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.

Current status and research progress on diagnosis and treatment of choroid plexus carcinoma / 中华放射肿瘤学杂志

Choroid plexus carcinoma is a relatively rare primary intracranial malignant tumor which is derived from the choroid plexus epithelium.It is classified as World Health Organization (WHO) grade Ⅲ and mainly occurs in children.Currently,maximal surgical resection is still the main therapeutic strategy.The clinical efficacy of postoperative adjuvant therapies remains controversial.Recent studies have promoted that postoperative combination of radiotherapy and chemotherapy can enhance the clinical prognosis and prolong the survival time for choroid plexus carcinoma patients undergoing sub-radical resection.In this review,relevant articles published in the recent 15 years were retrieved to summarize the current status and research progress on the diagnosis and treatment of choroid plexus carcinoma.

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 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 in an Adult

Choroid plexus carcinomas are extremely rare in adults. They can behave aggressively and their optimal management is uncertain. A 35-year-old woman was admitted with an episode of loss of consciousness. Magnetic resonance imaging showed a homogeneously enhancing mass in the trigone of the right lateral ventricle. Detailed examinations found no evidence of an extraneural primary focus. She underwent total removal of the tumor. Pathological diagnosis was confirmed as a choroid plexus carcinoma. She is doing well eight months after surgery.

Efficacy of Preoperative Chemotherapy in a Choroid Plexus Carcinoma

Choroid plexus carcinomas(CPCs) are rare neuroectodermal malginancies occuring mostly in young children of less than two years old, of which prognosis have been reported extremely grave. Generally, the extent of surgical resection has been regarded the most significant prognostic factor. However, the inherent hypervascularity and softness of these tumors make the total removal very difficult. We present our experience in a 18-months-old child with a large intraventricular CPC, of which total resection was achieved by the use of preoperative neoadjuvant chemotherapy with ICE regimen(Ifosfamide, Cisplatinum, Etoposide).

Huge Choroid Plexus Carcinoma in an Infant

Choroid plexus carcinoma is a rare malignant brain tumor that occurs predominantly in childhood. A 203-day-old infant was admitted to our hospital with macrocephaly and right hemiparesis. The skull protruded diffusely in the left parietal area. Brain magnetic resonance imaging) revealed a huge mass in the left ventricle. We performed an open biopsy and discovered a choroid plexus carcinoma. The tumor bled very easily and hemostasis was difficult. After three cycles of chemotherapy, we resected the entire mass in a staged operation. Chemotherapy enabled us to resect the entire tumor by reducing its size. We report this case to stress the benefits of preoperative chemo-therapy and review the relevant literature.

Surgical Outcome of Choroid Plexus Tumors in Adults : Experience of 5 Cases

A retrospective analysis of five adult patients with choroid plexus tumor(four choroids plexus papillomas, one choroids plexus carcinoma who underwent surgical resection at our institute between February 1991 and October 1995 was performed. The study group included two males and three females with a mean age of 34.0 years(range 21 to 24 years). Two of four choroids plexus papillomas were removed gross totally and one of these was irradiated postoperatively. The remaining two patients with choroids plexus papilloma underwent a subtotal surgical resection and one of them received radiation therapy. During follow-up, the authors could not find any difference in the size of the residual tumor or in the patient's KPS between the irradiated groups. There was also no response to postoperative radiation therapy in a patient with choroids plexus carcinoma who underwent a subtotal resection. The author's clinical experiences suggest that the use of radiation therapy in adult patients with choroids plexus tumor is not effective, and thus gross total resection should be attempted during the primary operation of such tumors.

Choroid Plexus Tumor Located in Extra-Ventricular Area : A Case of Pigmented Choroid Plexus Carcinoma: A Report of a Case

A Case of pigmented choroid plexus carcinoma is reported. The patient was a 35-year-old woman who had headache of 3-month duration, followed by ataxia for 1month before admission. Brain CT revealed a well enhanced mass at the left cerebello-pontine angle area as well as hydrocephalus. Subtotal removal of the tumor was performed via suboccipital craniectomy under the impression of meningioma because there was a mass only in the extraventricular area. The tumor was diagnosed by light microscopic examination. Pigmented choroid plexus carcinoma, which has been reported as low grade malignancy, is very rare and located always in the ventricles. In the case the tumor was subtotally removed and ventriculo-peritoneal shunt surgery and radiation therapy underwent later. The patient has been followed-up without recurrence for 1 year postoperatively.