Case Of Choroid Plexus Carcinoma Research Articles

Radiotherapeutic management of recurrent choroid plexus carcinoma with spine metastasis and delayed local brain recurrence in young adult male: A case report

Choroid plexus tumors or “Plexus-chorioideuscarcinomata” are rare malignancies with only a few reported cases. Choroid plexus carcinoma (CPC) is classified under the World Health Organization grade 3. CPC has fewer chances of occurrence in adults than in children. Recurrent CPCs have even more rare occurrences. Herein, we discuss a treated case of CPC in a 26-year-old adult male with spinal metastasis and delayed occurrence of local brain recurrence treated with radiotherapy for symptoms palliation. Clinical features, radiological findings, histopathological examination, and immunohistochemical findings are important in the diagnosis and management of such lesions. It is important to be aware of and know how to deal with such recurrent lesions.

Choroid plexus carcinoma with leptomeningeal spread in an adult: a\xa0case report and \xa0review of the\xa0literature

BackgroundChoroid plexus carcinoma is an intraventricular neoplasm originating from the choroid plexus epithelium and is of rare occurrence in adults. However, owing to the low prevalence of choroid plexus carcinoma, there is very limited information about the disease entity and treatment. Here we report a rare case of choroid plexus carcinoma in an adult patient.Case presentationA 46-year-old South Korean (East Asian) male presented with low back pain, headache, and diplopia. Magnetic resonance imaging demonstrated enhancing mass lesion in the left trigone, cerebellar with leptomeningeal spread. Surgery was performed via left parietal craniotomy, and the lesion was histologically confirmed to be choroid plexus carcinoma. The patient received adjuvant craniospinal irradiation for remnant mass and leptomeningeal spread. Magnetic resonance imaging performed immediately after completion of the treatment revealed a partial decrease in the size of the tumor. However, the patient expired died as a result of acute respiratory distress syndrome before follow-up of long-term outcome.ConclusionChoroid plexus carcinoma with leptomeningeal spread in adults is very important for rapid diagnosis and treatment. In the case of the presence of leptomeningeal spread, craniospinal irradiation can be considered as a treatment method, but may have serious complications. Hence, the technique should be applied with care.

Choroid Plexus Carcinoma of Third Ventricle: A Rare Case Report

Choroid plexus tumors are very rare tumors accounting for 0.5% of all brain tumors. They most commonly occur in childhood and present with features of raised intra-cranial pressure. World Health Organization (WHO) classifies them into 3 broad categories namely papilloma (grade 1), atypical papilloma (grade 2) and carcinoma (grade 3). These tumors are most commonly seen in the supratentorial compartment with lateral ventricle followed by third ventricle, being the most common site. Rarely, they may present in infratentorial region in adults. Dissemination through cerebrospinal fluid space is the inevitable natural course of the disease. Treatment requires total surgical resection with adjuvant chemoradiotherapy being controversial. In this study, we present a case of 7-year-old child who visited to the out-patient department of our center with progressive quadriparesis and altered sensorium and on imaging was found to have a well-defined, lobulated mass lesion with intense post contrast enhancement in posterior part of third ventricle with resultant obstructive hydrocephalus. The patient underwent craniotomy and on immuno-histopathological examination was diagnosed as a case of choroid plexus carcinoma. However, the patient succumbed to his illness a month after his surgery. The aim of this report is to highlight a rare entity, its diagnostic challenge and effect of early management in the form of surgery and chemo-radiotherapy.

An extremely rare case of choroid plexus carcinoma in the third ventricle of an infant – Case report and review of the literature

BackgroundChoroid plexus tumors are rare intraventricular tumors. Most of these tumors are benign choroid plexus papillomas. Choroid plexus carcinoma is the most malignant choroid plexus tumor and constitutes a small percentage of these tumors. The most common location of these tumors is the lateral ventricle and fourth ventricle in pediatric and adult patients respectively. Rare locations such as the third ventricle are reported in the literature with 55 reports of choroid plexus papilloma and just 3 cases of carcinoma located in the third ventricle. Case descriptionWe present an extremely rare case of choroid plexus carcinoma of the third ventricle in a 9 months old boy which was resected successfully through transcortical-transforaminal approach along with a review of literature available around different aspects of these tumors. ConclusionChoroid plexus carcinomas even in rare locations such as the third ventricle can be effectively resected through the transcortical-transforaminal approach, although post-operative subdural effusion can be a potential complication.

