Papiloma coroideo atípico en paciente de la tercera edad: Revisión bibliográfica a propósito de un caso / Atypical choroidal papilloma in an elderly patient: A literature review on a case

Introducción: Los tumores del plexo coroideo son raros. Contabilizan del 0,3 a 0,6% de la totalidad de los tumores cerebrales en adultos y del 10 a 20% en infantes; en quienes se ha registrado el 70% de estos y de los cuales al menos un 50% suceden en menores de dos años de edad. Objetivos: Esta publicación consiste en describir un caso de la tercera edad con papiloma atípico del cuarto ventrículo, la forma de resolución seleccionada y revisar la bibliografía del tema. Presentación del caso: Masculino de 71 años de edad que consulta por inestabilidad en la marcha y cefalea holocraneana intermitente. Al examen se muestra desorientado, con trastornos mnésicos, marcha magnética e incontinencia urinaria. Se realiza TC contrastada y posterior RM de cerebro con gadolinio objetivándose lesión espacio ocupante hipo-isointensa de 10 cc. Aprox. ocupando el 4to ventrículo, con realce intenso a la administración de contraste y ventriculomegalia asociada con edema transependimario. Intervención: Se realiza exéresis, logrando resección completa y mejoría clínica. Discusión: El papiloma atípico de plexo coroideo (Grado II) es una entidad intermedia que se distingue fundamentalmente del papiloma de grado I por su actividad mitótica; 2 o más mitosis en 10 campos. Conclusión: Este reporte, aborda una patología quirúrgicamente desafiante, potencialmente curable y clásicamente infantil, pero que también puede presentarse en la población geriátrica.

Introduction: Choroid plexus tumors are rare. They account for 0.3 to 0.6% of all brain tumors in adults and 10 to 20% in infants; in whom 70% of these have been registered and of which at least 50% occur in children under two years of age. Objectives: This publication consists of describing a case of the third age with atypical papilloma of the fourth ventricle, the selected form of resolution and reviewing the bibliography on the subject. Case presentation: 71-year-old male who consulted for gait instability and intermittent holocranial headache. On examination, he was disoriented, with memory disorders, magnetic gait, and urinary incontinence. Contrast-enhanced CT and subsequent MRI of the brain with gadolinium were performed, showing a 10 cc hypo-isointense occupying space lesion. Approx. occupying the 4th ventricle, with intense enhancement to contrast administration and ventriculomegaly associated with transependymal edema. Intervention: Exeresis is performed, achieving complete resection and clinical improvement. Discussion: Atypical choroid plexus papilloma (Grade II) is an intermediate entity that is fundamentally distinguished from grade I papilloma by its mitotic activity; 2 or more mitoses in 10 fields. Conclusion: This report addresses a surgically challenging pathology, potentially curable and classically infantile, but which can also occur in the geriatric population.

Carcinoma de plexo coroideo en paciente pediátrico: reporte de caso / Choroid plexus carcinoma in pediatric pacient: case report

El carcinoma de plexo coroideo se considera dentro de los tumores del cerebro como uno de los más infrecuentes, presentándose principalmente en la población pediátrica con mayor incidencia en hombres. Las principales manifestaciones clínicas son secundarias a la obstrucción del flujo de líquido cefalorraquídeo. Dentro de los estudios de imagen que se pueden emplear para realizar el diagnóstico están la tomografía computarizada y la resonancia magnética sin embargo el diagnóstico definitivo es el estudio anatomopatológico. Presentamos el caso clínico de un niño de 13 años de edad que acudió a la emergencia de pediatría del Hospital Mario Catarino Rivas por cefalea de dos meses de evolución que posteriormente presentó alteración de la marcha y hemiparesia derecha. El principal objetivo de tratamiento es la resección total sin embargo no se logra en todos los casos. Se ha propuesto el uso de quimioterapia adyuvante con o sin radiación, sin embargo, aún no se ha estandarizado este enfoque...

