Pineal germinoma: Difference between revisions
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==Overview== | ==Overview== | ||
Pineal germinoma is the most common tumor of the pineal gland accounting for 50% of all tumors and the majority (80%) of intracranial germ cell tumors.<ref name="overviewpg1">Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Pineal germinoma is a type of germ cell tumor. It refers to a tumor in the pineal gland that has a histology identical to two other tumors | Pineal germinoma is the most common [[tumor]] of the pineal gland accounting for 50% of all tumors and the majority (80%) of intracranial [[germ cell tumors]].<ref name="overviewpg1">Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Pineal germinoma is a type of [[germ cell tumor]]. It refers to a [[tumor]] in the [[pineal gland]] that has a [[histology]] identical to two other tumors, i.e. [[dysgerminoma]] in the [[ovary]] and [[seminoma]] in the [[testis]].<ref name="overviewpg2">Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | ||
==Pathophysiology== | ==Pathophysiology== | ||
===Pathogenesis=== | ===Pathogenesis=== | ||
*Pineal germinoma is a malignant neoplasm of the germinal tissue of the pineal region.<ref name="overviewpg2">Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | *Pineal germinoma is a [[malignant]] [[neoplasm]] of the germinal tissue of the [[Pineal gland|pineal]] region.<ref name="overviewpg2">Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | ||
*Germinomas are thought to originate from an error of development, when certain primordial germ cells fail to migrate properly. Germinomas lack histologic differentiation, whereas nongerminomatous germ cell tumors display a variety of differentiation. Like other germ cell tumors, germinomas can undergo malignant transformation.<ref name="overviewpg3">Natural history of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | *Germinomas are thought to originate from an error of development, when certain [[primordial germ cells]] fail to migrate properly. Germinomas lack [[histologic]] [[differentiation]], whereas nongerminomatous [[germ cell tumors]] display a variety of [[differentiation]]. Like other [[germ cell tumors]], germinomas can undergo [[malignant transformation]].<ref name="overviewpg3">Natural history of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | ||
===Associated Pathology=== | ===Associated Pathology=== | ||
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===Gross Pathology=== | ===Gross Pathology=== | ||
*On gross pathology, pineal germinoma is characterized by a mass whose external surface is smooth and bosselated (knobby) and the interior is soft, fleshy, and either cream-coloured, gray, pink, or tan.<ref name="grosspb1">Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | *On [[gross pathology]], pineal germinoma is characterized by a mass whose external surface is smooth and bosselated (knobby) and the interior is soft, fleshy, and either cream-coloured, gray, pink, or tan.<ref name="grosspb1">Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | ||
===Microscopic Pathology=== | ===Microscopic Pathology=== | ||
*On histopathological analysis, pineal germinoma is characterized by uniform large, round cells with vesicular [[nuclei]] and clear or finely granular cytoplasm that is [[eosinophilic]]. Typically, the stroma contains [[lymphocytes]] and approximately 20% of patients have sarcoid-like granulomas.<ref name="grosspb1">Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | *On [[histopathological]] analysis, pineal germinoma is characterized by uniform large, round cells with vesicular [[nuclei]] and clear or finely granular cytoplasm that is [[eosinophilic]]. Typically, the [[stroma]] contains [[lymphocytes]] and approximately 20% of patients have sarcoid-like [[granulomas]].<ref name="grosspb1">Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref> | ||
===Immunohistochemistry=== | ===Immunohistochemistry=== | ||
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
===Prevalence=== | ===Prevalence=== | ||
Pineal germinoma is the most common tumor of the [[pineal gland]], but accounts for less than 1% of all the [[brain tumor|intracranial tumors]]. Pineal germinoma accounts for 50% of all the pineal gland tumors and the majority (80%) of the intracranial germ cell tumors.<ref name="overviewpg1">Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref><ref name="epipg2">Epidemiology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on Dcember 2, 2015</ref> | Pineal germinoma is the most common tumor of the [[pineal gland]], but accounts for less than 1% of all the [[brain tumor|intracranial tumors]]. Pineal germinoma accounts for 50% of all the [[pineal gland tumors]] and the majority (80%) of the intracranial [[germ cell tumors]].<ref name="overviewpg1">Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref><ref name="epipg2">Epidemiology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on Dcember 2, 2015</ref> | ||
===Age=== | ===Age=== | ||
