Infratentorial atypical choroid plexus papilloma in an adult: A case report and literature review

Atypical choroid plexus papilloma is classified as WHO (World Health Organization) Grade II subtype of choroid plexus tumor, and it has intermediate pathological features, prognosis, and clinical outcome rates between choroid plexus papilloma and choroid plexus carcinoma. These tumors are more common in children compared with adults and are usually located in the lateral ventricles. We present a case of an adult with atypical choroid plexus papilloma located in the infratentorial region. A 41?year?old woman underwent evaluation for headache and dull aching neck pain. Magnetic resonance imaging (MRI) of the brain revealed a well?defined intraventricular mass lesion in the fourth ventricle and foramen of Luschka. She underwent craniotomy and gross total excision of the lesion. Histopathological and immunohistochemical findings confirmed the diagnosis of atypical choroid plexus papilloma (WHO Grade II). We discuss the various treatment options for this condition and review the relevant literature.

Malignant congenital orbital teratoma: Glioblastoma multiforme with primitive neuronal pattern and choroid plexus papilloma: A rare diagnosis at an unusual age

Childhood orbital teratomas are usually congenital lesions that are most often present at birth with progressive, massive unilateral proptosis. During the routine controls of 27-year-old woman between 26–27th weeks of pregnancy her fetal ultrasonography (USG) revealed a mass in the eye of the fetus, and termination was recommended. The family refused the termination option and in the 37th week of pregnancy, vaginal delivery is performed in an external medical center. The mass in the baby's eye was surgically removed. Microscopic examination revealed disorganized ocular tissues, adipose tissue, microcalcification, nerve plexuses as well as areas of neuronal nodules including hypercellular areas, palisatic necrosis, and microvascular proliferation. The immunprofile––patchy GFAP staining in the areas of cellular epithelioid and spindled cells that also show an focal and sparse expression p53 staining and a high proliferation rate in Ki67 staining––confirmed the hematoxylin–eosin (HE) impression of a teratoma with a component of glioblastoma (GBM). In this unique presentation of a malignant orbital teratoma with a GBM, we have identified three tumor components: (1) GBM component, (2) nodules of neuroglial tissue with mature neurons and BRAF mutation, and (3) papillary proliferation possibly representing a choroid plexus papilloma.

Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children / 中华医学杂志(英文版)

Background@#Decision-making concerning the treatment of choroid plexus tumor (CPT) in pediatric patients remains a topic of considerable debate. The aim of this work was to describe clinical features and prognostic risk factors of CPT in the pediatric population and to provide theoretical opinions regarding clinical decisions for CPT.@*Methods@#The data of 96 patients with CPT and younger than 14 years were retrospectively analyzed. Clinical characteristics such as pathological type of CPTs, rate and severity of hydrocephalus, treatment and outcome, and recurrence were investigated. For categorical variables, the Pearson's Chi-square test was performed. The Mann-Whitney U-test was used for comparisons between nonnormally distributed parameters. Log-rank test was used for progression-free survival (PFS).@*Results@#The study included 70 choroid plexus papilloma (CPP) cases, 17 atypical choroid plexus papilloma (aCPP) cases, and 9 choroid plexus carcinoma (CPC) cases. Compared with patients with CPP or aCPP, patients with CPC had a shorter disease course (median: CPP, 4 months; aCPP, 2 months; CPC, 1 month; H: 23.5, P 0.05). Patients with CPC experienced shorter time for recurrence than those with CPP or aCPP (χ = 40.1, P < 0.0001).@*Conclusions@#Our results indicated that CPP in the fourth ventricle could trigger serious clinical symptoms at an early stage, requiring early intervention. Adjuvant treatment might be necessary for patients with partially resected CPP, aCPP, and CPC to achieve a favorable outcome.

Sellar-Suprasellar Extraventricular Choroid Plexus Papilloma : A Case Report and Review of the Literature

Choroid plexus papillomas (CPPs) are relatively rare neuroectodermal tumors that develop from choroid plexus epithelial cells and are usually restricted to the ventricles. Extraventricular CPPs are very unusual and can be difficult to diagnose and treat. A 50-year-old male patient was admitted to our clinic complaining of headache and visual deterioration. Neurological examination found no abnormalities except decreased light perception and secondary optic atrophy in the left eye. Endocrine testing revealed normal levels of hormones produced by the pituitary and target glands. Magnetic resonance imaging of the brain revealed a huge regular-shaped lesion in the sellar-suprasellar region occupying the sella turcica and extending into the suprasellar cistern and planum sphenoidale. The lesion was completely excised by microsurgery via an ordinary left-sided pterional approach. Histopathology identified the lesion as a choroid plexus papilloma. Following the case report, literature on the origin, differential diagnosis, and treatment of this rare tumor is reviewed.