Choroid Plexus Carcinoma in Adults: Two Case Reports

Choroid plexus tumors are uncommon brain tumors that primarily occur in children. Most of these tumors originate from the intraventricular area, and the most common clinicalpresentation is increased intracranial pressure. Dissemination through the cerebrospinal fluid space is the inevitable natural course of the disease. Here, we present 2 rare cases of adult choroid plexus carcinoma (CPC), each with distinct clinical presentation and progression. The first case was a 40-year-old male who presented with multiple intraventricular masses. After surgical biopsy, radiation and intrathecal chemotherapy failed to elicit any response. The patient progressed with spinal cord dissemination and expired 1 year later. The second case presented with visual disturbance, and brain MRI revealed a large ovoid juxtaventricular mass with peritumoral edema. This 49-year-old female patient underwent craniotomy for what was thought to be a high-grade glioma; however, the mass was connected to the choroid plexus at the operative field. Her pathology specimen was diagnosed as CPC, and adjuvant systemic chemotherapy was administered. She has now been free of recurrence for 10 months. The description of the presentation and progression of these rare adult-onset CPC provides insight for the diagnosis and treatment of other rare instances of choroid plexus tumors.

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.

Carcinoma de plexo coroide: relato de caso / Choroid plexus carcinoma: case report

Aims: To describe a case of choroid plexus carcinoma which initial signs and symptoms were related to the upper respiratory tract, contrary to the classic symptomatology of this disease reported in the literature. Case description: A two years and eight months old boy was admitted due to acute respiratory failure. He was treated with antibiotics for suspected croup without success. After two weeks he presented neurological manifestations that led to investigation and diagnosis of choroid plexus carcinoma. Resection was contraindicated due to extension of the tumor and involvement of vital areas.Conclusions: The rapid evolution of choroid plexus carcinoma in this case points to the need for early suspicion of central nervous system disorders. This case may alert pediatricians to the need to consider the presence of a brain tumor causinginjury to the vagus nerve in cases of respiratory distress resistant to treatment.

Objetivos: Descrever um caso de carcinoma do plexo coroide no qual os sinais e sintomas iniciais foram relacionadas ao trato respiratório superior, ao contrário da sintomatologia clássica da doença reportada na literatura.Descrição do caso: Um menino de dois anos e oito meses de idade foi internado por insuficiência respiratória aguda.O paciente foi tratado com antibióticos para suspeita de crupe, sem sucesso, e após oito dias mostrou sintomatologia neurológica, que levou a uma investigação e ao diagnóstico de carcinoma do plexo coroide. A ressecção foi contra-indicadadevido à extensão do tumor e envolvimento de áreas vitais.Conclusões: A evolução rápida do carcinoma do plexo coroide neste caso mostra a necessidade da suspeita precoce de distúrbios do sistema nervoso central. Este caso pode alertar os pediatras para a necessidade de considerar a presença de um tumor cerebral levando a lesão do nervo vago em casos de dificuldade respiratória resistente ao tratamento.

Carcinoma de plexo coroide: relato de caso e revisão de literatura / Choroid plexus carcinoma: case report and literature review

Tumores do plexo coroide são tumores raros de origem neuroectodérmica, Correspondem a 0,4% a 0,6% de todos os tumores cerebrais, com incidência anual de 0,3 caso por milhão. Em sua maioria, são tumores benignos, raramente se apresentando na forma maligna. Os locais mais comumente relatados são os ventrículos. Relato de caso: Trata-se de carcinoma de plexo coroide em criança de 6 anos de idade que iniciou quadro com cefaleia, paresia do oculomotor, evoluindo com distúrbio de marcha. Foi submetida à biopsia, com confirmação histopatológica do carcinoma de plexo coroide. Esse paciente foi submetido à cirurgia, evolui bem com recuperação quase total no pós-cirúrgico imediato.

Tumors of the choroid plexus are rare tumors of neuro-ectodermal origin, correspond to 0.4% to 0.6% of all brain tumors, with an annual incidence of 0.3 cases per million.áThey are mostly benign tumors, rarely presenting as malignant.áThe sites most frequently reported are the ventricles.áCase report: This is choroid plexus carcinoma in ma child of 6 years of age she developed headache, oculomotor paresis progressing to gait disturbance.áThe patient underwent biopsy with histopathologic confirmation of choroid plexus carcinoma.áThis patient was submitted to surgery and progressed well, with full recovery in the immediate postoperative.

Melanotic Choroid Plexus Carcinoma of the Posterior Fossa

BACKGROUND: The pigmented or melanotic variant of choroid plexus carcinoma is very rarely encountered. CASE REPORT: We report herein a case of melanotic or pigmented choroid plexus carcinoma in the posterior fossa of an 11-year-old female. CONCLUSIONS: A histopathological study supported by immunohistochemistry helped the authors to diagnose this rare entity. How to differentiate this tumor from pigmented papillary medulloblastoma, cerebellar papillary ependymoma, and metastatic malignant melanoma is discussed.

