Successful treatment of an unresectable choroid plexus carcinoma in a patient with Li-Fraumeni syndrome

Choroid plexus carcinoma (CPC) is an uncommon central nervous system tumor requiring complete surgical excision for favorable outcome. The authors report the successful treatment of a 2-year-old patient with widely disseminated CPC and Li-Fraumeni syndrome. Following a partial resection of the tumor the patient received chemotherapy consisting of cyclophosphamide, etoposide, and carboplatin. There were no additional surgical procedures and radiation was not administered. Remarkably, the patient remains without evidence of active disease more than 3 years from the completion of therapy. Additional studies are necessary to determine whether this treatment plan can be beneficial to other patients with CPC and whether the patient's p53 mutation had an effect on outcome.     

Bilateral choroid plexus carcinoma of the lateral ventricles

This is a case report of a bilateral choroid plexus carcinoma (CPCa) of the lateral ventricles in whom contralateral ventricular dissemination was thought to be due to seeding. The disease was diagnosed by ultrasonography; CT and MRI confirmed the pre-operative diagnosis.     

Current management of choroid plexus carcinoma in children.

Eleven children with choroid plexus carcinoma are reviewed with respect to presentation, radiographic diagnosis, treatment and pathologic histology. These cases accumulated over a 33-year period and represent 39% of all of our choroid plexus tumors (n = 28). Characteristically, choroid plexus carcinomas are associated with a poor prognosis for long-term survival. Unfortunately, efforts at total resection are hindered in part by the extreme vascularity of the tumor which itself may be of considerable volume. We have recently treated four children who had biopsy or minimal resection of their tumor after which adjuvant chemotherapy was given. Chemotherapy is not curative but it does cause a reduction in tumor volume and, more importantly, has tended to reduce the tumor vascularity. This allows for a second stage operation which is more safely performed and typically allows for more complete tumor removal. At this point, our length of follow-up is insufficient to conclude if total removal in this manner significantly increases survival but it would seem that consistent gross total removal may be an initial step toward better overall management and prognosis in this tumor type.     

Childhood choroid plexus neoplasms

Fourteen children less than 2 years old with choroid plexus papilloma (CPP) were studied. Of these patients nine had the tumor in the lateral ventricles (LV), three in the fourth ventricle (4th V), one in the third ventricle (3rd V) and one in the third and left lateral ventricle (LLV). Ten cases showed benign CPP, three cases malignant CPP, and one case had a venous malformation of the choroid plexus (VMCP). Computed tomography (CT) was the best method for neuroradiological study, although a very small CPP in the 3rd V was not detected in an early study. Malignant and benign CPP can be differentiated with CT, especially if the study is performed with contrast media. Pneumoencephalography must be rejected because it is very dangerous and may be the cause of death in some cases. The follow-up of patients with CPP showed a poor prognosis, even after correctly removing the tumor and inserting a shunt.     

Secondary glioblastoma multiforme after treatment for primary choroid plexus carcinoma in childhood

A 15-year-old boy was diagnosed with choroid plexus carcinoma (CPC) of the right lateral ventricle. His metastatic work-up was negative. After complete macroscopic resection of the tumor, the patient was treated with chemotherapy consisting of vincristine, cisplatin, etoposide, and carboplatin, followed by radiotherapy for a total dose of 34.2 Gy on the whole craniospinal axis plus a boost of 19.8 Gy at the tumor region. The patient remained in complete clinical and radiologic remission over the next 5 years when a secondary malignant tumor, glioblastoma multiforme, a rare complication of the treatment of CPC, was diagnosed. This case reflects the necessity of thorough follow-up in long-term survivors of CPC.     

Malignant evolution of choroid plexus papilloma

Choroid plexus tumors are rare CNS neoplasms. The distinction between choroid plexus papilloma (CPP) and choroid plexus carcinoma (CPC) is made on the basis of clinical and histological criteria. Malignant evolution of CPP may occur, and the presence of mitotic figures in CPP may predict the likelihood of recurrence or malignant evolution. Close surveillance is mandated for these patients. We report on two such cases of CPP that transformed to CPC at the time of recurrence.     

