Epidemiological Characteristics and Prognosis Model of Pineal Region Tumors: A Retrospective Analysis Based on the SEER Database.

BACKGROUND: A pineal region tumor is a rare intracranial tumor, and its specific location leads to its own characteristics. This study aimed to provide some insight for medical practice in the care of pineal region tumors. We investigated the key epidemiological characteristics and survival prognosis of pineal tumors based on the epidemiological data from the Surveillance, Epidemiology, and End Results database. METHODS: Data of pineal region tumor patients from 1975 to 2019 were extracted from the Surveillance, Epidemiology, and End Results database. The data were divided into 3 pathologic groups: germ cell tumors, pineal parenchymal tumors, and other. The patients' overall survival (OS) was analyzed using the Kaplan-Meier method. The prognostic effects of the patient characteristics on OS were explored using the Cox proportional hazard model. The analysis results are presented as tabular data, Kaplan-Meier plots, forest plots, and nomograms. A calibration curve was used to verify the nomograms. All analyses were performed for all patients overall and stratified by pathological group using SPSS and R language. RESULTS: Based on the predefined inclusion and exclusion criteria, 628 patients were included in this study, of whom 440 (70.1%) were male and 188 (29.9%) were female. Most patients were aged 0-19 years. The pathological type was germinoma for 225 patients (35.8%). Age, surgery, behavioral code, and pathology were significant factors for OS. A calibration curve was used to verify that the nomograms had a good prediction effect. CONCLUSIONS: An intuitive nomogram was developed and verified and can predict the prognosis of patients with pineal tumors.

The Prognosis of Pineal Parenchymal Tumors: Development and Validation of a Nomogram Based on Surveillance, Epidemiology and End Results.

BACKGROUND: Pineal parenchymal tumors are exceedingly rare, and optimal disease management has yet to be defined. In this study, we aimed to identify prognostic factors and establish a predictive model for the prognosis of patients with pineal parenchymal tumors. METHODS: All patients with pineal parenchymal tumors in the Surveillance, Epidemiology and End Results database between 1975 and 2019 were reviewed. Data were summarized, and survival was modeled with Cox regression analyses. In addition, a nomogram predicting 5- and 10-year survival probability for pineal parenchymal tumors was developed and validated. RESULTS: We found 691 pineal parenchymal and 1961 pineal region neoplasms during 1975 and 2019 resulting in an incidence of 35%. In total, 441 patients were excluded due to incomplete data. The final cohort was subdivided into groups based on tumor histology: pineocytomas, pineoblastomas, and pineal parenchymal tumors of intermediate differentiation. Multivariate Cox analysis identified age and beam radiation as prognostic factors in pineoblastomas. Age, histology, tumor size, extent of resection, radiation, and chemotherapy were selected to build a clinical nomogram. The C-index for the nomogram was 0.795 (95% confidence interval 0.738-0.852). The calibration curves of the 5- and 10-year survival rates showed good agreement between the nomogram predictions and actual observations. CONCLUSIONS: This nomogram is a convenient and precise tool for clinicians to evaluate prognosis of pineal parenchymal tumors.

Prevalence Rate of Coexisting Rathke Cleft Cysts and Pineal Cysts: A Multicenter Cross-Sectional Study.

BACKGROUND: Rathke cleft cysts (RCCs) are benign sellar lesions originating from remnants of primitive ectoderm. They have not been previously linked to other cystic lesions, such as pineal cysts (PCs). Our objective was to perform a multicenter cross-sectional neuroimaging study to examine prevalence rates of coexisting RCC and PC. METHODS: We retrospectively queried prospectively maintained, institutional review board-approved, databases from the authors' centers. All patients undergoing transsphenoidal surgery for RCC between the years of 2011 and 2020 were included for analysis. Preoperative magnetic resonance imaging was reviewed to identify the coexistence of a PC. Patient demographics and neuroimaging characteristics were recorded. A control cohort comprised of 100 age- and sex-matched patients with nonfunctional pituitary adenoma (NFPA) who also underwent surgical intervention was utilized. RESULTS: Eighty-four patients with RCC were identified for analysis. A coexistent PC was identified in 40.5% (n = 34) of patients with RCC compared with 14.3% (n = 12) in the NFPA cohort (P < 0.001). There was no significant difference in PC size between patients with RCC and PA (8 vs. 8.8 mm, respectively; P = 0.77). Although the majority (85.7%; n = 72) of the RCC cohort were female patients, there was no sex predominance with respect to coexisting PC in either the RCC or PA cohort. CONCLUSIONS: This is the first study to report an increased prevalence of coexisting PC and RCC, possibly because of an embryologic link or other propensity for intracranial cyst formation. Additional studies in more generalizable populations can further explore the relation between RCC and PC, or other cyst formation.

