Pineocytoma

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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: Pineocytomas; Pinealocytoma; Pinealocytomas; PC; Pineal gland tumor; Brain tumor

Overview

Pineocytoma is a benign, slowly growing pineal parenchymal tumor.[1]

Pathophysiology

Gross Pathology

On gross pathology, pineocytoma is characterized by solid, sometimes with focal areas of cystic change, gray, well-circumscribed mass with or without hemorrhage.[2][3]

Microscopic Pathology

On microscopic histopathological analysis, pineocytoma is characterized by:[4]

  • Cytologically benign cells (uniform size of nuclei, regular nuclear membrane, light chromatin)
  • Pineocytomatous/neurocytic rosette, which is an irregular circular/flower-like arrangement of cells with a large meshwork of fibers (neuropil) at the centre

According to the WHO classification of tumors of the central nervous system, pineocytoma is classified into a WHO grade I tumor.[5]

Immunohistochemistry

Pineocytoma is demonstrated by positivity to tumor markers such as:[6][7][8][9]

Gallery

Differentiating Pineocytoma from other Diseases

Pineocytoma must be differentiated from:[13]

Epidemiology

Prevalence

  • Pineocytoma constitutes about 45% of the pineal parenchymal tumors.[9]
  • Pineocytoma constitutes about 0.4 - 1% of the intracranial neoplasms.[14]

Age

  • Pineocytoma is a rare disease that tends to affect all the age groups, most commonly in the second decade of life.[15]

Gender

  • Pineocytoma affects men and women equally.[15]

Natural History, Complication and Prognosis

Natural History

If left untreated, patients with pineocytoma may progress to develop seizures, obstructive hydrocephalus, local recurrence, and CSF metastasis.[16][17]

Complications

Common complications of pineocytoma include:[17][9]

Prognosis

  • Prognosis is generally excellent, and the 5-year survival rate of patients with pineocytoma is approximately 86%.[17]
  • Pineocytoma has the most favorable prognosis among all the pineal gland tumors.[18]

History and Symptoms

History

When evaluating a patient for pineocytoma, 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

Physical Examination

Compression of the superior colliculi can lead to a characteristic gaze palsy, known as Parinaud syndrome. Common physical examination findings of pineocytoma include:[16]

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

CT

  • Head CT scan may be diagnostic of pineocytoma.
  • Findings on CT scan suggestive of pineocytoma include a mass of intermediate density similar to the adjacent brain with peripheral calcifications.[19]

Gallery

MRI

  • Brain MRI may be diagnostic of pineocytoma.
  • Features on MRI suggestive of pineocytoma include:[19]
MRI component Findings

T1

  • Isointense to brain parenchyma

T2

  • Solid components are isointense to brain parenchyma
  • Areas of cystic change
  • Sometimes the majority of the tumor is cystic

T1 with gadolinium contrast

  • Solid components vividly enhance

Gallery

Treatment

The mainstay of therapy for pineocytoma is surgery (gross total or subtotal resection).[17][18]

References

  1. Pineocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pineocytoma. Accessed on November 18, 2015
  2. Pathology and radiographic features of pineocytoma. Dr Bruno Di Muzio and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineocytoma. Accessed on November 18, 2015
  3. Gross description of pineocytoma. Pathology Outlines 2015. http://pathologyoutlines.com/topic/cnstumorpineocytoma.html. Accessed on November 20, 2015
  4. Microscopic features of pineocytoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
  5. General feature of pineocytoma. Libre pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
  6. Microscopic description of pineocytoma causing hydrocephalus. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineocytoma-causing-hydrocephalus. Accessed on November 20, 2015
  7. Histology of pineocytoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineocytoma-with-astrocytic-differentiation-1. Accessed on November 20, 2015
  8. IHC features of pineocytoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 20, 2015
  9. 9.0 9.1 9.2 Hirato, Junko; Nakazato, Yoichi (2001). Journal of Neuro-Oncology. 54 (3): 239–249. doi:10.1023/A:1012721723387. ISSN 0167-594X. Missing or empty |title= (help)
  10. 10.0 10.1 10.2 Microscopic images of pineocytoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
  11. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  12. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  13. 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 20, 2015
  14. Clark, Aaron J.; Sughrue, Michael E.; Aranda, Derick; Parsa, Andrew T. (2011). "Contemporary Management of Pineocytoma". Neurosurgery Clinics of North America. 22 (3): 403–407. doi:10.1016/j.nec.2011.05.004. ISSN 1042-3680.
  15. 15.0 15.1 Epidemiology of pineocytoma. Dr Bruno Di Muzio and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineocytoma. Accessed on November 20, 2015
  16. 16.0 16.1 16.2 Clinical presentation of pineocytoma. Dr Bruno Di Muzio and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineocytoma. Accessed on November 20, 2015
  17. 17.0 17.1 17.2 17.3 Treatment and prognosis of pineocytoma. Dr Bruno Di Muzio and Dr Frank Gaillard et al. Radiopaedia 2015.http://radiopaedia.org/articles/pineocytoma. Accessed on November 20, 2015
  18. 18.0 18.1 Deshmukh, Vivek R.; Smith, Kris A.; Rekate, Harold L.; Coons, Stephen; Spetzler, Robert F. (2004). "Diagnosis and Management of Pineocytomas". Neurosurgery. 55 (2): 349–357. doi:10.1227/01.NEU.0000129479.70696.D2. ISSN 0148-396X.
  19. 19.0 19.1 Radiographic features of pineocytoma. Dr Bruno Di Muzio and Dr Frank Gaillard et al. Radiopeadia 2015. http://radiopaedia.org/articles/pineocytoma. Accessed on November 20, 2015
  20. 20.0 20.1 Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  21. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  22. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  23. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  24. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  25. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC


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