Pineal germinoma is a disease that tends to affect the children and young adult population.<ref name="epiagepb1">Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Most patients are 20 years or younger at the time of diagnosis. | Pineal germinoma is a disease that tends to affect the children and young adult population.<ref name="epiagepb1">Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Most patients are 20 years or younger at the time of [[diagnosis]]. | ||
===Gender=== | ===Gender=== | ||
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Common complications of pineal germinoma include:<ref name="nathispg1">Clinical presentation of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref><ref name="complicapg1">Treatment and prognosis of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> | Common complications of pineal germinoma include:<ref name="nathispg1">Clinical presentation of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref><ref name="complicapg1">Treatment and prognosis of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> | ||
*[[Obstructive hydrocephalus]] | *[[Obstructive hydrocephalus]] | ||
*Leptomeningeal spread | *[[Leptomeningeal]] spread | ||
===Prognosis=== | ===Prognosis=== | ||
*Prognosis is generally excellent, and the 5-year survival rate of patients with pineal germinoma is approximately 90%.<ref name="prognosispg1">Prognosis of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma#fn__22. Accessed on December 2, 2015</ref> | *[[Prognosis]] is generally excellent, and the [[Five year survival rate|5-year survival rate]] of patients with pineal germinoma is approximately 90%.<ref name="prognosispg1">Prognosis of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma#fn__22. Accessed on December 2, 2015</ref> | ||
*Multifocal or disseminated lesions are associated with poorer prognosis.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | *Multifocal or disseminated lesions are associated with poorer [[prognosis]].<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | ||
*Pineal germinoma usually presents with negative biological markers. The prognosis of patients with [[Human chorionic gonadotropin|β-HCG]] secreting germinoma has been suggested to be worse than that of patients with pure germinoma. | *Pineal germinoma usually presents with negative biological markers. The prognosis of patients with [[Human chorionic gonadotropin|beta-human chorionic gonadotropin]] ([[Human chorionic gonadotropin|β-HCG]] )secreting germinoma has been suggested to be worse than that of patients with pure germinoma. | ||
==History and Symptoms== | ==History and Symptoms== | ||
===History=== | ===History=== | ||
*When evaluating a patient for pineal germinoma, 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. | *When evaluating a patient for pineal germinoma, you should take a detailed history of the presenting [[symptom]] (onset, duration, and progression), other associated [[symptoms]], and a thorough [[Family history|family]] and past medical history review. | ||
===Symptoms=== | ===Symptoms=== | ||
*The clinical presentation of pineal germinoma is mainly from the [[obstructive hydrocephalus]] secondary to compression of the [[tectum]] of the midbrain and obstruction of the [[Cerebral aqueduct|aqueduct]].<ref name="symppg1">Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> | *The clinical presentation of pineal germinoma is mainly from the [[obstructive hydrocephalus]] secondary to compression of the [[tectum]] of the midbrain and obstruction of the [[Cerebral aqueduct|aqueduct]].<ref name="symppg1">Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> | ||
*Symptoms of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref> | *[[Symptoms]] of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref> | ||
:*[[Headache]]s | :*[[Headache]]s | ||
:*[[Nausea]] | :*[[Nausea]] | ||
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:*[[Fatigue]] | :*[[Fatigue]] | ||
:*[[anorexia|Loss of appetite]] | :*[[anorexia|Loss of appetite]] | ||
:*Hair loss | :*[[Hair loss]] | ||
:*Presyncopal episodes | :*Presyncopal episodes | ||
:*[[Personality pathology|Personality changes]] | :*[[Personality pathology|Personality changes]] | ||
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==Physical Examination== | ==Physical Examination== | ||
*Compression of the superior colliculi can lead to a characteristic gaze palsy, known as [[Parinaud syndrome]].<ref name="symppg1">Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> | *Compression of the superior [[Colliculus|colliculi]] can lead to a characteristic [[gaze palsy]], known as [[Parinaud syndrome]].<ref name="symppg1">Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> | ||
*Common physical examination findings of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref> | *Common [[physical examination]] findings of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref> | ||
===HEENT=== | ===HEENT=== | ||
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*Eyes that are constantly looking down ([[sunsetting sign]]) | *Eyes that are constantly looking down ([[sunsetting sign]]) | ||
*Deficiency in upward-gaze | *Deficiency in upward-gaze | ||
*Pupillary light-near dissociation (pupils respond to near stimuli but not light) | *[[Pupillary]] [[light-near dissociation]] (pupils respond to near stimuli but not light) | ||
