Tumors of the central nervous system. Classification of the World Health Organization 2021. Towards a paradigm shift. / Tumores del sistema nervioso central. Clasificación de la Organización Mundial de la Salud 2021. Hacia un cambio de paradigma.

The study of central nervous system (CNS) tumors is a subject of great interest and such knowledge is of great importance in medical practice. The classifications of CNS neoplasms began in the mid-19th century, until the World Health Organization (WHO) published, in 1979, the first edition of a useful systematic review for the purpose of establishing a common language for all medical specialties. To date, 5 updated editions of neoplastic taxonomy have been published. The fifth edition, from 2021, consolidates the paradigm shift brought about by molecular advances, although the transition between morphological and molecular biological characterization is still in progress. In this article, the new modifications introduced in the different most frequent families of tumors in pediatrics are analyzed, emphasizing useful information for pediatricians in their daily practice and multidisciplinary consultations.

El estudio de los tumores del sistema nervioso central (SNC) resulta ser un tema de gran consideración y su conocimiento reviste una alta importancia en la práctica médica. Las clasificaciones de las neoplasias del SNC comenzaron a mediados del siglo XIX hasta que en 1979 la Organización Mundial de la Salud (OMS) publicó la primera edición de una sistemática útil con el objetivo de establecer un lenguaje común para todas las especialidades médicas. Al día de hoy, 5 ediciones actualizaron la taxonomía neoplásica. La quinta edición del año 2021 consolida el cambio de paradigma dado por los avances moleculares, si bien todavía la transición se encuentra en proceso entre la caracterización morfológica y la biológica molecular. En este artículo, se analizan las nuevas modificaciones incorporadas en las diferentes familias tumorales más frecuentes en pediatría haciendo hincapié en aquella información de utilidad para el médico pediatra en su práctica diaria y la consulta multidisciplinaria.

Clinical Validation of Stimulated Raman Histology for Rapid Intraoperative Diagnosis of Central Nervous System Tumors.

Stimulated Raman histology (SRH) is an ex vivo optical imaging method that enables microscopic examination of fresh tissue intraoperatively. The conventional intraoperative method uses frozen section analysis, which is labor and time intensive, introduces artifacts that limit diagnostic accuracy, and consumes tissue. SRH imaging allows rapid microscopic imaging of fresh tissue, avoids tissue loss, and enables remote telepathology review. This improves access to expert neuropathology consultation in both low- and high-resource practices. We clinically validated SRH by performing a blinded, retrospective two-arm telepathology study to clinically validate SRH for telepathology at our institution. Using surgical specimens from 47 subjects, we generated a data set composed of 47 SRH images and 47 matched whole slide images (WSIs) of formalin-fixed, paraffin-embedded tissue stained with hematoxylin and eosin, with associated intraoperative clinicoradiologic information and structured diagnostic questions. We compared diagnostic concordance between WSI and SRH-rendered diagnoses. Also, we compared the 1-year median turnaround time (TAT) of intraoperative conventional neuropathology frozen sections with prospectively rendered SRH-telepathology TAT. All SRH images were of sufficient quality for diagnostic review. A review of SRH images showed high accuracy in distinguishing glial from nonglial tumors (96.5% SRH vs 98% WSIs) and predicting final diagnosis (85.9% SRH vs 93.1% WSIs). SRH-based diagnosis and WSI-permanent section diagnosis had high concordance (κ = 0.76). The median TAT for prospectively SRH-rendered diagnosis was 3.7 minutes, approximately 10-fold shorter than the median frozen section TAT (31 minutes). The SRH-imaging procedure did not affect ancillary studies. SRH generates diagnostic virtual histologic images with accuracy comparable to conventional hematoxylin and eosin-based methods in a rapid manner. Our study represents the largest and most rigorous clinical validation of SRH to date. It supports the feasibility of implementing SRH as a rapid method for intraoperative diagnosis complementary to conventional pathology laboratory methods.

Advances in treatment of elderly primary central nervous system lymphoma.

