure (ICP). This case report describes a middle-aged female who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea from a tumour located in the cerebellopontine angle (CPA). She underwent craniotomy with subtotal tumour resection and remained progression and rhinorrhea-free for several years. Upon clinical progression, the patient was treated with Cyberknife stereotactic radiosurgery. The patient clinically improved and demonstrated a favourable radiologic response to radiosurgery.

Hemifacial Spasm Associated with Compression of the Facial Colliculus by a Choroid Plexus Papilloma of the Fourth Ventricle.

BACKGROUND: Hemifacial spasm is an involuntary condition that involves muscles innervated by the ipsilateral facial nerve. There are secondary causes of hemifacial spasm that can produce a typical presentation of symptoms. Extrinsic compression of the facial colliculus at the floor of the fourth ventricle is responsible for <0.6% of the causes of secondary hemifacial spasm, as the cases with this origin reported in the literature are rare. CASE REPORTS: We present the case of a 43-year-old female with hemifacial spasm of typical characteristics 6 months after onset. Upon clinical examination, a severe contraction of the orbicularis oculi, orbicularis oris, and superficial muscles of the neck displaying 50 crisis per hour was revealed. Brain magnetic resonance imaging showed absence of the facial nerve vascular loop in the cisternal portion, with evidence of an intraventricular tumor in relation with the medial portion of the fourth ventricle at the facial colliculus level, indicating a secondary origin of hemifacial spasm. Preoperative electromyography demonstrated irritative electric activity in the muscular branches of the facial nerve. A telovelar approach was performed to the fourth ventricle with intraoperative electrophysiology monitoring, with immediate resolution of the irritative activity after complete tumor resection. The result of the histopathologic study was a choroid plexus papilloma. CONCLUSION: Fourth ventricle tumors with extrinsic compression of the facial colliculus represent <0.6% of the causes of hemifacial spasm. Its relationship with choroid plexuses papilloma is being described as the first case reported in the literature. Clinical correlation, imaging, and intraoperative findings in conjunction with intraoperative electrophysiology recordings allow to predict the resolution of symptoms after resecting the lesion.

Temporal Horn Choroid Plexus Papilloma Presenting with Seizures in Adulthood: Clinical Case Report and Review of the Literature.

BACKGROUND: Choroid plexus papillomas (CPPs) are benign World Health Organization grade I tumors that comprise 2%-4% of all brain tumors among children and less than 1% of brain tumors in adults. Most adult cases occur in the fourth ventricle, with only 1 previous report describing an adult patient with a temporal horn CPP. CASE DESCRIPTION: We report a rare case of a temporal horn CPP presenting in an adult with seizures. We performed a minimally invasive subtemporal approach for gross total resection of the lesion. CONCLUSIONS: CPP presenting in the temporal horn is rare among adults. We discuss the surgical nuances of the subtemporal approach for resection and review the literature regarding adult presentation of CPP and the treatment strategies for adult CPP.

Management of Choroid Plexus Tumors in Infants and Young Children Up to 4 Years of Age: An Institutional Experience.

BACKGROUND: Choroid plexus tumors (CPTs) are rare tumors characterized by papillary and intraventricular growth. The young age of presentation of such tumors, especially in infants, and the lack of consensus on adjuvant therapy in case of atypical choroid plexus papilloma (aCPP) and choroid plexus carcinoma (CPC) create dilemma for the management of such tumors. We discuss the presentation, management, complications, and outcome in 15 patients (children 4 years of age and younger) and review pertinent literature. METHODS: We retrospectively analyzed the case records of all patients with CPTs who were operated in our institute from January 2010 to March 2018. We found 15 patients in the age group of 0-4 years of age. The variables analyzed include age, sex, presentation, location, surgical approach, extent of resection, intraoperative blood loss, percentage of blood loss, blood transfused, histopathology, postoperative complications, and outcome. Images were obtained from picture archiving and communication system, and patient details and follow-up were obtained from discharge summary, operative notes, and hospital records. RESULTS: Ten patients had choroid plexus papilloma (CPP), 2 patients had aCPP, and 3 patients had CPC. The mean age was 15.2 months, whereas the median age was 8 months (range, 40 days-4 years). The mean blood loss was 329 mL, whereas the median blood loss was 175 mL. There were a total of 5 deaths, including 3 patients with CPC and 1 each with aCPP and CPP. CONCLUSIONS: CPTs are challenging tumors in infants and very young children because of the potential for massive blood loss. CPP is associated with lesser blood loss and favorable outcome compared with aCPP and CPC. Massive blood loss in CPC and aCPP can be life threatening as has been shown in our series. CPC has a rapid proliferation potential as shown in one of our cases. Attempts at decreasing vascularization of such tumors should be made by various methods, including preoperative embolization and neoadjuvant chemotherapy; however, a consensus on this is lacking.

Choroid plexus papilloma presenting as an occipital mass with neck pain: Case report.

