Variations of disseminated choroid plexus papilloma: 2 case reports and a review of the literature

BACKGROUND: Choroid plexus papillomas are typically considered benign lesions, but histology is not always predictive of their behavior. These tumors can metastasize anywhere along the neuraxis and may be intraventricular, subarachnoid, or intraparenchymal. We present 2 cases that illustrate the wide diversity with which choroid plexus papillomas can disseminate. CASE DESCRIPTIONS: The patient described in case 1 had a primary fourth ventricular choroid plexus papilloma that produced diffuse cystic subarachnoid and leptomeningeal lesions. Patient 2 also had a primary fourth ventricular tumor but with subsequent suprasellar and spinal drop metastases. Patient 1 was treated with temozolomide, resulting in regression of symptoms including headache and dizziness. Patient 2 has been treated with several modalities, including radiation therapy and chemotherapy, with slowing of symptom progression. CONCLUSIONS: Variations of choroid plexus papilloma dissemination include intraventricular, subarachnoid, and leptomeningeal nodules or cystic lesions, and intraparenchymal locations. There is no consensus on the most effective treatment for choroid plexus papilloma metastases; surgical resection, chemotherapy, and radiation therapy may all yield benefits. The prognosis for patients with disseminated choroid plexus papilloma can range from prolonged stable disease and symptoms to death within months.     

Lateral ventricle choroid plexus papilloma in childhood: management and complications

BACKGROUND: A review of the choroid plexus papilloma of the lateral ventricle in the pediatric age group is presented. The characteristic clinical features, imaging studies, preoperative, and operative approaches, as well as complications, will be included. METHODS: Among a group of 24 patients with ventricular choroid plexus papilloma treated by the authors over a 12-year period, seven patients were selected for this study. The selection included patients under the age of 8 years at the time of their presentation with choroid plexus papilloma of the lateral ventricle. Adult patients and children with choroid plexus papilloma at other sites or choroid plexus carcinoma were excluded. At least a 30-month follow-up period was available for all patients except one. Preoperative and postoperative imaging studies confirming total tumor resection were available for all patients. RESULTS: Developmental delays and regression were the most consistent clinical features. Magnetic resonance imaging (MRI) proved to be the diagnostic test of choice. The addition of magnetic resonance angiogram (MRA) obviates the need for angiography. Our attempts at tumor embolization failed. CONCLUSION: An intersulcal splitting approach to the lateral ventricular trigone, combined with a perioperative external ventricular drainage, may be of value in the avoidance of symptomatic subdural effusions.     

Benign choroid plexus papilloma. 2 local recurrences and intraventricular seeding

The authors report a case of choroid plexus papilloma developped in the fourth ventricle. After complete surgical resection, two local recurrences of the tumor occurred, and required new surgical procedures. Eight years after the first operation, a papilloma of the right lateral ventricle was resected. The histological examination showed benign choroid plexus papilloma after each operation. Recurrence and seeding of benign choroid plexus papillomas are infrequent in the literature. For this reason, a long-term follow-up, by clinical examination and CT scan, is necessary for those patients.     

Ectopic presacral choroid plexus cyst in a neonate

An unusual case of a presacral ectopic choroid plexus cyst in a neonate is described. After birth, a soft lump was noticed at the left buttock. Imaging studies including sonography and magnetic resonance imaging demonstrated a presacral cystic lesion extending to the buttocks, composed of several septated cystic masses with no connection to the spinal canal or rectum. After total resection, the tumor was diagnosed as an ectopic choroid plexus cyst. To our knowledge, this is the first case report in the English literature of a presacral ectopic choroid plexus cyst.     

Adult choroid plexus papilloma of the posterior fossa mimicking a hemangioblastoma. Case report

The authors report a case of cystic choroid plexus papilloma that originated in the posterior fossa. No connection with the ventricular system was found intraoperatively. Magnetic resonance (MR) and computerized tomography imaging did not furnish a diagnosis, but findings of pathological examinations were consistent with those of choroid plexus papilloma. The authors describe the different appearances of the tumor on MR images and discuss the differential diagnosis with other tumors of the posterior fossa.     