The management of older individuals (≥60 years) with primary central nervous system lymphoma remains a clinical challenge. Identification of optimal therapy and delivering adequate dose intensity are two of the major issues in treating elderly patients. Premorbid performance status and comorbidities influence individualised treatment approaches and geriatric assessment tools are increasingly utilised. Optimal induction treatment remains high-dose methotrexate-based immunochemotherapy, delivery is feasible in the majority of patients and the goal of treatment remains achieving complete remission. Consolidation strategies are also relevant in the elderly, aiming to maximise duration of response and quality of life (QoL). Potential options include high-dose therapy with haematopoietic stem cell consolidation, non-myeloablative chemotherapy and whole-brain radiotherapy. Efficacy of novel agents, such as Bruton tyrosine kinase inhibitors and lenalidomide, have been reported; these represent an alternative for elderly patients unfit for chemotherapy. Prognosis remains poor, improvement of outcomes in this age group is urgently needed.

CNS Tumors in Adolescents and Young Adults: The Need for a Holistic Specialized Approach.

CNS tumors are one of the most common causes of cancer-related death in the 15- to 39-year-old age group. The management of adolescents and young adults (AYAs) who are diagnosed with brain tumors presents unique endocrine, developmental, and psychosocial issues. AYAs are frequently diagnosed late, after a prolonged period of misdiagnosis. The epidemiology, biology, prognosis, and overall management of these tumors differ from those of both older and younger age groups. AYAs are usually in a transitional phase in their lives, and brain tumors in this age group carry a better prognosis than in older adults; thus, special attention should be paid to survivorship care. Fertility and other treatment-related sequelae that affect the quality of life, as well as the increased risk of secondary malignancies in long-term survivors, are such examples. Although most AYAs are managed by adult or, to a lesser extent pediatric, oncologists, a multidisciplinary approach in the setting of specialized centers with increased participation in clinical trials is preferable. End-of-life and palliative care remain an unmet need for these patients, because most physicians lack the training to discuss such issues with young patients.

Primary central nervous system lymphoma: time for diagnostic biomarkers and biotherapies?

PURPOSE OF REVIEW: Primary central nervous system lymphoma (PCNSL) is a rare cancer with a somber prognosis in older patients, which it affects predominantly. Only in recent years have molecular alterations characterizing PCNSL been thoroughly described. This opens possibilities for the use of targeted therapies. Developments in imaging and biomarkers have also great potential to help clinicians faced with diagnostic and prognostic uncertainties. RECENT FINDINGS: Several biomarkers for PCNSL, such as different microRNAs, which could be tested in cerebrospinal fluid and vitreous fluid, and IL-10, which has been shown to have excellent sensitivity and specificity in the cerebrospinal fluid, have emerged in the last years. Methotrexate-based regimens remain the gold standard first-line treatment, with recent studies looking at the best adjunctive molecules to methotrexate, including rituximab, and at the role of autologous stem cell transplantation. As mutations leading to the activation of nuclear factor-kappa-B signaling are found in most PCNSLs, with mutations of MYD88 and CD79B particularly, ibrutinib is studied as molecule of great interest and encouraging results have been found in pilot studies. There is also great interest in the immunomodulatory drugs (lenalidomide) and immunotherapy (anti-programmed cell death 1/programmed cell death 1 ligand 1). SUMMARY: Identification of molecular genetic and cytokine changes in tumor and liquid biopsies will have an increasing role in the diagnostic and follow-up of PCNSL but also in the treatment and management of the disease.

Central precocious puberty following the diagnosis and treatment of paediatric cancer and central nervous system tumours: presentation and long-term outcomes.

OBJECTIVES: To estimate the prevalence of central precocious puberty (CPP) after treatment for tumours and malignancies involving the central nervous system (CNS) and examine repercussions on growth and pubertal outcomes. DESIGN: Retrospective study of patients with tumours near and/or exposed to radiotherapy to the hypothalamus/pituitary axis (HPA). PATIENTS AND MEASUREMENTS: Patients with CPP were evaluated at puberty onset, completion of GnRH agonist treatment (GnRHa) and last follow-up. Multivariable analysis was used to test associations between tumour location, sex, age at CPP, GnRHa duration and a diagnosis of CPP with final height <-2SD score (SDS), gonadotropin deficiency (LH/FSHD) and obesity, respectively. RESULTS: Eighty patients (47 females) had CPP and were followed for 11·4 ± 5·0 years (mean ± SD). The prevalence of CPP was 15·2% overall, 29·2% following HPA tumours and 6·6% after radiotherapy for non-HPA tumours. Height <-2SDS was more common at the last follow-up than at the puberty onset (21·4% vs 2·4%, P = 0·005). Obesity was more prevalent at the last follow-up than at the completion of GnRHa or the puberty onset (37·7%, 22·6% and 20·8%, respectively, P = 0·03). Longer duration of GnRHa was associated with increased odds of final height <-2SDS (OR = 2·1, 95% CI 1·0-4·3) and longer follow-up with obesity (OR = 1·3, 95% CI 1·1-1·6). LH/FSHD was diagnosed in 32·6%. There was no independent association between CPP and final height <-2SDS, and LH/FSHD and obesity in the subset of patients with HPA low-grade gliomas. CONCLUSIONS: Patients with organic CPP experience an incomplete recovery of growth and a high prevalence of LH/FSHD and obesity. Early diagnosis and treatment of CPP may limit further deterioration of final height prospects.

