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Pineal/germ cell tumors and pineal parenchymal tumors

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Abstract

Introduction

Pineal region tumors (PRTs) are tumors arising from the pineal gland and the paraspinal structures. These tumors are rare and heterogeneous that account for 2.8–10.1% and 0.6–3.2% of tumors in children and in all ages, respectively. Almost all types and subtypes of CNS tumors may be diagnosed in this region. These tumors come from cells of the pineal gland (pinealocytes and neuroglial cells), ectopic primordial germ cells (PGC), and cells from adjacent structures. Hence, PRTs are consisted of pineal parenchyma tumors (PPTs), germ cell tumors (GCTs), neuroepithelial tumors (NETs), other miscellaneous types of tumors, cystic tumors (epidermoid, dermoid), and pineal cyst in addition. The symptoms of PRTs correlate to the increased intracranial cranial pressure due to obstructive hydrocephalus and dorsal midbrain compression. The diagnostic imaging studies are mainly MRI of brain (with and without gadolinium) along with a sagittal view of whole spine. Serum and/or CSF AFP/β-HCG helps to identify GCTs. The treatment of PRTs is consisted of the selection of surgical biopsy/resection, handling of hydrocephalus, neoadjuvant and/or adjuvant therapy according to age, tumor location, histopathological/molecular classification, grading of tumors, staging, and threshold value of markers (for GCTs) in addition.

Methods

In this article, we review the following focus points: 1. Background of pineal region tumors. 2. Pineal GCTs and evolution of management. 3. Molecular study for GCTs and pineal parenchymal tumors. 4. Review of surgical approaches to the pineal region. 5. Contribution of endoscopy. 6. Adjuvant therapy (chemotherapy, radiotherapy, and combination). 7. Future direction.

Results

In all ages, the leading three types of PRTs in western countries were PPTs (22.7–34.8%), GCTs (27.3–34.4%), and NETs (17.2–28%). In children and young adults, the leading PRTs were invariably in the order of GCTs (40–80.5%), PPTs (7.6–21.6%), NETs (2.4–37.5%). Surgical biopsy/resection of PRTs is important for precision diagnosis and therapy. Safe resection with acceptable low mortality and morbidity was achieved after 1970s because of the advancement of surgical approaches, CSF shunt and valve system, microscopic and endoscopic surgery. Following histopathological diagnosis and classification of types and subtypes of PRTs, in PPTs, through molecular profiling, four molecular groups of pineoblastoma (PB) and their oncogenic driver were identified. Hence, molecular stratified precision therapy can be achieved.

Conclusion

Modern endoscopic and microsurgical approaches help to achieve precise histopathological diagnosis and molecular classification of different types and subtypes of pineal region tumors for risk-stratified optimal, effective, and protective therapy. In the future, molecular analysis of biospecimen (CSF and blood) along with AI radiomics on tumor imaging integrating clinical and bioinformation may help for personalized and risk-stratified management of patients with pineal region tumors.

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Acknowledgements

The authors would like to thank Ku-Sheng Wu and Yu-Han Jung for editing the manuscript and the manuscript.

Funding

The clinical and basic research on pediatric brain tumors in TMUH is support by the funding from National Science and Technology Council (Taiwan) grant (MOST 111-2314-B-038-145-; MOST 111–2314-B-038 -131 -MY3); the Featured Areas Research Center Program within the framework of the Higher Education Sprout Project by the Ministry of Education (Taiwan) (MOE) (DP2-111-21121-01-C-04-01); and the Ministry of Health and Welfare (Taiwan) grant (MOHW112-TDU-B-222-124017).

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Authors and Affiliations

  1. Division of Neurosurgery, National Center for Child Health and Development, Okura 2-10-1, Setagaya-ku, 157-8535, Tokyo, Japan

    Hideki Ogiwara

  2. Division of Hematology and Oncology, Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung 807, Taiwan

    Yu-Mei Liao

  3. Division of Pediatric Neurosurgery, Department of Neurosurgery, Taipei Medical University Hospital, 252 Wuxing St, Taipei, 11031, Taiwan

    Tai-Tong Wong

  4. Institute of Clinical Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan

    Tai-Tong Wong

  5. Neuroscience Research Center, Taipei Medical University Hospital, Taipei, Taiwan

    Tai-Tong Wong

  6. Pediatric Brain Tumor Program, Taipei Cancer Center, Taipei Medical University, Taipei, Taiwan

    Tai-Tong Wong

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  1. Hideki Ogiwara
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Conceptualization: T.-T.W. Writing—original draft preparation: T.-T.W., H.O, Y.-M.L. Writing—review and editing: T.-T.W., H.O., Y.-M.L. All authors have read and agreed to the published version of the manuscript.

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Correspondence to Tai-Tong Wong.

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Ogiwara, H., Liao, YM. & Wong, TT. Pineal/germ cell tumors and pineal parenchymal tumors. Childs Nerv Syst 39, 2649–2665 (2023). https://doi.org/10.1007/s00381-023-06081-1

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