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.

Infección por Bartonella henselae con compromiso de sistema nervioso central, asociado a un papiloma del plexo coroídeo / Central nervous system infection by Bartonella henselae associated with a choroid plexus papilloma

Resumen La infección por Bartonella henselae es una enfermedad prevalente en nuestro país. En general, se presenta como la enfermedad por arañazo de gato o un síndrome febril prolongado. Existen manifestaciones atípicas dentro de las cuales está el compromiso óseo, cardíaco, hepatoesplénico y del sistema nervioso central. Se presenta el caso de una adolescente con historia de vómitos, fiebre y ataxia, en que se diagnosticó una infección por Bartonella henselae con compromiso del sistema nervioso central, asociada a un papiloma del plexo coroídeo. Este caso corresponde a una presentación inusual, de difícil diagnóstico. Su tratamiento es motivo de controversia, tanto en si es necesario tratar, la elección del antimicrobiano, como su duración.

Bartonella henselae infection is a prevalent illness in Chile. It presents generally as a cat scratch disease or as a prolonged fever syndrome. There are atypical manifestations, which include central nervous system, bone, cardiac and hepato-esplenic compromised. We present an adolescent case with a history of fever, vomiting and ataxia, whose diagnosis was a central nervous system infection by Bartonella henselae associated with a choroid plexus papilloma. This case corresponds to an unusual presentation, with a challenging diagnosis. It is controversial whether to treat this patient, which antimicrobial is the right choice and how long the treatment should be.

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.

Clinicopathologic features of endolymphatic sac tumor at cerebellopontine angle / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features and immunophenotype of endolymphatic sac tumor (ELST) and normal endolymphatic sac.</p><p><b>METHODS</b>The clinical and histologic features were evaluated in 5 cases of ELST. Eight cases of choroid plexus papilloma at cerebellopontine angle and 2 cases of normal endolymphatic sac were used as controls. Immunohistochemical study for vimentin, AE1/AE3, CK8/18, CK5/6, EMA, GFAP, synaptophysin, S-100 protein, CEA, TTF-1, VEGF, D2-40, calponin, calretinin and Ki-67 was carried out.</p><p><b>RESULTS</b>The age of onset of ELST ranged from 23 to 35 years (median = 24 years). The male-to-female ratio was 2:3. The clinical presentation was tinnitus, otalgia, hearing loss, otorrhagia with effusion and headache. The duration of symptoms ranged from 6 months to 10 years. Local recurrences were noted in 3 cases. Radiologically, the tumors were located at cerebellopontine angle and demonstrated petrous bone destruction. Histologic examination showed that the tumors had a papillary-glandular pattern. The papillae were covered by a single layer of low cuboidal cells. The tumor cells had distinct cell borders and contained eosinophilic to clear cytoplasm. The nuclei were slightly atypical and sometimes apically located. Focal dilated glandular structures with colloid-like material were also identified. The surrounding stroma was vascularized. All of the 5 cases had dural or petrous bone infiltration. Immunohistochemical study showed that all of the 5 cases were positive for AE1/AE3, CK8/18, CK5/6 and VEGF, 4 cases for EMA, 3 cases for calponin (focal), 2 cases for vimentin, 2 cases for S-100 protein, 1 case for GFAP and 1 case for synaptophysin (focal and weak). The Ki-67 index measured less than 1%. The staining for D2-40, calretinin, CEA and TTF-1 was negative. The 2 cases of the normal endolymphatic sac were positive for AE1/AE3 and CK8/18, and negative for CK5/6, EMA, S-100 protein, GFAP and synaptophysin. The 8 cases of choroid plexus papilloma were positive for synaptophysin. Seven cases were also positive for S-100 protein, 2 cases for GFAP and 1 case for D2-40. All of the 8 cases were negative for EMA, CK5/6 and calponin.</p><p><b>CONCLUSIONS</b>ELST is a rare slow-growing and potentially malignant tumor with a tendency of bone invasion and local recurrence. Distant metastasis is not observed. It must be distinguished from choroid plexus papilloma occurring at cerebellopontine angle. Correlation with clinical, radiologic and immunohistochemical findings would also be helpful.</p>

Diagnostic use of D2-40 and annexin-1 in ependymal tumors / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the diagnostic significance of D2-40 and annexin-1 in the ependymal tumors.</p><p><b>METHODS</b>To analyses the expression of D2-40, annexin-1, EMA and Ki-67 by immunohistochemistry in 52 cases of ependymal tumors (48 cases of ependymomas, 4 cases of choroid plexus papilloma) from Xuanwu Hospital from 2005 to 2009. Ten cases of corresponding normal brain tissue were also obtained as control.</p><p><b>RESULTS</b>Thirty-two of forty-eight (66.7%) cases of ependymomas were positive for D2-40. "Dot-like" and "ring-like" structures were commonly observed in ependymomas (55.3%, 21 of 38 cases) and anaplastic ependymomas (5 of 6 cases) with D2-40 staining. There was no difference in the expression between D2-40 and Ki-67 (r(s) = -0.013, P = 0.931). For annexin-1, 87.5% (42 of 48 cases) of the ependymomas were positive. The specific "granular structures" and cilium were observed in ependymomas (1 of 4 cases of myxopapillary ependymomas and 11 of 38 cases of ependymomas respectively) for annexin-1. The difference in expression between annexin-1 and Ki-67 was statistically significant (r(s) = -0.405, P = 0.005). D2-40 in combination of EMA and annexin-1 increased the positive rate to 100% in ependymomas. Choroid plexus papillomas were all positive for D2-40 and annexin-1. The control tissue was negative for D2-40 but positive for annexin-1 in the capillaries.</p><p><b>CONCLUSIONS</b>The specific structures are valuable in diagnosing of ependymal-genetic tumors, and are highlighted by D2-40 and annexin-1. D2-40 in combination of EMA and annexin-1 is a useful diagnostic marker for ependymal tumors.</p>

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.

