Assuntos
Ependimoma , Glândula Pineal , Humanos , Ependimoma/patologia , Ependimoma/cirurgia , Ependimoma/diagnóstico por imagem , Ependimoma/diagnóstico , Glândula Pineal/patologia , Glândula Pineal/diagnóstico por imagem , Pinealoma/patologia , Pinealoma/diagnóstico por imagem , Pinealoma/cirurgia , Pinealoma/diagnóstico , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/diagnóstico , Masculino , Imageamento por Ressonância Magnética , FemininoRESUMO
A rare case of pineoblastoma on cerebrospinal fluid cytology was reported in a 15-year-old girl. In the current paper, a rare case of pienoblastoma on CSF cytology has been described.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Feminino , Humanos , Adolescente , Pinealoma/diagnóstico , Técnicas Citológicas , CitodiagnósticoRESUMO
PURPOSE: To provide a treatment-focused review and develop basic treatment guidelines for patients diagnosed with pineal anlage tumor (PAT). METHODS: Prospectively collected data of three patients with pineal anlage tumor from Germany was combined with clinical details and treatment information from 17 published cases. RESULTS: Overall, 20 cases of PAT were identified (3 not previously reported German cases, 17 cases from published reports). Age at diagnosis ranged from 0.3 to 35.0 (median: 3.2 ± 7.8) years. All but three cases were diagnosed before the age of three years. For three cases, metastatic disease at initial staging was described. All patients underwent tumor surgery (gross-total resection: 9, subtotal resection/biopsy: 9, extent of resection unknown: 2). 15/20 patients were alive at last follow-up. Median follow-up for 10/15 surviving patients with available follow-up and treatment data was 2.4 years (0.3-6.5). Relapse was reported for 3 patients within 0.8 years after diagnosis. Five patients died, 3 after relapse and 2 from early postoperative complications. Two-year-progression-free- and -overall survival were 65.2 ± 12.7% and 49.2 ± 18.2%, respectively. All 4 patients who received intensive chemotherapy including high-dose chemotherapy combined with radiotherapy (2 focal, 2 craniospinal [CSI]) had no recurrence. Focal radiotherapy- and CSI-free survival rates in 13 evaluable patients were 46.2% (6/13) and 61.5% (8/13), respectively. CONCLUSION: PAT is an aggressive disease mostly affecting young children. Therefore, adjuvant therapy using intensive chemotherapy and considering radiotherapy appears to comprise an appropriate treatment strategy. Reporting further cases is crucial to evaluate distinct treatment strategies.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Neoplasias Supratentoriais , Adolescente , Adulto , Criança , Pré-Escolar , Humanos , Lactente , Adulto Jovem , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/cirurgia , Recidiva Local de Neoplasia/patologia , Glândula Pineal/cirurgia , Glândula Pineal/patologia , Pinealoma/diagnóstico , Pinealoma/cirurgia , Recidiva , Neoplasias Supratentoriais/patologia , Resultado do TratamentoRESUMO
Embryonal and pineal tumours represent a diverse group of central nervous system (CNS) neoplasms. While many of the small round blue cell tumours that make up the embryonal neoplasms share similar histologic qualities, there are several morphologic and cytologic characteristics that are useful in distinguishing different tumour types. Similarly, pineal parenchymal tumours represent clinically diverse tumours, ranging from benign to overtly malignant. The most recent iteration of the World Health Organization Classification of CNS Tumours expanded greatly on the significance of molecular alterations in brain tumour diagnostics. In this article, we summarize the salient cytologic and histologic features of CNS embryonal and pineal tumours, and highlight diagnostically relevant molecular alterations within each tumour type.
