Papillary tumor of the pineal region

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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]

Synonyms and Keywords: Papillary tumors of the pineal region; Papillary tumour of the pineal region; Papillary tumours of the pineal region; PTPR; Pineal parenchymal tumor; Pineal gland tumor; Brain tumor

Overview

Papillary tumor of the pineal region is located on the pineal gland which is located in the center of the brain and was introduced in the World Health Organization (WHO) classification of central nervous system (CNS) in 2007.[1] 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.[2]

Historical Perspective

Papillary tumor of the pineal region was first described in 2003 by A. Jouvet et al.[1]

Pathophysiology

Pathogenesis

  • Papillary tumor of the pineal region is made up of ependymal cells which form the papilla. The papilla is meant to be the 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 the blood vessels, nerve cells, and muscle cells.[3]
  • These tumors have no known predisposing genetic characteristics.
  • 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 (DNA). As the ependymal 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 eventually becomes a tumor.

Gross Pathology

On gross pathology, papillary tumor of the pineal region is characterized by a mass in the pineal region that has the following features:[4][5]

  • Solid, well-circumscribed
  • May be quite large (2-4cm)
  • Sometimes may be cystic
  • Mimics pineocytoma macroscopically

Microscopic Pathology

On microscopic histopathological analysis, papillary tumor of the pineal region is characterized by:[6]

  • Papillary growth pattern
  • Dense areas exhibiting ependymal features
  • Clear, vacuolated cytoplasm
  • Rosettes

According to the 2007 World Health Organization (WHO) classification of tumors of the nervous system, papillary tumor of the pineal region is classified as a WHO grade II/III tumor.[7]

WHO Grade Description

Grade II

Grade II tumors are relatively slow growing. They have a slightly abnormal microscopic appearance and can spread into nearby normal tissue. Grade II tumors can also recur as a higher-grade tumor.

Grade III

Grade III tumors are malignant. In these types of tumors, the cells are actively reproducing abnormal cells. The cells of the tumor will often grow into nearby normal brain tissue. Grade III tumors tend to recur as higher-grade tumors.

Gallery

Immunohistochemistry

Papillary tumor of the pineal region is demonstrated by positivity to tumor markers such as:[5][9][10]

Gallery

Differentiating Papillary Tumor of the Pineal Region from other Diseases

Papillary tumor of the pineal region must be differentiated from:[12]

Epidemiology and Demographics

Prevalence

  • Papillary tumor of the pineal region is extremely rare, constituting approximately 0.4 - 1% of all the central nervous system tumors.[13]

Age

  • Patients of all age groups may develop papillary tumor of the pineal gland, ranging from 5-66 years of age.[14]
  • More commonly, papillary tumor of the pineal gland tends to affect the adult population.[13]
  • The mean age at diagnosis is 31.5 years.[13]

Natural History, Complications and Prognosis

Natural History

  • If left untreated, patients with papillary tumor of the pineal region may progress to develop seizures, obstructive hydrocephalus, local recurrence, and leptomeningeal seeding.[15][16]

Complications

Common complications of papillary tumor of the pineal region include:[16]

History and Symptoms

History

  • When evaluating a patient for papillary tumor of the pineal region, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review.

Symptoms

  • The clinical presentation of papillary tumor of the pineal region is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct.[15]
  • Symptoms of papillary tumor of the pineal region include:

Physical Examination

Compression of the superior colliculi can lead to a characteristic gaze palsy, known as Parinaud syndrome. Common physical examination findings of papillary tumor of the pineal region include:[15]

HEENT

  • Bulging soft spots (fontanelles)
  • Eyes that are constantly looking down (sunsetting sign)
  • Deficiency in upward-gaze
  • Pupillary light-near dissociation (pupils respond to near stimuli but not light)
  • Convergence-retraction nystagmus

