Papillary tumor of the pineal region
Citation, DOI & article data
Papillary tumors of the pineal region are seen in a wide range of ages, reported from 5 to 66 years, while the mean age is in the fourth decade (30-40 years of age) 3,5. They show no significant sex predilection, perhaps marginally more commonly encountered in females 3,5.
As with all other pineal region masses, the clinical presentation is mainly from obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct, being headache of short duration is the most common presentation 5. Compression of the superior colliculi can also lead to a characteristic gaze palsy, known as Parinaud syndrome.
Papillary tumors of the pineal region are thought to arise from specialized ependymocytes of the subcommissural organ located in the lining of the posterior commissure rather than from the pineal gland itself 1-3. It is also hypothesized that some, or perhaps many, previously published descriptions of unusual pineal region tumors (e.g. choroid plexus papillomas and papillary ependymomas) actually represent papillary tumors of the pineal region, as histologically they are difficult to distinguish, requiring immunohistochemistry 1.
These tumors are considered WHO grade 2 or 3 tumors, although precise grading criteria are not yet available 3.
Papillary tumors are well-circumscribed lesions and their size may range from 2.5 to 4 cm. They may also have a cystic component 5.
Papillary tumors of the pineal region demonstrate variable morphology ranging from solid to predominantly papillary, reminiscent of ependymomas, including the presence of ependymal rosettes 3. Areas of necrosis are sometimes identified 3,4.
Papillary tumors of the pineal region have a fairly characteristic immunohistochemical profile that allows them to be distinguished from other pineal parenchymal tumors.
- cytokeratins (AE1/3, CAM5.2, KL1, CK18): positive
- S100: positive
- vimentin: positive
- tansthyretin: positive
- neuron-specific enolase: positive
- MAP2: positive
- GFAP: variable
Papillary tumors of the pineal region are typically well-defined masses with variable T1 signal, high T2 signal, and heterogeneous postcontrast enhancement and are without pathognomic features 6-8. Cystic areas within the mass are commonly seen 6. T1 high signal has been described and is considered to be related to secretory inclusions containing glycoprotein or protein 1 but is considered a non-specific finding 7.
Treatment and prognosis
Screening of the entire neural axis is required as CSF dissemination has been reported in up to 7% of cases 4,6,7. These masses are typically treated with surgical resection and postoperative radiotherapy 7. 5-year survival rates are 73% 3,6.
History and etymology
In 2003, Jouvet et al reported six cases and named this entity as papillary tumor of pineal region 5. This tumor was first described in the WHO 2007 classification of brain tumors and was named as “A rare neuroepithelial tumor of the pineal region in adults, characterized by papillary architecture and epithelial cytology, immunopositivity for cytokeratin and ultrastructural features suggesting ependymal differentiation" 5.
All pineal region lesions which are known to have papillary architecture are included in the differential diagnosis of PTPR. These include pineal parenchymal tumors, papillary ependymoma, choroid plexus ependymoma/carcinoma, papillary meningioma, and metastases 5.
- 1. Chang A, Fuller G, Debnam J et al. MR Imaging of Papillary Tumor of the Pineal Region. AJNR Am J Neuroradiol. 2008;29(1):187-9. doi:10.3174/ajnr.A0784 - Pubmed
- 2. Jouvet Anne, François Fauchon, Pawel Liberski et al. “Papillary Tumor of the Pineal Region.” The American Journal of Surgical Pathology 27, no. 4 (2003). [Link].
- 3. Hasselblatt M, Huang A, Vasiljevic A, Jones DTW, Orr BA, Snuderl M, Papillary tumour of the pineal region. In: WHO Classification of Tumours Editorial Board. Central nervous system tumours. Lyon (France): International Agency for Research on Cancer; 2021. (WHO classification of tumours series, 5th ed.; vol. 6). https://publications.iarc.fr/601
- 4. Dumrongpisutikul N, Intrapiromkul J, Yousem D. Distinguishing Between Germinomas and Pineal Cell Tumors on MR Imaging. AJNR Am J Neuroradiol. 2012;33(3):550-5. doi:10.3174/ajnr.A2806 - Pubmed
- 5. Patil. Papillary Tumor of Pineal Region: A Rare Entity. Asian J Neurosurg. 2016;11(4):453. doi:10.4103/1793-5482.145373 - Pubmed
- 6. Smith A, Rushing E, Smirniotopoulos J. From the Archives of the AFIP: Lesions of the Pineal Region: Radiologic-Pathologic Correlation. Radiographics. 2010;30(7):2001-20. doi:10.1148/rg.307105131 - Pubmed
- 7. Vandergriff C, Opatowsky M, O'Rourke B, Layton K. Papillary Tumor of the Pineal Region. Proc (Bayl Univ Med Cent). 2012;25(1):78-9. doi:10.1080/08998280.2012.11928791 - Pubmed
- 8. Kim Y, Kim J, Park C et al. Papillary Tumor of Pineal Region Presenting with Leptomeningeal Seeding. Neuropathology. 2010;30(6):654-60. doi:10.1111/j.1440-1789.2010.01108.x - Pubmed