Endoscopic coagulation of choroid plexus hyperplasia

Hydrocephalus is a clinical disorder resulting from an imbalance between the production of CSF and its resorption, of which the latter is mostly a disadvantage. In rare cases of choroid plexus papilloma or carcinoma, hydrocephalus is due to an overproduction of CSF. Choroid plexus hyperplasia (CPH) is a distinct clinicopathological entity in which the enlarged choroid plexus produces large amounts of CSF. Historically, patients with CPH were treated by shunt procedures or by microsurgical removal of the choroid plexus, which is associated with a high complication rate. In this paper the authors show that endoscopic plexus coagulation can result in restoring the equilibrium of the intracranial fluid volumes, resulting in shunt independency. In this way, both the shunt-related complications and the bleeding risks of microsurgical plexectomy are avoided. In instances of hydrocephalus, thorough efforts should be made to demonstrate the underlying pathophysiology to choose the optimal treatment, of which shunt procedures should receive the least priority.     

Pediatric choroid plexus carcinoma: Biologically and clinically in need of new perspectives

Choroid plexus (CP) carcinoma is a rare pediatric brain neoplasm. Recent studies have highlighted the potential of genome‐wide methylation and gene expression profiling to provide additional layers of information to improve tumor risk‐stratification. There is a lack of data regarding the best therapy, and approaches have been heterogeneous. Despite multidisciplinary treatment approaches, the outcome remains guarded and treatments have been based on case series and expert opinions. In this study, we discuss the recent wealth of data regarding CP carcinoma molecular biology and current management. We also briefly highlight the remaining barriers to formulate the best treatment strategies, and future therapeutic potentials.     

Symptomatic third ventricular choroid plexus cysts

We describe the imaging findings in 3 children with choroid plexus cysts (CPC) at the foramen of Monro. All CPC measured less than 2 cm and produced symptoms of raised intracranial pressure when located at the foramen of Monro where there was obstruction to the cerebrospinal fluid (CSF) flow. Among the imaging studies done on our patients, CPC were depicted best by cranial sonography and CT-ventriculography and missed by standard CT and MRI. Misdiagnosis can lead to inappropriate shunting with adverse effects. Two of our patients had relief of symptoms after resection of the cyst. One patient with inoperable cardiac defects died and had no surgery performed. Serial CT and sonographic studies in this patient showed progression in the size of the cyst and ventriculomegaly. Cranial sonography and CT-ventriculography are the modalities of choice in evaluating ventriculomegaly when the diagnosis of occult obstructive CPC is entertained in children. Cranial sonography is indicated in infants with an open fontanelle and CT-ventriculography is reserved for older children with hydrocephalus which is not responding to shunting.     

Infantile myofibromatosis involving the choroid plexus

We present a case of infantile myofibromatosis manifest as a choroid plexus mass followed by spontaneous regression. Infantile myofibromatosis is a common juvenile fibrous disorder occurring in infancy and early childhood. Intracranial involvement in infantile myofibromatosis is rare. It generally occurs in the dura with calvarial invasion and secondary brain compression.     

Magnetic resonance imaging in the diagnosis and management of choroid plexus carcinoma in children

Background: Choroid plexus carcinoma (CPC) is a rare tumour occurring in childhood. The MRI features are not well described. Objective: To characterise the MRI features of CPC in children and to describe the role of MRI in post-surgical management. Materials and methods: The pre- and post-operative MRI examinations of five children with histologically proven CPC treated at this hospital were reviewed. Results: The tumour was supratentorial in two patients and infratentorial in three patients. Pre-operative MRI showed heterogeneous enhancing tumours with irregular margins, parenchymal invasion and surrounding oedema. Cystic change within the tumour was present in all patients. Curvilinear signal voids were seen in two patients and punctate signal voids in one. All patients had hydrocephalus. In one patient a fourth-ventricular metastasis was present at diagnosis. In all cases, surgical resection was incomplete. Follow-up found long-term survival in one patient who had been treated with chemotherapy and further surgery. Conclusion: The MRI characteristics of CPC are nonspecific, but intraventricular location with tumour heterogeneity, parenchymal invasion and oedema may be useful features in diagnosis. MRI is valuable in the initial diagnosis, surgical planning and follow-up of children with these rare tumours. Received: 6 October 2000 Revised: 6 February 2001 Accepted: 5 March 2001     

