Pineal parenchymal tumors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

The various types of pineal parenchymal tumors include:[1]

 
 
 
 
 
 
 
 
 
 
 
 
 
Pineal parenchymal tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pineocytoma
 
Pineal parenchymal tumor with intermediate differentiation
 
Papillary tumor of the pineal region
 
Pineoblastoma
 
 
 
 


Pineal parenchymal tumors arise from pineocytes or their precursors, and they are distinct from other pineal gland neoplasms such as astrocytic and germ cell tumors.

Pineocytoma (WHO grade I) is a slow-growing pineal parenchymal neoplasm that primarily occurs in young adults. Pineocytomas account for fewer than 1% of all brain tumors and comprise approximately 45% of all pineal parenchymal tumors. Adults aged 25 to 35 years are most frequently affected. No specific cytogenetic abnormalities or molecular genetics exist with this tumor. The 5-year survival rate has been reported to be as high as 86%.

Pineoblastoma (WHO grade IV) is a highly malignant primitive embryonal tumor of the pineal gland that manifests primarily in children. Pineoblastomas are rare brain tumors that comprise approximately 45% of all pineal parenchymal tumors. No specific cytogenetic abnormalities or molecular genetics exist with this tumor. Tumors similar to pineoblastomas in appearance have been observed in patients with familial (bilateral) retinoblastoma. Projected 1-, 3-, and 5-year survival rates of pineoblastoma patients treated by various modalities are 88%, 78%, and 58%, respectively.

Pineal parenchymal tumors with intermediate differentiation (WHO grade II/III) are monomorphous tumors exhibiting moderately high cellularity, mild nuclear atypia, occasional mitosis, and the absence of large pineocytomatous rosettes. They comprise approximately 10% of all pineal parenchymal tumors and occur in all age groups. No specific cytogenetic abnormalities or molecular genetics exist with this tumor. Clinical behavior is variable.

Papillary tumor of the pineal region was introduced in the World Health Organization (WHO) classification of central nervous system (CNS) in 2007.[2] It is 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.[3]

Diagnosis

Pineoblastoma

References

  1. Pineal parenchymal tumors. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-parenchymal-tumours. Accessed on November 18, 2015
  2. Papillary tumors of the pineal region. Wikipedia 2015. https://en.wikipedia.org/wiki/Papillary_tumors_of_the_pineal_region. Accessed on November 24, 2015
  3. Papillary tumour of the pineal region (PTPR). Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopedia 2015. http://radiopaedia.org/articles/papillary-tumour-of-the-pineal-region. Accessed on November 24, 2015

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