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Epidemiology, diagnosis, and optimal management of glioma in adolescents and young adults.

ABSTRACT: Neoplasms of the central nervous system (CNS) are the most frequently encountered solid tumors of childhood, but are less common in adolescents and young adults (AYA), aged 15-39 years. Gliomas account for 29%-35% of the CNS tumors in AYA, with approximately two-thirds being low-grade glioma (LGG) and the remaining being high-grade glioma (HGG). We review the epidemiology, work-up, and management of LGG and HGG, focusing on the particular issues faced by the AYA population relative to pediatric and adult populations. Visual pathway glioma and brainstem glioma, which represent unique clinical entities, are only briefly discussed. As a general management approach for both LGG and HGG, maximal safe resection should be attempted. AYA with LGG who undergo gross total resection (GTR) may be safely observed. As age increases and the risk factors for recurrence accumulate, adjuvant therapy should be more strongly considered with a strong consideration of advanced radiation techniques such as proton beam therapy to reduce long-term radiation-related toxicity. Recent results also suggest survival advantage for adult patients with the use of adjuvant chemotherapy when radiation is indicated. Whenever possible, AYA patients with HGG should be enrolled in a clinical trial for the benefit of centralized genetic and molecular prognostic review and best clinical care. Chemoradiation should be offered to all World Health Organization grade IV patients with concurrent and adjuvant chemotherapy after maximal safe resection. Younger adolescents with GTR of grade III lesions may consider radiotherapy alone or sequential radiotherapy and chemotherapy if unable to tolerate concurrent treatment. A more comprehensive classification of gliomas integrating pathology and molecular data is emerging, and this integrative strategy offers the potential to be more accurate and reproducible in guiding diagnostic, prognostic, and management decisions.

SUBMITTER: Diwanji TP

PROVIDER: S-EPMC5624597 | biostudies-other | 2017

REPOSITORIES: biostudies-other

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Epidemiology, diagnosis, and optimal management of glioma in adolescents and young adults.

Diwanji Tejan P TP Engelman Alexander A Snider James W JW Mohindra Pranshu P

Adolescent health, medicine and therapeutics 20170922

Neoplasms of the central nervous system (CNS) are the most frequently encountered solid tumors of childhood, but are less common in adolescents and young adults (AYA), aged 15-39 years. Gliomas account for 29%-35% of the CNS tumors in AYA, with approximately two-thirds being low-grade glioma (LGG) and the remaining being high-grade glioma (HGG). We review the epidemiology, work-up, and management of LGG and HGG, focusing on the particular issues faced by the AYA population relative to pediatric ...[more]

PMID: 28989289

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Project description:Lymphangioleiomyomatosis (LAM) is an uncommon disease presented as diffuse thin-walled cystic changes in the lung. The main differential diagnoses include pulmonary Langerhans' histiocytosis (PLCH), Birt-Hogg-Dubé syndrome (BHD), lymphoid interstitial pneumonia (LIP), and amyloidosis. A combination of clinical, radiological, and pathological approaches as well as genetic testing will clarify the diagnosis in most cases. LAM is a disease almost exclusively in women. Dyspnea, pneumothorax, and hemoptysis are common presentations in LAM patients. LAM is also a lymphatic disorder affecting lymphatic vessels and lymph nodes. Chylothorax, chylous ascites, and lymphangiomyomas are frequently seen. LAM can present sporadically as a single entity or as part of tuberous sclerosis complex (TSC). Angiomyolipoma (AML) is a characteristic extra-pulmonary lesion, either found in association with sporadic or TSC-related LAM. High-risk populations should be screened for LAM, including adult women with TSC and female patients with spontaneous pneumothorax, AMLs in the kidney, and diffuse cystic lung diseases. Definitive diagnosis of LAM is based on a high level of clinical suspicion on presentation supported by pathological findings or by a distinct feature, such as a history of TSC, AMLs in the kidney, chylothorax, or chylous ascites. Vascular endothelial growth factor-D (VEGF-D) in serum is a noninvasive and reliable diagnostic biomarker. In experienced centers, trans-bronchial lung biopsy (TBLB) provides a convenient and safe way to obtain lung specimens for diagnostic purposes. An effective treatment for LAM is now available, namely using a mechanistic target of rapamycin (mTOR) inhibitor such as sirolimus. Efficacy of sirolimus has been confirmed in clinical trials. Research in other molecular-targeted therapies is under investigation. A previously little-known rare disease with no cure is now better understood with regards to its pathogenesis, diagnosis, and management. In this review, current knowledge in diagnosis and differential diagnosis of LAM will be discussed, followed by the discussion of therapy with mTOR inhibitors.

Project description:Importance:Individuals with psychotic disorders have increased mortality, and recent research suggests a marked increase shortly after diagnosis. Objective:To use population-based data to examine overall and cause-specific mortality after first diagnosis of a psychotic disorder. Design, Setting, and Participants:This cohort study used records from 5 integrated health systems that serve more than 8 million members in 5 states. Members aged 16 through 30 years who received a first lifetime diagnosis of a psychotic disorder from September 30, 2009, through September 30, 2015, and 2 comparison groups matched for age, sex, health system, and year of diagnosis were selected from all members making an outpatient visit (general outpatient group) and from all receiving a first diagnosis of unipolar depression (unipolar depression group). Exposures:First recorded diagnosis of schizophrenia, schizoaffective disorder, mood disorder with psychotic symptoms, or other psychotic disorder in any outpatient, emergency department, or inpatient setting. Main Outcomes and Measures:Death within 3 years after the index diagnosis or visit date, ascertained from health system electronic health records, insurance claims, and state mortality records. Results:A total of 11 713 members with first diagnosis of a psychotic disorder (6976 [59.6%] men and 4737 [40.4%] women; 2368 [20.2%] aged 16-17 and 9345 [79.8%] aged 18-30 years) were matched to 35 576 outpatient service users and 23 415 members with a first diagnosis of unipolar depression. During the year after the first diagnosis, all-cause mortality was 54.6 (95% CI, 41.3-68.0) per 10 000 in the psychotic disorder group compared with 20.5 (95% CI, 14.7-26.3) per 10 000 in the unipolar depression group and 6.7 (95% CI, 4.0-9.4) per 10 000 in the general outpatient group. After adjustment for race, ethnicity, and preexisting chronic medical conditions, the relative hazard of death in the psychotic disorder group compared with the general outpatient group was 34.93 (95% CI, 8.19-149.10) for self-inflicted injury or poisoning and 4.67 (95% CI, 2.01-10.86) for other type of injury or poisoning. Risk of death due to heart disease or diabetes did not differ significantly between the psychotic disorder and the general outpatient groups (hazard ratio, 0.78; 95% CI, 0.15-3.96). Between the first and third years after diagnosis, all-cause mortality in the psychotic disorder group decreased from 54.6 to 27.1 per 10 000 persons and injury and poisoning mortality decreased from 30.6 to 15.1 per 10 000 persons. Both rates, however, remained 3 times as high as in the general outpatient group (9.0 per 10 000 for all causes; 4.8 per 10 000 for injury or poisoning). Conclusions and Relevance:Increases in early mortality underscore the importance of systematic intervention for young persons experiencing the first onset of psychosis. Clinicians should attend to the elevated suicide risk after the first diagnosis a psychotic disorder.

Dataset Information

Epidemiology, diagnosis, and optimal management of glioma in adolescents and young adults.

Publications

Epidemiology, diagnosis, and optimal management of glioma in adolescents and young adults.

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