Neurological

Treatment

  • The predominant therapy for papillary tumor of the pineal region is surgical resection. Adjunctive chemotherapy and radiation may be required.[17]
  • Patients with papillary tumor of the pineal region will develop hydrocephalus in majority of the cases and they will require CSF diversion. Ventriculo-peritoneal (V-P) shunt placement is a viable option with low morbidity and mortality rate. However, shunt malfunction in this population is as high as 20%. In addition, tumor metastasis through a CSF shunt has been reported. Endoscopic third ventriculostomy (ETVC) is an alternative option, which also permits a biopsy of the tumor in the same procedure. Ahn et al. reported that the biopsy samples, obtained in the lateral ventricle or pineal region, were more favorable towards a successful diagnosis than those in the thalamus or tectal region. Neuroendoscopic biopsy procedures have been proven safe with low complication rates.[18]

References

  1. 1.0 1.1 Papillary tumors of the pineal region. Wikipedia 2015. https://en.wikipedia.org/wiki/Papillary_tumors_of_the_pineal_region. Accessed on November 24, 2015
  2. 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
  3. Characteristics of papillary tumor of the pineal region. Wikipedia 2015. https://en.wikipedia.org/wiki/Papillary_tumors_of_the_pineal_region. Accessed on November 24, 2015
  4. Gross features of papillary tumour of pineal region (PTPR). Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Papillary_tumour_of_pineal_region_.28PTPR.29. Accessed on November 24, 2015
  5. 5.0 5.1 Li, Z (2014), Clinicopathological characteristics of papillary tumor of the pineal region, Chinese Journal of Contemporary Neurology & Neurosurgery, retrieved 11/25/2015  Check date values in: |access-date= (help)
  6. 6.0 6.1 Microscopic features of papillary tumour of pineal region (PTPR). Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Papillary_tumour_of_pineal_region_.28PTPR.29. Accessed on November 24, 2015
  7. Grading of papillary tumor of the pineal region. Wikipedia 2015. https://en.wikipedia.org/wiki/Papillary_tumors_of_the_pineal_region. Accessed on November 24, 2015
  8. Rosa Junior M, da Rocha AJ, Zanon da Silva A, Rosemberg S (2015). "Papillary Tumor of the Pineal Region: MR Signal Intensity Correlated to Histopathology.". Case Rep Neurol Med. 2015: 315095. PMC 4320939Freely accessible. PMID 25688307. doi:10.1155/2015/315095. 
  9. IHC features of papillary tumor of the pineal region. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Papillary_tumour_of_pineal_region_.28PTPR.29. Accessed on November 24, 2015
  10. Histological and immunohistochemical features of papillary tumor of the pineal region. Wikipedia 2015. https://en.wikipedia.org/wiki/Papillary_tumors_of_the_pineal_region. Accessed on November 24, 2015
  11. 11.0 11.1 IHC images of papillary tumor of the pineal region. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Papillary_tumour_of_pineal_region_.28PTPR.29. Accessed on November 24, 2015
  12. Differential diagnosis of pineal region mass. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-region-mass. Accessed on November 24, 2015
  13. 13.0 13.1 13.2 Epidemiology of papillary tumor of the pineal region. Wikipedia 2015. https://en.wikipedia.org/wiki/Papillary_tumors_of_the_pineal_region. Accessed on November 24, 2015
  14. Epidemiology of papillary tumor of the pineal gland. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/papillary-tumour-of-the-pineal-region. Accessed on November 24, 2015
  15. 15.0 15.1 15.2 Clinical presentation of papillary tumor of the pineal region. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/papillary-tumour-of-the-pineal-region. Accessed on November 24, 2015
  16. 16.0 16.1 Vandergriff C, Opatowsky M, O'Rourke B, Layton K (2012). "Papillary tumor of the pineal region.". Proc (Bayl Univ Med Cent). 25 (1): 78–9. PMC 3246862Freely accessible. PMID 22275792. 
  17. Papillary tumour of the pineal region. Operative Neurology 2015. http://operativeneurosurgery.com/doku.php?id=papillary_tumour_of_the_pineal_region. Accessed on November 25, 2015
  18. Alexiou, George A (2012). "Management of pineal region tumours in children". Journal of Solid Tumors. 2 (2). ISSN 1925-4075. doi:10.5430/jst.v2n2p15. 

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