Background Considering that pediatric high-grade gliomas (HGGs) are biologically distinct from their adult counterparts, the objective of this study was to define the landscape of HGGs in adolescents and young adults (AYAs). Methods We performed a multicentric retrospective study of 112 AYAs from adult and pediatric Ile-de-France neurosurgical units, treated between 1998 and 2013 to analyze their clinicoradiological and histomolecular profiles. The inclusion criteria were age between 15 and 25 years, histopathological HGG diagnosis, available clinical data, and preoperative and follow-up MRI. MRI and tumoral samples were centrally reviewed. Immunohistochemistry and complementary molecular techniques such as targeted/next-generation sequencing, whole exome sequencing, and DNA-methylation analyses were performed to achieve an integrated diagnosis according to the 2016 World Health Organization (WHO) classification. Results Based on 80 documented AYA patients, HGGs constitute heterogeneous clinicopathological and molecular groups, with a predominant representation of pediatric subtypes (histone H3-mutants, 40%) but also adult subtypes (isocitrate dehydrogenase [IDH] mutants, 28%) characterized by the rarity of oligodendrogliomas, IDH mutants, and 1p/19q codeletion and the relative high frequency of “rare adult IDH mutations” (20%). H3G34-mutants (14%) represent the most specific subgroup in AYAs. In the H3K27-mutant subgroup, non-brainstem diffuse midline gliomas are more frequent (66.7%) than diffuse intrinsic pontine gliomas (23.8%), contrary to what is observed in children. We found that WHO grade has no prognostic value, but molecular subgrouping has major prognostic importance. Conclusions HGGs in AYAs could benefit from a specific classification, driven by molecular subtyping rather than age group. Collaborative efforts are needed from pediatric and adult neuro-oncology teams to improve the management of HGGs in AYAs. Key Points 1. Pediatric subtypes (H3G34 and H3K27 mutants) were the most common HGGs in AYAs. 2. Contrary to what is observed in children, non-brainstem diffuse midline gliomas are more frequent than diffuse intrinsic pontine gliomas. 3. WHO grade has no prognostic value contrary to molecular subgrouping.
This clinical report describes 8 cases of branchial remnant cysts (BRC) in the horse. The horses presented with bimodal age distribution, with 5 cases in mature horses (age 8-21 years) and 3 in foals (age 1, 6 and 10 months). Mature cases presented for dysphagia or intermittent oesophageal obstruction (2/5), and retropharyngeal swelling (3/5), whereas respiratory stridor and visible mass were presenting complaints in the foals. Presence of a right-sided (5/8) or dorsally located (2/8) palpable retropharyngeal mass of 3-35 cm diameter was noted clinically; one left-sided mass was identified as an incidental finding at necropsy. Ultrasonography typically revealed a thick-walled cyst containing hypoechoic fluid with dependent hyperechoic masses consistent with blood clots. Radiographs and upper airway endoscopy were also consistent with a retropharyngeal mass. Fluid cytology revealed chronic haemorrhage in 6/8 cases, and squamous epithelial cells in one case. Histopathology in all cases demonstrated an epithelium-lined cyst with no smooth muscle or thyroid tissue. Two cases was subjected to euthanasia; one due to concurrent laryngeal anomalies and one due to financial constraints. The remaining 5 cases were treated via surgical excision. Post operatively, right laryngeal hemiplegia was observed in 4/5 cases. All previous reports of BRC in the horse have described juvenile individuals. Brachial remnant cyst should be considered a differential diagnosis for mature horses with masses of the throatlatch area and can be definitively diagnosed by the presence of squamous epithelium in aspirated fluid or by histopathology of the excised mass. Right recurrent laryngeal nerve damage is a common complication of surgery.
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