Intracranial growing teratoma syndrome (iGTS): an international case series and review of the literature.

PURPOSE: Intracranial growing teratoma syndrome (iGTS) is a rare phenomenon of paradoxical growth of a germ cell tumor (GCT) during treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of iGTS in Western countries. METHODS: Pediatric patients from 22 North American and Australian institutions diagnosed with iGTS between 2000 and 2017 were retrospectively evaluated. RESULTS: From a total of 777 cases of central nervous system (CNS) GCT, 39 cases of iGTS were identified for an overall frequency of 5%. Pineal region was a more frequent location for iGTS as compared to cases of GCT without iGTS (p < 0.00001). In patients with an initial tissue diagnosis of GCT, immature teratoma was present in 50%. Serum AFP or ßhCG was detectable in 87% of patients (median values 66 ng/mL and 44 IU/L, respectively). iGTS occurred at a median of 2 months (range 0.5-32) from diagnosis, in the majority of patients. All patients underwent surgical resection, leading to gross total resection in 79%. Following surgery, all patients resumed adjuvant therapy or post treatment follow-up for GCT. At a median follow-up of 5.3 years (range 0.2-11.8), 37 (95%) of patients are alive, including 5 with stable residual mass. CONCLUSION: iGTS occurs in 5% of patients with GCT in Western countries. Tumors of the pineal region and GCT containing immature teratoma appear to be associated with a higher risk of developing iGTS. Complete surgical resection is the mainstay of treatment. Overall survival of patients developing iGTS remains favorable.

Outcome and prognostic features in paediatric pineoblastomas: analysis of cases from the Surveillance, Epidemiology, and End Results registry (1990-2007).

BACKGROUND: Paediatric pineoblastomas are rare central nervous system tumours. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. METHODS: Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated clinical and pathological factors associated with outcome in paediatric pineoblastomas. Paediatric patients (< 16 years old) with pineoblastomas diagnosed between 1990 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analysed included patient's age at diagnosis, gender, race, tumour spread and size, surgical resection and the use of adjuvant radiotherapy. RESULTS: Seventy-eight patients were identified from the database. Twelve patients were excluded as 11 had no surgery and one patient was excluded as the surgical status was unknown. Analysis of the remaining 66 patients revealed a median age at diagnosis of 5.5 years. Three patients underwent biopsy. Seventeen patients underwent full and partial resection, respectively. A further 46 patients underwent surgery the nature of which was not recorded. Thirty-nine patients (59.1%) received adjuvant radiotherapy. Eight patients (12.1%) had both surgery (full or partial resection) and radiotherapy. The median overall survival was 40.5 months. Univariate analysis demonstrated that older age at diagnosis was the only positive predictor of overall survival. CONCLUSION: This study represents the largest analysis of paediatric pineoblastomas to date. The only clinically relevant prognostic factor was older age at diagnosis. The role of surgery and adjuvant radiotherapy on overall survival remains to be defined.

Retinoblastoma and Neuroblastoma Predisposition and Surveillance.

Retinoblastoma (RB) is the most common intraocular malignancy in childhood. Approximately 40% of retinoblastomas are hereditary and due to germline mutations in the RB1 gene. Children with hereditary RB are also at risk for developing a midline intracranial tumor, most commonly pineoblastoma. We recommend intensive ocular screening for patients with germline RB1 mutations for retinoblastoma as well as neuroimaging for pineoblastoma surveillance. There is an approximately 20% risk of developing second primary cancers among individuals with hereditary RB, higher among those who received radiotherapy for their primary RB tumors. However, there is not yet a clear consensus on what, if any, screening protocol would be most appropriate and effective. Neuroblastoma (NB), an embryonal tumor of the sympathetic nervous system, accounts for 15% of pediatric cancer deaths. Prior studies suggest that about 2% of patients with NB have an underlying genetic predisposition that may have contributed to the development of NB. Germline mutations in ALK and PHOX2B account for most familial NB cases. However, other cancer predisposition syndromes, such as Li-Fraumeni syndrome, RASopathies, and others, may be associated with an increased risk for NB. No established protocols for NB surveillance currently exist. Here, we describe consensus recommendations on hereditary RB and NB from the AACR Childhood Cancer Predisposition Workshop. Clin Cancer Res; 23(13); e98-e106. ©2017 AACRSee all articles in the online-only CCR Pediatric Oncology Series.