*Convergence-retraction [[nystagmus]] | *Convergence-retraction [[nystagmus]] | ||
*[[Papilledema]] | *[[Papilledema]] | ||
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==CT== | ==CT== | ||
*Head CT scan may be diagnostic of pineal germinoma. | *Head [[Computed tomography|CT scan]] may be diagnostic of pineal germinoma. | ||
*Findings on CT scan suggestive of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref><ref name="ctpg1">CT radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref> | *Findings on [[CT scan]] suggestive of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref><ref name="ctpg1">CT radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref> | ||
:*[[Obstructive hydrocephalus]] | :*[[Obstructive hydrocephalus]] | ||
:*Large midline mass in the pineal region | :*Large midline mass in the [[Pineal gland|pineal]] region | ||
::*Hyperdense compared to normal brain | ::*Hyperdense compared to normal brain | ||
::*Vivid contrast enhancement | ::*Vivid contrast enhancement | ||
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===Gallery=== | ===Gallery=== | ||
==MRI== | ==MRI== | ||
*Brain MRI may be diagnostic of pineal germinoma. | *[[Brain]] [[MRI]] may be diagnostic of pineal germinoma. | ||
*Features on MRI suggestive of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref><ref name="mripg1">MRI radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref> | *Features on [[MRI]] suggestive of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref><ref name="mripg1">MRI radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref> | ||
{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px align=center" | {| style="border: 0px; font-size: 90%; margin: 3px; width:1000px align=center" | ||
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{| class="wikitable" | {| class="wikitable" | ||
|+ | |+ | ||
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Management Options | ! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Management Options for Pineal Gland Tumors | ||
|- | |- | ||
|'''Radiation''' | |'''Radiation''' | ||
| | | | ||
* Postoperative adjuvant RT is frequently (but not universally) recommended, and local control is dose-dependent. | * Postoperative [[Adjuvant therapy|adjuvant RT]] is frequently (but not universally) recommended, and local control is dose-dependent. | ||
* The incidence of leptomeningeal recurrence was significantly lower among patients receiving CSI compared with those who did not. | * The [[incidence]] of [[leptomeningeal]] recurrence was significantly lower among patients receiving CSI compared with those who did not. | ||
* The five-year survival rates | * The [[Five-year survival rate|five-year survival rates]] are 86 and 49 percent for [[pineocytomas]] and non-[[pineocytoma]] PPTs, respectively. | ||
* Adjuvant RT is not universally recommended after gross total resection of a pineocytoma | * [[Adjuvant therapy|Adjuvant RT]] is not universally recommended after gross total resection of a [[pineocytoma]] | ||
|- | |- | ||
|'''Stereotactic radiosurgery''' | |'''Stereotactic radiosurgery''' | ||
| | | | ||
* Stereotactic radiosurgery (SRS) is emerging as a useful treatment alternative for pineocytomas, although experience is limited. | * [[Stereotactic radiosurgery]] (SRS) is emerging as a useful treatment alternative for [[pineocytomas]], although experience is limited. | ||
* The precise radiation fields that are defined by MRI or CT-computerized treatment planning minimize damage to the surrounding brain, and the risks of general anesthesia and craniotomy are avoided. | * The precise radiation fields that are defined by [[MRI]] or [[CT-scans|CT]]-computerized treatment planning minimize damage to the surrounding [[brain]], and the risks of [[general anesthesia]] and [[craniotomy]] are avoided. | ||
* SRS is increasingly being used to treat pineal region tumors, either as an additional therapy after conventional treatments or as a primary treatment. | * SRS is increasingly being used to treat [[Pineal gland|pineal]] region [[tumors]], either as an additional therapy after conventional treatments or as a primary treatment. | ||
* Due to the low rate of side effects, IRS may develop into an attractive alternative to microsurgery in de novo diagnosed pineocytomas. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation. | * Due to the low rate of side effects, IRS may develop into an attractive alternative to [[microsurgery]] in de novo diagnosed [[pineocytomas]]. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation. | ||
|- | |- | ||
|'''Chemotherapy as part of multimodality therapy''' | |'''Chemotherapy as part of multimodality therapy''' | ||
| | | | ||
* The similarity of pineoblastomas to medulloblastomas in terms of their clinical behavior and tendency for leptomeningeal seeding has led to the use of similar chemotherapy regimens in patients with pineoblastoma as part of a multimodality approach. | * The similarity of [[pineoblastomas]] to [[medulloblastomas]] in terms of their clinical behavior and tendency for [[leptomeningeal]] seeding has led to the use of similar [[chemotherapy]] regimens in patients with [[pineoblastoma]] as part of a multimodality approach. | ||