Prognostic impact of B-cell lymphoma 6 in primary CNS lymphoma.

BACKGROUND: We investigated the prognostic significance of B-cell differentiation status and common B-cell differentiation markers in a post hoc analysis of 119 patients with primary CNS lymphoma (PCNSL) homogeneously receiving high-dose methotrexate (HDMTX)-based chemotherapy within the prospective G-PCNSL-SG1 trial. METHODS: We evaluated protein expression of B-cell lymphoma 2 (BCL2), BCL6, CD10, and multiple myeloma oncogene 1/interferon regulatory factor 4 (MUM1/IRF4) by immunohistochemistry and analyzed the association with survival. RESULTS: The median follow-up of all patients was 67.5 months. Median progression-free survival (PFS) was 10.61 months (95% CI: 4.23-17.00). Median overall survival (OS) was 28.85 months (95% CI: 17.96-39.73). Eighty-nine tumors expressed BCL2 (92.7%), 24 (20.5%) expressed CD10, 60 (54.1%) expressed BCL6, and 87 (79.0%) expressed MUM1/IRF4. On the basis of the Hans algorithm, 80 tumors (73.4%) were classified to the non-germinal center B group, suggesting a post-germinal center origin of PCNSL. Expression of BCL6 (cutoff point 30%), but none of the other markers, was associated with shorter PFS (P = .047) and OS (P = .035). On multivariate analysis, BCL6 expression was associated with shorter PFS (hazard ratio: 1.95, 95% CI: 1.22-3.12, P = .005) but not OS (hazard ratio: 1.85, 95% CI: 0.71-4.80, P = .21). Classification according to Hans algorithm and expression status of the single B-cell markers BCL2, CD10, and MUM1/IRF4 did not correlate with prognosis. CONCLUSION: The findings are limited by the fact that only 23% of all G-PCNSL-SG1 patients could be included in the analysis. If validated in an independent cohort, BCL6 may assume clinical relevance as an unfavorable prognostic biomarker in PCNSL.

Predictive molecular markers in metastases to the central nervous system: recent advances and future avenues.

Metastases to the central nervous system (CNS) are common in several cancer types. For most primary tumors that commonly metastasize to the CNS, molecular biomarker analyses are recommended in the clinical setting for selection of appropriate targeted therapies. Therapeutic efficacy of some of these agents has been documented in patients with brain metastases, and molecular testing of CNS metastases should be considered in the clinical setting. Here, we summarize the clinically relevant biomarker tests that should be considered in neurosurgical specimens based on the current recommendations of the European Society of Medical Oncology (ESMO) or the National Comprehensive Cancer Network (NCCN) for the most relevant primary tumor types: lung cancer (EGFR mutations, ALK rearrangement, BRAF mutations), breast cancer (HER2 amplification, steroid receptor overexpression), melanoma (BRAF mutations), and colorectal cancer (RAS mutations). Furthermore, we discuss emerging therapeutic targets including novel oncogenic alterations (ROS1 rearrangements, FGFR1 amplifications, CMET amplifications, and others) and molecular features of the tumor microenvironment (including immune-checkpoint molecules such as CTLA4 and PD-1/PD-L1). We also discuss the potential role of advanced biomarker tests such as next-generation sequencing and "liquid biopsies" for patients with CNS metastases.