Unusual Radiologic Findings and Pathologic Growth Patterns on Choroid Plexus Papillomas

OBJECTIVE: Choroid plexus papillomas (CPPs) are generally regarded as benign tumors with typical radiologic and pathologic findings. However, they sometimes have unusual findings. We have analyzed radiologic findings and pathologic growth patterns on CPPs. METHODS: The study group included 5 male and 5 female patients (age range, 3 months to 58 years : median, 29 years). The study group included 3 pediatric and 7 adult patients. All patients underwent surgery; 9 patients had a gross total resection and 1 patient had a subtotal resection. We analyzed the radiologic findings (location, size, mottle-like appearance, enhancement, calcifications, and hydrocephalus) and pathologic growth patterns (typical papillary, papillary and solid, and papillary and tubular). RESULTS: The median follow-up duration was 21.3 months (range, 4-47.8 months). There were no recurrences after initial treatment. All patients had benign CPPs. Pediatric CPPs were 3.2 cm masses (range, 2.7-4 cm) with homogeneous enhancement and a mottle-like appearance, which pathologically showed the papillary growth pattern. Hydrocephalus was present in all pediatric patients. Postoperatively, subdural hygroma had occurred in two patients. In adults, CPPs were located in the fourth ventricle in 6 patients and suprasellar area in 1 patient. The size varied from 0.5-4.2 cm. Hydrocephalus and calcifications occurred in 3 and 4 patients, respectively. Three patients showed the heterogeneous enhancement without a mottle-like appearance and pathologically showed combined papillary and solid growth in 2 patients and papillary and tubular growth in one. Postoperatively, two patients with large masses had injuries of the brainstem and underwent shunt procedures for aggravation of hydrocephalus. CONCLUSION: CPPs may show unusual radiologic findings, which preoperatively give the difficulty to be differentiated from other tumors. CPPs with unusual radiologic findings showed the combined pathologic growth patterns.

Adult choroid plexus papilloma: cause of sudden death.

Choroid plexus papilloma (CPP) is a rare, benign neoplasm, relatively more common in childhood. It is associated with signs and symptoms of increased intracranial pressure, frequently in association with obstructive hydrocephalus. CT and MRI are the investigations of choice and are diagnostic. Sudden deaths have been reported, but are very unusual. A 41 year old male was brought for medico-legal autopsy examination on ground of sudden death. He was reported to have headaches over a long period of time. On autopsy examination, massive sub-arachnoid hemorrhage was seen on both the cerebral hemispheres and cerebellum. A cyst measuring about 1 cm diameter was found in choroid plexus of right lateral ventricle. On histopathological examination, it was found to be a choroid plexus papilloma. Calcification was also evident in the papilloma. From medico-legal aspect, the present case reveals an unusual cause for sudden death in an adult male. The pathology could have been diagnosed easily by CT scan or MRI. When diagnosed, it has good survival rate, the morbidity depending on the extent of pathological effects. The present case was likely to have survived having minimal effects with appropriate treatment had he been diagnosed. The pathology is rare and a suspicion for this pathology in the adult male was not expected, but a CT scan to investigate chronic headache was warranted. Absence of such a suggestion leading to death, which could have been preventable, is sufficient ground for charge of professional negligence.

Atypical Choroid Plexus Papilloma in an Adult

We present an extremely rare case of the atypical choroid plexus papilloma in an adult which developed at the trigone of right lateral ventricle. A 62-year-old woman presented with the history of intermittent and gradually progressive headache and left side hemiparesis for 6 months. The brain magnetic resonance image showed highly enhanced and well demarcated mass at the trigone of lateral ventricle attached to the choroid plexus. Gross total resection was performed by transcortical approach via the middle temporal gyrus. The tumor was diagnosed as an atypical choroid plexus papilloma. She had no neurologic deficit after the surgery. We report a case of atypical choroid plexus papilloma in adult and introduce newly classified pathologic characteristics of this tumor.

Spinal Drop Metastasis from a Posterior Fossa Choroid Plexus Papilloma

Choroid plexus papillomas (CPPs) are typically considered as benign tumors, with a favorable long-term prognosis. Drop metastasis of CPP into the spinal subarachnoid space is rare. We report a 42-year-old woman who presented with headache and back pain 6 years after removal of a posterior fossa CPP. Magnetic resonance imaging revealed mass lesions in the lumbosacral subarachnoid space and recurrent intracranial tumor. The lesions were resected and histologically diagnosed was CPP. We consider that CPP can spread via cerebrospinal fluid pathways and cause spinal drop metastasis. Therefore, it is necessary to evaluate the whole spinal axis and to perform periodic follow-up examinations in patients with CPP.

