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

Age

  • Pineocytoma is a rare disease that tends to affect all 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]
  • Clark et al. after performing a systematic review of the literature reported that the 1- and 5-year progression free survival (PFS) rates for patients that underwent resection versus the biopsy group were 97% and 90%, and 89% and 75% respectively. The 1- and 5-year PFS rates for the gross total resection group versus the group undergoing subtotal resection combined with radiation therapy were 100% and 94%, and 100% and 84% respectively.[19]

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.[20]

Gallery

MRI

  • Brain MRI may be diagnostic of pineocytoma.
  • Features on MRI suggestive of pineocytoma include:[20]
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][19]
  • The main goal of open surgery on pineocytoma is the complete tumor removal with minimal morbidity, whenever possible. However, even if gross total excision cannot be achieved, establishment of an accurate diagnosis, maximal cytoreduction, and restoration of the CSF pathway may be achieved.
  • Radiotherapy administration to subtotally resected tumor is not associated with an increase in either tumor control or survival.[19]
  • Stereotactically guided iodine-125 seed implantation has been proposed as a potential alternative to microsurgery in de novo diagnosed pineocytomas, since it was proven efficient and safe.
  • Patients with pineocytoma 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.[19]

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. ISSN 0167-594X. doi:10.1023/A:1012721723387. 
  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. ISSN 1042-3680. doi:10.1016/j.nec.2011.05.004. 
  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. ISSN 0148-396X. doi:10.1227/01.NEU.0000129479.70696.D2. 
  19. 19.0 19.1 19.2 19.3 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. 
  20. 20.0 20.1 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
  21. 21.0 21.1 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
  26. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC

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