Large choroid plexus teratoma: A rare cause of congenital hydrocephalus.

Teratomas form the most common type of congenital brain tumors, frequently presenting as stillbirth. The largest neonatal series of intracranial teratomas reported a 12% survival rate. Although the first teratoma of the lateral ventricle was reported in 1961 by Maier, neonatal intracranial teratoma of the lateral ventricle is an extremely rare entity. We report here a large intracranial poorly differentiated teratoma arising from choroid plexus of lateral ventricle. This typically presented at birth with a large congenital hydrocephalus.

Tumores primarios del sistema nervioso central en Cartagena, 2001- 2006 / Primary central nervous system tumours reported in Cartagena, 2001-2006

Objetivos Determinar la frecuencia de los tumores primarios del sistema nervioso central, diagnosticados en Cartagena de Indias durante el periodo de 2001-2006 y, determinar las características demográficas, epidemiológicas y clínicas de los pacientes con tumores del sistema nervioso central de una institución de la ciudad, en el mismo periodo. Métodos Se realizó un estudio descriptivo de vigilancia epidemiológica pasiva. Se tomaron los reportes de patología con diagnóstico nuevo de tumor primario del sistema nervioso central de todos laboratorios de Cartagena y se analizaron las historias clínicas disponibles de estos casos. Se estimaron las tasas de incidencia general, por año, género, grupos de edad y tipo histológico con intervalos de confianza al 95 por ciento. Además se calcularon razones estandarizadas de morbilidad. Resultados Durante los años 2001 y 2006 se encontraron 390 casos. La tasa incidencia general fue de 6,91/100 000 personas-año. El tipo histológico más frecuente fue meningioma (3,46/100 000 personas-año). Sólo se determinó la procedencia en el 43,1 por ciento de los casos. Las razones estandarizadas de morbilidad fueron más altas en Cartagena con respecto a las de los Estados Unidos, el Instituto Nacional de Cancerología de Colombia y el Registro Poblacional de Cáncer de Cali. Conclusiones Hubo una frecuencia de tumores primarios del sistema nervioso central más elevada en Cartagena que en el resto del país. Se recomienda mejorar los sistemas de registro y vigilancia para determinar la magnitud real del problema y fomentar investigaciones en busca de factores de riesgo.

Objectives Determining the frequency of primary central nervous system tumours diagnosed in Cartagena; Colombia, from 2001-2006 and determining the demographic, epidemiological and clinical characteristics of patients having central nervous system tumours reported by a single institution in Cartagena between 2001 and 2006. Methods A passive epidemiological surveillance descriptive study was carried out. The pathology reports of new diagnosed central nervous system primary tumours from all laboratories in Cartagena were taken and the available clinical records regarding these cases were analysed. The overall incidence rate and incidence rates by year, gender, age and histological type were estimated, with 95 percent confidence intervals. Standardised morbidity rates were also calculated. Results There were 390 such cases during 2001-2006. The overall incidence rate was 6.91/100,000 people-year. Meningiomas were the most frequently occurring histological types (3.46/100,000 people-year). The provenance could only be determined in 43.1 percent of cases. Standardised morbidity rates were higher in Cartagena regarding those reported in the United States and by the Colombian National Cancer Institute and the Population-based Cali Cancer Registry. Conclusions There was a higher incidence of primary central nervous system tumours in Cartagena than in the rest of the country. Registry and surveillance systems should be improved and research into risk factors encouraged.

Rhabdoid choroid plexus carcinoma: a rare histological type

Primary central nervous system atypical teratoid/rhabdoid tumors mostly occur during early childhood and are almost invariably fatal. These tumors show similar histological and radiological features to primitive neuroectodermal tumor, meduloblastoma and choroid plexus carcinoma, but present different biological behaviors. We present the case of an 18 year-old man who presented headache, vomiting and ataxia. CT-scan and MRI revealed a posterior fossa tumor. A gross total resection was performed. An intraoperative study showed papillary-like tumors with large cells and mitotic features. Histological examination showed two different main growth patterns: solid sheets of undifferentiated polygonal cells with papillary features and rhabdoid cells. Immunohistochemically, these rhabdoid cells were positive for vimentin, epithelial membrane antigen, smooth-muscle actin, cytokeratin, S-100 protein, and glial fibrillary acidic protein. Electro-microscopically, the typical rhabdoid cells contained whorled bundles of intermediate filaments in their cytoplasm. A rhabdoid tumor is a clinicalpathological entity and emphasizes the necessity to distinguish this unique tumor from other pediatric central nervous system neoplasms. Cytopathological features, immunohistochemistry and electro-microscopy differential diagnoses are discussed.