Surgical Removal of a Huge Atypical Choroid Plexus Papilloma

The authors present a case of huge atypical choroid plexus papilloma in both lateral and third ventricles. A 2-year-old girl presented with lethargy and vomiting. Magnetic resonance images disclosed a huge enhancing mass, which probably arose from the left lateral ventricle and extended to the right lateral and the third ventricles. Total removal of the mass via the interhemispheric transcallosal approach was done. Histologically, an atypical choroid plexus papilloma was identified. Second operative treatment, subduro-peritoneal shunt, was performed due to postoperative subdural effusion. The patient was discharged two weeks after the second surgical procedure without a neurological deficit.

A Case of Choroid Plexus Papilloma of a Child with Anorexia and Failure to Thrive / 대한소아신경학회지

Choroid plexus papilloma is a very rare disease in children. The manifestations of the disease differ according to the tumor size and the location. Increased CSF production by the tumor, hydrocephalus and symptoms of increased ICP are the main symptoms. The first line of treatment is surgical excision, but sometimes excision itself is very difficult due to the site and the size of the mass. Adjunctive radiation therapy or chemotherapy is not recommended. Since gross total resection without adjunctive therapy offers highest likelihood of success. Significant prognostic factors are sex, age, time of diagnosis, the duration between the appearance of symptoms and diagnosis, tumor volume, tumor site and the extent of surgery. We report a case of choroid plexus papilloma, in a child with anorexia and failure to thrive.

Two Primary Intracranial Tumors of Different Histology: Report of a Case with a Choroid Plexus Papilloma and a Concurrent Vestibular Schwannoma in the Cerebellopontine Angle

A very rare case of multiple primary intracranial tumors is reported. A 41-year-old female patient was referred for surgery with a cerebellopontine angle (CPA) tumor. Medical history and MRI study showed typical findings of a right acoustic neuroma with a hydrocephalus. Neurological, dermatological, and ocular examinations revealed no evidence of neurofibromatosis. During surgery, a red-colored cauliflower like mass was found in the right CPA. The roof of the fourth ventricle could be seen through the lateral recess after removal of the tumor. Another mass, a 1.5-cm sized schwannoma protruding through the right internal auditory meatus, was removed by the transmeatal approach. Although the tumor masses were in contact and compressed against each other, there was a clear demarcation between them. Histological examination confirmed that the first mass was a typical choroid plexus papilloma with fibrovascular core, and that the second was a schwannoma. The patient recovered without any new neurological deficit. Result of a Medline search indicated that this rare combination of multiple primary tumors has not been reported previously.

Encapsulated Choroid Plexus Papilloma with Surrounding Large Cyst

The case of a 6-month-old male infant with a choroid plexus papilloma showing unusual features is presented. This case is very unusual because the tumor did not have any connection with the choroid plexus but was attached to the normal brain parenchyme, encapsulated with thick hyalinized membrane and surrounded with large multi-septated cyst. At the surgery, it was confirmed that the tumor was in fact located at the right temporal lobe without having any connection to the normal choroid plexus and surrounding cyst was not lateral ventricle. Histopathological examination revealed a choroid plexus papilloma. We present this unusual case of choroid plexus papilloma with review of literature. Also, the pathogenesis of the tumor capsule and surrounding cyst is discussed.

Surgical Outcome of Choroid Plexus Tumors in Adults : Experience of 5 Cases

A retrospective analysis of five adult patients with choroid plexus tumor(four choroids plexus papillomas, one choroids plexus carcinoma who underwent surgical resection at our institute between February 1991 and October 1995 was performed. The study group included two males and three females with a mean age of 34.0 years(range 21 to 24 years). Two of four choroids plexus papillomas were removed gross totally and one of these was irradiated postoperatively. The remaining two patients with choroids plexus papilloma underwent a subtotal surgical resection and one of them received radiation therapy. During follow-up, the authors could not find any difference in the size of the residual tumor or in the patient's KPS between the irradiated groups. There was also no response to postoperative radiation therapy in a patient with choroids plexus carcinoma who underwent a subtotal resection. The author's clinical experiences suggest that the use of radiation therapy in adult patients with choroids plexus tumor is not effective, and thus gross total resection should be attempted during the primary operation of such tumors.

Transient Mutism following Resection of 4th Ventricle Choroid Plexus Papilloma in a 6-Year-Old Girl

Transient mutism has ben known as a rare complication following a posterior fossa approach to cerebellar tumors, although its pathophysiology remains unclear. Cerebellar mutism may be defined as a transient mutism occurring after a posterior fossa tumor approach in children with unimpaired consciousness, unimpaired symbolic functions, no detectable deficit of cranial nerves or peripheral organs of speech, and no lesions of long pathways in the course of the cranial nerves at the level of the brain stem. Forty two cases in children, and only two cases in adults have been found in the literature. We report a case of transient mutism with oropharyngeal apraxia after total excision of 4th ventricle choroids plexus papilloma in a 6-year-old girl. Postoperatively, the patient became mute and refused food intake without disturbance of consciousness, or lower cranial nerve function. Computed tomography(CT) and magnetic resonance image(MRI) demonstrated no abnormal findings except the surgical lesion in the posterior fossa. The mutism lasted for 3 weeks, but she gradually became fluent. The mechanism of "cerebellar mutism" is briefly discussed with literature review.