Pineal cysts-A benign association with familial retinoblastoma.

Patients with familial/heritable retinoblastoma (RB) are at increased risk of developing second malignancies throughout life, including a pineoblastoma (trilateral RB [TRB]) in early childhood. Current guidelines recommend regular surveillance brain imaging for those with heritable RB until 5 years of age. The presence of pineal cysts has been reported in patients with RB. Pineal cysts are thought to arise due to focal degeneration of the pineal gland and can be found incidentally. The finding of pineal abnormalities including cysts in children with RB on imaging is disconcerting, as it raises the possibility of an underlying malignancy, specifically a pinealoblastoma. The authors reviewed the imaging findings and clinical significance of pineal cysts in 69 patients diagnosed with RB at our center between December 1999 and November 2015. Twenty-six patients had pineal cysts found on brain magnetic resonance imaging (MRI) scans performed either at diagnosis or follow-up. Thirty-eight of 69 patients had underlying heritable RB. Nineteen of 38 familial RB patients had a pineal cyst compared with 3 out of 26 with sporadic RB (P = .004). In the majority, the imaging characteristics and size of the cysts remained stable or resolved. In this cohort, pineal cysts were detected at significantly increased frequency in heritable RB. This may be a benign association or may reflect abnormal underlying biology of pineal tissue in individuals highly susceptible to malignancy. Imaging characteristics can be helpful in distinguishing between benign and malignant lesions. The presence of a pineal cyst in patients with unilateral disease may be a useful indicator of underlying heritable RB.

Magnetic resonance imaging biomarkers indicate a central venous hypertension syndrome in patients with symptomatic pineal cysts.

BACKGROUND: While most pineal cysts (PCs) are asymptomatic, some PCs are accompanied with symptoms of variable severity. We suggested that symptom severity in symptomatic patients with non-hydrocephalic PCs relates to venous compression causing central venous hypertension. This study explored whether possible magnetic resonance imaging (MRI) biomarkers of central venous hypertension could differentiate the severity of symptoms in individuals with non-hydrocephalic PCs. METHODS: The study included all individuals with PCs and MRI available for analysis followed conservatively within the department from 2003 to 2014. Severity of symptoms at follow-up were assessed from a questionnaire. Suggested MRI biomarkers indicative of central venous hypertension were explored, in addition to MRI measures of cyst size, aqueduct stenosis, and tectal compression. RESULTS: The study included 66 patients. As compared to the 27/66 patients (41%) with "None-Moderate" symptoms at follow-up, the 39/66 patients (59%) with "Much-Severe" symptoms presented with significantly altered indices of central venous hypertension (tectum-splenium-cyst ratio and indices of thalamic and periventricular edema). PC grading based on MRI biomarkers of central venous hypertension differentiated the severity of symptoms. CONCLUSION: The results indicate an association between severity of symptoms and MRI biomarkers of central venous hypertension in symptomatic individuals with non-hydrocephalic PCs.

Incidental Pineal Cysts: Is Surveillance Necessary?

OBJECTIVE: The natural history of incidental pineal cysts is poorly understood. Neurosurgeons and neuroradiologists are more frequently faced with this disease in the advent of higher-resolution magnetic resonance imaging (MRI) scanning. We aim to suggest a suitable surveillance strategy for these patients. METHODS: All patients who had MRI of the brain between June 2007 and January 2014 (n = 42,099) at The Walton Centre for Neurology and Neurosurgery were included. Radiologic reports containing the terms "pineal" and "cyst" were reviewed to identify patients. RESULTS: A total of 281 patients were identified with pineal cysts. The principal indication for head MRI was headache (50.2%), although no symptoms were deemed attributable to pineal disease. A total of 178 patients (63.3%) were female, and the age at diagnosis ranged from 16 to 84 years. The median size of pineal cyst at diagnosis was 10 mm. A total of 181 patients had subsequent follow-up at a median time of 6 months (range, 1-68). Eleven pineal cysts (6%) changed size during the follow-up period. Four patients had a reduction in cyst size; the median change was 2.5 mm. A further 7 pineal cysts increased in cyst size; the median change was 2 mm. No patients developed complications. CONCLUSIONS: Incidental pineal cysts typically show a benign course. In the adult population, they do not require long-term neurosurgical follow-up, because pineal cysts tend to remain a stable size. In asymptomatic patients, we recommend a single follow-up MRI scan at 12 months to confirm diagnosis. The patient should then be discharged if the cyst remains stable.