Assuntos
Neoplasias Embrionárias de Células Germinativas , Glândula Pineal , Pinealoma , Humanos , Pinealoma/patologia , Pinealoma/diagnóstico , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/diagnóstico , Glândula Pineal/patologia , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genéticaRESUMO
BCOR is expressed in a new brain tumour entity, i.e. 'CNS tumour with BCOR internal tandem duplication' (HGNET BCOR) but not in several other high grade paediatric brain tumours investigated. Immunohistochemical detection of BCOR expression may therefore serve as a potential diagnostic marker. Nevertheless, in rare paediatric glioma cases recurrent EP300-BCOR fusions were detected, which resulted in strong BCOR immunopositivity. We have therefore examined other, not analysed so far, types of central nervous system (CNS) tumours, pineoblastoma and germinoma, to assess a potential involvement of BCOR in these tumours. Levels of BCOR RNA expression were investigated by NanoString nCounter system analysis in a series of altogether 66 high grade paediatric tumours, including four pineoblastoma cases. Immunohistological detection of BCOR was performed in eight pineoblastoma, five germinoma and four atypical teratoid rhabdoid tumours (ATRTs), all located in the pineal region. We detected BCOR expression in all pineoblastomas, at the RNA and protein levels, but not in germinomas and ATRTs. Further analysis of pineoblastoma samples did not reveal the presence of either BCOR internal tandem duplication or BCOR fusion involvement. Positive immunohistological BCOR nuclear reaction in pineoblastoma may therefore differentiate this type of tumour from other high grade tumours located in the pineal region.
Assuntos
Neoplasias Encefálicas , Germinoma , Glândula Pineal , Pinealoma , Tumor Rabdoide , Humanos , Criança , Pinealoma/diagnóstico , Pinealoma/genética , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , RNA , Proteínas Proto-Oncogênicas , Proteínas Repressoras/genéticaRESUMO
Pineal region tumors fall into five broad categories: benign pineal region tumors, glial tumors, papillary tumors, pineal parenchymal tumors, and germ cell tumors. Genetic and transcriptional studies have identified key chromosomal alterations in germinomas (RUNDC3A, ASAH1, LPL) and in pineocytomas/pineoblastomas (DROSHA/DICER1, RB1). Pineal region tumors generally present with symptoms of hydrocephalus including nausea, vomiting, papilledema, and the classical Parinaud's triad of upgaze paralysis, convergence-retraction nystagmus, and light-near pupillary dissociation. Workup requires neuroimaging and tissue diagnosis via biopsy. In germinoma cases, diagnosis may be made based on serum or CSF studies for alpha-fetoprotein or beta-HCG making the preferred treatment radiosurgery, thereby preventing the need for unnecessary surgeries. Treatment generally involves three steps: CSF diversion in cases of hydrocephalus, biopsy through endoscopic or stereotactic methods, and open surgical resection. Multiple surgical approaches are possible for approach to the pineal region. The original approach to the pineal region was the interhemispheric transcallosal first described by Dandy. The most common approach is the supracerebellar infratentorial approach as it utilizes a natural anatomic corridor for access to the pineal region. The paramedian or lateral supracerebellar infratentorial approach is another improvement that uses a similar anatomic corridor but allows for preservation of midline bridging veins; this minimizes the chance for brainstem or cerebellar venous infarction. Determination of the optimal approach relies on tumor characteristics, namely location of deep venous structures to the tumor along with the lateral eccentricity of the tumor. The immediate post-operative period is important as hemorrhage or swelling can cause obstructive hydrocephalus and lead to rapid deterioration. Adjuvant therapy, whether chemotherapy or radiation, is based on tumor pathology. Improvements within pineal surgery will require improved technology for access to the pineal region along with targeted therapies that can effectively treat and prevent recurrence of malignant pineal region tumors.
Assuntos
Neoplasias Encefálicas , Glioma , Hidrocefalia , Glândula Pineal , Pinealoma , Humanos , Pinealoma/diagnóstico , Pinealoma/genética , Pinealoma/cirurgia , Glândula Pineal/patologia , Glândula Pineal/cirurgia , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/patologia , Glioma/patologia , Hidrocefalia/patologia , Ribonuclease III , RNA Helicases DEAD-boxRESUMO
BACKGROUND: Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children. Even with intensive multimodal treatment, the prognosis of many types is guarded, and treatment-related toxicity is significant. Recent advances in molecular diagnostics allowed the discovery of novel entities and inter-tumor subgroups, with opportunities for improved risk-stratification and treatment approaches. SUMMARY: Medulloblastomas separate into four distinct subgroups with distinct clinicopathologic characteristics, and data from recent clinical trials for newly diagnosed medulloblastoma support subgroup-specific treatment approaches. Atypical teratoid rhabdoid tumor (ATRT), embryonal tumor with multilayered rosettes (ETMR), and pineoblastoma, as well as other rare embryonal tumors, can be distinguished from histologically similar tumors by virtue of characteristic molecular findings, with DNA methylation analysis providing a strong adjunct in indeterminate cases. Methylation analysis can also allow further subgrouping of ATRT and pineoblastoma. Despite the dire need to improve outcomes for patients with these tumors, their rarity and lack of actionable targets lead to a paucity of clinical trials and novel therapeutics. KEY MESSAGES: (1) Embryonal tumors can be accurately diagnosed with pediatric-specific sequencing techniques. (2) Medulloblastoma risk stratification and treatment decisions should take into account molecular subgroups. (3) There is a dire need for a novel collaborative clinical trial design to improve outcomes is rare pediatric embryonal tumors.