Intrinsic brainstem choroid plexus papilloma. Case report

The authors report an intrinsic brainstem lesion that was diagnosed initially as a pontine cavernoma, which finally proved to be a choroid plexus papilloma. Choroid plexus papillomas are rare tumors of the central nervous system and are usually intraventricular in location. The occurrence of this tumor in an intraparenchymal location is extremely rare, and its occurrence within the brainstem is previously unreported. The authors also report a trial of chemotherapy with lomustine in the management of the residual tumor.     

Aggressive choroid plexus papilloma

A young woman, who was initially treated for an Arnold-Chiari malformation, was found 7 years later to have a posterior third ventricular tumor. A superior cerebellar approach allowed total resection of the mass, which was an aggressive choroid plexus papilloma. The patient was treated with whole cerebrospinal axis radiation. There is no clinical or computed tomographic evidence of recurrence after 4 years. The literature is reviewed and a discussion of aggressive choroid plexus papilloma (carcinoma) is presented.     

Choroid plexus papilloma with neuropil-like islands

Choroid plexus papilloma may display unusual histologic features, but the presence of neuronal differentiation at the light microscopic level has not yet been described. We thus report a choroid plexus papilloma with neuropil-like islands located within the lateral ventricle of an 11-year-old girl. The absence of atypical histologic features associated with recurrence (particularly increased mitotic activity) and recurrence-free follow-up upon gross total resection are compatible with a diagnosis of choroid plexus papilloma (WHO grade I). This case further emphasizes the capacity of choroid plexus tumor cells toward neuronal differentiation, and expands the spectrum of tumors, which may contain neuropil-like islands.     

Choroid plexus papilloma in the posterior third ventricle in infancy: a case report]

Choroid plexus papillomas represent approximately 0.5% of all intracranial tumors, but they are found in the third ventricle only infrequently. We report a case of choroid plexus papilloma in the third ventricle which is difficult to differentiate from a pineal region tumor. A 4-month-old female presented with bulging fontanelle and sunset phenomenon. A CT scan and MRI showed marked hydrocephalus caused by a tumor extending from the posterior third ventricle to the peneal body. Preoperatively, we diagnosed the lesion as a pineoblastoma. The tumor was totally removed through the occipital transtentorial approach. Pathological examination of the tumor revealed a typical choroid plexus papilloma. The operation was uneventful, and she has grown normally without recurrence of the tumor for three years since the operation. A sagittal gadolinium-enhanced MRI was useful in our case to differentiate a choroid plexus papilloma from a pineal region tumor, because the former extended into the aqueduct forming the shape of the letter V while the latter compressed the aqueduct downward.     

A choroid plexus papilloma of the third ventricle in the neonatal period--a case report

The incidence of choroid plexus papilloma is about 0.5% of all intracranial tumors, but choroid plexus papilloma in the third ventricle is rare. Fortuna reported 56 cases of choroid plexus papilloma of the third ventricle in 1979. Among them, the neoplasm of the third ventricle in the neonatal period was not found. This report is a case of choroid plexus papilloma of the third ventricle in the neonatal period. A 34 day-old female was admitted to our service with complaints of head enlargement, vomiting, and convulsive seizures on January 8, 1981. Enlarged head had been noticed by her family within the first two weeks. On admission, a marked congenital hydrocephalus was diagnosed by CT scan with symmetrical dilated ventricles and no abnormal high or low density area, and V-P shunt was performed on the next day. But several days later, she suffered from progressive abdominal distension, which was disclosed due to CSF overproduction by a choroid plexus papilloma. When the tumor was recognised by enhanced CT scan, her general condition was too poor to attempt a surgical procedure, and she died on March, 19, 1981. Histologically the neoplasm was a typical choroid plexus papilloma of the third ventricle. So far as we know, this case is the first one of the choroid plexus papilloma of the third ventricle in the neonatal period.     