Occurrence of Pineal Gland Tumors in Combined Chronic Toxicity/Carcinogenicity Studies in Wistar Rats.

Pineal gland tumors are very rare brain lesions in rats as well as in other species including humans. A total of 8 (out of 1,360 examined) Wistar rats from 3 different combined chronic toxicity/carcinogenicity or mere carcinogenicity studies revealed pineal gland tumors. The tumors were regarded to be spontaneous and unrelated to treatment. The morphology and immunohistochemical evaluation led to the diagnosis malignant pinealoma. The main characteristics that were variably developed within the tumors were the following: cellular atypia, high mitotic index, giant cells, necrosis, Homer Wright rosettes, Flexner-Wintersteiner rosettes and pseudorosettes, positive immunohistochemical reaction for synaptophysin, and neuron-specific enolase. The pineal gland is not a protocol organ for histopathological examination in carcinogenicity studies. Nevertheless, the pineal gland can occasionally be encountered on the routine brain section or if it is the origin of a tumor protruding into the brain, the finding will be recorded. Therefore, although known to be a rare tumor in rats, pineal neoplasms should be included in the list of possible differential diagnoses for brain tumors, especially when the tumor is located in the region of the pineal body.

Incidence of pineal tumours. A review of the literature.

The knowledge of the incidence of pineal tumours is important not only for diagnostic care but also for its therapeutic programme. We reviewed the incidence of pineal tumours reported in literature in an attempt to establish if a difference existed between pineal gland tumours and the pineal region tumours as different authors use both expressions to indicate the same thing. The rate of frequency of these tumours is useful to guide the therapeutic choice for patients as the decisional tree is completely different for either germ cell tumours, pineal gland tumours or pineal gliomas and eventually papillary tumours of the pineal region. According to the French Register of pineal tumours, true pineal tumours represent: 27% pineal parenchymal tumours (PPT), 27% germ cell tumours, 17% gliomas, 8% papillary tumours. True pineal gland tumours are represented by: pineocytomas - (13%), pineal parenchymal tumours with intermediary differentiation PTT-ID - (66%) and pinealoblastomas - (21%). There was no statistical difference found between the French register and the Lyon series concerning histological diagnosis. It seemed to us important to discover its true incidence by comparing the data published in the literature and to stress the utility of the French Register for these uncommon tumours not only for recording new histological cases but also to document clinical symptomatology, therapeutic programmes, length of follow-up and clinical results for each patient treated. A better understanding of their natural history and improved evaluation of different treatments and their complications should contribute to improve clinical results.

An unusual location for a choroid plexus papilloma: the pineal region.

PURPOSE: Choroid plexus papillomas (CPP) are rare benign neoplasms of the central nervous system that occur most often in children during the first decade of life. They occur most often in the lateral ventricle. It is extremely rare for a CPP to occur in the pineal region. We describe the case of a child with a CPP located in the pineal region, who was initially diagnosed with obstructive hydrocephalus by cranial computed tomography (CT). METHODS: A 9-year-old female patient presented with complaints of visual disturbance, nausea, and vomiting. Magnetic resonance imaging (MRI) showed a poor contrast-enhanced pineal-localized lesion. Anatomical variations within the patient caused her surgery to proceed using a supratentorial-occipital interhemispheric approach. RESULTS: The tumor was totally removed, and a histological examination revealed the tumor to be a typical CPP. The patient received follow-up neurological and ophthalmologic examinations at 3, 6, 9, 12, 24, and 36 months postoperatively, which demonstrated her progressive improvement. CONCLUSIONS: CPPs may have a wide range of locations and resulting symptoms. However, the pineal region is a rarely encountered location, particularly for pediatric patients. It is of great value to correctly differentiate neoplasms such as germ cell tumors, pineocytomas, meningiomas, and astrocytomas, so that patients receive the correct diagnosis and treatment approach.

Outcome and prognostic features in adult pineoblastomas: analysis of cases from the SEER database.

