Endoscopic removal of a cystic choroid plexus papilloma of the third ventricle: a case report and review of the literature

Cystic choroid plexus papilloma (CPP) of the third ventricle is rare. The authors have reported a case of cystic CPP resected successfully through neuroendoscopic approach. An 18-year-old young man was admitted to our hospital, presented with blurred vision and paroxysmal, intermittent, severe headache for 1 month as well as nausea and vomiting. The findings of neurological examination were normal except for a bilateral papilledema. Magnetic resonance and computerized tomography revealed a cystic lesion in the third ventricle, which did not furnish a definite diagnosis. The tumor was totally resected through neuroendoscopic approach and was found in histopathological examination to be a CPP. The patient retained free of symptoms at 2 year follow-up. To the best of the authors’ knowledge, this is the first reported case of a cystic CPP of the third ventricle involved adult and endoscopic surgery might be a preferred technique to it.     

Oncocytic variant of choroid plexus papilloma

Summary Two cases of oncocytic plexus papilloma are presented which were in the fourth ventricle and cerebello-pontine angle in women aged 70 and 63 years, respectively. The cytoplasm of the transformed tumor cells was enlarged, eosinophilic, and granular; and it was characterized ultrastructurally by innumerable mitochondria. Most of them had lamellar cristae, a clear matrix, and contained dense granules. These oncocytes are identical with oncocytes of the human thyroid, parathyroid, bronchi, kidneys, adrenals, salivary gland and myocardium, and non-neoplastic choroid plexus epithelium. As far as we know, this is the first report of an oncocytic variant of the choroid plexus papilloma. Two unusual cases are described.     

Ultrastructure of disseminated choroid plexus papilloma

Summary A disseminated choroid plexus papilloma (DCPP) with a malignant change in the cervico-spinal leptomeninges observed 4 years after the removal of choroid plexus papilloma (CPP), originating from the fourth ventricle, was studied under the electron microscope. Although the ultrastructure of intracranial CPP has been reported by several authors, there are just a few reports on DCPP. The present tumor was ultrastructurally very similar to normal choroid plexus, but the lack of the capillary fenestration and of invaginations of the epithelial basal plasmalemmas suggested that the epithelium was deprived of secretory function.     

Choroid plexus papilloma of the III ventricle in an infant

A case of choroid plexus papilloma of the III ventricle associated with hydrocephalus in an 1-month-old boy is reported. The tumor was removed after placement of a ventriculoperitoneal shunt; however, the histologically mainly benign tumor grew very rapidly. The cause of this rapid growth may have been a drop in the intraventricular pressure after the shunting procedure.     

First report of occurrence of choroid plexus papilloma and medulloblastoma in the same patient

Introduction Choroid plexus papilloma is a benign epithelial brain tumour showing a striking predilection for infants and occurring most frequently in the lateral and fourth ventricles. Medulloblastoma, on the other hand, is a primitive neuroectodermal tumour and is the most frequent malignant brain tumour of the posterior fossa in children. In this study, we report a metachronous occurrence of choroid plexus papilloma and medulloblastoma in the same patient, which has not been reported before to the best of our knowledge. Case report The authors describe the case of a girl who presented with an atypical choroid plexus papilloma on the posterior wall of the left lateral ventricle at 3 months of age that was resected completely. She was followed up regularly after surgery and made good progress with normal development. At 8 years of age, she presented with right cerebellar medulloblastoma. Discussion The authors review literature for incidence and aetiology of the two tumours.     