Recurrent choroid plexus carcinoma

Plexus-chorioideuscarcinomataor malignant plexus papillomas are very rare malignancy; only few cases have been described in the literature. A 2.5-year-old girl, known case of choroid plexus carcinoma, presented with complaints of weakness of right upper limb and lower limb, decreased activity, altered sensorium and vomiting. After re-evaluation, she underwent re-excision of tumor and chemotherapy started.

Choroid plexus carcinoma: A case report and literature review

Choroid plexus carcinoma is a rare tumor representing less than 1% of all brain tumors. In adult, the incidence is extremely rare making the diagnosis difficult. Majority of choroid plexus tumor is found in the ventricle. However, ectopic sites such as intracranial extraventricular or spine have been reported. We report a case of choroid plexus carcinoma in a 39-year-old man. The clinical presentation, pathology and management are discussed.

CASE REPORT: CHOROID PLEXUS CARCINOMA IN THE POSTERIOR CRANIAL FOSSA — PATHOLOGICAL ASSESSMENT AND TREATMENT IN A DOG

Choroid plexus tumors (PTs) are rare in dogs. Herein, we reported a canine case of choroid plexus carcinoma (CPC) and its clinical treatment. A 10-year-old male Shar Pei presented a two-month history of progressive uncoordinated change in head and gait posture. Magnetic resonance imaging (MRI) showed a lesion in posterior cranial fossa characterized by iso- to hypo-intensity in T1 and iso- to hyper-intensity in T2 with a homogeneous contrast uptake. The case received a one-month course of steroid therapy, and then performed by suboccipital craniectomy. The histopathological examination was described as CPC. The neurological symptoms were immediately and significantly improved after surgery. Follow-up MRI demonstrated that approximately 60% of the tumor was resected. To our knowledge, the clinical outcome of CPC was very poor. In this study, we provided an effective surgical approach, suboccipital craniectomy, for CPC in the posterior cranial fossa to avoid excess brain damage.

A typical radiological presentation in a case of choroid plexus carcinoma

A typical radiological presentation in a case of choroid plexus carcinoma

Choroid plexus carcinoma in an adult

Choroid plexus carcinoma is a very rare tumor in adults. Here we report a rare case of choroid plexus carcinoma in an adult patient. A 24-year-old male presented with a right temporal intraventricular tumor with a cystic component also extending up to the cortex. Histological examination revealed complex papillary structures and glandular spaces showing stratification and multilayering of cells with nuclear crowding and numerous mitotic figures and large areas of necrosis. The patient went through a complete search for a possible primary keeping in mind the differential diagnosis of metastatic carcinoma that is more common in adults but there was no evidence of any other tumor. Finally a diagnosis of choroid plexus carcinoma was rendered. Immunohistochemical analysis for p53 protein showed positivity. Choroid plexus carcinoma is exceptionally rare in adults but cases do occur.

A novel TP53 somatic mutation involved in the pathogenesis of pediatric choroid plexus carcinoma

SummaryBackgroundChoroid plexus carcinoma (CPC) is an uncommon, aggressive, malignant, central nervous system neoplasm that typically occurs in children, presenting with the signs and symptoms of intracranial hypertension and cerebrospinal fluid obstruction.Case ReportWe report the case of a 2.5-year-old girl with CPC. The tumor was subtotally removed by microsurgery, followed by gamma knife radiosurgery for the residual lesion. H&E staining indicated that this was a rare case of CPC. Neuropathological studies, assayed by immunohistochemical staining, showed that the tumor sample was positive to antibodies against S-100, CgA, AE1/AE3 (cytokeratin), Ki-67, INI1 and TP53, and was negative to antibodies against Nestin, GFAP, CD133, EMA and AFP. Moreover, stainings for transthyretin and vimentin were focally positive. Interestingly, direct DNA sequencing of the paraffin-embedded tumor sample identified a novel R248Q mutation in the TP53 gene. In contrast to previous reports suggesting that TP53 germline mutations were associated with the pathogenesis of CPC, here we provide a rare case of CPC with TP53 somatic mutation, as evidence that the peritumoral tissue possesses the non-mutant TP53 allele.ConclusionsOur finding suggests that TP53 somatic mutations, in addition to its germline mutations, may also be involved in the pathogenesis of pediatric CPC.

