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; 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, well-circumscribed mass with or without hemorrhage.[2]

Microscopic Pathology

On microscopic histopathological analysis, pineocytoma is characterized by:[3]

  • 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.[4]

Immunohistochemistry

Pineocytoma is demonstrated by positivity to tumor markers such as:[5][6]

Gallery

Differentiating Pineocytoma from other Diseases

Pineocytoma must be differentiated from:[9]

  • Pineal parenchymal tumor with intermediate differentiation
  • Papillary tumor of the pineal region
  • Pineoblastoma
  • Pineal germinoma
  • Pineal embryonal carcinoma
  • Pineal choriocarcinoma
  • Pineal yolk sac carcinoma (endodermal sinus tumor)
  • Pineal teratoma
  • Pineal cyst
  • Astrocytoma of the pineal gland
  • Meningioma near pineal gland
  • Pineal metastasis
  • Cavernoma in pineal region
  • Aneurysm in pineal region

Epidemiology

Age

  • Pineocytoma is a rare disease that tends to affect all the age groups, most commonly in the second decade of life.[10]

Gender

  • Pineocytoma affects men and women equally.[10]

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.[11][12]

Complications

Common complications of pineocytoma include:[12]

  • Local recurrence
  • CSF metastasis

Prognosis

Prognosis is generally excellent, and the 5-year survival rate of patients with pineocytoma is approximately 86%.[12]

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 also lead to a characteristic gaze palsy, known as Parinaud syndrome.[11]
  • Common physical examination findings of pineocytoma include:
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.[13]

Gallery

MRI

  • Brain MRI may be diagnostic of pineocytoma.
  • Features on MRI suggestive of pineocytoma include:[13]
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.[12]

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. Microscopic features of pineocytoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
  4. General feature of pineocytoma. Libre pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
  5. Microscopic description of pineocytoma causing hydrocephalus. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineocytoma-causing-hydrocephalus. Accessed on November 20, 2015
  6. Histology of pineocytoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineocytoma-with-astrocytic-differentiation-1. Accessed on November 20, 2015
  7. 7.0 7.1 7.2 Microscopic images of pineocytoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
  8. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  9. 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
  10. 10.0 10.1 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
  11. 11.0 11.1 11.2 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
  12. 12.0 12.1 12.2 12.3 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
  13. 13.0 13.1 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
  14. 14.0 14.1 Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  15. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  16. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  17. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  18. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  19. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC


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