Medulloblastoma

• Article • • 19 March 2021 The transcriptional landscape of Shh medulloblastoma • • • • • • ORCID: orcid.org/0000-0002-0253-8512 • ORCID: orcid.org/0000-0001-7984-3147 • ORCID: orcid.org/0000-0003-0222-4182 • • • ORCID: orcid.org/0000-0002-3602-2290 • • • ORCID: orcid.org/0000-0003-3136-9241 • • ORCID: orcid.org/0000-0003-4064-8066 • • • • • ORCID: orcid.org/0000-0002-1008-192X • • • • ORCID: orcid.org/0000-0002-8632-430X • • • • • ORCID: orcid.org/0000-0001-5718-6465 • ORCID: orcid.org/0000-0001-5752-9763 • • • • • • ORCID: orcid.org/0000-0002-8730-2276 • • ORCID: orcid.org/0000-0002-7942-6674 • • • • • • • • • • ORCID: orcid.org/0000-0002-0668-9529 • • ORCID: orcid.org/0000-0003-2485-3692 • • • • • ORCID: orcid.org/0000-0003-2757-6782 • • • • • ORCID: orcid.org/0000-0002-3789-8120 • • • ORCID: orcid.org/0000-0002-7395-1937 • ORCID: orcid.org/0000-0002-4053-0052 • ORCID: orcid.org/0000-0003-4298-2746 • • • • • • • • • • • • • • ORCID: orcid.org/0000-0002-8731-1121 • • • • • • ORCID: orcid.org/0000-0002-6557-895X • • ORCID: orcid.org/0000-0003-3394-2208 • ORCID: orcid.org/0000-0002-0905-2742 • • • • ORCID: orcid.org/0000-0003-0185-8861 • ORCID: orcid.org/0000-0002-2299-2309 • ORCID: orcid.org/0000-0002-8515-0934 • • ORCID: orcid.org/0000-0001-7146-7175 • ORCID: orcid.org/0000-0002-8858-0294 • … • ORCID: orcid.org/0000-0001-7009-3466 Show authors Nature Communications volume 12, Article number: 1749 ( 2021) Sonic hedgehog medulloblastoma encompasses a clinically and molecu...

• m ə ˌ d ʌ l oʊ b l æ ˈ s t oʊ m ə/ Headaches Nausea Vomiting Tiredness Clumsiness Dizziness Change in vision Handwriting problems Usual onset Between 5 and 9 years old Frequency About 500 children diagnosed annually in the United States Medulloblastoma is a common type of primary The brain is divided into two main parts, the larger Historically, medulloblastomas have been classified as a Medulloblastomas are invasive, rapidly growing tumors that, unlike most brain tumors, spread through the The cumulative relative survival rate for all age groups and histology follow-up was 60%, 52%, and 47% at 5 years, 10 years, and 20 years, respectively, with children doing better than adults. Signs and symptoms [ ] Signs and symptoms are mainly due to secondary increased Extraneural metastasis to the rest of the body is rare, and when it occurs, it is in the setting of relapse, more commonly in the era prior to routine chemotherapy. Pathogenesis [ ] Medulloblastomas are usually found in the vicinity of the fourth ventricle, between the Although medulloblastomas are thought to originate from immature or embryonal cells at their earliest stage of development, the cell of origin depends on the subgroup of medulloblastoma. WNT tumors originate from the lower rhombic lip of the brainstem, while SHH tumors originate from the external granular layer. Currently, medulloblastomas are thought to arise from cerebellar stem cells that have been prevented from dividing and differentiating into th...

Purpose of Review Molecular subtyping in medulloblastoma (MB) has diagnostic and prognostic values which impact therapy. This paper provides guidance for the clinician caring for pediatric and adult patients with medulloblastoma in the modern era. Recent Findings Medulloblastoma comprises four molecularly distinct subgroups: wingless activated (WNT), sonic hedgehog activated (SHH), group 3, and group 4. Risk stratification before and after the discovery of molecular subgroups aims at minimizing toxicity by reducing radiation and chemotherapy doses in low-risk patients while maintaining favorable overall survival (OS). The mainstay of newly diagnosed medulloblastoma treatment is surgery, radiation therapy, and chemotherapy, except for children under 6 years of age, where high-dose chemotherapy with autologous stem cell rescue is used to avoid or delay radiotherapy, preventing neurocognitive sequelae. Management of recurrent/refractory medulloblastoma remains a challenge with immunotherapy and small-molecule inhibitors forming the backbone of novel strategies. Summary Recent innovations in medulloblastoma research allow us to better understand pathogenesis and molecular characteristics resulting in advanced risk stratification models, new therapeutic approaches, and overall improved survival and quality of life. Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance • Kunscher LJ. Harvey Cushing and medulloblastoma...