Complete removal of intraventricular cpps almost always cures the condition and it has been estimated that the survival rate at 5 years for cpps after removal is approximately 100%. Choroid plexus papilloma an overview sciencedirect topics. Case series of choroid plexus papilloma in children at. Choroid plexus papilloma developing in a dermoid cyst of ovary. As cpts grow, they block the flow of cerebrospinal fluid. The female patient, aged 32 years, presented with a 6 month history of left loin pain postpartum. If you continue browsing the site, you agree to the use of cookies on this website.
The interventricular extension of choroid plexus papillomas. Other investigations to be considered to exclude metastasis from choroid plexus carcinoma are mri of spine to rule out leptomeningeal spread and also a cerebrospinal fluid cytology. Twothirds of the tumors are found in children and all of these are located in the lateral ventricles. Their biological behaviour cannot be predicted in an individual case. We present to you the case of a 1yearold child who presented with the signs and symptoms of hydrocephalous and was diagnosed as choroid plexus papilloma on histopathology. Choroid plexus tumors are rare intraventricular tumors 1% of all intracranial tumors that occur mainly in children. Prognostic implications of atypical histologic features in. Grade i of iv benign rare pdf choroid plexus tumors are rare intracranial tumors which account for only 0. Palade university of medicine and pharmacy carol davila bucharest 1st neurosurgery clinic introduction choroid plexus tumors cpt are defined as papillary neoplasms originating. Choroid plexus papilloma choroid plexus carcinoma atypical choroid plexus papilloma. Files are available under licenses specified on their description page. Choroid plexus papillomas oxford academic journals. On histology, the w ho classification recognizes choroid plexus papilloma cpp. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.
Neuropathology service, institute of pathology, university of berne, switzerland. Microscopically, the tumor cells resemble normal ependymal cells and are arranged in perivascular pseudorosettes tumor cell nuclei located at some distance from a central vessel with delicate processes radiating toward the vessel wall, tubular structures like the central canal of the spinal cord, and papillary formations. However, the involved choroid is not enlarged and there is no associated hydrocephalus or interventricular extension. The prognosis for choroid plexus papilloma cpp is determined mainly by how completely the lesion is removed during surgery. Histopathological examination diagnosed choroid plexus papilloma. The authors report the successful total removal of a rare intracranial tumor in the right lateral ventricle of a 15yearold girl with epileptic seizures. Choroid plexus papilloma of the fourth ventricle case. It consists of epithelial cells, fenestrated blood vessels, and the stroma, dependent on various physiological or pathological conditions, which may contain fibroblasts, mast cells, macrophages, granulocytes or other infiltrates, and a rich extracellular matrix.
Choroid plexus papilloma is a rare, slowgrowing, histologically benign intracranial neoplasm that is commonly located in the ventricular system. The prognostic significance of atypical histologic features in choroid plexus tumors remains uncertain. Final histopathology revealed sinonasal papilloma with both inverted and exophytic growth patterns associated with moderate to severe dysplasia involving approximately 50% of the lesion, but no invasive carcinoma. High survival unless becomes malignant then 5 year survival is 26% although histology does not predict behavior case reports 49 year old woman with oncocytic variant of choroid plexus papilloma arch pathol lab med 2004. Results on immunohistochemical features are scanty and controversial even regarding normal plexus. Tumors of the choroid plexus epithelium are rare and account for only 0. Prognostic implications of atypical histologic features in choroid. Choroid plexus tumors are intraventricular papillary neoplasms derived from. Histopathologic examination showed fronds of fibrovascular tissue covered by. Choroid plexus papilloma sethi d, arora r, garg k, tanwar. Pathology by systems nervous system central nervous system brain atypical choroid plexus papilloma atypical choroid plexus papilloma monday 24 april 2017. Read choroid plexus papilloma diagnosed by crush cytology, diagnostic cytopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Although the lateral ventricles are the most common location, these tumors can present in the third or fourth ventricles, especially in adults. Within this family of tumors, there are benign and malignant variants, typically classified as choroid plexus papilloma cpp and choroid plexus carcinoma cpc, respectively.
The histopathology was suggestive of choroid plexus papilloma n 19, atypical choroid. Cpp is most common in young children, but it can also appear in adults. Choroid plexus papilloma diagnosed by crush cytology. Choroid plexus papillomas are an uncommon, benign who grade i neuroepithelial intraventricular tumor which can occur in both the pediatric more common and adult population on imaging, these tumors are usually identified in the fourth ventricle in adults and in the lateral ventricles in the pediatric population. Therefore, a series of 164 choroid plexus tumors was evaluated for the presence of atypical histologic features, including mitotic activity, increased cellularity, nuclear pleomorphism, blurring of papillary growth pattern, and necrosis.
