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Papillary tumors of the pineal region
Papillary tumors of the pineal region (PTPR) were first described by A. Jouvet et al. in 2003 〔Jouvet A et al.,(2003), Papillary tumor of the pineal region. American Journal of Surgical Pathology, 27(4), 505-512.〕 and were introduced in the World Health Organization (WHO) classification of Central Nervous System (CNS) in 2007.〔Jouvet A, Nakazato Y, Scheithauer BW, Paulus W,(2007). Papillary tumor of the pineal region. WHO classification of tumours of the central nervous system, chapter 7, Eds: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, IARC, Lyon, France 128-129.〕 Papillary Tumors of the Pineal Region are located on the pineal gland which is located in the center of the brain. The pineal gland is located on roof of the diencephalon. It is a cone shaped structure dorsal to the midbrain tectum.〔Jorg-Christian Tonn, M. W., J.T. Rutka. (2010). Oncology of CNS Tumors (2 ed.): Springer-Verlag Berlin Heidelberg.〕 The tumor appears to be derived from the specialized ependymal cells of the subcommissural organ.〔〔Kevin Buffenoir, P. R., Michel Wager, Sarah Ferrand, Alix Coulon, Jean Luc Blanc, Benoit Bataille, Antoine Listrat. (2008). Papillary Tumor of the Pineal Region in a Child: Case Report and Review of the Literature. Childs Nervous System, 24, 379-384.〕〔M. Kern, P. R., G. Lee, P. Watson. (2006). Papillary tumor of the pineal region- a new pathological entity. Clinical Neuropathology, 25(4), 185-192.〕 Papillary tumors of the central nervous system and particularly of the pineal region are very rare and so diagnosing them is extremely difficult.〔Chang AH, F. G., Debnam JM, Karis JP, Coons SW, Ross JS, Dean BL. (Jan. 2008). MR imaging of papillary tumor of the pineal region. Division of Neuroradiology, 29(1), 187-189.〕
== Characteristics ==
Pineal region tumors are normally composed of a variety of cells including astrocytes, ganglion cells, blood vessels, and pinealocytes, which are the cells of this organ. Pinealocytes are specialized neurons, which are rich in monoaminergic neurotransmitters, including, serotonin, norepinephrine, and melatonin.〔 Specifically, papillary tumors of this region are made up of ependymal cells which form papilla. The papilla is meant to be surface cells. The ependymal cells line the inside of the ventricles of the brain. These cells have proteins that make up the characteristics of the tumor. These proteins arise from blood vessels, nerve cells and muscle cells.〔Janss, Anna. Personal Interview. 24 Oct. 2011.〕
These tumors, papillary tumors in this case, have no known predisposing genetic characteristics, as stated by Dr. Janss when I interviewed her. She said that there is nothing that links the likelihood of getting this tumor type to genetics, meaning nothing is inherited. However, technically speaking, there is genetics involved, just not in the sense of inheritability. The tumor cells may differ in their structure and function, but they all have normal function, which is directed by the deoxyribonucleic acid, or DNA. There are meant to be certain cells in a specific area, for the pineal region these are ependymal cells, and the cells divide into millions. As these cells divide, their genetic material is being copied. In a tumor, there is a collection of mutations that are typos in the genetic material, which disrupts the function of the normal cell. Therefore, the cells differentiate from what they were meant to be. If the abnormal cells continue to grow, divide, and produce more abnormal cells, the mass of abnormal cells may eventually become a tumor.〔〔Virginia Stark-Vance, M. L. D. (2011). 100 Questions and Answers About Brain Tumors (2 ed.): Jones and Bartlett Publishers.〕
Papillary tumors of the pineal region are normally well circumscribed. They are reported to be fairly large ranging between 2.5 to 4.0 centimeters. These tumors sometimes feature a cystic component.〔Federico Roncaroli, B. S. (2007). Papillary Tumor of the Pineal Region and Spindle Cell Oncocytoma of the Pituitary: New Tumor Entities in the 2007 WHO Classification. Brain Pathology, 17, 314-318.〕
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