The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia

Background Bilateral choroid plexus hyperplasia is a rare condition often associated with cerebrospinal fluid (CSF) overproduction. CSF overproduction is usually so high that the placement of a CSF ventriculoperitoneal shunt almost always results in progressive ascites leading to the necessity of removing the inserted shunt device. A direct surgical treatment of the hyperplastic choroid plexuses is then mandatory. Endoscopic coagulation of the choroid plexuses has been recently proposed as an alternative to open surgical plexectomy. However, the effectiveness of the procedure in controlling CSF overproduction is still debated.Technique We report a case of bilateral choroid plexus hyperplasia in which an extensive bilateral endoscopic coagulation of the choroid plexuses failed to reduce the CSF formation rate sufficiently. A one-stage bilateral open surgical plexectomy was performed.Results The procedure succeeded to control CSF overproduction. Intraoperative blood loss during the surgical removal of the choroid plexuses was significantly reduced due to the previous coagulation of their surface.Conclusion On these grounds, we suggest that endoscopic choroid plexuses coagulation, even when failing to normalize CSF production, may still be considered as a valid adjuvant procedure in the management of this condition.     

Outcomes of the endoscopic endonasal approach for tumors in the third ventricle or invading the third ventricle

We aimed to retrospectively analyze the surgical and clinical outcomes of the endoscopic endonasal approach (EEA) for tumors in the third ventricle or invading the third ventricle. In total, 82 patients who had undergone surgical treatment using the EEA for tumors involving the third ventricle were enrolled in this study. This cohort study comprised 46 male and 36 female patients. The median age was 37 years (range, 5–76), and the median follow-up duration was 56.5 months (range, 6–117). Seventy-six patients had craniopharyngiomas, and 6 had gangliocytomas, gangliogliomas, astrocytomas, diffuse midline gliomas and lymphomas. Gross total removal was performed in 71 (86.5%) of the 82 patients, subtotal tumor removal in 7 patients and partial removal or biopsy in 4 patients. The pituitary stalk was preserved in 20 cases. Visual function improved in 40 (81.6%) of 49 patients. Endocrine function worsened in 41 (50%) of 82 patients. Hypothalamic function improved in 16 (72.7%) of 22 cases. Postoperative obesity occurred in 3 (20.0%) of 15 children and 11 (23.9%) of 46 adult patients. The postoperative cerebrospinal fluid leakage rate was 3.6%. Postoperative meningitis occurred in 18 (21.9%) cases. Permanent diabetes insipidus was identified in 73 (89.0%) of 82 patients. Tumor recurrence was observed in 10 patients (12%). The EEA appears to be a safe and effective treatment modality for tumors in the third ventricle or involving the third ventricle. However, more cases and long-term follow-up outcomes are required to confirm the clinical efficacy of the EEA.     

经胼胝体-脉络膜裂入路的显微解剖学研究

目的 探讨经胼胝体-脉络膜裂入路的显微解剖学特征和临床应用价值.方法 显微镜下对12例成人尸头湿标本进行侧脑室、第三脑室区显微解剖,观察侧脑室、脉络丛、脉络膜裂及第三脑室顶的解剖学特点;在3例成人尸头新鲜标本上模拟经胼胝体-脉络膜裂入路手术,观察与本人路相关的重要解剖结构并测量相关距离.结果 脉络丛位于侧脑室底部内侧,脉络膜裂位于丘脑和穹窿之间,可借助与之相连的脉络丛进行辨认.切开脉络膜裂穹窿带到达第三脑室顶中间帆后,于两侧大脑内静脉间分离打开第三脑室顶即到达第三脑室腔.经测量,大脑内侧缘冠状缝处至胼胝体沟的距离为(39.12±3.94)mm,大脑内侧缘冠状缝处至室间孔的距离为(61.53±4.02)mm,室间孔后缘至穹窿与胼胝体融合处的距离为(28.65±2.23)mm,前后连合的距离为(25.94±2.16)mm,室间孔至乳头体的长度为(19.62±1.79)mm.结论 与其他手术人路相比,经胼胝体-脉络膜裂入路循自然裂隙进入第三脑室,具有路径直接、术野暴露广泛等特点,值得临床推广应用.     

