Brain Tumors in Adolescents and Young Adults: A Review.

Brain tumors account for the majority of cancer-related deaths in adolescents and young adults (AYAs), defined as individuals aged 15 to 39. AYAs constitute a distinct population in which both pediatric- and adult-type central nervous system (CNS) tumors can be observed. Clinical manifestations vary depending on tumor location and often include headaches, seizures, focal neurological deficits, and signs of increased intracranial pressure. With the publication of the updated World Health Organization CNS tumor classification in 2021, diagnoses have been redefined to emphasize key molecular alterations. Gliomas represent the majority of malignant brain tumors in this age group. Glioneuronal and neuronal tumors are associated with longstanding refractory epilepsy. The classification of ependymomas and medulloblastomas has been refined, enabling better identification of low-risk tumors that could benefit from treatment de-escalation strategies. Owing to their midline location, germ cell tumors often present with oculomotor and visual alterations as well as endocrinopathies. The management of CNS tumors in AYA is often extrapolated from pediatric and adult guidelines, and generally consists of a combination of surgical resection, radiation therapy, and systemic therapy. Ongoing research is investigating multiple agents targeting molecular alterations, including isocitrate dehydrogenase inhibitors, SHH pathway inhibitors, and BRAF inhibitors. AYA patients with CNS tumors should be managed by multidisciplinary teams and counselled regarding fertility preservation, psychosocial comorbidities, and risks of long-term comorbidities. There is a need for further efforts to design clinical trials targeting CNS tumors in the AYA population.

Is superolateral Hoffa fat pad edema a consequence of impingement between lateral femoral condyle and patellar ligament?

PURPOSE: To evaluate whether knee extensor mechanism features are associated with superolateral Hoffa fat pad edema at magnetic resonance imaging. MATERIALS AND METHODS: Institutional review board approval and written consent from all patients were obtained. Patients with superolateral Hoffa fat pad edema (n = 30) and a control group without edema of the fat pad (n = 60) were evaluated prospectively with magnetic resonance (MR) imaging. Demographic data and extensor mechanism features were compared, including trochlear depth, lateral trochlear inclination, patellar tilt angle, patellar height ratio, distance between patellar ligament and lateral trochlear facet, distance from the tibial tubercle to the trochlear groove, patellar facet asymmetry, and patellar ligament abnormalities. RESULTS: The following variables were associated with superolateral Hoffa fat pad edema in the multivariable models: patellar height ratio (P = .023), shortest distance between patellar ligament and lateral trochlear facet (P < .001), and distance from the tibial tubercle to the trochlear groove (P = .046). Of all demographic and degenerative variables, only age was significantly associated, with younger patients more likely to have superolateral Hoffa fat pad edema (P < .009). CONCLUSION: A high-riding patella, a short distance between the patellar ligament and the lateral trochlear facet, and an increased distance from the tibial tubercle to the trochlear groove are associated with superolateral Hoffa fat pad edema at MR imaging. These results are suggestive of impingement between the lateral femoral condyle and the posterior aspect of the patellar ligament in these patients.

[An unusual adrenal tumor: Ewing tumor]. / Une tumeur surrénalienne inhabituelle : tumeur d'Ewing.

We report the case of a voluminous tumor of the adrenal diagnosed in a young pregnant woman at 26(th) week of amenorrhea. Morphologically, a soft white tumor with haemorragic areas was observed, made of sheets of monomorphous, medium sized, spindle-shaped to polygonal, with high mitotic activity. Tumorous cells expressed cytokeratins AE1/AE3, EMA, and CD99 (expression of vimentin is not relevant). Contemplated diagnoses included poorly differentiated synovialosarcoma, sarcomatoid carcinoma and Ewing tumor. Thanks to molecular biology, showing the specific transcript of Ewing/peripheral primitive neuroectodermal tumor (pPNET) EWS/FLI1, the diagnosis of this atypical tumor in an unusual location was performed. Indeed, 75% of Ewing tumors involve bones (especially, the diaphysis of long bones) and 20 to 25% soft tissues. Primitive visceral involvement is rare; less than 10 cases of adrenal involvement have been reported. The hypothesis that Ewing cell's origin is a mesenchymal stem cell, which may derive from neural crest cell, could explain the uncommon adrenal involvement. Diagnosis of Ewing tumor is based on pathologic and molecular findings, especially in atypical cases.