The cytologic findings in choroid plexus carcinoma: Report of a case with differential diagnosis

Choroid plexus carcinoma is a rare tumor of the choroid plexus that shows frank cytologic features of malignancy including frequent mitoses, increased cellularity, nuclear pleomorphism, loss of papillary architecture, and necrosis. It occurs predominantly in the pediatric population and is associated with a poor prognosis. We report the cerebrospinal fluid and intraoperative squash preparation cytologic findings of a case of choroid plexus carcinoma arising in the lateral ventricle of a 16-year-old girl who developed tumor recurrence in cerebrospinal fluid 6 years after initial resection. To the best of our knowledge, there are only a few reports in the English literature describing the cytologic features of choroid plexus carcinoma. Relevant differentials and the usefulness of ancillary studies in diagnosis are also discussed.

Choroid plexus tumors: An institutional series of 25 patients

Choroid plexus tumors (CPT) are rare neoplasms that pose considerable treatment challenges. This study reviews a single institute's experience with 25 patients of CPT and attempts to contribute to the general body of knowledge on CPT. A retrospective analysis of the case records of 25 patients operated for CPT since January 1998 and having a minimum of 1 year follow-up. The study group included 12 (48%) cases of choroid plexus papilloma (CPP), 09 (36%) cases of choroid plexus carcinoma (CPC) and 4 cases of atypical CPP. The mean age at presentation was 18.6 years (range, 6 months to 54 years; SD, 18.7) and a male preponderance was noted (17:8). Raised intracranial pressure was the commonest presenting symptom (72%). The tumors were distributed as follows: lateral ventricle (16; 64%), fourth ventricle (5; 20%), fourth ventricle with cerebellopontine angle extension (3; 12%), and third ventricle (1; 4%). A complete surgical excision was achieved in 11 cases of CPP and 8 cases of CPC. Operative complications include pneumocephalus (40%), focal deficits (36%), subdural effusion (32%), and persistent hydrocephalus requiring shunt (24%). All patients with CPP had a good outcome at the end of a mean follow-up of 5.4 years, whereas the median survival for patients with CPCs who underwent a subtotal resection with adjuvant therapy was 36 months. CPTs include a spectra ranging from CPP to CPC. Radiologic and histologic characterization of these tumors is difficult and newer immunohistochemical and genetic studies should be done to differentiate them from each other. Total excision offers a good prognosis and should be attempted for all forms of CPTs. CPPs carry a good prognosis, and adjuvant therapy is not indicated even after partial excision. CPCs and atypical CPCs carry a poor prognosis, and adjuvant therapy improves survival marginally after total excision. Spinal drop metastases are common for CPC and screening of the spine for possible metastasis should be part of the routine preoperative and postoperative investigation protocol.

A case of choroid plexus carcinoma with spinal metastasis in an adult

The author presents a rare case of choroid plexus carcinoma originated from right lateral ventricle and progressive multiple spinal seeding through cerebrospinal pathway after gross total resection of brain lesion in a 60-year old woman. The patient presented with one-year history of headache. nausea and vomiting. In spite of radical resection of the ventricular tumor followed by chemotherapy and radiation therapy over the cerebrospinal axis. the patient expired with respiratory failure one and half year after surgery.

Choroid plexus carcinoma: Report of two cases

Choroid plexus carcinomas (CPCs) are rare malignant counterparts of choroid plexus papilloma which occur in infants and children with a predilection for the posterior fossa and have a poor prognosis. We report two cases of CPC diagnosed in a 5-year-old boy and a 12-year-old boy and discuss the clinicopathologic features.

Choroid Plexus Carcinoma Secreting Carbohydrate Antigen 19-9 in an Adult-Case Report-

A 53-year-old man presented with a choroid plexus carcinoma in the body of the right lateral ventricle manifesting as gait disturbance due to left mild hemiparesis and papilledema. Subtotal removal of the tumor was performed. Histological examination revealed well-differentiated papillary adenocarcinoma. Immunohistochemical examination showed intense reactivity for carbohydrate antigen 19-9 (CA19-9). The serum level of CA19-9 was significantly high, but decreased rapidly after radiation therapy. Systemic examinations found no malignancy in other organs. This second case of choroid plexus carcinoma producing CA19-9 indicates that CA19-9 may be a useful marker of choroid plexus carcinoma.

Mutational analysis of hSNF5/INI1 and TP53 genes in choroid plexus carcinomas

We report here the mutational analysis of hSNF5/INI1 and TP53 genes performed on 11 specimens of choroid plexus carcinomas (CPC) in which a large number of abnormalities has been detected by molecular biology techniques. Loss of heterozygosity (LOH) analysis performed on six tumors revealed losses on chromosomes 1, 3, 5, 9, 10, 13, 16, 18, and 22. However, there were no abnormalities on 17p and mutations of the TP53 gene have been observed for two tumors comprising exons 5 and 7, respectively. Exon 4 of hSNF5/INI1 was mutated in one tumor with LOH restricted to the hSNF5/INI1 locus. There was no coexistence of mutations in both analyzed genes. Our analysis confirms the presence of the hSNF5/INI1 mutations and proves involvement of TP53 mutations in sporadic cases of CPC.

