{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T21:42:00Z","timestamp":1793050920758,"version":"4.5.0"},"reference-count":104,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2022,7,27]],"date-time":"2022-07-27T00:00:00Z","timestamp":1658880000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Pineal region tumors are rare intracranial tumors, accounting for less than 1% of all adult intracranial tumor lesions. These lesions represent a histologically heterogeneous group of tumors. Among these tumors, pineal parenchymal tumors and germ cell tumors (GCT) represent the most frequent types of lesions. According to the new WHO 2021 classification, pineal parenchymal tumors include five distinct histotypes: pineocytoma (PC), pineal parenchymal tumors of intermediate differentiation (PPTID), papillary tumor of the pineal region (PTPR), pinealoblastoma (PB), and desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant; GCTs include germinoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma, teratoma, mixed GCTs. Neuroradiological assessment has a pivotal role in the diagnostic work-up, surgical planning, and follow-up of patients with pineal masses. Surgery can represent the mainstay of treatment, ranging from biopsy to gross total resection, yet pineal region tumors associated with obstructive hydrocephalus may be surgically managed via ventricular internal shunt or endoscopic third ventriculostomy. Radiotherapy remains an essential component of the multidisciplinary treatment approach for most pineal region tumors; however, treatment volumes depend on the histological subtypes, grading, extent of disease, and the combination with chemotherapy. For localized germinoma, the current standard of care is chemotherapy followed by reduced-dose whole ventricular irradiation plus a boost to the primary tumor. For pinealoblastoma patients, postoperative radiation has been associated with higher overall survival. For the other pineal tumors, the role of radiotherapy remains poorly studied and it is usually reserved for aggressive (grade 3) or recurrent tumors. The use of systemic treatments mainly depends on histology and prognostic factors such as residual disease and metastases. For pinealoblastoma patients, chemotherapy protocols are based on various alkylating or platinum-based agents, vincristine, etoposide, cyclophosphamide and are used in association with radiotherapy. About GCTs, their chemosensitivity is well known and is based on cisplatin or carboplatin and may include etoposide, cyclophosphamide, or ifosfamide prior to irradiation. Similar regimens containing platinum derivatives are also used for non-germinomatous GCTs with very encouraging results. However, due to a greater understanding of the biology of the disease\u2019s various molecular subtypes, new agents based on targeted therapy are expected in the future. On behalf of the EURACAN domain 10 group, we reviewed the most important and recent developments in histopathological characteristics, neuro-radiological assessments, and treatments for pineal region tumors.<\/jats:p>","DOI":"10.3390\/cancers14153646","type":"journal-article","created":{"date-parts":[[2022,7,27]],"date-time":"2022-07-27T21:11:05Z","timestamp":1658956265000},"page":"3646","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":57,"title":["Diagnosis and Treatment of Pineal Region Tumors in Adults: A EURACAN Overview"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1316-2132","authenticated-orcid":false,"given":"Giuseppe","family":"Lombardi","sequence":"first","affiliation":[{"name":"Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5662-8978","authenticated-orcid":false,"given":"Pietro Luigi","family":"Poliani","sequence":"additional","affiliation":[{"name":"Pathology Unit, Department of Molecular and Translational Medicine, University of Brescia, P.le Spedali Civili 1, 25121 Brescia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5130-3971","authenticated-orcid":false,"given":"Renzo","family":"Manara","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit, University of Padua, Via VIII Febbraio 2, 35122 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moncef","family":"Berhouma","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Lyon University Hospital, 69007 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1239-1603","authenticated-orcid":false,"given":"Giuseppe","family":"Minniti","sequence":"additional","affiliation":[{"name":"Radiation Oncology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, Via Banchi di Sotto 55, 53100 Siena, Italy"},{"name":"IRCCS NEUROMED, 86077 Pozzilli (IS), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emeline","family":"Tabouret","sequence":"additional","affiliation":[{"name":"Neuro-Oncologye Department, Aix-Marseille University, La Timone Hospital, AP-HM, Team 8 GlioME, CNRS 7051, Inst. Neurophysiopathol, 13005 Marseille, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evangelia","family":"Razis","sequence":"additional","affiliation":[{"name":"Third Department of Medical Oncology, Hygeia Hospital, 15123 Athens, Greece"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Cerretti","sequence":"additional","affiliation":[{"name":"Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vittorina","family":"Zagonel","sequence":"additional","affiliation":[{"name":"Department of Oncology, Oncology 1, Veneto Institute of Oncology IOV-IRCCS, Via Gattamelata 64, 35128 Padua, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Weller","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital and University of Zurich, 8006 Zurich, Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5290-1204","authenticated-orcid":false,"given":"Ahmed","family":"Idbaih","sequence":"additional","affiliation":[{"name":"Sorbonne Universit\u00e9, Inserm, CNRS, UMR S 1127, Institut du Cerveau, ICM, AP-HP, H\u00f4pitaux Universitaires La Piti\u00e9 Salp\u00eatri\u00e8re\u2014Charles Foix, Service de Neurologie 2-Mazarin, 75013 Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,7,27]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1097\/NEN.0000000000000093","article-title":"PTEN Mutations and Activation of the PI3K\/Akt\/MTOR Signaling Pathway in Papillary Tumors of the Pineal Region","volume":"73","author":"Goschzik","year":"2014","journal-title":"J. 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