RATIONALE: Choroid plexus papillomas are rare benign central nervous system neoplasms arising from choroid plexus epithelium. They are most often located in the lateral ventricle, followed by the fourth and third ventricles and, rarely, in the cerebellopontine angle. PATIENT CONCERNS: We report an uncommon case of a 17-year-old boy who presented with neck pain that had lasted for more than 1 month, with accompanying pain and numbness in his upper extremities. His conditions included slight dizziness, nausea, diplopia, paresthesia, and an unsteady gait. Magnetic resonance imaging (MRI) showed huge cerebellopontine angle tumor that extended to the front medulla oblongata. DIAGNOSIS: Choroid plexus papilloma (WHO I) was diagnosed in this patient. INTERVENTIONS: The patient was referred for neurosurgical intervention. The very large neoplasm was subtotally resected. OUTCOMES: The symptoms of the patient were gradually alleviated after surgery and subsequent radiotherapy treatment, but unfortunately, follow-up of 2 years later revealed that the disease was recurrent and the young man passed away. LESSONS: Neck pain is related to many factors. The case provided an awareness of the origin of severe intracranial disease. It is mandatory to take a thorough clinical assessment with a holistic approach.

[Central nervous system infection by Bartonella henselae associated with a choroid plexus papilloma]. / Infección por Bartonella henselae con compromiso de sistema nervioso central, asociado a un papiloma del plexo coroídeo.

Bartonella henselae infection is a prevalent illness in Chile. It presents generally as a cat scratch disease or as a prolonged fever syndrome. There are atypical manifestations, which include central nervous system, bone, cardiac and hepato-esplenic compromised. We present an adolescent case with a history of fever, vomiting and ataxia, whose diagnosis was a central nervous system infection by Bartonella henselae associated with a choroid plexus papilloma. This case corresponds to an unusual presentation, with a challenging diagnosis. It is controversial whether to treat this patient, which antimicrobial is the right choice and how long the treatment should be.

Treating Cerebrospinal Fluid Rhinorrhea without Dura Repair: A Case Report of Posterior Fossa Choroid Plexus Papilloma and Review of the Literature.

BACKGROUND: Choroid plexus papilloma revealed by nontraumatic cerebrospinal fluid (CSF) rhinorrhea has only been described 5 times, to our knowledge, in the literature. The challenges in this situation are to recognize CSF leak, to rapidly understand the pathophysiology of the leak, and to choose the best treatment strategy in emergency. We report an original case of posterior fossa choroid plexus papilloma revealed by CSF leak. We then discuss the surgical strategy and the pathophysiology of CSF leak, which is explained, in this case, by both hyperproduction of CSF and local skull base erosion. CASE DESCRIPTION: We report the case of a 47-year-old man who has developed spontaneous rhinorrhea, right hearing loss, and confusion. A choroid plexus papilloma of the right cerebellomedullary cistern was diagnosed. Hydrocephalus and pneumocephalus were associated with an erosion of the homolateral skull base. The patient underwent surgical total tumor removal by a median suboccipital approach after implantation of a temporary external ventricular drainage. The patient recovered completely without any recurrence of CSF rhinorrhea. The 5-month postoperative images show total bone re-growth and resolution of hydrocephalus. CONCLUSIONS: Our case shows that 1) causal reasoning is of major importance when dealing with CSF rhinorrhea and that 2) dura repair can be avoided when treating CSF leak secondary to posterior fossa choroid plexus papilloma.

Spinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report.

Choroid plexus papillomas (CPPs) are typically benign tumors that can occur in any age group but are more commonly found in pediatric patients. Although these tumors are benign, there are several reports in adult patients of distant metastases present either at the time of diagnosis or occurring months to years after initial resection. Here, the authors report the case of a 14-year-old boy who presented with symptoms of elevated intracranial pressure due to obstructive hydrocephalus that was caused by a large fourth ventricular mass. Preoperative imaging included a full MRI of the spine, which revealed an intradural lesion that encased the distal sacral nerve roots at the tip of the thecal sac and was concerning for a drop metastasis. The patient underwent gross-total resection of both the fourth ventricular and sacral tumors with histology of both lesions consistent with benign CPP (WHO Grade I). In addition, the authors review prior reports of both pediatric and adult patients in whom benign CPPs have metastasized with either benign or atypical pathology found at a distant site. Taking into account this unusual case and reports in the literature, patients with even benign CPPs may warrant initial and routine follow-up imaging of the total neural axis in search of the rare, but possible, occurrence of drop metastasis.