Papillary ependymoma: its differential diagnosis from choroid plexus papilloma

Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. Here, we report two cases of papillary ependymomas regarding their unique clinicopathologic features and differential points from choroid plexus papilloma. Brain MRI revealed a large mass in the left lateral ventricle in one case and a 3cm sized mass in the pineal area and the 3rd ventricle in the other. Microscopically, the tumor was characterized by papillary and tubular structures. Immunohistochemically, the tumor cells in both cases expressed cytokeratins(CK22 and CAM 5.2) but did not express glial fibrillary acidic protein(GFAP), vimentin, epithelial membrane antigen, and S100 protein. This is a very unusual immunohistochemical feature for papillary ependymoma. Ultrastructurally, the tumor showed a mosaic pattern of tumor cells with frequent intercellular microrosettes having a few stubby microvilli, a few cilia and zonulae adherentes. The cytoplasmic processes were markedly reduced compared to conventional ependymoma. The cytoplasm did not contain intermediate filaments. Interestingly, the mitochondria showed abnormal features with a pleomorphic shape and abnormal cristae in both cases. These ultrastructural features enabled differentiation between papillary ependymoma and choroid plexus papilloma in addition to the light microscopic findings.

Choroid Plexus Papilloma in Third and Fourth Ventricle

Choroid plexus papilloma(CPP) is a rare intraventricular neoplasm occurring primarily in the lateral ventricle of children and the 4th ventricle of adults. The 3rd ventricle is an extremely rare site for CPP to occur. The authors report two cases of CPP of the 3rd ventricle in a 2-month-old girl and a 4-month-old boy, and one case of CPP of the 4th ventricle in a 17-year-old male. Two CPP of third ventricle were totally removed by transcortical-transventricular approach and transcallosal subchoroidal approach, respectively. The CPP in the fourth ventricle was totally removed by suboccipital approach. The subdural hygroma and hydrocephalus due to CSF overproduction was managed with subduroperitoneal shunt and ventriculoperitoneal shunt.

Two Cases of Choroid Plexus Papilloma of the 4th Ventricle and Cerebellopontine Angle in Adult

The choroids plexus papillomas are rare neoplasms of central nervous system, constituting about 0.5% of intracranial tumors. They are most common in children, especially in the first two year of life. In adult, they usually occur within posterior fossa, but cerebellopontine(CP) angle choroids plexus tumor is extremely rare. The authors report two cases of 4th ventricle and CP angle choroids plexus papilloma in adult.

A Case of Lateral Ventricle Choroid Plexus Papilloma in an Infant

Choroid plexus papillomas are uncommon neoplasms of the central nervous system, accounting for 0.4% to 0.1% of all intracranial tumors. We present a choroid plexus papilloma in the trigone of the lateral ventricle in an 8 months old infant with hydrocephalus. We successfully controlled hydrocephalus and subdural fluid collection after total removal of the mass.

Congenital Choroid Plexus Papilloma: Report of a case

The choroid plexus papilloma is a rare tumor. Its incidence is 0.4-0.6% of all intracranial tumors. Most cases of this tumor are found in the young subject, especially infant and childhood but its congenital form is very rare. The clinical and pathologic findings of congenital choroid plexus papilloma are similar to that of any age. But the cilia on the cell surface are common in the former and very rare in the latter. We present a case of congenital choroid plexus papilloma of the lateral ventricle in a 2 month-old male baby. He had suffered from a sudden onset of convulsions and a drowsy mental state for 2 days. The CT scan revealed a large intraventricular tumor in the left lateral ventricle with hydrocephalus. After ventriculo-peritoneal shunt(V-P shunt), his symptoms were improved. But the hydrocephalus was aggravated due to malfunction of V-P shunt, and he recieved the operation after 4 months. The gross examination revealed a large ovoid papillary tumor(4x3x3cm). On light microscopic examination, the tumor showed papillary structure lined by columnar eosinophilic cells. Some cells had cilia. The electron microscopic finding showed intercellular junction, microvilli and cilia. The tumor cells were positive for cytokeratin, vimentin and S-100 protein.

A Case of Lateral Ventricle Choroid Plexus Papilloma in a Child

The choroid plexus papillomas are uncommon neoplasms of the central nervous system, and constitute 0.5~0.6 percent of intracranial tumors. These tumor are surgically treatable and the encouraging results of surgical removal justify an aggeressive surgical approach. We present a case of lateral ventricle choroid plexus papilloma in a child with communicating hydrocephalus.