Glioblastoma multicêntrico originado em dois locais incomuns: cerebelo e tálamo / Multicentric glioblastoma arising in two unusual sites: cerebellum and thalamus

Multicentric glioblastomas (MGBM) arising in infra/supratentorial regions are uncommon lesions. The authors report a case of MGBM in a 61 year-old female patient, who presented a sudden onset of left hemiplegia. The magnetic resonance imaging (MRI) showed two expansive large lesions affecting cerebellum and thalamus, with strong contrast enhancement. The patient underwent resection of the cerebellar lesion. Microscopy revealed a high grade glial neoplasm exhibiting high mitotic index, areas of necrosis and microvascular proliferation. The neoplastic cells showed positive immunoexpression for glial fibrillary acidic protein (GFAP). The morphological findings were consistent with glioblastoma (GBM). The patient was referred to radiotherapy, with discrete signs of tumor regression after a 60-day clinical follow-up.

Glioblastomas multicêntricos (GBMM) originados em regiões infra/supratentoriais são lesões incomuns. Os autores relatam um caso de GBMM em paciente do sexo feminino, 61 anos de idade, que apresenta quadro súbito de hemiplegia esquerda. O exame de ressonância magnética (RM) mostrou duas lesões expansivas volumosas, com forte impregnação pelo contraste no cerebelo e no tálamo. A paciente foi submetida à ressecção da lesão cerebelar. À microscopia, foi identificada uma neoplasia glial de alto grau exibindo alto índice mitótico, áreas de necrose e proliferação microvascular. As células neoplásicas revelaram imunoexpressão positiva para proteína glial acídica (GFAP). O conjunto das alterações morfológicas foi consistente com glioblastoma. A paciente foi encaminhada para radioterapia, com sinais discretos de regressão tumoral após acompanhamento clínico de 60 dias.

The management of primary central nervous system lymphoma related to AIDS in the HAART era.

PURPOSE OF REVIEW: The review highlights the recent advances in the pathogenesis, diagnosis and treatment of AIDS-related primary CNS lymphoma (AIDS-PCNSL). RECENT FINDINGS: The incidence of AIDS-PCNSL has decreased in the era of highly active antiretroviral therapy (HAART). The prognosis has improved and this most probably in relation both with the HAART-induced immunologic status recovery and subsequently the possibility to use more aggressive treatment strategies. Immunomodulatory effect of HAART seems also to have an indirect antitumor activity on the disease. SUMMARY: The treatment strategy for AIDS-PCNSL in the HAART era tends to become similar to that of the immunocompetent population. In the absence of randomized trials devoted to AIDS-PCNSL, most current national comprehensive cancer network guidelines recommend the use of high-dose methotrexate-based chemotherapy combined or not with whole-brain radiotherapy as initial treatment in addition to HAART. The objective for the future would be that prognosis of AIDS-PCNSL catch up with that of the immunocompetent patients with special attention to systemic and neurocognitive tolerance of the treatments.

Prognostic value of immunohistochemical profile and response to high-dose methotrexate therapy in primary CNS lymphoma.

Several biomarkers have been identified as prognostic factors in primary central nervous system lymphoma (PCNSL). However, the correlation between the histogenetic origin of PCNSL and the response to therapy is still unclear. To elucidate the utility of immunophenotypic markers in predicting clinical outcomes, we investigated 27 immunocompetent patients with PCNSL treated with high-dose methotrexate therapy. Of the 27 patients, 25 received whole-brain radiotherapy after high-dose methotrexate. Immunostaining for CD5, CD10, BCL-6, and MUM-1 was used to determine the immunophenotypic profile of diffuse large B-cell lymphoma of PCNSL. We then evaluated whether immunophenotypic markers were associated with the response to therapy or patients' survival. The response to induction high-dose methotrexate therapy was determined by magnetic resonance imaging after three courses of i.v. high-dose methotrexate. We categorized B-cell lymphomas into three known subtypes: germinal center B-cell (GCB), activated-GCB, and post-GCB subtypes according to immunohistochemical profile. All the BCL-6-positive samples were co-positive for MUM-1 and therefore classified into activated-GCB subtype. BCL-6 expression in this study was associated with poor progression-free survival (P = 0.038). No immunophenotypic markers or subtypes had a significant effect on the response to high-dose methotrexate therapy. However, the response itself was a significant predictor for both progression-free survival (P < 0.001) and overall survival (P = 0.003). Further investigation is needed to assess BCL-6 as a potential prognostic factor in PCNSL.