BACKGROUND: Adult pineoblastomas (PBL) are rare central nervous system tumors. Patient and treatment factors associated with outcome are poorly defined and limited to small retrospective case series and single case reports. Using the Surveillance, Epidemiology, and End Results (SEER) cancer registry, we investigated clinicopathological factors associated with outcome in adult PBL. METHODS: Adult patients (≥16 years old) with PBL diagnosed between 1990 and 2007 were identified from the SEER database. Kaplan-Meier survival analysis and Cox models were used to examine the effect of variables on overall survival. The variables analyzed included patient's age at diagnosis, gender, race, tumor location, uni-focal or multi-focal tumor, tumor size, surgical resection, and the use of adjuvant radiotherapy. RESULTS: Ninety-five patients were identified, with a median age at diagnosis of 39.2 years. Sixty-one patients (64 %) underwent surgery and 44 patients (47.4 %) received adjuvant radiotherapy. Forty-two patients (44 %) had both surgery and radiotherapy. The median overall survival was 176 months. Univariate analysis identified younger age at diagnosis, uni-focal and localized disease as important predictors of overall survival. On multivariate analysis, only age at diagnosis and localized disease emerged as important prognostic factors. CONCLUSIONS: Despite the numerous limitations of the SEER database, this study represents the largest analysis of adult PBL to date. Clinically relevant prognostic factors were younger age of diagnosis and localized disease. The role of surgery and adjuvant radiotherapy remains to be defined. Our data suggest these treatment modalities may not influence overall survival.

Hidrocefalia triventricular secundaria a pineocitoma diagnosticado por endoscopía: presentación de un caso / Endoscopic diagnosis of triventricular hydrocephalus secondary to pineocitoma: a case report

Introducción: El pineocitoma es un tumor benigno del parénquima pineal, de crecimiento lento cuya sintomatologíadepende de la hidrocefalia no comunicante por obstrucción del Acueducto de Silvio y a efectos compresivos de estructuras nerviosas vecinas.Caso clínico: Se presenta un paciente masculino, de la piel blanca, con cuadro clínico de hipertensión endocraneanasecundaria a hidrocefalia triventricular. Le realizamos Fenestración Endoscópica premamilar del piso del tercer ventrículo y biopsia que diagnostico un pineocitoma. En el estudio tomográfico simple de cráneo solo existió diagnóstico de la primera entidad.Conclusiones: Se demuestra así, la ventaja que resulta de la neuroendoscopía del tercer ventrículo, no solo con fines terapéuticos sino también diagnósticos(AU)

Introduction: The Pineocitoma it is a benign pineal parenchyma tumor with slow growth. It´s symptoms depend on the noncommunicant hydrocephalus for obstruction of the Silvio aqueduct and compressive effects of surrounding nervous structures.Case report: In a white skin masculine patient, with clinical picture of intracranial hypertension secondary to triventricular hydrocephalus, we carry out a Fenestration Endoscopic premamilar of the floor of the third ventricle and biopsy that turnedout to be a Pineocitoma. In the simple CT scan of alone skull diagnosis of the first entity existed.Conclusions: By this way, is demonstrated the advantages of the neuroendoscopic of the third ventricle, not only for therapeutic ends but also diagnostic purposes(AU)

Neuroendoscopy in the management of pineal tumors.

INTRODUCTION: Endoscopy has been applied for the management of pineal tumors and the associated obstructive hydrocephalus. The techniques include endoscopic third ventriculostomy (ETV), endoscopic biopsy, and endoscopic-assisted open surgery of tumor in selected cases. The purpose of this study is to report our experience and techniques of endoscopic surgeries for pineal tumors in children. METHODS: This is a retrospective review of 36 ETVs, 25 endoscopic transventricular biopsies (ETB), 2 endoscopic transnasal transsphenoid biopsies, and 5 endoscopic-assisted open resections of pineal tumors. RESULTS: One patient died of delay tumor hemorrhage after ETV and ETB were excluded. With a mean follow-up period of 5.4 years, ETV failure occurred in 7 of 35 patients with long-term success rate of 80.0%. Histological diagnosis was established in 21 of 25 (84.0%) patients through ETBs. Tissue diagnosis was obtained in two selected patients with bifocal germ cell tumors by endoscopic transnasal transsphenoid approach to the intrasellar lesion. Direct radical resections were achieved in four of five selected patients of pineal tumors with hydrocephalus by endoscopic-assisted open surgery.

Childhood pineal parenchymal tumors: clinical and therapeutic aspects.

The aim of our study was to investigate the correlation of the clinical characteristic of pineal parenchymal tumors in children and adolescent with histopathological diagnosis and patient survival. Records of 27 patients with histologically diagnosed pineocytomas (n=16) and pineoblastoma (n=11) consecutively treated between 1991 and 2001 were reviewed retrospectively to identify factors predictive of aggressiveness. Among analyzed epidemiological, clinical, and radiological factors, we found that independent prognostic indicator in patients with childhood pineal parenchymal tumor was the extent of surgical resection.

