Primary Choroid Plexus Papilloma Research Articles

MR Findings of Primary Choroid Plexus Papilloma of the Cerebellopontine Angle: Report of Three Cases and Literature Reviews

MR Findings of Primary Choroid Plexus Papilloma of the Cerebellopontine Angle: Report of Three Cases and Literature Reviews

A sacro‐caudal spinal cord choroid plexus papilloma in a shar‐pei dog

A seven-year-old shar-pei dog was referred because of severe lumbosacral pain and faecal incontinence of 20 days' duration. Neurological examination was characterised by plegic tail, absence of perineal reflex, dilated anus, perineum and tail analgesia, and severe lumbosacral pain. The neurological clinical signs were suggestive of a selective lesion involving sacral and caudal spinal cord segments and/or related nerve roots. A magnetic resonance imaging of lumbosacral spine was performed and was suggestive of an intradural lesion. Primary or secondary neoplasia was considered as the most probable differential diagnosis. The dog was euthanased upon the owner's request. Histopathological examination confirmed the presence of an intradural-extramedullary neoplastic tissue enveloping intradural tract of spinal nerve roots. On the basis of histological and immunohistochemical findings, a diagnosis of well-differentiated choroid plexus papilloma was made. To the authors's knowledge, this is the first case of primary or metastatic spinal choroid plexus papilloma in dogs.

Culture models to study leukocyte trafficking across the choroid plexus

BackgroundA critical point during the course of central nervous system infection is the influx of leukocytes from the blood into the brain across the blood-brain barrier (BBB) and the blood-cerebrospinal fluid barrier (BCSFB). However, experimental in vitro models to investigate leukocyte transmigration across cultured choroid plexus epithelial cells have been lacking so far.MethodsWe have developed a porcine and human “inverted” culture insert system that enables leukocyte transmigration specifically from the physiologically relevant basolateral side. The models use primary porcine choroid plexus epithelial cells (PCPEC) and human choroid plexus papilloma cells (HIBCPP). As a prerequisite for a functional barrier, we optimized culture conditions in which cells are maintained in serum-containing medium until high barrier function is reached. Leukocyte transmigration through the plexus epithelial cells is analysed by three-dimensional Apotome®-imaging and electron microscopy, and the route of transmigration through the plexus epithelial cells, i.e. transcellular as well as paracellular, can be determined.DiscussionAs a functionally relevant porcine and human BCSFB model, PCPEC and HIBCPP respectively, offer a wide range of options for analysis of disease-related mechanisms at the choroid plexus epithelium, especially involving human pathogens. Moreover, our in vitro models facilitate the investigation of leukocyte entry into the CNS via the blood-CSF barrier.

Primary choroid plexus papilloma in the pituitary fossa: case report and literature review

Choroid plexus papillomas are rare tumors of the central nervous system and are usually confined to the ventricular system. We illustrated a primary choroid plexus papilloma in the pituitary fossa. A 31-year-old female presented with amenorrhea and intermittent galactorrhoea, with no visual complaints in the last 2 years. Endocrine testing showed no hormone excess or deficiency of the pituitary and target glands, except for a higher prolactin level (56 ng/ml). A sharply circumscribed regular mass in the sellar region occupying the entire sella turcica and extending into the suprasellar cistern was demonstrated on MR imaging with gadolinium diethylenetriamine pentaacetic acid. The patient underwent an endonasal trans-sphenoidal approach. Complete microsurgical excision and complete preservation of the normal pituitary gland was achieved, with normal prolactin level. The histopathology showed that the lesion was a choroid plexus papilloma. Theories of the origin, the differential diagnosis, and treatment of the rare tumor are discussed.

Primary choroid plexus papilloma of the sacral nerve roots

The authors describe a unique case of a choroid plexus papilloma of the sacral nerve roots. This 60-year-old woman was admitted to the hospital because of a 1-year history of sacral pain, rectal and urinary bladder retention, and paradoxical episodic incontinence. Physical examination revealed sensory abnormalities in the S-2 dermatomes and poor rectal and bladder sphincter contractions. Contrast-enhanced spinal MR imaging showed a well-circumscribed, ovoid, homogeneously enhancing mass at the S1-2 level suggesting a diagnosis of ependymoma or schwannoma, and surgery allowed the identification and complete removal of a soft gray mass intimately adhering to the sacral nerve roots. Histological examination revealed a tumor consisting of papillary structures lined by a single layer of columnar cells, with an immunophenotype that satisfied the diagnostic criteria of choroid plexus papilloma. After diagnosis, contrast-enhanced brain MR imaging excluded the presence of a primary choroid plexus papilloma in the cerebral ventricles, thus ruling out a drop metastasis along the CSF pathways. A review of the literature did not reveal any similar cases of choroid plexus papilloma, and so the authors also discuss the inclusion of primary or metastatic papillary tumors in this unusual location as part of the differential diagnosis.