Assuntos
Neoplasias Embrionárias de Células Germinativas , Pré-Escolar , Humanos , Lactente , Neoplasias Encefálicas/patologia , Neoplasias do Sistema Nervoso Central/patologia , Neoplasias Cerebelares , Meduloblastoma/diagnóstico , Meduloblastoma/genética , Meduloblastoma/terapia , Neoplasias Embrionárias de Células Germinativas/genética , Neoplasias Embrionárias de Células Germinativas/terapia , Glândula Pineal/patologia , Pinealoma/diagnóstico , Pinealoma/genética , Pinealoma/terapia , Tumor Rabdoide/diagnóstico , Tumor Rabdoide/genética , Tumor Rabdoide/terapia , Ensaios Clínicos como AssuntoRESUMO
OBJECTIVE: Pineal region tumors account for 2.7%-11% of all CNS tumors in children. In this series, the authors present their surgical results and long-term outcomes from a pediatric pineal region tumor cohort. METHODS: A total of 151 children aged 0-18 years were treated from 1991 to 2020. Tumor markers were collected in all patients; if positive, chemotherapy was performed, and if negative, biopsy was performed, preferably endoscopically. Resection was performed when there was a residual germ cell tumor (GCT) lesion after chemotherapy. RESULTS: The distribution based on histological type, as verified by markers, biopsy, or surgery, was germinoma (33.1%), nongerminomatous GCT (NGGCT) (27.2%), pineoblastoma (22.5%), glioma (12.6%), and embryonal tumor (atypical teratoid rhabdoid tumor) (3.3%). A total of 97 patients underwent resection, and gross-total resection (GTR) was achieved in 64%; the highest GTR rate (76.6%) was found in patients with GCTs, and the lowest (30.8%) was found in those with gliomas. The supracerebellar infratentorial approach (SCITA) was the most common, performed in 53.6% of patients, followed by the occipital transtentorial approach (OTA), performed in 24.7% of patients. Lesions were biopsied in 70 patients, and the diagnostic accuracy was 91.4. The overall survival (OS) rates at 12, 24, and 60 months as stratified by histological type were 93.7%, 93.7%, and 88% for patients with germinomas; 84.5%, 63.5%, and 40.7% for patients with pineoblastomas; 89.4%, 80.8%, and 67.2% for patients with NGGCTs; 89.4%, 78.2%, and 72.6% for patients with gliomas; and 40%, 20%, and 0% for patients with embryonal tumors, respectively (p < 0001). The OS at 60 months was significantly higher in the group with GTR (69.7%) than in the group with subtotal resection (40.8%) (p = 0.04). The 5-year progression-free survival was 77% for patients with germinomas, 72.6% for patients with gliomas, 50.8% for patients with NGGCTs, and 38.9% for patients with pineoblastomas. CONCLUSIONS: The efficacy of resection varies by histological type, and complete resection is associated with higher OS rates. Endoscopic biopsy is the method of choice for patients presenting with negative tumor markers and hydrocephalus. For tumors restricted to the midline and with extension to the third ventricle, a SCITA is preferred, whereas for lesions with extension toward the fourth ventricle, an OTA is preferred.