Benign papillomas are reported to account for approximately 80% of neoplasms, and carcinomas, 20%. Choroid plexus papillomas cpps are uncommon tumors of the central nervous system arising from the choroid plexus. Histopathology results showed papillary structures with a. Choroid plexus papillomas cpps are rare, usually slow growing neoplasms. Choroid plexus papilloma cpp is a benign neoplasm that arises from the ventricular choroid plexus. The epithelium of the choroid plexus originates from specialisation of primitive ventricular neuroepithelial cells at certain sites of the neural tube when these cells become apposed to intraventricular infoldings of the adjacent primitive mesenchyme from which the tela choroidea is derived 26, 37. They are classified as a who grade i tumor if typical and who grade ii if atypical. Choroids plexus papillomas are benign, but occasionally an invasive variant can be found. Choroid plexus tumors are uncommon neoplasms derived from choroid plexus epithelium and characterized by papillary and intraventricular growth. The predominant clinical presentation is raised intracranial pressure. Choroid plexus papilloma, also known as papilloma of the choroid plexus, is a rare benign neuroepithelial intraventricular who grade i lesion found in the choroid plexus. Choroid plexus papilloma cpp ia a noncancerous benign tumor of the choroid plexus, a network of blood vessels in the brain which surrounds the ventricles and produces the fluid surrounding the brain and spinal cord cerebrospinal fluid, or csf.
Grossly, the tumors are soft, globular, friable pink masses with irregular projections and high vascularity. Papillomas and carcinomas of the choroid plexus in. Several lines of evidence suggest that simian virus 40 sv40 can cause these tumors. Discussion choroid plexus papilloma is a rare epithelial neoplasm, accounting for less than 1% of intracranial tumors. We experienced a case of choroid plexus papilloma associated with fluidelectrolyte imbalance due to overdrainage after evd, which. Choroid plexus papilloma cpp is an uncommon intracranial tumor of neuroepithelial origin. Cpps most commonly occur in children but may occur in adults. Choroid plexus neoplasms are rare intraventricular tumors, which account for approximately 2% of pediatric brain tumors 1,2 but are comparatively more common in neonates 5%20% of perinatal brain tumors 3.
Department of anatomic pathology, faculty of medical sciences, state. The choroid plexus is an epithelialendothelial vascular convolute within the ventricular system of the vertebrate brain. All the signs and symptoms in the child had resolved postoperatively and he was discharged three weeks later. Therefore, histopathology with identification of the papillary pattern associated to immunophenotyping was a decisive diagnostic strategy for the conduction of the case. Choroid plexus papilloma genetic and rare diseases. Pdf choroid plexus tumors are rare intracranial tumors which account for only 0.
Choroid plexus papilloma radiology reference article. Choroid plexus papilloma is an uncommon, benign neuroepithelial intraventricular tumor which can occur in both the pediatric more common and adult population. Choroid plexus carcinoma is a rare tumor representing less. Choroid plexus neoplasms are rare, intraventricular, primary central nervous system cns tumors derived from choroid plexus epithelium that are seen predominantly in children. Choroid plexus carcinomas and papillary ependymomas invade the local ependyma and glial tissue. Thirteen cases of choroid plexus tumors and five samples of normal fetal choroid plexus were submitted to immunohistochemical study using a panel of epithelial, neuronal and stromal. Choroid plexus tumors are graded based on the world health organization who classification scheme and include choroid plexus papilloma cpp who grade i see the f. Choroid plexus papilloma statpearls ncbi bookshelf. The histopathology was suggestive of choroid plexus papilloma n 19, atypical choroid plexus papilloma n 1, and choroid plexus carcinoma n 2.
Choroid plexus papilloma ade wijaya, md february 2018 slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. It leads to increased cerebrospinal fluid production, thus causing increased intracranial pressure and hydrocephalus choroid plexus papilloma occurs in the lateral ventricles of children and in the fourth ventricle of adults. Experimental evidence suggests that differentiation of this epithelium. In this context, papilla refers to the projection created by the tumor, not a tumor. Choroid plexus tumors are intraventricular tumors arising from choroid plexus epithelium. This page was last edited on 23 november 2019, at 14. Choroid plexus papilloma saint lukes health system. The clinical features, radiological characteristics, and treatment have been discussed in this study for a pediatric population. In adults most of the tumors are present in the fourth ventricle. Ennsting2 reported acase of subarachnoid bleeding in which carotid angiograph\r demonstrated avascular tumon supplied by the. Choroid plexus papilloma developing in a dermoid cyst of. The pathophysiological characteristics of associated hydrocephalus, surgical management, and oncological issues related to. Histology was the most important prognostic factor, as one, five, and 10year projected survival rates were 90, 81, and 77% in choroid plexuspapilloma.
The histopathology of choroid plexus papilloma and choroid plexus. Choroid plexus with degenerative changes may be difficult to differentiate histologically from a choroid plexus papilloma 17. Choroid plexus papillomas cpps are more common than choroid plexus carcinomas cpcs in a ratio of a least 5. Choroid plexus papilloma arising in a mature cystic.
This can increase pressure on the brain and cause the skull to get bigger. Choroid plexus tumors cpts begin in the brain within areas called ventricles that are filled with cerebrospinal fluid. The common sites of these tumors are the lateral ventricles in children and the fourth ventricle in adults. In adults, cpp most often happens near the back of the brain. All structured data from the file and property namespaces is available under the creative commons cc0 license. Herein, to the best of our knowledge, we report the third published case of a choroid plexus papilloma arising in a mature cystic teratoma. Thirteen cases of choroid plexus tumors and five samples of normal fetal. It is an important albeit rare cause of hydrocephalous.
Pdf choroid plexus papilloma cpp is a benign neoplasm that arises from the ventricular choroid. Choroid plexus papillomas are extremely rare vascular tumors that are often located in the ventricular areas of infants and children of both sexes. Histopathological examination showed a benign tumor formed by mature cartilage and choroid plexus papilloma. Cpcs, resulting in significant overlapping histology with atypical teratoidrhabdoid tumors atrts fig.
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