Management of disseminated choroid plexus papilloma: a case study.

Choroid plexus papilloma (CPP) is a low-grade neoplasm of the choroid plexus that typically occurs in childhood and comprises under 1% of adult brain tumors. In this article, we report our experience delivering craniospinal irradiation (CSI) to a highly unusual case of disseminated CPP in an adult patient 8 years after an initial near total resection. The patient remains free of disease progression 3 years after completing radiation.

The relative patient benefit of gross total resection in adult choroid plexus papillomas.

Choroid plexus papillomas are rare neuroepithelial tumors found mainly in children. Although well studied in the pediatric population, there is a paucity of literature focusing specifically on adults. We sought to assess the relative advantage of gross total resection (GTR) and further characterize the natural history of this disease in adults. A comprehensive PubMed search was performed to identify adults who underwent surgical resection for choroid plexus papillomas with clearly reported age, tumor location, and extent of resection. Kaplan-Meier analysis was used to assess progression-free survival (PFS) and overall survival (OS). Multivariate analysis was performed using Cox proportional hazards models. A total of 193 patients were identified with a mean age of 39.9 ± 1.1 years. GTR was achieved in 72% of patients with subtotal resection (STR) in 28%. GTR was associated with a significant increase in both PFS (p = 0.015) and OS (p = 0.004) compared to STR. In a multivariate Cox proportional hazards model we found that only GTR was associated with recurrence (hazard ratio [HR] = 0.47, 95% confidence interval [CI] 0.25-0.90), while both age (HR = 1.03, 95% CI 1.00-1.05) and GTR (HR = 0.36, 95% CI 0.17-0.78) were associated with OS. Interestingly, our observed recurrence and death rates were higher than those in previously published studies. These findings demonstrate the benefit of GTR for the treatment of choroid plexus papillomas in adults. Our analysis suggests that these lesions are not as indolent as previously thought and while GTR is preferred, it is not always curative.

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...

Choroid plexus papillomas: a single institutional experience.

OBJECTIVE: To determine the long-term outcome of resected choroid plexus papillomas (CPPs). METHODS: Medical records and histologic specimens were reviewed for 41 patients (19 male, 22 female; median age, 36 years; range, 6 months to 74 years) with CPP seen between 1974 and 2000. Tumor locations were as follows: 76%, fourth ventricle; 17%, lateral ventricle, and 7%, third ventricle. Fifty-six percent had a gross total resection (GTR) and 44% had a subtotal resection (STR). Median follow-up was 6.5 years. RESULTS: Five-year local control, distant brain control, and overall survival were 84%, 92%, and 97%, respectively. Comparison of GTR and STR at 5 years showed a significant increase in local control (100% vs. 68%; P = 0.04) but not in overall survival (100% vs. 94%). Even after STR, only 50% of patients required a subsequent resection for recurrence. Addition of radiation therapy to initial STR did not seem to influence outcomes. At first relapse, GTR was accomplished in 1 patient, and only STR was accomplished in the others. Addition of radiation therapy to STR in our study led to disease control in half the patients treated, and STR alone led to disease control in only a quarter of the patients. Second relapses were treated palliatively with radiation therapy. CONCLUSIONS: Surgical resection is the treatment of choice for CPPs. After initial STR, reoperations for recurrence are required only half the time. Therefore, there seems to be no role for radiation therapy after initial STR. For STRs at first relapse, local control outcome is poor.

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.