Ossifying choroid plexus papilloma recurring with features of atypical papilloma

A case of ossifying choroid plexus papilloma (CPP) with an unusual clinical course is reported. The patient was a 46‐year‐old woman who underwent surgery for a 3‐cm tumor occupying the fourth ventricle. The tumor showed typical histopathological features of CPP, and the formation of psammoma bodies and mature bone trabeculae was prominent in the stroma. The tumor recurred locally after a dormant period of 15 years, and the recurrent lesion showed invasion of the cerebellum as well as increased cellularity, cellular stratification, nuclear atypism, and mitotic activity, all of which were consistent with a diagnosis of “atypical” CPP. The recurrent tumor did not show ossification of the stroma, although many psammoma bodies were found. There have been very few reported cases of ossifying CPP, and all of the previous cases behaved in a benign fashion. The present case is the first report of ossifying CPP that showed postoperative recurrence with progression to atypical CPP after an unusually long dormant period.     

Xanthogranulomatous colloid cyst of the third ventricle

Summary This is a case report of a xanthogranulomatous colloid cyst of the 3rd ventricle. Posible etiologies for this rare entiry, along with the clinical problems that may be associated with it, and the differential diagnosis are discussed.     

Malignant choroid plexus papilloma of the IV ventricle

We report a case of malignant choroid plexus papilloma (MCPP) of the IV ventricle in a child with prominent extraventricular expansive growth, mostly into the cerebellopontine angle. Interestingly enough, the tumor was entirely covered by thin, smooth, membranous, fibrous tissue, probably derived from the pia mater. In addition, hydrocephalus was not observed. The reason for the lack of hydrocephalus remains speculative, but it is possible that the presence of pure, nonexpansive, fibrous covering on the tumor might have suppressed the tumor growth to some extent, resulting in progressively increased intratumoral pressure. This, in turn, caused the suppression of excessive cerebrospinal fluid production by the tumor cells. From the diagnostic standpoint, immunohistochemical studies, using antiepithelial membrane antigen (EMA) and antitissue polypeptide antigen (TPA), were demonstrated to be useful for diagnosing the tumor.     

Focal glial differentiation and oncocytic transformation in choroid plexus papilloma

Summary The histiopathological features of a choroid plexus papilloma in a 27-year-old male are described. The tumor displayed marked oncocytic transformation and glial differentiation of the epithelium in areas in which there was also marked sclerosis of the fibrovascular cores. Non-membrane-bound bodies of intermediate filaments characterized ultrastructurally the cells with glial differentiation.Support was derived from a grant-in-aid, Department of Pathology, University of Alabama at Birmingham     

Clinicopathologic correlations in epithelial choroid plexus neoplasms: a study of 52 cases

Summary Sixty-seven tumor specimens of epithelial choroid plexus neoplasms obtained by 60 biopsies and 7 autopsies from 52 patients were investigated. Diagnoses of the first operations were choroid plexus papilloma (PP; 32 cases), choroid plexus papilloma with histological atypies (atypical PP; 6 cases), and choroid plexus carcinoma (PC; 14 cases). Carcinoembryonic antigen was expressed by 2 of the 3 biopsies autoptically recognized as metastatic carcinomas and by 2 autopsy cases of PC, while it was absent in all biopsies of true choroid plexus tumors. Tumor cells positive for transthyretin (TTR, prealbumin), S-100 protein (S100), and glial fibrillary acidic protein (GFAP) were detected in 39, 46 and 13, respectively, of the 49 cases of true choroid plexus tumors. Fourth ventricle tumors expressed more S100 (number of positive tumor cells) than lateral ventricle tumors, PP more S100 and TTR than atypical PP/PC. Tumors from patients 20 years of age and older expressed more GFAP and TTR than tumors from younger patients. Of the 30 patients with complete follow-up 19 were alive 2 to 11 years after surgery, including 7 recurrencies. Eleven died from the tumor 4 months to 7 years after surgery. The following histopathologic features (in order of decreasing significance) were correlated with poor prognosis (recurrency or fatal outcome): less than 50% of the tumor cells heavily positive for S100, presence of mitoses, absence of TTR-positive cells, brain invasion by cell nests, absence of marked stromal edema, and presence of necrotic areas. Our results indicate that some histologic features correlate significantly with poor prognosis and that immunohistochemical results correlate with tumor localization, age, and malignancy.     