Rapid regrowth of an atypical choroid plexus papilloma located in the cerebellopontine angle

Choroid plexus papilloma usually occurs in the lateral or the fourth ventricle. Primary choroid plexus papilloma of the cerebellopontine angle, as described here, is an uncommon lesion. A 42-year-old man presented with a 1-month history of dysphagia and gait unsteadiness. CT scans and MRI showed a large extra-axial tumor in the right cerebellopontine angle. Pathological study revealed that the lesion was choroid plexus papilloma. Repeat imaging conducted 1 year after the operation showed that the tumor had recurred with distinct cystic features. Pathological examination again revealed increased mitotic activity and supported a diagnosis of atypical choroid plexus papilloma.

Primary choroid plexus papilloma of the sellar region

Choroid plexus papillomas are rare tumors of the central nervous system and are usually confined to the ventricle system. We describe a choroid plexus papilloma located in the sella turcica that was identified pathologically. A 49-year-old woman with a 5-year history of progressive visual deterioration in the left eye was admitted to our hospital. Neurological examination found no abnormality except for the perception of light and secondary optic atrophy in her left eye. Careful endocrine testing did not show any hormone excess or deficiency of the pituitary and target glands. Magnetic resonance imaging demonstrated a hemorrhagic mass in the sellar region, which occupied the entire sella turcica and extended to the suprasellar cistern. The mass was excised via an endonasal trans-sphenoidal approach. Histologic examination proved that the lesion was a choroid plexus papilloma. Choroid plexus papillomas arising in and occupying the sella are rare, although some radiographic patterns of ectopic choroid plexus papillomas have been reported. A review of the current literature revealed no similar cases of a choroid plexus papilloma. We discuss the different radiological appearances of choroid plexus papillomas, the differential diagnosis, and treatment.

Malignant progression in choroid plexus papillomas

Malignant progression of choroid plexus papillomas has been occasionally reported, but this issue has not yet been systematically addressed. Frequency and extent of malignant progression were examined in a retrospective series of 124 primary choroid plexus papillomas using uniform histological criteria. After gross-total resection and a mean follow-up period of 59 months, 12 recurrences necessitating neurosurgical intervention had occurred in the 103 cases of choroid plexus papilloma (World Health Organization [WHO] Grade I) and 21 cases of atypical choroid plexus papilloma (WHO Grade II). The proportion of recurring tumors was higher in cases of atypical choroid plexus papilloma than in cases of choroid plexus papilloma (six [29%] of 21 compared with six [6%] of 103, respectively; p < 0.05). In the majority (10 of 12) of the recurrences, there was a close correspondence between the primary tumor and the recurrence with respect to features identified on routine histological examination as well as Ki 67 (MIB-1) proliferation indices (median value 4% for both primary and recurrent tumors; range 0-15% for primary compared with 0-12% for recurrent tumors). However, two patients experienced a transition from a choroid plexus papilloma (WHO Grade I) and an atypical choroid plexus papilloma (WHO Grade II) to choroid plexus carcinomas (WHO Grade III). Recurrent tumor growth after gross-total resection is rare in choroid plexus papillomas, but malignant progression to choroid plexus carcinoma does occur in a small percentage of tumors.

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.

Concomitant choroid plexus papillomas involving the third and fourth ventricles: A case report and review of the literature

Choroid plexus papillomas (CPP) are histopathologically benign and rare central nervous system (CNS) neoplasms arising from the epithelium of the choroid plexus. The most common site of presentation of these lesions is in the fourth ventricle in adults and lateral ventricles in children. Third ventricular CPP are uncommon. In this study, we present a case of a 66-year-old woman with complaints of progressive confusion, lethargy, and weakness who was found to have concomitant third and fourth ventricular masses on imaging studies. The patient underwent a biopsy of the third ventricular mass. The biopsy was followed by staged resections of the fourth and third ventricular masses, respectively. Pathology from the biopsy and both resections was benign CPP. Multifocal concomitant CPP is rare. Concomitant CPPs may be secondary to mere coincidental tumor occurrence or to biologic seeding of cerebrospinal fluid (CSF) from a primary CPP despite otherwise benign histopathology. The primary treatment for CPP is surgical resection. Post-operative chemotherapy or radiation for CPP is of controversial benefit.

Primary Choroid Plexus Papilloma Located in the Suprasellar Region

Primary Choroid Plexus Papilloma Located in the Suprasellar Region

Primary choroid plexus papilloma of the cerebellopontine angle: MR imaging

The authors report the CT and MR findings of a rare benign primary choroid plexus papilloma developed in the CPA with invasion of the neighboring structures, apparently the first description of such a case. Differential diagnosis with other CPA tumors is discussed. Papillomas should be considered among the vascular, calcified enhancing masses of the CPA even in adulthood.

Primary choroid plexus papilloma of the cerebellopontine angle

A case of choroid plexus papilloma of the cerebellopontine angle, extending from the upper cervical region to the level of the tentorial notch, in a 50-year-old woman is presented. The differential diagnosis of tumors in the cerebellopontine angle and the role of surgical treatment vs. radiation therapy are discussed. (Neurosurgery 12:334-336, 1983)

Primary choroid plexus papilloma of the cerebellopontine angle. Case report.

Primary choroid plexus papilloma of the cerebellopontine angle. Case report.