Assuntos
Neoplasias Encefálicas , Germinoma , Glioma , Glândula Pineal , Pinealoma , Masculino , Criança , Humanos , Pinealoma/cirurgia , Pinealoma/diagnóstico , Pinealoma/patologia , Glândula Pineal/cirurgia , Glândula Pineal/patologia , Glioma/cirurgia , Glioma/patologia , Germinoma/patologia , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/patologiaRESUMO
PURPOSE: Pineal parenchymal tumors of intermediate differentiation (PPTIDs), which were recognized in the 2007 World Health Organization (WHO) classification, are rare, accounting for less than 1% of all central nervous system tumors. This rarity and novelty complicate the diagnosis and treatments of PPTID. We therefore aimed to evaluate the clinicopathological significance of this tumor. METHODS: At 11 institutions participating in the Kyushu Neuro-Oncology Study Group, data for patients diagnosed with PPTID were collected. Central pathology review and KBTBD4 mutation analysis were applied to attain the diagnostically accurate cohort. RESULTS: PPTID was officially diagnosed in 28 patients: 11 (39%) with WHO grade 2 and 17 (61%) with WHO grade 3 tumors. Median age was 49 years, and the male:female ratio was 1:2.1. Surgery was attempted in all 28 patients, and gross total resection (GTR) was achieved in 46% (13/28). Adjuvant radiotherapy and chemotherapy were administered to, respectively, 82% (23/28) and 46% (13/28). The 5-year progression-free survival (PFS) and overall survival rates were 64.9% and 70.4% respectively. Female sex (p = 0.018) and GTR (p < 0.01) were found to be independent prognostic factors for PFS and female sex (p = 0.019) was that for OS. Initial and second recurrences were most often leptomeningeal (67% and 100% respectively). 80% (20/25) of patients harbored a KBTBD4 mutation. CONCLUSIONS: Female sex and GTR were independent prognostic factors in our patients with PPTID. Leptomeningeal recurrence was observed to be particularly characteristic of this tumor. The rate of KBTBD4 mutation observed in our cohort was acceptable and this could prove the accuracy of our PPTID cohort.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Pinealoma/genética , Pinealoma/terapia , Pinealoma/diagnóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/diagnóstico , Estudos de Coortes , Intervalo Livre de Progressão , Glândula Pineal/patologia , Estudos RetrospectivosRESUMO
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.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Humanos , Pinealoma/diagnóstico , Pinealoma/epidemiologia , Pinealoma/terapia , Nomogramas , Prognóstico , Programa de SEER , Neoplasias Encefálicas/epidemiologia , Neoplasias Encefálicas/terapia , Glândula Pineal/patologiaRESUMO
Pineocytoma is a rare tumor. It is rare for pineocytoma to present as leptomeningeal metastasis. We present a rare case of pineocytoma with malignant transformation and leptomeningeal metastasis after subtotal tumor resection and adjuvant radiotherapy. This case was a 58-year-old male with an unsteady gait for 2 months. Enhanced brain magnetic resonance imaging revealed a heterogeneous mass involving the pineal region. The initial pathological diagnosis of pineocytoma was confirmed after subtotal tumor resection. Two years after adjuvant radiotherapy to the primary site, the magnetic resonance imaging showed C2 and T2 metastatic lesions, with the final pathological diagnosis being pineal parenchymal tumor (PPT) with intermediate differentiation after the removal of T2 intramedullary tumor. After that adjuvant radiotherapy at the cervical and thoracic spinal cord was completed. There was no recurrence of the tumor 1 year after the radiotherapy. We report a rare case of pineocytoma with malignant transformation to PPT with intermediate differentiation and leptomeningeal dissemination.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Masculino , Humanos , Pessoa de Meia-Idade , Pinealoma/diagnóstico , Pinealoma/radioterapia , Pinealoma/cirurgia , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/radioterapia , Neoplasias Encefálicas/patologia , Radioterapia Adjuvante , Glândula Pineal/patologia , Medula Espinal/patologia , Transformação Celular Neoplásica/patologiaAssuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Sarcoma , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/genética , RNA Helicases DEAD-box/genética , Humanos , Glândula Pineal/patologia , Pinealoma/diagnóstico , Pinealoma/genética , Pinealoma/patologia , Ribonuclease III/genética , Sarcoma/patologiaAssuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Neoplasias da Retina , Retinoblastoma , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Humanos , Lactente , Glândula Pineal/diagnóstico por imagem , Glândula Pineal/patologia , Pinealoma/diagnóstico , Pinealoma/genética , Pinealoma/patologia , Neoplasias da Retina/diagnóstico , Neoplasias da Retina/genética , Neoplasias da Retina/patologia , Retinoblastoma/diagnóstico , Retinoblastoma/genética , Retinoblastoma/patologiaAssuntos
