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.     

Neonatal diagnosis of tuberous sclerosis

Intracranial tumors are rare in the neonatal period, and generally the most common histological types are astrocytoma, medulloblastoma, choroid plexus papilloma and neuroectodermal tumors. The early diagnosis of these tumors is often very difficult. The authors report a case of a full-term newborn who presented with opisthotonus. A subependymal mass was detected by cerebral ultrasonography, and when the child was 1 month of age depigmentations appeared on the trunk and on the right leg, confirming the suspicion of tuberous sclerosis. At 3 months of age the child suffered infantile spasm with hypsarrhythmia. The developmental delay, the marked progressive neurological deterioration and the daily seizures suggested surgical resection. Histologic studies showed a subependymal giant cell astrocytoma such as typically occurs in tuberous sclerosis.     

Atypical choroid plexus papilloma in adults: case report and literature review

IntroductionAtypical choroid plexus papilloma is a rare tumour, recently defined by the World Health Organisation and with very few cases reported to date. Choroid plexus tumours are rare intraventricular neoplasms originating from choroid plexus epithelium, with benign papillomas being more frequent than carcinomas. Most of these lesions appear in children, mainly located in the lateral ventricles. Those arising from the fourth ventricle are more frequent in adulthood. Surgery with complete resection can be curative in papillomas, with 5-year survival rates close to 100% and occasional recurrences. Radical surgery in carcinomas is difficult and usually requires adjuvant therapy, prognosis is worse and survival rates are lower. We report a case of atypical choroid plexus papilloma in adulthood located in the fourth ventricle, describing the different therapeutic options and reviewing the existing literature.Case report38-year old male with an atypical choroid plexus papilloma located in the fourth ventricle, totally resected by posterior fossa craniectomy and telovellar approach.ConclusionAtypical choroid plexus papillomas are associated with an increased risk of recurrence or malignant transformation when compared to “typical” papillomas. At present, complete surgical excision and close follow-up seems to be the most reliable treatment. Adjuvant chemotherapy or radiotherapy should be considered in cases of partial resection, recurrence or dissemination.     

Malignant choroid plexus papilloma. An autopsy case report (author's transl)

Malignant choroid plexus papilloma is a rare disease. The autopsy case of malignant choroid plexus papilloma being suggestive of the youngest in Japan, was reported. This 8-months-old baby had normal delivery history, and the development and growth were not eventful. The patient admitted to the University hospital because of projecting vomiting and meningeal irritating signs at 7.5 months in age, and septic meningitis was most suspected. He died 2 weeks afte the admission. At autopsy, a large papillary tumor with marked necrosis and hemorrhage was seen in the right lateral ventricle. The right lateral ventricle was almost replaced by the tumor. The metastasis to the brain base and the sheeding to the subarachinoidal space of the cerebellum were noted.     

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.     

不典型脉络丛乳头状瘤1例：临床及病理研究

目的探讨不典型脉络丛乳头状瘤的临床特点、病理诊断及鉴别诊断。方法分析1例4岁男性不典型脉络丛乳头状瘤病人的临床资料，光镜下观察病理形态特征并行免疫组化研究。结果CT显示右侧颞叶脑实质占位。MRI显示右侧侧脑室囊实性占位。病理形态多样，部分区域分化差，细胞密度增加、核分裂3-5个／10HPF，呈片状方式生长。免疫组化结果：角蛋白（CK）、波形蛋白（Vimentin）阳性，上皮膜抗原（EMA）弱阳性；S-100、胶质纤维酸性蛋白（GFAP）等均为阴性。Ki-67阳性率局部〉5％。结论病人年龄、肿瘤准确定位等临床资料对病理诊断较为重要；严格掌握肿瘤的诊断标准并辅以合理的免疫组化检查，对诊断与鉴别诊断极为重要。     

A large choroid plexus papilloma removed by the cerebellomedullary fissure approach. Case report and review of the literature

We report a case of large choroid plexus papilloma of the fourth ventricle in a 23-year-old woman. She presented with severe headache, dysphagia, and gait disturbances. Horizontal nystagmus, ataxic gait and quadriparesis were detected on initial examination. Imaging studies showed a large mass in the left side of brain stem and a marked hydrocephalus. The tumour was removed by microsurgical dissection of the cerebellomedullary fissure. We have discussed the effectiveness of this approach for removal of bulky tumors of the fourth ventricle and reviewed the literature about its benefits and potential hazards.     