A Case of Difficult Management of Fluid-electrolyte Imbalance in Choroid Plexus Papilloma

A 22-month-old boy presented with nausea and gradual deterioration of gait disturbance. Computed tomography (CT) demonstrated an intraventricular mass lesion in the right lateral ventricle. He was referred to our department 3 weeks after onset. Acute hydrocephalus gradually proceeded 4 days after admission, and external ventricular drainage (EVD) was performed. EVD revealed cerebrospinal fluid (CSF) overproduction (800–1,500 mL/day) under constant pressure of 10 cm H₂O above external auditory meatus. Magnetic resonance imaging showed a multi-lobular mass in the inferior horn of the right lateral ventricle. A choroid plexus tumor was suspected. The ratio of blood urea nitrogen:creatinine (BUN:Cre) remained between 30 and 40, and hemoglobin was between 14.0–17.0 mg/dL, suggesting marked dehydration. Serum sodium varied between 117 and 140 mmol/L, and serum potassium between 2.2 mmol/L and 6.9 mmol/L. The amount of EVD was unstable and fluid balance management was difficult. Hypotonic fluid with sodium chloride supplement was used to adjust thefluid and electrolyte imbalance. Surgical removal of the tumor was performed6 days after EVD and tumor was grossly and totally removed. The high BUN:Cre ratio decreased to about 15 and hemoglobin recovered to 7.5–9.0 mg/dL after removal. Electrolytes returned to the normal range. Overproduction of CSF also markedly improved to < 300 mL/day. Histopathological examination diagnosed choroid plexus papilloma.We experienced a case of choroid plexus papilloma associated with fluid-electrolyte imbalance due to over-drainage after EVD, which could not be effectively controlled before tumor removal. Cautious fluid management and emergent surgical resection might be required to managethe overproduction of CSF and fluid-electrolyteimbalance.     

Choroid plexus papilloma presenting with cerebrospinal fluid rhinorrhea and otorrhea: case report

A 52-year-old woman presented with right rhinorrhea and right otorrhea manifesting as aural fullness for 2 years caused by a choroid plexus papilloma in the right cerebellomedullary cistern. Computed tomography and magnetic resonance imaging revealed a well defined lobulated mass at the foramen of Luschka, which extended towards the right cerebellomedullary cistern with slight dilation of the ventricular systems. The tumor was totally resected via a right lateral suboccipital approach. Histological examination revealed a choroid plexus papilloma. Postoperative course was uneventful, just after the operation rhinorrhea ceased completely, and hearing of the right ear dramatically improved. Choroid plexus papillomas rarely cause cerebrospinal fluid (CSF) rhinorrhea. Total removal of the tumor resulted in the cessation of CSF leaks.     

Intracranial metastasis of a choroid plexus papilloma originating in the cerebellopontine angle region: a case report

The authors present a rare case in which a choroid plexus papilloma originating in the left cerebellopontine angle region metastasized and formed a space-occupying lesion in the right temporal region. A 46-year-old woman with choked disks presented with two separate mass lesions in the right temporal and the left cerebellopontine angle regions. Magnetic resonance imaging exhibited the relationship between the tumors and the surrounding structures. To our knowledge, no case has been reported in which a choroid plexus papilloma originating in the cerebellopontine angle region formed a space-occupying lesion in the right temporal region.     