* Chemotherapy has been used to delay radiation therapy in very young children, for whom the long-term neurocognitive and developmental side effects of craniospinal irradiation (CSI) are a major concern. | * [[Chemotherapy]] has been used to delay [[radiation therapy]] in very young children, for whom the long-term [[neurocognitive]] and developmental side effects of craniospinal [[irradiation]] (CSI) are a major concern. | ||
* The importance of radiation therapy as a component of the initial treatment of supratentorial primitive neuroectodermal tumors (PNETs) is also supported by the German HIT-SKK87 and HIT-SKK92 protocols, as well as the Canadian pediatric brain tumor protocol | * The importance of [[radiation therapy]] as a component of the initial treatment of [[supratentorial]] [[primitive neuroectodermal tumors]] ([[Primitive neuroectodermal tumor|PNETs]]) is also supported by the German HIT-SKK87 and HIT-SKK92 protocols, as well as the Canadian [[pediatric]] [[brain tumor]] protocol | ||
|} | |} | ||
*Patients with pineal germinoma | *Patients with pineal [[germinoma]] may develop [[hydrocephalus]] in majority of the cases and they will require [[CSF]] diversion. [[Ventriculoperitoneal shunt|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 [[Midbrain tectum|tectal region]]. Neuroendoscopic [[biopsy]] procedures have been proven safe with low complication rates.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | ||
==References== | ==References== | ||
Latest revision as of 01:28, 3 June 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Synonyms and keywords: Pineal gland germinoma; Pineal germinomas; Germinoma of the pineal gland; Pineal dysgerminoma; Pineal dysgerminomas; Pineal gland tumor; Brain tumor
Overview
Pineal germinoma is the most common tumor of the pineal gland accounting for 50% of all tumors and the majority (80%) of intracranial germ cell tumors.[1] Pineal germinoma is a type of germ cell tumor. It refers to a tumor in the pineal gland that has a histology identical to two other tumors, i.e. dysgerminoma in the ovary and seminoma in the testis.[2]
Pathophysiology
Pathogenesis
- Pineal germinoma is a malignant neoplasm of the germinal tissue of the pineal region.[2]
- Germinomas are thought to originate from an error of development, when certain primordial germ cells fail to migrate properly. Germinomas lack histologic differentiation, whereas nongerminomatous germ cell tumors display a variety of differentiation. Like other germ cell tumors, germinomas can undergo malignant transformation.[3]
Associated Pathology
- Pineal germinoma may be associated with Down syndrome.[4]
Gross Pathology
- On gross pathology, pineal germinoma is characterized by a mass whose external surface is smooth and bosselated (knobby) and the interior is soft, fleshy, and either cream-coloured, gray, pink, or tan.[5]
Microscopic Pathology
- On histopathological analysis, pineal germinoma is characterized by uniform large, round cells with vesicular nuclei and clear or finely granular cytoplasm that is eosinophilic. Typically, the stroma contains lymphocytes and approximately 20% of patients have sarcoid-like granulomas.[5]
Immunohistochemistry
Pineal germinoma is demonstrated by positivity to tumor markers such as:[6]
Differentiating Pineal Germinoma from other Diseases
Pineal germinoma must be differentiated from:[7][8]
- Pineocytoma
- Pineal parenchymal tumor with intermediate differentiation
- Papillary tumor of the pineal region
- Pineoblastoma
- Pineal embryonal carcinoma
- Pineal choriocarcinoma
- Pineal yolk sac carcinoma
- Pineal teratoma
- Pineal cyst
- Astrocytoma of the pineal gland
- Meningioma near pineal gland
- Pineal metastasis
- Cavernoma in pineal region
- Aneurysm in pineal region
Epidemiology and Demographics
Prevalence
Pineal germinoma is the most common tumor of the pineal gland, but accounts for less than 1% of all the intracranial tumors. Pineal germinoma accounts for 50% of all the pineal gland tumors and the majority (80%) of the intracranial germ cell tumors.[1][9]
Age
Pineal germinoma is a disease that tends to affect the children and young adult population.[10] Most patients are 20 years or younger at the time of diagnosis.
Gender
Males are more commonly affected with pineal germinoma than females. The male to female ratio is approximately 13 to 1.[10]
Natural History, Complications and Prognosis
Natural History
If left untreated, patients with pineal germinoma may progress to develop seizures, obstructive hydrocephalus, and CSF metastasis.[11]
Complications
Common complications of pineal germinoma include:[11][12]
Prognosis
- Prognosis is generally excellent, and the 5-year survival rate of patients with pineal germinoma is approximately 90%.[13]
- Multifocal or disseminated lesions are associated with poorer prognosis.[14]
- Pineal germinoma usually presents with negative biological markers. The prognosis of patients with beta-human chorionic gonadotropin (β-HCG )secreting germinoma has been suggested to be worse than that of patients with pure germinoma.