Vertebral and spinal cavernous angiomas associated with familial cerebral cavernous malformation.

BACKGROUND: Cerebral cavernous malformations are vascular malformations that affect the CNS and have been associated with cutaneous, retinal, and hepatic lesions. Until now, vertebral hemangiomas associated with CCM have been described only in one case. The coexistence of intracranial and spinal cavernous angiomas in familial CCM is extremely rare. In addition to previous studies, the occurrence of spinal, vertebral, and cutaneous cavernous angiomas is now described in different members of a large family with CCM. CASE DESCRIPTION: Our study reports a previously described family (IFCAS-07) with 12 members affected by autosomal dominant cavernous angiomas: 11 had CCM either alone or associated with hepatic or retinal angiomas, and one had only hepatic angioma. In all 11 members affected by CCM, the mutation of CCM1 gene was detected. During the follow-up, 8 subjects underwent a spinal MRI: 2 because they were symptomatic (thoracic paresthesias, enuresis, back pain) and 6 as a screening examination. Spinal MRI showed in 5 subjects spinal cavernous angiomas either alone or associated with vertebral hemangiomas. CONCLUSIONS: To our knowledge, this is the largest family reported with different subjects affected by CCM associated with multiple cavernous angiomas throughout (brain and spinal cord) and besides (retina, skin, liver, and vertebral column) the CNS. Comprehensive care of patients with familial CCM includes screening of all the tissues that can be affected and appropriate management by specialists. We emphasize the importance of spinal MRI in the diagnosis of spinal and vertebral cavernous angiomas in all patients affected by familial CCM.

Cilengitide: an integrin-targeting arginine-glycine-aspartic acid peptide with promising activity for glioblastoma multiforme.

BACKGROUND: Glioblastoma multiforme (GBM), a highly invasive and vascular cancer, responds poorly to conventional cytotoxic therapy. Integrins, widely expressed in GBM and tumor vasculature, mediate cell survival, migration and angiogenesis. Cilengitide is a potent alphavbeta3 and alphavbeta5 integrin inhibitor. OBJECTIVE: To summarize the preclinical and clinical experience with cilengitide for GBM. METHODS: Preclinical studies and clinical trials evaluating cilengitide for GBM were reviewed. RESULTS/CONCLUSIONS: Cilengitide is active and synergizes with external beam radiotherapy in preclinical GBM models. In clinical trials for recurrent GBM, single-agent cilengitide has antitumor benefits and minimal toxicity. Among newly diagnosed GBM patients, single-arm studies incorporating cilengitide into standard external beam radiotherapy/temozolomide have shown encouraging activity with no increased toxicity and have led to a planned randomized Phase III trial.

A pilot study of addition of amifostine to melphalan, carboplatin, etoposide, and cyclophosphamide with autologous hematopoietic stem cell transplantation in pediatric solid tumors-A pediatric blood and marrow transplant consortium study.

Limited information is available regarding the use of amifostine in pediatric hematopoietic stem cell transplant (HSCT) patients. Melphalan, carboplatin, etoposide +/- cyclophosphamide is a commonly used preparatory regimen in pediatric solid tumor HSCT. Therefore, we decided to determine the feasibility of the addition of amifostine (750 mg/m b.i.d. x 4 d) to melphalan (200 mg/m), carboplatin (1200 mg/m), and etoposide (800 mg/m) (level 1) and escalating doses of cyclophosphamide (3000 mg/m and 3800 mg/m, levels 2 and 3, respectively) followed by autologous HSCT. Thirty-two patients with a variety of pediatric solid tumors were studied. Seventeen patients were accrued at level 1, 9 at level 2, and 6 at level 3. Major toxicities during the administration of the preparatory regimen were hypocalcemia, emesis, and hypotension. Hypocalcemia required aggressive calcium supplementation during the conditioning phase. No dose limiting toxicities were encountered at level 3. Amifostine at 750 mg/m b.i.d. for 4 days can be administered with a double alkylator regimen consisting of melphalan (200 mg/m), cyclophosphamide (up to 3800 mg/m), carboplatin (1200 mg/m), and etoposide (800 mg/m) with manageable toxicities.