显微手术治疗低龄儿童幕上脉络丛肿瘤

目的总结6岁以下低龄儿童幕上脉络丛肿瘤的临床特点和手术经验。方法回顾性分析2004年1月至2010年12月显微手术治疗的13例6岁以下儿童脉络丛肿瘤患者的临床资料。本组13例,平均年龄为19．4月;肿瘤位于侧脑室7例、第三脑室内6例。结果肿瘤全切除10例,次全切除2例,部分切除1例。术后病理证实12例为脉络丛乳头状瘤,l例为脉络丛乳头状癌。术后随访3～92个月,平均38．1月;1例脉络丛乳头状癌患者术后6月复发并转移而死亡;1例脉络丛乳头状瘤患者术后16个月死于颅内感染;余11例存活。结论儿童幕上脉络丛肿瘤大部分为良性肿瘤,显微手术全切除肿瘤可达到满意的疗效。     

Bilateral cerebrovascular accidents in incontinentia pigmenti

Choroid plexus papillomas are rare tumors that are confined to areas in which the choroid plexus is normally located. In children, choroid plexus papillomas are predominantly located in the lateral ventricles. Clinically they present with signs of raised intracranial pressure, such as vomiting and increasing head size. Here we report on the clinical, radiologic, and histologic findings of a 4-year-old female who was found to have a tumor in the posterior fossa that had all the histologic hallmarks of a choroid plexus papilloma. This tumor did not originate from the roof of the fourth ventricle as expected but from the ependymal lining covering the median rostral medulla near the pontomedullary junction, a location that so far has not been reported.     

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.     

Immunohistochemical evidence of endothelin-1 in human choroid plexus

Summary An immunohistochemical investigation was carried out on 17 specimens of human choroid plexus obtained post mortem, 1 biopsy of normal choroid plexus including part of the lateral ventricle and 1 papilloma of the choroid plexus removed surgically. The material was fixed in formalin. Paraffin and cryostat sections were used. A polyclonal antiserum to endothelin-1 served as a primary antibody. The avidin-biotin-peroxidase method was applied to demonstrate the immunoreaction. The epithelial cells of the choroid plexuses, the choroid papilloma and most ependymal cells of the lateral ventricle showed a distinct brown reaction product in their cytoplasm indicating antigenic sites to endothelin-1. The reaction was of lesser intensity in the ependymal cells. The connective tissue in choroid plexus was unstained. A positive immunoreaction was present in the walls of some vessels in the choroid plexus in cryostat sections. This is the first report on the presence of antigenic sites to endothelin-1 in the epithelial cells of the human choroid plexus. The role of endothelin in these cells should be investigated to ascertain if the cells synthesize this biologically active peptide or if it is merely bound to receptors in them.Supported by grants from Swedish Medical Research Council, project 03020, 1987 Års stiftelse för strokeforskning, Selanders stiftelse, Åhlen-stiftelsen and Stiftelsen Gamla Tjänarinnor, Stockholm Sweden     

Brain tumors during the first year of life

Among 341 childhood brain tumors treated at Northwestern University--Children's Memorial Hospital during the years 1967-1980, there were 39 children (11%) who presented during the first year of life. Half of the total number of childhood choroid plexus papillomas, meningeal sarcomas and teratomas we treated occurred in this particular age group. Supratentorial tumors were more common than infratentorial, a rate of 1.8:1. Medulloblastoma and benign astrocytoma were the most common histological types. Hydrocephalus was present in 82% of the children and papilledema in 28%, so that progressive enlargement of head circumference was the most common reason for referral. For the 37 patients who underwent surgical removal or biopsy of the tumor, the 1-month mortality rate was 19% and the 1-, 3- and 5-year survival rates were 46, 30 and 22%, respectively. Whenever tolerated, roentgen therapy was given. Most of the 24 deaths occurred within 6 months of the time of diagnosis. 5 patients (1 each with malignant astrocytoma, medulloblastoma, meningeal sarcoma, and 2 with choroid plexus papilloma) are still alive 5 years later, without neurological or mental deficit, and with no sign of recurrence. There were three exceptions to Collin's rule. Among the 15 survivors, 5 suffer mental retardation.     