History and Symptoms
History
- When evaluating a patient for pineal germinoma, 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 pineal germinoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct.[15]
- Symptoms of pineal germinoma include:[16]
- Headaches
- Nausea
- Vomiting
- Seizures
- Hearing loss
- Sleepiness
- Irritability
- Slowed speech
- Fatigue
- Loss of appetite
- Hair loss
- Presyncopal episodes
- Personality changes
- Double vision
- Trouble coordinating motor movements
- Difficulty walking
- Slowed growth
Physical Examination
- Compression of the superior colliculi can lead to a characteristic gaze palsy, known as Parinaud syndrome.[15]
- Common physical examination findings of pineal germinoma 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
- Papilledema
Neurological
- Mental retardation
- Muscle spasms
- Hemiparesis
- Loss of bladder control
- Ataxia
- Tremor
- Dysmetria
- Pronator drift
CT
- Head CT scan may be diagnostic of pineal germinoma.
- Findings on CT scan suggestive of pineal germinoma include:[16][17]
- Large midline mass in the pineal region
- Hyperdense compared to normal brain
- Vivid contrast enhancement
- Calcification: usually representing "engulfed" pineal calcification
Gallery
MRI
- Brain MRI may be diagnostic of pineal germinoma.
- Features on MRI suggestive of pineal germinoma include:[16][18]
| MRI component | Findings |
|---|---|
|
T1 |
|
|
T2 |
|
|
T1 with gadolinium contrast [T1 C+ (Gd)] |
|
|
Diffuse weighted imaging [DWI] |
|
Gallery
-
![Sagittal MRI with contrast demonstrating a large enhancing mass centered on the pineal region. It is heterogeneous with areas of cystic change. There is marked compression of the tectum with resulting obstructive hydrocephalus. A little surrounding edema is also present.[19]](/images/9/93/MRI_of_pineal_germinoma_1.jpg)
Sagittal MRI with contrast demonstrating a large enhancing mass centered on the pineal region. It is heterogeneous with areas of cystic change. There is marked compression of the tectum with resulting obstructive hydrocephalus. A little surrounding edema is also present.[19]
![Sagittal MRI with contrast demonstrating a large enhancing mass centered on the pineal region. It is heterogeneous with areas of cystic change. There is marked compression of the tectum with resulting obstructive hydrocephalus. A little surrounding edema is also present.[19]](/index.php/File:MRI_of_pineal_germinoma_1.jpg)
Treatment
- The mainstay of therapy for pineal germinoma is radiotherapy, since it is highly radiosensitive.[14]
- In children, there is an attempt to reduce the toxicity of radiation therapy by the use of chemotherapy in combination with reduced dose radiation to decrease the volume of normal tissue irradiated by stereotactic radiotherapy.
- The various chemotherapeutic agents that may be used for the treatment of pineal germinoma include:[14][20]
| Management Options for Pineal Gland Tumors | |
|---|---|
| Radiation |
|
| Stereotactic radiosurgery |
|
| Chemotherapy as part of multimodality therapy |
|
- Patients with pineal germinoma may 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.[14]
References
- ↑ 1.0 1.1 Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
- ↑ 2.0 2.1 Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015
- ↑ Natural history of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015
- ↑ Tan HW, Ty A, Goh SG, Wong MC, Hong A, Chuah KL (2004). "Pineal yolk sac tumour with a solid pattern: a case report in a Chinese adult man with Down's syndrome". J Clin Pathol. 57 (8): 882–4. doi:10.1136/jcp.2004.016659. PMC 1770394. PMID 15280413.
- ↑ 5.0 5.1 Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015
- ↑ Histology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on December 2, 2015
- ↑ Differential diagnoses of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ Epidemiology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on Dcember 2, 2015
- ↑ 10.0 10.1 Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
- ↑ 11.0 11.1 Clinical presentation of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015
- ↑ Treatment and prognosis of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
- ↑ Prognosis of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma#fn__22. Accessed on December 2, 2015
- ↑ 14.0 14.1 14.2 14.3 Alexiou, George A (2012). "Management of pineal region tumours in children". Journal of Solid Tumors. 2 (2). doi:10.5430/jst.v2n2p15. ISSN 1925-4075.
- ↑ 15.0 15.1 Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
- ↑ 16.0 16.1 16.2 16.3 Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS; et al. (2015). "Intracranial germinoma". Proc (Bayl Univ Med Cent). 28 (1): 43–5. PMC 4264708. PMID 25552796.
- ↑ CT radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015
- ↑ MRI radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ Treatment and prognosis of pineal germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 3, 2015