Infección por Bartonella henselae con compromiso de sistema nervioso central, asociado a un papiloma del plexo coroídeo / Central nervous system infection by Bartonella henselae associated with a choroid plexus papilloma

Resumen La infección por Bartonella henselae es una enfermedad prevalente en nuestro país. En general, se presenta como la enfermedad por arañazo de gato o un síndrome febril prolongado. Existen manifestaciones atípicas dentro de las cuales está el compromiso óseo, cardíaco, hepatoesplénico y del sistema nervioso central. Se presenta el caso de una adolescente con historia de vómitos, fiebre y ataxia, en que se diagnosticó una infección por Bartonella henselae con compromiso del sistema nervioso central, asociada a un papiloma del plexo coroídeo. Este caso corresponde a una presentación inusual, de difícil diagnóstico. Su tratamiento es motivo de controversia, tanto en si es necesario tratar, la elección del antimicrobiano, como su duración.

Bartonella henselae infection is a prevalent illness in Chile. It presents generally as a cat scratch disease or as a prolonged fever syndrome. There are atypical manifestations, which include central nervous system, bone, cardiac and hepato-esplenic compromised. We present an adolescent case with a history of fever, vomiting and ataxia, whose diagnosis was a central nervous system infection by Bartonella henselae associated with a choroid plexus papilloma. This case corresponds to an unusual presentation, with a challenging diagnosis. It is controversial whether to treat this patient, which antimicrobial is the right choice and how long the treatment should be.

Typical Symptoms of Normal-Pressure Hydrocephalus Caused by Choroid Plexus Papilloma in the Cerebellopontine Angle.

BACKGROUND: Choroid plexus papillomas (CPPs) are rare benign intracranial tumors. We report a rare case of CPP manifesting with typical symptoms of normal-pressure hydrocephalus rather than obstructive hydrocephalus. CASE DESCRIPTION: A 45-year-old woman presented with a 6-year history of headache and typical symptoms of normal-pressure hydrocephalus, including gait disturbance, urinary incontinence, and cognitive dysfunction, in addition to the more common symptoms of CPP, such as lower cranial nerve dysfunctions and ataxia. The CPP arose from the right lateral recess of the fourth ventricle and extended to the cerebellopontine angle. Magnetic resonance imaging did not show any radiographic evidence of obstruction of aqueduct, foramen of Magendie, or foramen of Luschka on the contralateral side. We speculated that hydrocephalus was associated with cerebrospinal fluid overproduction by the CPP originating from the choroid plexus of the lateral recess and extending laterally into the cerebellomedullary fissure (CMF). Surgical excision was performed with a trans-CMF approach. The symptoms of normal-pressure hydrocephalus disappeared. In addition to improved clinical symptoms, radiographic findings showed mitigation of ventricular dilatation. CONCLUSIONS: The hydrocephalus was of a communicating type and caused by CSF overproduction. The lateral route of the trans-CMF approach is useful for the dissection of tumors that extend into the unilateral cerebellomedullary cistern through the CMF. This approach not only enables sufficient exposure for resection but also avoids damage to the surrounding structures.

Feasibility and value of preoperative embolization of a congenital choroid plexus tumour in the premature infant: An illustrative case report with technical details.

We present a case of a preterm boy (born at 35 weeks of pregnancy) who was delivered urgently by a caesarean section due to placental abruption. The baby was found to have a tense fontanelle leading to imaging that showed a 5.5 cm right intraventricular mass centred in the atrium, hydrocephalus and extensive surrounding vasogenic oedema. The mass was avidly enhancing with a few large associated vessels suggesting high vascularity. The condition of the baby was acutely deteriorating. He had multiple seizures followed by persistent low level of consciousness in the third day of life. His clinical decline prompted decision to proceed with surgical resection and preoperative embolization. A large single feeder (the right posterolateral choroidal artery) was embolized leading to near complete obliteration of the tumour blush. Embolization likely facilitated subsequent surgery the next day. This is based on an estimated blood loss of 250 ml in this 3 kg baby without haemodynamic instability. Gross total resection was achieved with a single surgery and the diagnosis was choroid plexus papilloma. Utilization of this combined approach in this preterm baby had led to survival in this potentially curable disease.

DYSPHONIA AS AN UNCOMMON PRESENTATION OF PONTOCEREBELLAR CHOROID PLEXUS PAPILLOMA.

A case is presented of a patient with dysphonia, hearing loss and ataxia due to vestibulocochlear and vagal nerve compression by choroid plexus papilloma in the cerebellopontine angle. Choroid plexus papillomas are rare tumors usually arising in the lateral and fourth ventricle, and rarely found in the cerebellopontine angle, making the neuroimaging characteristics usually not sufficient for diagnosis. Patients usually present with headache and hydrocephalus but tumors in the cerebellopontine angle can cause vestibulocochlear dysfunction and cerebellar symptoms. Dysphonia along with hearing loss was a dominant symptom in the case presented. After complete surgical removal of the tumor, deterioration of dysphonia was noticed; it could be explained as peripheral vagal nerve neuropathy due to tumor compression and intraoperative manipulation. In this case report, we describe dysphonia as an uncommon presentation of a rare posterior fossa tumor. To our knowledge, a case of choroid plexus papilloma presenting with dysphonia has not been described before. Our case extends the differential diagnosis of dysphonia from the otorhinolaryngological to the neurosurgical field.