Polyploidy in atypical grade II choroid plexus papilloma of the posterior fossa

Cytogenetic studies of choroid plexus tumors, particularly for atypical choroid plexus papillomas, have been rarely described. In the present report, the cytogenetic investigation of an atypical choroid plexus papilloma occurring at the posterior fossa of a 16‐year‐old male is described. Comparative genome hybridization analysis demonstrated gains of genetic material from almost all chromosomes. Chromosome losses involved 19p, regional losses at chromosome X and loss of chromosome Y. The presence of polyploid cells was confirmed by fluorescence in situ hybridization analysis with probes directed to centromeric regions. Furthermore, the microscopic analysis of cultures showed nuclear buds, nucleoplasmic bridges, and micronuclei in 23% of tumor cells suggesting the presence of complex chromosomal abnormalities. Previous cytogenetic studies on choroid plexus papillomas showed either normal, hypodiploid or hyperdiploid karyotypes. To the best of our knowledge, this is the first report of polyploidy in choroid plexus papilloma of intermediate malignancy grade. Although the mechanisms beneath such genome duplication remain to be elucidated, the observed abnormal nuclear shapes indicate constant restructuring of the tumor's genome and deserves further investigation.     

Immunocytochemical detection of insulin-like growth factor II (IGF-II) in choroid plexus papilloma: a possible marker for differential diagnosis

BACKGROUND: Insulin-like growth factor II (IGF-II) has been detected in the choroid plexus of animals by means of immunohistochemistry and in situ hybridization, and this factor is thought to play an important role in the central nervous system (CNS). Little is known, however, about the presence and localization of this substance in the choroid plexus and in choroid plexus papilloma in humans. MATERIALS AND METHODS: 5 normal choroid plexus and 10 choroid plexus papillomas were examined for IGF-II by immunocytochemistry. IGF-II was not detected in normal human choroid plexus, whereas it was found in choroid plexus papilloma. Furthermore, to assess the possibility that IGF-II could serve as an immunohistological marker of choroid plexus papilloma, we used the same technique to examine paraffin-embedded samples from various kinds of brain tumors. RESULTS AND CONCLUSION: Our results suggest that IGF-II may be a useful marker for choroid plexus papilloma in differential diagnosis.     

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.     

Choroid plexus papilloma containing bone

Summary The presence of bone in choroid plexus papillomas is a rare event. The authors report a case of choroid plexus papilloma of the IVth ventricle containing bone in a 18-year-old man, and review two previously reported cases, as well as the mechanisms proposed for the presence of bone and cartilage in neuroepithelial neoplasms.     

Choroid plexus papilloma in a 4-month-old child: a case report

We report a case of choroid plexus papilloma arising from the right lateral ventricle in a 4-month-old infant. Because of intermittent vomiting, abdominal pain, and diarrhea, the child was initially treated with intravenous rehydration, which aggravated the symptoms, and a bulging fontanel developed. Before a planned lumbar puncture, cranial ultrasound was performed, which disclosed a choroid plexus papilloma. The tumor was removed surgically, and the child made an uneventful recovery. We discuss the diagnostic value of cranial ultrasound compared to computed tomographic (CT) scan and angiography.     

Primary choroid plexus papilloma of the cerebellopontine angle presenting as brain stem tumor in child.

A case of a primary choroid plexus papilloma of the cerebellopontine angle in an 8-year-old female is presented. The clinical features of progressive cranial nerve palsies and cerebellar signs in the absence of intracranial hypertension initially suggested an intrinsic brain stem lesion. Whereas a radionuclide brain scan demonstrated abnormal uptake in the region of the pons, vertebral angiography and pneumoencephalography were diagnostic of an angle mass. At surgery, the tumor proved to be a choroid plexus papilloma that was totally confined to the cerebellopontine angle. This patient represents the thirteenth reported case of a primary cerebellopontine angle papilloma and the first such case occurring in a child.     

Papiloma del plexo coroideo del tercer ventrículo. Descripción de un caso

Choroid plexus papilloma is an uncommon tumour of the central nervous system, accounting for less than 1% of all intracranial neoplasm. The usual locations are the lateral ventricle in infants and children and the fourth ventricle in adults. The third ventricle is a rare location, with few cases reported in the literature.We describe the case of a 3-month-old boy who was admitted to our centre with signs of raised intracranial pressure. Neuroimaging studies showed a third ventricular mass with associated hydrocephalus. The patient underwent complete tumour removal through a transfrontal approach and ventriculo-peritoneal shunt surgery. Postoperative course of the child was uneventful and follow-up magnetic resonance imaging revealed no residual tumour. Histopathology of the resected lesion confirmed the diagnosis of choroid plexus papilloma.We discuss the clinical, radiological and histological features of this infrequent type of tumours.