Papiloma do plexo coroide do ângulo pontocerebelar: relato de caso / Primary choroid plexus papilloma of the cerebellopontine angle: case report

Contexto: Os papilomas dos plexos coroides (PPC) têm localização típica intraventricular pela sua relação com os plexos coroides. Apenas 9 por cento dos PPC estão localizados no ângulo pontocerebelar (APC), mas estes estão, na sua maioria, em relação com os plexos coroides do quarto ventrículo que afloram dos orifícios de Luschka. Já foram, no entanto, descritos alguns casos raros de PPC sem qualquer relação com os plexos coroides, tanto na fossa posterior como, até mesmo, no canal raquidiano. A recorrência local é rara e ainda mais rara é a disseminação pelo neuroeixo via espaço subaracnoide. Objetivo: Apresentar um caso de PPC com características atípicas: localização no APC, mas sem relação com o plexo coroide do quarto ventrículo, invasão óssea local, disseminação extradural e irrigação a partir de ramos arteriais meníngeos. Foi encontrado um caso semelhante na literatura, descrito em 1999. Alertamos para as características atípicas do PPC que poderão estar relacionadas com o diferente comportamento que este caso apresentou e que deve merecer uma reflexão sobre a melhor estratégia terapêutica...

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.

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.

Choroid plexus papilloma associated with developmental delay.

Choroid plexus papillomas are rare, benign tumors of neuroectodermal origin usually observed in the lateral ventricles of children. The usual presenting signs of choroid plexus papillomas are related to hydrocephalus and increased intracranial pressure. A child presented to us with clinical features of delayed milestones, which was later diagnosed as a case of choroid plexus papilloma with hydrocephalus. He underwent complete excision of the tumour with gradual recovery of milestones.

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.

Choroid plexus papilloma of the posterior third ventricle during infancy & childhood: report of two cases with management morbidities.

We report two cases of posterior third ventricular choroid plexus papilloma, one in an 8-month-old infant and another in a two-year-old child. These cases presented with features of obstructive hydrocephalus. Both these patients underwent a ventriculo-peritoneal (VP) shunt surgery prior to the tumor excision. Following the VP shunt surgery both patients developed ascitis requiring exteriorization of the abdominal end of the shunt. There was a clear proof of CSF overproduction: 1400-1500 ml/day in the eight-month-old infant and 900-1200 ml/day in the two-year-old child. In the former it was transient and could be treated with revision of the VP shunt whereas in the second case a ventriculo-arterial shunt had to be done. In the second case a staged reduction cranioplasty was also performed for an enormously enlarged head (head circumference--74 cm). Interesting clinical and radiological findings and useful management strategies are described.

Papiloma de plexos coroides / Choroid plexus papiloma

El papiloma de los plexos coroides es un tumor de baja incidencia, responsable de menos del 1 por ciento de las neoplasias cerebrales. Mas del 65 por ciento aparece en pacientes menores de 2 años. En Los archivos del hospital Pediátrico de la Misericordia de Bogotá se encontraron seis casos de este tumor. Se revisan sus características clínicas, histopatológicas y los aspectos principales de la literatura al respecto

Various Tumors in the 4th Ventricle in Adults: MRI Findings

Primary fourth ventricular neoplasms are common in children but rare in adults, and the disease categories encountered differ according to the patient's age. This study reviewed the records of patients aged 16 years or over, who underwent magnetic resonance (MR) imaging and were found to have fourth ventricular lesions. Most patients then underwent surgical resection, leading to specific pathologic diagnosis. The various fourth ventricular tumors encountered were ependymoma (n=8), subependymoma (n=1), choroid plexus papilloma (n=3), astrocytoma (n=3), medulloblastoma (n=1), lymphoma (n=2), epidermoid cyst (n=2), meningioma (n=1), melanoma (n=1), cavernous hemangioma (n=1) and metastasis (n=1). We describe the various tumors located mainly in the fourth ventricle and review their clinical presentation and the radiological findings, the majority of which were nonspecific. In some cases, however, specific signal intensity or the growth pattern of the tumors was useful for differential diagnosis.

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

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.

Choroid plexus papilloma and Aicardi syndrome: case report

A case of Aicardi syndrome associated with a choroid plexus papilloma of the third and both lateral ventricles in a 15 months old baby girl is repported. The tumor was completely removed via three craniotomies. Reports of the literature with the association of choroid plexus papilloma and Aicardi syndrome are rare. We suggest that children diagnosed with Aicardi syndrome should routinely undergo imaging studies of the brain, such as computed tomography or magnetic resonance

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.