An adult case of malignant choroid plexus papilloma in the lateral ventricle and the cerebellopontine angle revealed simultaneously

An adult case of malignant choroid plexus papilloma is very rare. This report is an adult case of malignant choroid plexus papilloma revealed in the lateral ventricle and in the cerebellopontine (CP) angle simultaneously. A 37-year-old man was admitted to the hospital complaining of headache, nausea, and a floating sensation on August 29, 1984. Neurological examination on admission revealed bilateral papilledema, left dysmetria and horizontal nystagmus. CT scan revealed a slightly high density round mass in the right lateral ventricle and a cystic mass with mural nodule in the left CP angle. The intraventricular mass and mural nodule were enhanced moderately and homogeneously. The initial surgery was for removal of the CP angle tumor, and 8 days later removal of the lateral ventricle tumor was carried out. The histology of these tumors was the same and revealed malignant choroid plexus papilloma. Postoperative radiation therapy was carried out 70Gy to the brain (whole brain; 50Gy, focal; 20Gy) and 30Gy to the whole spine. About 2 years later paraparesis, lower cranial nerve palsy, and disturbance of consciousness had progressed gradually. He died of the severe recurrence of the tumor in the brain stem, and multiple dissemination in the spinal cord on September 6, 1987. There was no recurrence of tumor in the right lateral ventricle. This is a very rare case of malignant choroid plexus papilloma which was revealed in both the supra- and infratentorial regions simultaneously. They may have developed independently or they may have arisen through subarachnoid seeding. Radical removal of the tumor is important to prevent recurrence of malignant choroid plexus papilloma.     

Choroid plexus papilloma of the cerebellopontine angle. Presentation of a case and review of the literature

Choroid plexus papillomas of the cerebellopontine angle are quite rare. Up to date, 19 cases surgically removed have been reported (table I). We describe the case of a 25 year old man with a choroid plexus papilloma (C.P.P.) who presented only two syncopes in the interval of 4 months. A computed tomographic scan revealed a calcified right extra-axial tumor occupying the cerebellopontine angle and a moderate degree of obstructive hydrocephalus. A vertebral angiogram showed that the tumor was avascular. A right suboccipital craniectomy was performed and the mass was excised in totality. The patient was discharged from hospital 2 weeks after surgery. 10 months later, neurological findings were normal. Histological examination exhibited a typical papilloma of the choroid plexus. The differential diagnosis of tumors in the C.P.P. angle is discussed. Total surgical excision in the treatment of choice. Radiation therapy could be used when the resection is incomplete.     

Choroid plexus tumors in children: significance of stromal invasion

OBJECTIVE: A group of choroid plexus tumors fit the cellular criteria for choroid plexus papilloma (CPP) except for invasion into the adjacent parenchyma, with associated loss of the normal villus architecture at the site of invasion. These tumors retain a benign cellular appearance. In the existing literature, it is unclear whether these tumors are classified as choroid plexus carcinomas or as CPPs. In our experience, although evidence of invasion is present, these tumors tend to exhibit benign behavior. We suggest that stromal invasion of this type remains consistent with a benign clinical course, although surgical results may demonstrate higher morbidity rates, given the invasive nature of the tumors. The failure to classify these tumors as CPPs may explain some of the variability in outcomes reported for various studies. METHODS: To study this question, clinical and histological data for children diagnosed with CPPs (either with or without stromal invasion) between 1985 and 1995 were examined. Only cases with pre- and postoperative magnetic resonance imaging data were included in the series. RESULTS: A total of 12 patients with CPPs were identified, with 4 tumors being notable for stromal invasion. After gross total tumor removal, none of the eight children with CPPs received adjuvant therapy at our institution; all are alive without evidence of tumor recurrence after surgical excision (mean, 108 mo). Of the four patients with stromal invasion, three underwent gross total resection. The one patient who underwent subtotal resection received chemotherapy at another facility. All four of these patients are alive, after a mean of 100 months of follow-up monitoring. CONCLUSION: It is recommended that CPPs with a benign cellular appearance but with evidence of local parenchymal invasion and loss of the normal villus architecture at the site of invasion be classified as CPPs. Patients with these tumors respond to surgical therapy alone, without the need for adjuvant treatment.     