Immunohistochemical evidence of endothelin-1 in human choroid plexus

Summary An immunohistochemical investigation was carried out on 17 specimens of human choroid plexus obtained post mortem, 1 biopsy of normal choroid plexus including part of the lateral ventricle and 1 papilloma of the choroid plexus removed surgically. The material was fixed in formalin. Paraffin and cryostat sections were used. A polyclonal antiserum to endothelin-1 served as a primary antibody. The avidin-biotin-peroxidase method was applied to demonstrate the immunoreaction. The epithelial cells of the choroid plexuses, the choroid papilloma and most ependymal cells of the lateral ventricle showed a distinct brown reaction product in their cytoplasm indicating antigenic sites to endothelin-1. The reaction was of lesser intensity in the ependymal cells. The connective tissue in choroid plexus was unstained. A positive immunoreaction was present in the walls of some vessels in the choroid plexus in cryostat sections. This is the first report on the presence of antigenic sites to endothelin-1 in the epithelial cells of the human choroid plexus. The role of endothelin in these cells should be investigated to ascertain if the cells synthesize this biologically active peptide or if it is merely bound to receptors in them.Supported by grants from Swedish Medical Research Council, project 03020, 1987 Års stiftelse för strokeforskning, Selanders stiftelse, Åhlen-stiftelsen and Stiftelsen Gamla Tjänarinnor, Stockholm Sweden     

第三脑室脑膜瘤(九例报告及文献回顾)

目的探讨第三脑室脑膜瘤的组织发生学、临床特点和外科治疗。方法报道9例经影像学、手术和病理学证实的第三脑室脑膜瘤，并回顾相关文献。结果第三脑室前部脑膜瘤5例，第三脑室后部脑膜瘤4例，平均发病年龄25．6岁，无明显性别差异；最常见的症状是梗阻性脑积水，缺乏硬脑膜附着为其影像学特征。肿瘤全切除6例。结论第三脑室脑膜瘤是一种罕见的脑室内脑膜瘤，起源于中间帆脉络组织或脉络丛，经半球间裂-胼胝体-穹隆间入路是合理的手术路径选择。     

Expanded endonasal approach: a fully endoscopic completely transnasal resection of a skull base arteriovenous malformation

Objective and importance Vascular lesions with an intraosseus nidus involving the skull base are uncommon and challenging [Gianoli GJ, Amedee RG Vascular malformation of the sphenoid sinus. Ear Nose Throat J. 70:373–375;(1991), Malik GM, Mahmood A, Mehta BA Dural arteriovenous malformation of the skull base with intraosseous vascular nidus. Report of two cases. J. Neurosurg 81:620–623;(1994)]. We present a pediatric patient, with a life-threatening arteriovenous malformation (AVM) of the sphenoid sinus, clivus, and ventral skull base, who failed routine multimodality management of AVMs. An entirely transsphenoidal fully endoscopic resection was used to resect this ventral cranial base AVM with an intraosseus nidus located in the clivus. Clinical presentation A 4-year-old female presented with recurrent, life-threatening hemorrhages from a clival and ventral skull base AVM of the entire clivus and ventral skull base. The patient had been temporized from the age of 2–4 years with multiple internal and external carotid arterial particulate and alcohol embolizations, including both external and internal carotid artery embolizations, intracranial ligation of the right internal carotid artery, and gamma knife irradiation. Despite these multiple interventions, the patient had persistent, life-threatening hemorrhages from arterial recanalization and recruitment requiring intubation, tracheostomy, and nasopharyngeal packing. Intervention The patient underwent a three-stage surgical intervention to resect the AVM. An open subfrontal approach, as the first procedure, provided minimal access to the feeding vessels and was therefore aborted. A two-stage image-guided fully endoscopic approach via a sublabial midface approach without external incisions was performed. Postoperative angiography revealed minimal residual shunting in the pharynx and cavernous sinus. The patient has been free of significant hemorrhages over the past three years. Conclusion Technological advances in endoscopic surgery and image guidance are now allowing for purely endoscopic surgical treatment of previously unresectable lesions with acceptable morbidity. We report the successful and safe resection of a ventral cranial base AVM via a fully endoscopic approach. This paper reports the first AVM with a purely intraosseus nidus of the ventral skull base and demonstrates the ability to deal with complex ventral skull base lesions using a fully endoscopic transsphenoidal technique.     