Neoplasias Encefálicas , Síndrome de Secreção Inadequada de HAD , Glândula Pineal , Pinealoma , Síndrome de Emaciação , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/diagnóstico por imagem , Humanos , Síndrome de Secreção Inadequada de HAD/etiologia , Pinealoma/diagnóstico , Pinealoma/diagnóstico por imagem , Síndrome de Emaciação/complicaçõesRESUMO
Pineal parenchymal tumor of intermediate differentiation (PPTID) is a WHO grade II and III tumor arising from pineal parenchymal cells. PPTID is a rare tumor accounting for less than 1% of all primary central nervous system neoplasms. Therefore, reports describing the clinical characteristics and biological features of PPTID are lacking. Moreover, the therapeutic strategy remains controversial. The current study aimed to evaluate treatment results and problems of contemporary therapeutic modalities of PPTID based on its features compared with other pineal parenchymal tumors. A comprehensive systematic literature review of 69 articles was performed, including articles on PPTID (389 patients) and similar tumors. Patient demographics, disease presentation, imaging characteristics, biological features, and current therapeutic options and their results were reviewed. We found that histopathological findings based on current WHO classification are well associated with survival; however, identifying and treating aggressive PPTID cases with uncommon features could be problematic. A molecular and genetic approach may help improve diagnostic accuracy. Therapeutic strategy, especially for grade III and aforementioned uncommon and aggressive tumors, remains controversial. A combination therapy involving maximum tumor resection, chemotherapy, and radiotherapy could be the first line of treatment. However, although challenging, a large prospective study would be required to identify ways to improve the clinical results of PPTID treatment.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/terapia , Terapia Combinada , Humanos , Glândula Pineal/cirurgia , Pinealoma/diagnóstico , Pinealoma/cirurgia , Estudos ProspectivosRESUMO
PURPOSE: To determine the risk of patients with an early diagnosis of heritable retinoblastoma being diagnosed with TRb (or pineoblastoma) asynchronously in a later stage and its effect on screening. METHODS: We updated the search (PubMed and Embase) for published literature as performed by our research group in 2014 and 2019. Trilateral retinoblastoma (TRb) patients were eligible for inclusion if identifiable as unique and the age at which TRb was diagnosed was available. The search yielded 97 new studies. Three new studies and eight new patients were included. Combined with 189 patients from the previous meta-analysis, the database included 197 patients. The main outcome was the percentage of asynchronous TRb in patients diagnosed before and after preset age thresholds of 6 and 12 months of age at retinoblastoma diagnosis. RESULTS: Seventy-nine per cent of patients with pineoblastoma are diagnosed with retinoblastoma before the age of 12 months. However, baseline MRI screening at time of retinoblastoma diagnosis fails to detect the later diagnosed pineal TRb in 89% of patients. We modelled that an additional MRI performed at the age of 29 months picks up 53% of pineoblastomas in an asymptomatic phase. The detection rate increased to 72%, 87% and 92%, respectively, with 2, 3 and 4 additional MRIs. CONCLUSIONS: An MRI of the brain in heritable retinoblastoma before the age of 12 months misses most pineoblastomas, while retinoblastomas are diagnosed most often before the age of 12 months. Optimally timed additional MRI scans of the brain can increase the asymptomatic detection rate of pineoblastoma.