Brain tumor-like lesion in Behçet disease.

We report a patient with longstanding Behçet disease who presented sudden onset of headache and facial paresis. The magnetic resonance imaging (MRI) showed a mass in the right thalamus, extending to the lentiform nucleus, subthalamic area, right cerebral peduncle and deep subcortical white matter. Stereotactic brain biopsy disclosed gliosis with no signs of malignancy. The diagnosis of a pseudotumoral form of neuro-Behçet disease was done and she was treated with pulse methylpredinisolone and intravenous cyclophosphamide. After 8 weeks she had improved and a new MRI showed disappearance of the tumor-like lesion. The differential diagnosis, especially with central nervous system tumor is emphasized.

El eje hipotálamo-hipófisis en el niño: consideraciones de imagen / The hypothalamus-hypophysis axis in the child: imaging considerations

Este trabajo aborda una revisión general con resonancia magnética (RM) de los aspectos anatómicos del eje hipotálamo-hipofisario en el grupo pediátrico y de sus alteraciones estructurales. El mayor rendimiento diagnóstico se ha producido en el hipopituitarismo congénito, que frecuentemente se expresa en el niño por un déficit de hormona del crecimiento, al mejorar la delimitación del complejo malformativo de la línea media. La ausencia de visualización total o parcial del tallo hipofisario y la presencia de una neurohipófisis ectópica han cambiado el concepto clásico del enanismo hipofisario idiopático, y han acotado un grupo de pacientes con esta alteración estructural que muestran una mayor resistencia al tratamiento con hormona y una frecuente aparición evolutiva de otras deficiencias adenohipofisarias. La demostración de una hipoplasia aislada de la adenohipófisis puede orientar el diagnóstico de un hipopituitarismo de origen genético. Los tumores de la región supraselar y de la región posterior del tercer ventrículo, craneofaringiomas, gliomas y germinomas, son la causa más frecuente de disfunción endocrina adquirida, aunque ésta suele aparecer como resultado de las actuaciones terapéuticas sobre aquellos. Los adenomas hipofisarios son tumores poco frecuentes en el niño y predominan los microadenomas en esta edad. La histiocitosis de células de Langerhans se muestra como un aumento de grosor y captación del tallo hipofisario y su diagnóstico se apoya en las manifestaciones asociadas en otros órganos; en ausencia de éstas, el diagnóstico diferencial más importante de estos hallazgos es el germinoma. El estudio radiológico del eje hipotálamo-hipófisis debe incluir imágenes con contraste y extenderse al resto del sistema nervioso central en función de la información clínica aportada

This paper provides a general review with MRI of anatomical aspects of the hypothalamus-hypophysis axis in the pediatric group and of its structural alterations. The greatest diagnostic yield has been found in congenital hypopituitarism, frequently expressed in the child by growth hormone deficit, through an improvement of the delimitation of the malformative complex of the middle line. Absence of total or partial visualization of the pituitary stem and the presence of ectopic neurohypophysis have changed the classical concept of idiopathic pituitary dwarfism, delimiting a group of patients with this structural alteration that show greater resistance to hormone treatment and frequent evolutive appearance of other adenopituitary deficiencies. Demonstration of an isolated hypoplasia of the adenopituitary may orient the diagnosis of a hypopituitarism of genetic origin. The tumors of the suprasellar regional and the posterior region of the III ventricle, craniopharyngioma, gliomas and germinomas are the most frequent cause of acquired endocrine dysfunction, although this generally appears as a result of the therapeutic actions on them. Hypopituitary adenomas are rare tumors in the child and microadenomas predominate at this age. Langerhan cell histiocytosis is shown as an increase in thickness and uptake of the pituitary stem and its diagnosis is supported by the associated manifestations in other organs. In absence of these, the most important differential diagnosis of these findings is the germinoma. The radiology study of the hypothalamus-hypophysis axis should include images with contrast and extend to the rest of the central nervous system based on the clinical information supplied

Proton magnetic resonance spectroscopy in immunocompetent patients with primary central nervous system lymphoma.