Is there a requirement for adjuvant therapy for choroid plexus carcinoma that has been completely resected?

Choroid plexus carcinomas (CPC) are rare central nervous system malignancies. While attempted surgical resection is imperative, the benefit of adjuvant therapy, particularly in the setting of a gross total resection (GTR), is unclear. We reviewed all pediatric cases of CPC reported in the literature between 1985 and 2000. Seventy-five cases of CPC were identified. Mean age at the time of diagnosis was 26 months. Thirty-seven patients had a GTR and 38 patients had a subtotal resection (STR). Thirty-eight cases (51%) were alive and 37 cases (49%) were deceased at time of publication. For cases with a GTR, survival was 84% compared to an 18% survival for patients with a STR. For patients with a GTR, all forms of adjuvant therapy were statistically equivalent. Our retrospective literature review confirms the importance of GTR in the therapy of CPC, with GTR alone being the single most important predictor of survival. The prognosis is poor for any patient with a STR, with the exception of those patients for whom adjuvant therapy allowed for an eventual GTR. The small number of patients receiving a GTR and no further therapy precluded a statistical comparison of no therapy in the setting of a GTR versus any form of adjuvant therapy. However, all four of these patients are alive, raising the possibility that adjuvant therapy in the setting of a GTR may not be required.

Usefulness of synaptophysin immunohistochemistry in an adult case of choroid plexus carcinoma

Choroid plexus carcinoma in an adult case is a very rare tumor and difficult to differentiate from metastatic tumors. The authors report a case of a 49-year-old female with choroid plexus carcinoma who previously had multiple carcinomas. In this case, synaptophysin immunohistochemistry proved to be extremely helpful for the histological diagnosis. [Neurol Res 2000; 22: 478-480]

Choroid plexus carcinoma: report of 15 cases

Choroid plexus carcinoma (CPC) are rare central nervous system tumours derived from choroid plexus epithelium, affecting mainly children under 3 years of age. We present a clinical, epidemiological and histopathological study of 15 cases of CPC. Ten patients were male. Age ranged from 4 months to 21 years (mean=3,4). The lateral ventricles were affected in 73,3% of cases. Main symptom were: hydrocephalus (62.5%), intracranial hypertension (25%) and convulsion (12.5%). The patients were treated by surgery with partial resection in 75% of cases and total resection in 25%. There was one death due to surgical complication, 85.7% of patients had recurrence of tumours with mean survival rate of 13.6% months after diagnosis. Only one patient remain alive 5 years after initial treatment. These results support the poor prognosis and high mortality rate of CPC.

Management of Choroid Plexus Tumours in Children: 20 Years Experience at a Single Neurosurgical Centre

Objective: Tumours of the choroid plexus are rare tumours of neuro-ectodermal origin, accounting for less than 1% of all intracranial tumours. Most cases present in children less than 2 years of age. While choroid plexus carcinomas (CPC) are reported to have an extremely poor prognosis, choroid plexus papillomas (CPP) are generally regarded as benign tumours with a very favourable long-term outcome. Management dilemmas are associated with the choice of surgical procedure, tumour vascularity, the treatment of hydrocephalus and the value of adjuvant therapy. The objective of this study was to review our experience with this rare tumour over a 20-year period. Methods: Patients were identified from the Great Ormond Street Neurosurgical Brain Tumour Database. Over a 20-year period (1979–1999), 34 children were identified with a choroid plexus tumour. There were 25 cases of CPP and 9 cases of CPC. A retrospective review of case notes, radiological imaging, operation reports and pathology was performed. Results: The median age at presentation was 17 months (1–138) for CPP and 13 months (2–102) for CPC. There was no sex difference for CPP. However, 8 of the 9 CPCs were male (89%). A complete surgical resection was achieved in all 25 cases of CPP and in 3 cases of CPC (33%). The median survival for CPPs was 75.5 months (2–228), with a median follow-up of 73.5 months (2–228). The median survival for CPCs was 6 months (1–90), with a median follow-up of 6 months (1–90). Conclusion: With modern neurosurgical practise, a cure should be the aim for all children with CPP. There is no evidence that adjuvant therapy has any role in the primary management of these children. However, CPC still has an extremely poor prognosis, and the efficacy of adjuvant therapy remains to be established.