Assuntos
Neoplasias Encefálicas/diagnóstico , Diagnóstico Precoce , Glândula Pineal , Pinealoma/diagnóstico , Neoplasias da Retina/diagnóstico , Retinoblastoma/diagnóstico , Humanos , Lactente , Imageamento por Ressonância MagnéticaRESUMO
BACKGROUND: The survival of pineoblastoma patients is low, particularly in infants and those with metastatic disease. This study aimed to analyze the prognostic factors affecting the outcome of Pineoblastoma in different age groups. METHODS: A retrospective study included 33 patients. Twenty-two patients older than 3 years had upfront surgery, followed by induction CSI then 6 cycles of chemotherapy. Eleven patients younger than 3 years underwent surgery, followed by induction chemotherapy then radiation therapy. Focal irradiation (54 Gy) was administrated in six patients, and CSI (23.4 Gy) with booster dose 30.6 Gy to the tumor bed in two patients followed by 4 cycles of chemotherapy. RESULTS: Patient's age showed a significant impact on the outcome (P value = 0.001 for EFS and 0.002 for OS). The metastases' presence did not impact the outcome negatively. The survival of patients with metastatic disease did not differ between age groups. However, age had a significant impact on the outcome of M0 disease, with 3-year EFS and OS of 65.3% and 74%, respectively, in the older group compared to 0% for both rates in younger patients. CSI showed a positive impact on survival. For all cases, the 3-year OS and EFS were 46.7% and 44.4%, respectively. CONCLUSIONS: A multimodality approach is needed to treat this aggressive disease. Inadequate dose intensity affected our patients' outcome negatively. A more aggressive approach using high-dose chemotherapy or CSI may be required to improve infantile pineoblastoma's dismal outcome. Focal radiotherapy is not an efficacious treatment in infants due to its high-metastatic potential. Molecular typing should be considered to label patients who need a more intensified approach.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/radioterapia , Terapia Combinada , Humanos , Lactente , Pinealoma/diagnóstico , Pinealoma/radioterapia , Prognóstico , Estudos Retrospectivos , Resultado do TratamentoAssuntos
Carcinomatose Meníngea/diagnóstico por imagem , Carcinomatose Meníngea/cirurgia , Transtornos da Motilidade Ocular/diagnóstico por imagem , Transtornos da Motilidade Ocular/cirurgia , Pinealoma/diagnóstico por imagem , Pinealoma/cirurgia , COVID-19/complicações , Terapia Combinada , Diagnóstico Diferencial , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Hidrocefalia/etiologia , Imunoterapia , Imageamento por Ressonância Magnética , Masculino , Melanócitos/patologia , Melanoma/cirurgia , Melanoma/terapia , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Nistagmo Patológico/etiologia , Transtornos da Motilidade Ocular/patologia , Pinealoma/diagnóstico , Distúrbios Pupilares/etiologiaRESUMO
BACKGROUND: Pineoblastomas (PB) are rare tumors of the central nervous system and are more common in children. There is no consensus about standard of care. The objective of this study is to analyze the outcome of children with PB. METHODS: Six patients with PB who were diagnosed between 1990-2012 were evaluated retrospectively. Demographics, age of diagnosis, first complaint, tumor region, diagnosis type, seeding metastasis to the spinal axis or cerebrospinal fluid (CSF), treatment and survival of these patients were recorded. RESULTS: Three patients had subtotal resection and all patients received chemotherapy and craniospinal irradiation (CSI) after diagnosis. Median follow-up after treatment was 5.5 (range:1-19) years. Two patients are alive with no evidence of disease for 7.5 and 10 years, one of whom was diagnosed with papillary thyroid carcinoma 9.5 years after treatment. One of the patients who died had lived for 19 years after diagnosis. CONCLUSIONS: Pineoblastomas are rare but very aggressive tumors; more effective treatment strategies are needed. Survivors should be followed up for late effects such as second malignancies and endocrine deficiencies.
Assuntos
Neoplasias Encefálicas , Glândula Pineal , Pinealoma , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Criança , Humanos , Pinealoma/diagnóstico , Pinealoma/terapia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Growing teratoma syndrome is a well-recognized condition associated with both intracranial and extracranial nongerminomatous germ cell tumors (NGGCTs), which mostly manifest as rapid growth of cystic and solid components during or within several months after treatment. Here, we report a patient with NGGCT who experienced slow growth of intracranial growing teratoma syndrome with intraventricular lipid accumulation over 10 years without any clinical symptoms. Considering the clinicopathologic heterogeneity of this syndrome, long-term clinical and radiologic follow-up is required for all patients with intracranial NGGCT.