Choroid plexus papillomas of the III ventricle in childhood

Ten infants and children with choroid plexus papilloma of the III ventricle are presented. Hydrocephalus of various degrees was present in all patients, and seven patients had bilateral ventriculoperitoneal shunts prior to craniotomy. All patients were investigated with computed tomography and angiography. Tumor was resected through the transfrontal-transventricular approach in nine and through a transcallosal approach in one. One patient died intraoperatively due to an uncontrollable hemorrhage from a subependymal vein at its point of entry into the homolateral internal cerebral vein, and another died shortly after surgery due to hypothalamic trauma. The remaining eight patients are alive without recurrence over a minimum follow-up period of 3 years; three have mental retardation and seizure disorder. Despite this tumor's deep location and vascularity and occurrence in infancy, choroid plexus papillomas of the III ventricle can be successfully resected. Appropriate care for hydrocephalus and intra- and postoperative management are important.     

Disseminated choroid plexus papillomas in adults: A case series and review of the literature

Choroid plexus papillomas (CPPs) are uncommon, usually intraventricular, low-grade tumors, accounting for less than 1% of all intracranial neoplasms and 2–4% of brain tumors in children. Dissemination of CPPs to multiple levels of the neuraxis has been seldom observed. Thus far, only 26 adult patients have been reported in the English language literature. With some exceptions, disseminated CPPs have been observed in adults and involved multiple sites along the cerebrospinal fluid pathways. Occasionally, intraparenchymal extension has been documented, and secondary involvement of the suprasellar region has been reported in only five patients. Postoperative treatment of CPPs has not been standardized. Most recommended therapies have been extrapolated from a series of atypical papillomas or carcinomas of the choroid plexus in children. We herein report a series of three patients of disseminated choroid plexus papillomas providing additional insights into this relatively rare entity.     

An immunohistochemical study of cytokeratin and glial fibrillary acidic protein in chroid plexus papilloma

Summary Cellular localization of cytokeratin and glial fibrillary acidic protein (GFAP) was examined in two normal choroid plexuses and five choroid plexus papillomas by the peroxidase-antiperoxidase (PAP) method and double immunofluorescence (IFL) microscopy. Cytokeratin was observed in the majority of epithelial cells in all samples of normal and neoplastic choroid plexuses. On the other hand, GFAP was observed in some of the constituent epithelial cells in two cases of papilloma. Most of these GFAP-positive papilloma cells were simultaneously positive for cytokeratin, as could be seen by the PAP stainings of serial sections and by the double IFL stainings of the same sections. From these findings, it was suggested that normal and neoplastic choroid plexus epithel cells usually express cytokeratin and that some of the neoplastic cells can simultaneously express boty cytokeratin and GFAP.     

Flexible and floating choroid plexus cyst of the third ventricle: an ultrasonographic video documentation

Introduction Choroid plexus cysts can lead to isolation of the lateral ventricles and distension of the third ventricle. We present an ultrasonographic video documentation of an infant with variably shaped and localized choroid plexus cyst of the third ventricle. Case report An infant had periods of increased intracranial pressure with changing dilatation of the first to third ventricle. Cerebral ultrasonography of the not crying boy demonstrated a choroid plexus cyst limply hanging down from the roof of the third ventricle to the beginning of the aqueduct of Sylvius. During crying, the cyst prolapsed from the third into left lateral ventricle and was strangled by the foramen of Monro. Endoscopic cyst fenestration and third ventriculostomy continuously solved the problem of intermittent hydrocephalus occlusus. Conclusion Depending not only on localization and size but also on cyst form and cerebrospinal fluid pressure, a single choroid plexus cyst can cause various obstructions of cerebrospinal fluid pathways.