Los tumores de tipo rabdoide primarios en cualquier sitio son raros y en el sistema nervioso central son extremadamente raros y ocurren principalmente en niños, el tumor teratoide/rabdoide es el tumor más frecuente dentro de este grupo y de evolución clínica fatal. El tumor neuroectodermico primitivo, medulobalstoma y al carcinoma de plexos coroides son tumores generalmente muestran aspectos clínicos radiológicos e histológicos similares, con evolución diferente. Presentamos el caso de un hombre joven de 18 años que inició con cefalea vómitos y ataxia. La imagen de TC muestra tumor en fosa posterior. Se realizó resección total del tumor. En el estudio transoperatorio se observó neoplasia maligna de células grandes con mitosis y que formaba estructuras papilares. Histológicamente se observaron dos patrones diferentes, uno formado por nidos sólidos de células poligonales indiferenciadas con formación de estructuras papilares y el otro con abundantes células grandes de aspecto rabdoide. Por inmunohistoquímica las células de aspecto rabdoide fueron positivas para vimentina, antigeno de membrana epitelial, actina de músculo liso, citoqueratina, y proteína S-100, La PGAF fue focalmente positiva. Se realizó microscopía electrónica de las células rabdoides que mostraban nidos irregulares de filamentos intermedios intracitoplasmicos y lamina basal. El tumor rabdoide cerebral es clínica como histológicamente una rara entidad en la población pediátrica. Se discute el aspecto citopatológicas de inmunohistoquímica y de microscopia electrónica y sus diagnósticos diferenciales.

Extraneural metastases in anaplastic ependymoma.

Ependymoma are rare glial neoplasm, it rarely metastasize outside the central nervous system. We present a case of anaplastic ependymoma with extraneural metastases with review of literature. A ten-year-old male child presented with anaplastic ependymoma of choroid plexus and treated with craniospinal radiotherapy in 1998. He had intracranial recurrence in 2004, confirmed by biopsy. He was given adjuvant chemotherapy in form of PCV. At 10 months after completion of chemotherapy, he developed extracranial scalp metastasis and so was treated with palliative local radiation therapy to the scalp metastasis and systemic chemotherapy with oral Etoposide. Scalp metastasis completely disappeared and ataxia improved. After five cycles of chemotherapy, the patient had progression of disease in form of scalp and cervical lymph node metastasis confirmed by fine needle aspiration cytology, biopsy and immunohistochemistry. He was given salvage chemotherapy (carboplatin + ifosfamide + etoposide) at 3-weekly. He had partial response and was still on chemotherapy till May 2007.

Papillomas and carcinomas of the choroid plexus: histological and immunohistochemical studies and comparison with normal fetal choroid plexus

CONTEXTO: Os tumores do plexo coróide são raros. Os resultados de dados imuno-histoquímicos são escassos e controversos, o mesmo valendo para o plexo coróide normal. MÉTODO: Treze casos de tumores do plexo coróide e cinco exemplares de plexo coróide fetal normal foram submetidos a estudo imuno-histoquímico, utilizando-se marcadores para antígenos epiteliais, neurais e estromais. RESULTADOS/CONCLUSAO: Os achados histológicos mais relevantes foram células claras em 3/5 papilomas (PP) e 7/8 carcinomas (CA) e em todos os 5 plexos fetais; células rabdóides, desmoplasia e proliferação vascular foram encontradas, respectivamente, em 3, 4 e 5 casos de 6 CA pouco diferenciados, mas não nos PP e CA bem diferenciados. A pancitoqueratina AE1/AE3 foi fortemente positiva em todos os 13 casos, mesmo no componente indiferenciado do CA pouco diferenciado, em que a reatividade foi focal em 3 casos e difusa em outros 3. A citoqueratina de baixo peso molecular (35bH11) não foi expressa em nenhum dos 8 CA, mas estava presente em todos os 5 PP. Em 4/6 CA pouco diferenciados houve reatividade para actina de músculo liso (1A4) em 10-30% das células. Este achado ocorreu também em um caso sem células rabdóides. Laminina não foi detectada em nenhum dos 6 CA pouco diferenciados, mas estava presente em 4 PP e em 2 CA bem diferenciados. Todos os 5 plexos fetais expressaram GFAP.