Nodular lesions of choroid plexus in Hurler disease

Neuropathologic examination of six brains from children with Hurler disease revealed nodular lesions in the glomus of choroid plexus caused by proliferation of vacuolated pericytes, fibroblasts, and arachnoid cells on the background of collagenized and myxoid stroma. This localization of lesions can be explained by the presence of a rich vascular network, as well as cellular heterogeneity greater in the glomus than in other parts of the choroid plexus or in the brain parenchyma. The development of nodules did not correlate with the age, severity of hydrocephalus, or the degree of expansion of the perivascular spaces in the brain.     

Carcinoma of the choroid plexus: A pediatric experience

Carcinoma of the choroid plexus (CCP) is a rare tumor of the central nervous system which occurs more frequently in patients under 3 years of age. We report 10 cases of CCP in children. There were 5 males and 5 females, aged 5 to 84 months at diagnosis (median 32 months). Eight CCP were located in the lateral ventricle and 2 in the fourth ventricle. All patients underwent initial surgery, with complete resection in 5 cases. Postoperative treatment included radiotherapy alone in 4 patients, chemotherapy alone in 4, both in 1 patient, and no further treatment in one patient. Six patients remain in first continuous complete remission with a follow-up of 12 to 156 months (mean 34 months). Five of these six patients had a complete surgical resection of their tumor and 4 of them received chemotherapy alone. A comparison with the series in the literature suggests that total resection of the tumors is the major prognostic factor for survival. Recourse to additional treatments to prevent local relapses and CNS dissemination seems necessary. In our opinion, radiotherapy should be avoided as far as possible because of its deleterious effect in very young children. As some of the long term survivors did not receive radiation treatment, we conclude that CCP can be treated post operatively using chemotherapy protocols designed for infants with malignant brain tumors. © 1993 Wiley-Liss, Inc.     

Efficacy of sequential chemotherapy including methotrexate and doxorubicin in an infant with partially resected choroid plexus carcinoma

This report refers to a 3-month-old male, with a residual choroid plexus carcinoma following partial resection, who was successfully treated with sequential chemotherapy without any postoperative radiation therapy. Along with carboplatin, we also used doxorubicin and methotrexate, hypothesizing that, given the patient's age, the blood-brain barrier should not hamper drug delivery to the tumor. According to this hypothesis, the treatment achieved complete remission of the disease, which lasts 27 months after the diagnosis. This result deserves further studies to assess the possible curative role of chemotherapy in very young patients suffering from choroid plexus carcinoma.     

Clinical features of infants with subependymalgerminolysis and choroid plexus cysts

BACKGROUND: Periventricular cysts are not rare findings in neonates. However, they are sometimes associated with serious clinical complications, such as congenital viral infections and anomalies. METHODS: We performed a retrospective follow-up study on newborns who had periventricular cysts on routine cranial ultrasound examination. RESULTS: We followed 13 infants (three preterm) with periventricular cysts. Ten had single or multiple germinolysis cysts and the remaining three had choroid plexus cysts. All infants had various kinds of underlying complications, including congenital viral infection (two with cytomegalovirus and one with rubella),Sotos syndrome (n = 4), intrauterine growth retardation (n = 5), large-for-dates(n = 4), congenital heart disease (n = 1),myelomeningocele (n = 1) and other minor anomalies. All cases of germinolysis except for one developed a neurodevelopmental abnormality and/or delay. In contrast,all three cases with choroid plexus cysts appeared to develop well,despite the underlying complications. CONCLUSIONS: Germinolysis cysts seem to be associated with systemic diseases and should be treated as a high-risk sign for impaired neurological development.