Choroid plexus papilloma of the third ventricle in infancy: a case report]

The incidence of choroid plexus papilloma is about 0.5% of all intracranial tumors, but they are only infrequently found in the third ventricle. This is a case report of choroid plexus papilloma of the third ventricle in an infant. A make child in his 7th month of age was admitted because of large head circumference, on the 28th February, 1989. CT scan demonstrated hydrocephalus caused by a tumor in the third ventricle. Total removal of the tumor was performed on the 8th of March. Histologically the tumor was found to be choroid plexus papilloma. The patient suffered from postoperative subdural effusion and convulsions, so we performed ventriculoperitoneal shunt on the 14th April, and convulsions were controlled by zonisamid.     

Segmental myoclonus as the sole manifestation of a choroid plexus papilloma in the posterior fossa. Case report.

The authors describe the case of a 22-year-old woman with involuntary contractions of the sternocleidomastoid and trapezius muscles that resulted in turning movements of the head. The jerks displayed the clinical and neurophysiological characteristics of segmental myoclonus (SM) restricted to muscles supplied bilaterally by the first four cervical segments. Magnetic resonance imaging disclosed a tumor in the midline above the cisterna magna that was later histologically proven to be a choroid plexus papilloma. The patient's involuntary movements did not extend to other muscle groups or, in particular, to the palate, as one might have expected in the case of brainstem lesions. Myoclonus was the sole clinical manifestation of the tumor in this patient; other signs and symptoms invariably reported in other cases of posterior fossa papilloma, such as increased intracranial pressure or cranial nerve palsies, were absent. Release from suprasegmental control is suggested as a possible pathophysiological mechanism in this case of SM.     

Surgical treatment for choroid plexus tumors in the fourth ventricle: brain stem infiltration hinders total extirpation

The significance of surgery for choroid plexus tumors is well established, but surgical resection of those in the fourth ventricle has not been evaluated. This study reviewed five consecutive patients with choroid plexus tumors in the fourth ventricle treated in our institute between 1996 and 2005, focusing on the factors that hindered total extirpation. Two cases were choroid plexus papillomas, and three cases were choroid plexus carcinomas. Preoperative T2-weighted magnetic resonance imaging showed a diffuse high-intensity lesion in the brain stem in four patients. Infiltration into the fourth ventricle floor was apparent in all five patients during surgery, which hindered total resection of the tumors without neurological deterioration. Intraoperative bleeding was well controlled in all five patients by cauterizing the feeding arteries at the early stage of surgery through the telovelar approach. Performance status was improved in all patients postoperatively. All patients with choroid plexus carcinomas underwent radiation therapy after the surgical removal. No patient suffered tumor progression within the follow-up of 24–129 months (mean 64 months). Total resection of choroid plexus tumors in the fourth ventricle is difficult because of invasion into the fourth ventricle floor. Adjuvant therapy for choroid plexus tumors with brain stem infiltration must be established.     

Melanosis of choroid plexus papilloma of the lateral ventricle: a case report

A case of pigmented, well differentiated, choroid plexus papilloma is reported. A 45-year-old man came to our hospital complaining of attacks of vertigo and nausea. Skull X-ray films and CT scan demonstrated a calcified tumor in the left middle fossa. Tumor removal was done, and it was found that the tumor occupied the inferior horn of the left lateral ventricle. Histological examination revealed well differentiated choroid plexus papilloma containing various shaped pigment granules in the cytoplasm. The pigment granules in the tumor cells were stained black by Fontana-Masson method for melanin. The stain was negative in a section subjected to oxidative bleach. Granules were PAS-positive and autofluorescence. Therefore, the pigment granules had properties of both melanin and lipofuscin. In the electron microscopic study the ultrastructural appearance of the pigment was similar to that of neuromelanin, but not to that of pre-melanosome of neural crest origin. The pigment in this case seemed to be formed by "melanization" of lipofuscin as Barden's theory of pseudo-peroxidation. There are several case reports of pigmented choroid plexus carcinoma. However, no case of pigmented, well differentiated choroid plexus papilloma has, so far, been reported.