Pineocytoma
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
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Intermediate magnification micrograph of a pineocytoma. HPS stain.[7]
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High magnification micrograph of a pineocytoma. HPS stain.[7]
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Very high magnification micrograph of a pineocytoma. HPS stain.[7]
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The tumor cells are diffusely positive for synaptophysin and neurofilament.[8]
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
- The clinical presentation of pineocytoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct.[11]
- Symptoms of pineocytoma include:
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
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Different patterns of pineal calcification: Exploded calcification of tumors of pineal cell origin and engulfed calcification by germinomas.[14]
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Single image following biopsy and third ventriculostomy demonstrates a pineal region soft tissue mass. A small amount of blood is seen in the occipital horn on the right. Ventricles remain dilated.[14]
MRI
- Brain MRI may be diagnostic of pineocytoma.
- Features on MRI suggestive of pineocytoma include:[13]
MRI component | Findings |
---|---|
T1 |
|
T2 |
|
T1 with gadolinium contrast |
|
Treatment
The mainstay of therapy for pineocytoma is surgery.[12]
References
- ↑ Pineocytoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pineocytoma. Accessed on November 18, 2015
- ↑ 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
- ↑ Microscopic features of pineocytoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
- ↑ General feature of pineocytoma. Libre pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineocytoma. Accessed on November 18, 2015
- ↑ Microscopic description of pineocytoma causing hydrocephalus. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineocytoma-causing-hydrocephalus. Accessed on November 20, 2015
- ↑ Histology of pineocytoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineocytoma-with-astrocytic-differentiation-1. Accessed on November 20, 2015
- ↑ 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
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ 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
- ↑ 10.0 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
- ↑ 11.0 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
- ↑ 12.0 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
- ↑ 13.0 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
- ↑ 14.0 14.1 Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC