Pineal gland tumor: Difference between revisions

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* [[Brain metastasis]]
* [[Brain metastasis]]
For more information [[Astrocytoma differential diagnosis|click here]].
For more information [[Astrocytoma differential diagnosis|click here]].
==Differentiating features among different various Pineal Gland Tumors==
{| class="wikitable"
|+
! rowspan="2" |Tumors
! rowspan="2" |Grade
! rowspan="2" |Pathalogic Features
! rowspan="2" |5-year survival
! rowspan="2" |Cerebrospinal fluid (CSF) dissemination
! colspan="2" |Imaging
|-
!CT
!MRI
|-
|Pineocytoma
|
* Grade I lesion
|
* Small, uniform, mature cells that resemble pineocytes
* Lobular architecture and pineocytomatous rosettes
|
* 86%–100%
|
* Rare
|
* Well demarcated, usually less than 3 cm, and iso- to hyperattenuating.
|
* Hypo- to isointense on T1-weighted images and hyperintense on T2-weighted images.
|-
|Pineal Parenchymal Tumor of Intermediate Differentiation
|
* Grade II or III
|
* Gross inspection, PPTID is similar in appearance to pineocytoma.
* It is a well-circumscribed lesion without evidence of necrosis.
* Histologic evaluation reveals diffuse sheets of uniform cells and the formation of small rosettes, with features intermediate between those of pineocytoma and those of pineoblastoma.
* Low to moderate levels of mitotic activity and nuclear atypia are seen.
|
* 39%–74%
|
* Rare
|
* No specific imaging findings
|
* No specific imaging findings
* Demonstrate high signal intensity on T2-weighted images and enhance on postcontrast images
* Cystic areas may also be seen
|-
|Pineoblastoma
|
* Grade IV
|
* Pineo-blastomas are highly cellular embryonal neoplasms that resemble other primitive neuroectodermal neoplasms of the central nervous system.
* Cells have scant cytoplasm and are arranged in diffuse sheets.
* Homer-Wright rosettes (neuroblastic differentiation) or Flexner-Wintersteiner rosettes (retinoblastic differentiation) may be seen, and hemorrhage or necrosis may be present.
* Infiltration into adjacent structures and craniospinal dissemination commonly occur
|
* 58%
|
* Most common cause of the death
|
* Lobulated, typically hyperattenuating mass, an appearance that reflects its highly cellular histologic features.
|
* Ill-defined enhancing pineal mass with resultant hydrocephalus
|-
|Papillary Tumor of the Pineal Region
|
* Grade II or III
|
* PTPRs are well-circumscribed lesions that can measure up to 5 cm and may have a cystic component.
* At histologic evaluation, they demonstrate an epithelial-like growth pattern, papillary features, rosettes, and perivascular pseudorosettes.
* The immunohistochemical findings help differentiate PTPR from other lesions in the pineal region, especially ependymoma and choroid plexus papilloma.
|
* 73%
|
* 75% times
|
* No specific imaging findings
|
* Well-circumscribed lesions with variable signal intensity on T1-weighted images, high signal intensity on T2-weighted images, and enhancement on postcontrast images.
* Cystic areas are commonly present.
* Hyperintensity on T1-weighted images has been described, which is hypothesized to be related to secretory inclusions containing protein or glycoprotein.
|-
|Germinoma
|
* Grade II
|
* Account for 1%–2% of all cranial neoplasms
* 65% of intracranial germinomas occur in the pineal region
|
* >79%
|
* The possibility of CSF seeding necessitates imaging evaluation of the entire neuroaxis
|
* Sharply circumscribed, hyperattenuating mass that engulfs the pineal calcifications
|
* Demonstrates a solid mass that may have cystic components.
* Germinomas are iso- to hyperintense to gray matter on T1- and T2-weighted images 
* Avid, homogeneous enhancement on postcontrast images
|-
|Teratoma
|
* Grade III
|
* Mature teratoma reveals a lobulated neoplasm with a complex mixture of adult-type tissues from all three embryonic germ layers.
* Skin and skin appendages may be seen due to the ectodermal component.
* The mesoderm contributes to the presence of cartilage, bone, fat, and smooth and skeletal muscle.
* Respiratory or enteric epithelium arises from the endodermal component.
* Immature teratomas contain incompletely differentiated tissue elements that resemble fetal tissue.
|
|
|
* Multiloculated, lobulated lesion with foci of fat attenuation, calcification, and cystic regions
|
* T1-weighted MR images may show foci of T1 shortening due to fat and variable signal intensity related to calcification.
* On T2-weighted images, the soft-tissue component is iso- to hypointense.
|-
|Pineal Cyst
|
* Grade II
|
* Pineal cysts occur in all age ranges but are most predominant in adults 40–49 years of age; studies demonstrate a female predominance.
* Their origin is debated, with some suggesting they result from degenerative changes in the gland.
* These lesions are typically asymptomatic and are usually 2–15 mm in size.
* Follow-up studies have indicated that these lesions remain stable in size over time.
* When they exceed 15 mm, patients may become symptomatic, typically with headache or visual changes.
* Intracystic hemorrhage (“pineal apoplexy”) and acute hydrocephalus rarely occur; resultant death has been reported
|
* 75% of pineal cysts remained stable during a period of 0.5–9.1 years.
|
* Rare
|
* No specific imaging findings
|
* Round or oval, thin-walled, and well-circumscribed.
* They typically demonstrate signal intensity similar to that of CSF on T1- and T2-weighted images
|-
|Epidermoid and Dermoid Cysts
|
* Grade II III
|
* The wall of epidermoid cysts is composed of simple stratified squamous epithelium, and the cyst contents consist of layers of keratinaceous debris, which impart a “pearly” appearance to the gross specimen.
* Dermoid cysts contain dermal appendages (hair follicles, sweat glands.
* Both lesions slowly expand over time and rupture can result in chemical meningitis, which may be fatal.
|
* N/A
|
* N/A
|
* Epidermoid cysts have low attenuation, similar to that of CSF.
* Dermoid cysts have a more variable appearance, and areas of low attenuation may be seen due to a lipid component—not due to fat, which is of mesodermal origin.
* Peripheral calcifications may be seen in both lesions.
|
* Epidermoid cysts are hypointense on T1-weighted images and hyperintense on T2-weighted images, with signal intensity similar to that of CSF.
* They insinuate into adjacent structures and encase nerves and blood vessels, making resection difficult.
|-
|Astrocytoma
|
* Grade I II III IV
|
* Uncommon.
* They derive from stromal astrocytes
* In the pineal region they arise from the splenium of the corpus callosum, the thalamus, or the tectum of the midbrain.
* Rarely, they may arise from the neuronal elements within the pineal gland.
|
* Grade 2
** 34% without treatment
** 70% with [[radiation therapy]]
*  The [[prognosis]] is worst for these grade 4 [[glioma]]s.<ref name="pmid15497115">{{cite journal |vauthors=See SJ, Gilbert MR |title=Anaplastic astrocytoma: diagnosis, prognosis, and management |journal=Semin. Oncol. |volume=31 |issue=5 |pages=618–34 |date=October 2004 |pmid=15497115 |doi= |url=}}</ref><ref name="pmid12187956">{{cite journal |vauthors=Korshunov A, Golanov A, Sycheva R |title=Immunohistochemical markers for prognosis of anaplastic astrocytomas |journal=J. Neurooncol. |volume=58 |issue=3 |pages=203–15 |date=July 2002 |pmid=12187956 |doi= |url=}}</ref><ref name="pmid2990664">{{cite journal |vauthors=Burger PC, Vogel FS, Green SB, Strike TA |title=Glioblastoma multiforme and anaplastic astrocytoma. Pathologic criteria and prognostic implications |journal=Cancer |volume=56 |issue=5 |pages=1106–11 |date=September 1985 |pmid=2990664 |doi= |url=}}</ref>
**Few patients survive beyond 3 years.
|
* Uncommon
|
*In [[low grade astrocytoma]]:
**Poorly demarcated mass
**Low density
**No inhancement inside the tumor
**In few cases we might see some [[calcification]] and [[Cyst|cystic]] changes inside the mass
*In [[high grade astrocytoma]]:
**Poorly demarcated mass
**Low density
**There are partial enhancement inside the [[tumor]] mass
|
* Bulbous enlargement of the tectal plate is noted.
* The lesion is typically isointense on T1-weighted images
* Hyperintense on T2-weighted images with no to minimal enhancement on postcontrast images
|-
|Lipoma
|
* No Grading
|
* Abnormal differentiation of the meninx primitiva, which is the undifferentiated mesenchyme that surrounds the developing brain and normally develops into the leptomeninges and subarachnoid space.
* Lipomas represent malformations and not neoplasms.
* Blood vessels and nerves course through them, making resection difficult if required.
|
* Malignant transformation of lipomas into [[Liposarcoma|liposarcomas]] is very rare
* Liposarcomas are not produced from pre-existing benign lesions
|
* Uncommon
|
* Low attenuation, consistent with fat
|
* hyperintense on T1-weighted images with saturation on fat-saturated images
|}
==Pathophysiology==
==Pathophysiology==
* Due to the pineal gland's location, any tumor or cyst formation would lead to the compression of the [[aqueduct of Sylvius]].   
* Due to the pineal gland's location, any tumor or cyst formation would lead to the compression of the [[aqueduct of Sylvius]].   

Revision as of 20:20, 5 May 2019

Pineal gland tumor Main page

Patient Information

Overview

Causes

Classification

Pineocytoma
Pineal teratoma
Pineal yolk sac tumor
Pineal choriocarcinoma
Pineal embryonal carcinoma
Pineal germinoma
Pineal gland cyst

Pathophysiology

Differentiating pineal gland tumor from Other Diseases

Differentiating features among different various Pineal Gland Tumors


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D., Sujit Routray, M.D. [2]

Synonyms and keywords: Pineal gland tumors; Pineal gland cancer; Pineal gland cancers; Pineal gland neoplasm; Pineal gland neoplasms; Neoplasm of the pineal gland; Neoplasms of the pineal gland; Cancer of the pineal gland; Cancers of the pineal gland; Astrocytoma; Meningioma; Pineal gland; Parinaud syndrome; Brain tumor

Overview

The pineal gland is an endocrine gland that is located in the posterior aspect of the cranial fossa in the brain. The pineal gland is responsible for the secretion of melatonin hormone that regulates the in the circadian cycle sleep and wakefulness. The blood supply of the pineal gland is derived from the posterior cerebral artery from its choroidal branches. The internal cerebral vein drains the blood from the epiphysis cerebri. Histologically the gland consists of cells called pinealocytes and supporting cells. Several different tumors can arise from the pineal gland. True pineal cell tumors include pineocytoma, pineoblastoma, and mixed pineal tumors. Tumors that may occur in this region but are not necessarily pineal tumors include germinoma, non-germinoma (eg, teratoma, endodermal sinus tumor, embryonal cell tumor, choriocarcinoma, and mixed tumors), meningioma, astrocytoma, ganglioglioma, and dermoid cysts. Diagnosis of the type of tumor is crucial for treatment. The primary symptom of the tumor would be hydrocephalus. If the pineal gland invades the thalamus, it can cause weakness and loss of sensation in half of the body. Invasion of the hypothalamus would disrupt sleep, impede temperature and water regulation, and cause weight gain. An MRI is important when trying to see the location and size of the tumor. A biopsy is required when trying to determine the type of tumor. Usually, a biopsy is done via a stereotactic or endoscopic procedure. Sometimes biomarkers are used to detect the presence of the tumor, and if these are found in the CSF and blood, then a biopsy might not be needed. Some of these chemicals are beta-human chorionic gonadotropin, carcinoembryonic antigen, and a-fetoprotein.

Classification

Pineal gland tumors are broadly divided into four subcategories. The various types of pineal gland tumors include:[1]

1. Pineal parenchymal tumors: Pineal parenchymal tumors arise directly from the normal functional cells of the pineal gland, pineal parenchymal cells (pineocytes or their precursors), and they are distinct from other pineal gland neoplasms such as astrocytic and germ cell tumors. These tumors are formed after the embryological development of the pineal gland.
2. Pineal germ cell tumors: They are tumors which arise from the embryological abnormalities. They are derived from the germ cells, including sex cells, of the pineal gland during the developmental process of the pineal gland.
3. Astrocytoma of the pineal gland: They arise from the astrocytes, which are a particular kind of star-shaped, glial brain cells around the pineal gland.
4. Pineal metastasis: Pineal metastasis is a cancer that has metastasized to the pineal gland from another location in the body.

 
 
 
 
 
 
 
 
 
 
 
 
Pineal gland tumors
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 

Differentiating pineal gland tumor from Other Diseases

Pineal gland tumormust be differentiated from other diseases that cause seizure, visual disturbance, and constitutional symptoms, such as:

For more information click here.

Differentiating features among different various Pineal Gland Tumors

Tumors Grade Pathalogic Features 5-year survival Cerebrospinal fluid (CSF) dissemination Imaging
CT MRI
Pineocytoma
  • Grade I lesion
  • Small, uniform, mature cells that resemble pineocytes
  • Lobular architecture and pineocytomatous rosettes
  • 86%–100%
  • Rare
  • Well demarcated, usually less than 3 cm, and iso- to hyperattenuating.
  • Hypo- to isointense on T1-weighted images and hyperintense on T2-weighted images.
Pineal Parenchymal Tumor of Intermediate Differentiation
  • Grade II or III
  • Gross inspection, PPTID is similar in appearance to pineocytoma.
  • It is a well-circumscribed lesion without evidence of necrosis.
  • Histologic evaluation reveals diffuse sheets of uniform cells and the formation of small rosettes, with features intermediate between those of pineocytoma and those of pineoblastoma.
  • Low to moderate levels of mitotic activity and nuclear atypia are seen.
  • 39%–74%
  • Rare
  • No specific imaging findings
  • No specific imaging findings
  • Demonstrate high signal intensity on T2-weighted images and enhance on postcontrast images
  • Cystic areas may also be seen
Pineoblastoma
  • Grade IV
  • Pineo-blastomas are highly cellular embryonal neoplasms that resemble other primitive neuroectodermal neoplasms of the central nervous system.
  • Cells have scant cytoplasm and are arranged in diffuse sheets.
  • Homer-Wright rosettes (neuroblastic differentiation) or Flexner-Wintersteiner rosettes (retinoblastic differentiation) may be seen, and hemorrhage or necrosis may be present.
  • Infiltration into adjacent structures and craniospinal dissemination commonly occur
  • 58%
  • Most common cause of the death
  • Lobulated, typically hyperattenuating mass, an appearance that reflects its highly cellular histologic features.
  • Ill-defined enhancing pineal mass with resultant hydrocephalus
Papillary Tumor of the Pineal Region
  • Grade II or III
  • PTPRs are well-circumscribed lesions that can measure up to 5 cm and may have a cystic component.
  • At histologic evaluation, they demonstrate an epithelial-like growth pattern, papillary features, rosettes, and perivascular pseudorosettes.
  • The immunohistochemical findings help differentiate PTPR from other lesions in the pineal region, especially ependymoma and choroid plexus papilloma.
  • 73%
  • 75% times
  • No specific imaging findings
  • Well-circumscribed lesions with variable signal intensity on T1-weighted images, high signal intensity on T2-weighted images, and enhancement on postcontrast images.
  • Cystic areas are commonly present.
  • Hyperintensity on T1-weighted images has been described, which is hypothesized to be related to secretory inclusions containing protein or glycoprotein.
Germinoma
  • Grade II
  • Account for 1%–2% of all cranial neoplasms
  • 65% of intracranial germinomas occur in the pineal region
  • >79%
  • The possibility of CSF seeding necessitates imaging evaluation of the entire neuroaxis
  • Sharply circumscribed, hyperattenuating mass that engulfs the pineal calcifications
  • Demonstrates a solid mass that may have cystic components.
  • Germinomas are iso- to hyperintense to gray matter on T1- and T2-weighted images
  • Avid, homogeneous enhancement on postcontrast images
Teratoma
  • Grade III
  • Mature teratoma reveals a lobulated neoplasm with a complex mixture of adult-type tissues from all three embryonic germ layers.
  • Skin and skin appendages may be seen due to the ectodermal component.
  • The mesoderm contributes to the presence of cartilage, bone, fat, and smooth and skeletal muscle.
  • Respiratory or enteric epithelium arises from the endodermal component.
  • Immature teratomas contain incompletely differentiated tissue elements that resemble fetal tissue.
  • Multiloculated, lobulated lesion with foci of fat attenuation, calcification, and cystic regions
  • T1-weighted MR images may show foci of T1 shortening due to fat and variable signal intensity related to calcification.
  • On T2-weighted images, the soft-tissue component is iso- to hypointense.
Pineal Cyst
  • Grade II
  • Pineal cysts occur in all age ranges but are most predominant in adults 40–49 years of age; studies demonstrate a female predominance.
  • Their origin is debated, with some suggesting they result from degenerative changes in the gland.
  • These lesions are typically asymptomatic and are usually 2–15 mm in size.
  • Follow-up studies have indicated that these lesions remain stable in size over time.
  • When they exceed 15 mm, patients may become symptomatic, typically with headache or visual changes.
  • Intracystic hemorrhage (“pineal apoplexy”) and acute hydrocephalus rarely occur; resultant death has been reported
  • 75% of pineal cysts remained stable during a period of 0.5–9.1 years.
  • Rare
  • No specific imaging findings
  • Round or oval, thin-walled, and well-circumscribed.
  • They typically demonstrate signal intensity similar to that of CSF on T1- and T2-weighted images
Epidermoid and Dermoid Cysts
  • Grade II III
  • The wall of epidermoid cysts is composed of simple stratified squamous epithelium, and the cyst contents consist of layers of keratinaceous debris, which impart a “pearly” appearance to the gross specimen.
  • Dermoid cysts contain dermal appendages (hair follicles, sweat glands.
  • Both lesions slowly expand over time and rupture can result in chemical meningitis, which may be fatal.
  • N/A
  • N/A
  • Epidermoid cysts have low attenuation, similar to that of CSF.
  • Dermoid cysts have a more variable appearance, and areas of low attenuation may be seen due to a lipid component—not due to fat, which is of mesodermal origin.
  • Peripheral calcifications may be seen in both lesions.
  • Epidermoid cysts are hypointense on T1-weighted images and hyperintense on T2-weighted images, with signal intensity similar to that of CSF.
  • They insinuate into adjacent structures and encase nerves and blood vessels, making resection difficult.
Astrocytoma
  • Grade I II III IV
  • Uncommon.
  • They derive from stromal astrocytes
  • In the pineal region they arise from the splenium of the corpus callosum, the thalamus, or the tectum of the midbrain.
  • Rarely, they may arise from the neuronal elements within the pineal gland.
  • Uncommon
  • Bulbous enlargement of the tectal plate is noted.
  • The lesion is typically isointense on T1-weighted images
  • Hyperintense on T2-weighted images with no to minimal enhancement on postcontrast images
Lipoma
  • No Grading
  • Abnormal differentiation of the meninx primitiva, which is the undifferentiated mesenchyme that surrounds the developing brain and normally develops into the leptomeninges and subarachnoid space.
  • Lipomas represent malformations and not neoplasms.
  • Blood vessels and nerves course through them, making resection difficult if required.
  • Malignant transformation of lipomas into liposarcomas is very rare
  • Liposarcomas are not produced from pre-existing benign lesions
  • Uncommon
  • Low attenuation, consistent with fat
  • hyperintense on T1-weighted images with saturation on fat-saturated images

Pathophysiology

  • Due to the pineal gland's location, any tumor or cyst formation would lead to the compression of the aqueduct of Sylvius.
  • The aqueduct of Sylvius allows the cerebrospinal fluid to circulate out.
  • When there is a blockage in aqueduct of Sylvius by an abnormal pineal gland, the passage of the duct is blocked, and CSF pressure builds up, leading to hydrocephalus.
  • Increase in intracranial pressure can even be life-threatening, prompting emergency treatment.
  • The hydrocephalus can be relieved by the placement of a VP shunt or ventriculostomy.
  • Vision changes would also occur due to an involvement of the tectal region.
    • The tectal region helps dictate eye movements.
    • Fault in the tectal region causes double vision, an issue with focusing on objects, and eye movement impairment.
  • The pineal gland can cause Parinaud syndrome due to the increasing size of the gland compressing the pretectal area and superior colliculi of the midbrain.
  • The thalamus can be affected, and if so, there can be disturbances on that side of the body which would result in weakness and loss of sensation.
  • The tumor's effect on the hypothalamus will lead to weight gain, disruption of sleep, disruption of temperature control, and water regulation.
  • Cerebellar involvement would result in motor impairment.
    • If the tumor of the pineal gland is present in childhood, then endocrine dysfunctions can also result such as precocious pseudopuberty, diabetes insipidus, and a slowed growth rate.

References

  1. Pineal region mass. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-region-mass. Accessed on November 18, 2015
  2. See SJ, Gilbert MR (October 2004). "Anaplastic astrocytoma: diagnosis, prognosis, and management". Semin. Oncol. 31 (5): 618–34. PMID 15497115.
  3. Korshunov A, Golanov A, Sycheva R (July 2002). "Immunohistochemical markers for prognosis of anaplastic astrocytomas". J. Neurooncol. 58 (3): 203–15. PMID 12187956.
  4. Burger PC, Vogel FS, Green SB, Strike TA (September 1985). "Glioblastoma multiforme and anaplastic astrocytoma. Pathologic criteria and prognostic implications". Cancer. 56 (5): 1106–11. PMID 2990664.


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