BACKGROUND: Magnetic resonance spectroscopy imaging (MRSI) non-invasively evaluates the metabolic profile of normal and abnormal brain tissue. Primary central nervous system lymphoma (PCNSL) is a highly aggressive tumor responsive to high-dose methotrexate based regimens. Patients often have complete responses but relapses are common. We characterized the MR spectra of PCNSL patients, correlated MRSI with MRI and evaluated whether early recurrence could be detected by MRSI. METHODS: Patients with PCNSL had multi-voxel MRSI before, during, and after treatment. The region of interest was defined using axial FLAIR images. Metabolites assessed were N-acetyl-aspartate (NAA), choline (Cho), creatine (Cr), lipid, and lactate. Ratios of Cho/Cr, NAA/Cho, and NAA/Cr were calculated and correlated with MRI. Overall survival (OS), progression free survival (PFS), and relative risks of each of the ratios were determined. RESULTS: MRSI was performed on 11 men and seven women; median age of 59. Sixty-seven MRSI studies were performed, 17 baseline and 48 follow-up studies. Median ratios in 16 pretreated patients were Cho/Cr-1.90, NAA/Cho-0.39, and NAA/Cr-1.27. Two patients had lipid at baseline, five had lactate and two had both. MRSI correlated with tumor response or progression on MRI; in three patients MRSI suggested disease progression prior to changes on MRI. Univariate analysis of metabolite ratios, lipid, and lactate revealed that none significantly affected PFS or OS. Kaplan-Meier analysis of the presence or absence of lipid, lactate or both revealed a trend for increased PFS. CONCLUSION: MRSI and MRI correlate with tumor response or progression and may allow early detection of disease recurrence. The presence or absence of lipid and/or lactate may have prognostic significance. Further research using MRSI needs to be done to validate our findings and determine the role of MRSI in PCNSL.

[Diagnostic imaging of cancer]. / A képalkotó vizsgálómódszerek alkalmazása daganatok esetében. "Javaslat a képalkotók alkalmazásához".

Rapid development of imaging techniques provided much more precise methodology of diagnosis, staging and dynamics of cancer. Nowadays the onco-radiodiagnostic units are able to select the optimal imaging technique based on established international protocols. These protocols provide the basis of cancer diagnosis, therapy control and clinical research. The onco-radiodiagnostic unit is essential part of the oncoteam managing the cancer patients. Follow-up protocols are now equally important compared to those of the diagnosis and staging, requiring a continuous interaction between radiologists and physicians. The comprehensive cancer centers with all the necessary imaging techniques are the optimal organizations where professional and economic priorities both can be considered for the benefit of cancer patients.

Utilidad de las altas dosis de quimioterapia en tumores del SNC en niños / Usefulness of high-dose chemotherapy in children with CNS tumors

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Tumores del sistema nervioso central en la infancia: nuestra experiencia / Central nervous system tumours in childhood: our experience

1) Pensamos que nuestra experiencia reafirma una vez más la importancia de difundir a todo nivel la existencia de esta patología. 2) Consideramos fundamental que la asistencia de los niños con tumores sea encarada precozmente y por un equipo que agrupe a diversos especialistas, con medios, experiencia y planes preestablecidos, superando todo individualismo. 3) Incluímos en esta casuística muchos tumores histológicamente benignos, pero que por su localización y/o evolución pueden comportarse como malignos. 4) Con respecto a los tumores del SNC, la presentación en uno y otro sexo no mostró diferencias significativas. 5) La edad de los pacientes, revela un franco predominio en la segunda infancia. 6) Como es habitual en estos casos, la localización de los tumores mostró un ligero predominio de los infratentoriales sobre los supratentoriales. 7) Con respecto al diagnóstico histológico, se observó una mayor frecuencia de astroblastomas, craneofaringiomas y meduloblastomas. 8) Dado que este tipo de lesiones suele tener localizaciones inabordables, es importante contar con planes de estudio adecuados, dentro de los cuales, en la actualidad la tomografía computada ocupa un destacadísimo lugar. 9) Considerando el importante papel que juega la competencia inmune en esta patología, consideramos que todo centro oncológico debe contar con un laboratorio de inmunología que permita el estudio y seguimiento adecuado de estos enfermos. 10) La oncología pediátrica debe ser una realidad y el manejo, planes, personal, terapeutas, equipos y medios deben ser pediátricos y no diseñados para adultos y luego adaptados a los niños, hecho que significaría negar a la pediatría como entidad... (TRUNCADO)(AU)