Tumores de plexo coróide: estudo epidemiológico comparativo de 24 casos / Choroid plexus tumours: epidemiologic comparative study of 24 cases

Tumores derivados do plexo coróide são neoplasias raras do sistema nervoso central que acometem principalmente crianças. Este estudo apresenta uma série de 38 pacientes com tumores de origem neuroectodérmica, dentre os quais 24 possuem diagnóstico histológico e/ou imunoistoquímico de Carcinoma do Plexo Coróide (CPC). Destes, 16 eram do gênero masculino. O local predominantemente acometido foi o ventrículo lateral esquerdo. As manifestações clínicas mais freqüentes foram hidrocefalia, hipertensão intracraniana e crises convulsivas. Todos os pacientes foram submetidos a tratamento cirúrgico. Houve um óbito no per-operatório e outro no pós-operatório imediato. Nove pacientes tiveram recidiva, evoluindo a óbito em tempo médio de 12,3 meses. Sete estão vivos. Os demais casos não apresentavam dados disponíveis. Em Curitiba, o CPC tem uma alta prevalência em relação a outros tumores do plexo coróide, podendo estar relacionado a algum agente patogênico.

Lesões expansivas do plexo coróide: [revisão] / Choroid plexus mass lesions: [review]

As lesões expansivas do plexo coróide constituem um grupo bastante amplo e heterogêneo de doenças e seus simuladores. Tumores, infecções, anomalias congênitas, hemorragias, cistos e fenômenos degenerativos são alguns dos exemplos de causas de lesões expansivas do plexo coróide. No presente trabalho fizemos revisão da literatura pertinente, descrevendo os achados de imagem e ilustrando-os com alguns casos do nosso serviço. Apesar de não existir na literatura descrição de sinais patognomônicos, a avaliação criteriosa e sistemática das características das lesões pode sugerir determinada etiologia.

Choroid plexus mass lesions encompass a broad and heterogeneous group of diseases and their simulators. Tumors, infections, congenital anomalies, hemorrhage, cysts and degenerative diseases are some examples of mass lesions affecting the choroid plexus. In this article we review the current literature, describing the imaging findings and illustrating choroid plexus mass lesions with some cases diagnosed at our facility. Despite the inexistence of pathognomonic signs, a careful and systematic evaluation of the imaging characteristics may suggest many etiologies.

Choroid plexus carcinoma in an infant

Choroid plexus carcinoma is a rare intracranial neoplasm, affecting mainly very young children. The commonest site is within the lateral ventricles and the prognosis is very poor. We report a seven month old baby boy who presented with raised intracranial pressure and seizures. Brain CT scan showed large intraventricular mass with calcification and hydrocephalus. Total macroscopic resection of the tumour was performed and diagnosis of choroid plexus carcinoma was made. However, the patient died 11 days after the tumour excision. The histopathology of this rare childhood neoplasm is discussed.

Choroid plexus papillomas of the cerebellopontine angle.

The cerebellopontine angle (CPA) is a rare site for the growth of choroid plexus papilloma (CPP). The clinicoradiological diagnosis of this tumor in the CPA is difficult because of the nonspecific clinical presentation and radiological features. Five cases of choriod plexus papilloma (3 males, 2 females) operated upon at this center are reviewed. All the cases were operated upon by retromastoid suboccipital craniectomy. As they all presented with a typical CPA syndrome without any distinctive radiological feature, a clinicoradiological diagnosis of CPP could not be reached in any of these. The diagnosis of CPP could only be suspected at the operation table and established on histopathological examination. Two patients developed recurrences at the primary site following surgery. One patient developed recurrence twice despite gross total excision of tumor in each sitting. Subsequently, this patient remained symptom free for a follow-up period of 1 year. Another patient developed recurrence 2 years following surgery, but he died due to septicemia and aspiration pneumonitis. Therefore definitive surgery could not be performed. Radiotherapy was offered to one of the patients having residual mass post operatively, to render her symptom free for a 4 year follow-up. The remaining two patients have also showed progressive improvement in their symptoms following surgery for 4 years on follow-up. Hydrocephalus was a common feature in all the cases preoperatively, but only one required shunt CSF diversion, because of rapid deterioration in visual equity. In all other cases, hydrocephalus was managed conservatively. Surgery remains the main modality of treatment for CPP, both for primary and recurrent tumors, but radiotherapy may have a role in cases of recurrence, which are quite frequent.