[Primary central nervous system tumours reported in Cartagena, 2001-2006]. / Tumores primarios del sistema nervioso central en Cartagena, 2001- 2006.

OBJECTIVES: Determining the frequency of primary central nervous system tumours diagnosed in Cartagena; Colombia, from 2001-2006 and determining the demographic, epidemiological and clinical characteristics of patients having central nervous system tumours reported by a single institution in Cartagena between 2001 and 2006. METHODS: A passive epidemiological surveillance descriptive study was carried out. The pathology reports of new diagnosed central nervous system primary tumours from all laboratories in Cartagena were taken and the available clinical records regarding these cases were analysed. The overall incidence rate and incidence rates by year, gender, age and histological type were estimated, with 95 % confidence intervals. Standardised morbidity rates were also calculated. RESULTS: There were 390 such cases during 2001-2006. The overall incidence rate was 6.91/100,000 people-year. Meningiomas were the most frequently occurring histological types (3.46/100,000 people-year). The provenance could only be determined in 43.1 % of cases. Standardised morbidity rates were higher in Cartagena regarding those reported in the United States and by the Colombian National Cancer Institute and the Population-based Cali Cancer Registry. CONCLUSIONS: There was a higher incidence of primary central nervous system tumours in Cartagena than in the rest of the country. Registry and surveillance systems should be improved and research into risk factors encouraged.

Tumores primarios del sistema nervioso central en Cartagena, 2001- 2006 / Primary central nervous system tumours reported in Cartagena, 2001-2006

Objetivos Determinar la frecuencia de los tumores primarios del sistema nervioso central, diagnosticados en Cartagena de Indias durante el periodo de 2001-2006 y, determinar las características demográficas, epidemiológicas y clínicas de los pacientes con tumores del sistema nervioso central de una institución de la ciudad, en el mismo periodo. Métodos Se realizó un estudio descriptivo de vigilancia epidemiológica pasiva. Se tomaron los reportes de patología con diagnóstico nuevo de tumor primario del sistema nervioso central de todos laboratorios de Cartagena y se analizaron las historias clínicas disponibles de estos casos. Se estimaron las tasas de incidencia general, por año, género, grupos de edad y tipo histológico con intervalos de confianza al 95 por ciento. Además se calcularon razones estandarizadas de morbilidad. Resultados Durante los años 2001 y 2006 se encontraron 390 casos. La tasa incidencia general fue de 6,91/100 000 personas-año. El tipo histológico más frecuente fue meningioma (3,46/100 000 personas-año). Sólo se determinó la procedencia en el 43,1 por ciento de los casos. Las razones estandarizadas de morbilidad fueron más altas en Cartagena con respecto a las de los Estados Unidos, el Instituto Nacional de Cancerología de Colombia y el Registro Poblacional de Cáncer de Cali. Conclusiones Hubo una frecuencia de tumores primarios del sistema nervioso central más elevada en Cartagena que en el resto del país. Se recomienda mejorar los sistemas de registro y vigilancia para determinar la magnitud real del problema y fomentar investigaciones en busca de factores de riesgo.

Objectives Determining the frequency of primary central nervous system tumours diagnosed in Cartagena; Colombia, from 2001-2006 and determining the demographic, epidemiological and clinical characteristics of patients having central nervous system tumours reported by a single institution in Cartagena between 2001 and 2006. Methods A passive epidemiological surveillance descriptive study was carried out. The pathology reports of new diagnosed central nervous system primary tumours from all laboratories in Cartagena were taken and the available clinical records regarding these cases were analysed. The overall incidence rate and incidence rates by year, gender, age and histological type were estimated, with 95 percent confidence intervals. Standardised morbidity rates were also calculated. Results There were 390 such cases during 2001-2006. The overall incidence rate was 6.91/100,000 people-year. Meningiomas were the most frequently occurring histological types (3.46/100,000 people-year). The provenance could only be determined in 43.1 percent of cases. Standardised morbidity rates were higher in Cartagena regarding those reported in the United States and by the Colombian National Cancer Institute and the Population-based Cali Cancer Registry. Conclusions There was a higher incidence of primary central nervous system tumours in Cartagena than in the rest of the country. Registry and surveillance systems should be improved and research into risk factors encouraged.