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| __NOTOC__
| | [[Syncope]] is classified into three types: |
| | | * [[Cardiac]] |
| ===Management options of Retinoblastoma===
| | * [[Neurogenic]] |
| :*
| | * [[Vasovagal syncope|vasovagal]] |
| {| class="wikitable"
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| |+
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| ! colspan="2" |Treatment options for Intraocular tumor<ref name="urlRetinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#_13 |title=Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute |format= |work= |accessdate=}}</ref>
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| |Unilateral retinoblastoma
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| * Enucleation followed by chemotherapy
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| *Conservative ocular salvage approaches:
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| **Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy
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| **Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy)
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| |Bilateral retinoblastoma
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| * Enucleation for large intraocular tumors, followed by risk-adapted chemotherapy when the eye and vision cannot be saved
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| * Conservative ocular salvage approaches when the eye and vision can be saved:
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| **Chemoreduction with either systemic or ophthalmic artery infusion chemotherapy with or without intravitreal chemotherapy
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| **Local treatments (cryotherapy, thermotherapy, and plaque radiation therapy)
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| **EBRT
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| |Cavitary retinoblastoma
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| * Systemic and/or intra-arterial chemotherapy
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| |Progressive or recurrent intraocular retinoblastoma
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| * Enucleation
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| * Radiation therapy (EBRT or plaque radiation therapy)
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| * Local treatments (cryotherapy or thermotherapy)
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| * Salvage chemotherapy (systemic or intra-arterial)
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| * Intravitreal chemotherapy
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| |+
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| ! colspan="2" |Treatment options for Extraocular tumor<ref name="urlRetinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute">{{cite web |url=https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq#_13 |title=Retinoblastoma Treatment (PDQ®)—Health Professional Version - National Cancer Institute |format= |work= |accessdate=}}</ref>
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| |Orbital and locoregional retinoblastoma
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| * Chemotherapy
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| * Radiation therapy
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| |CNS disease
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| * Systemic chemotherapy and CNS-directed therapy
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| * Systemic chemotherapy followed by myeloablative chemotherapy and stem cell rescue
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| |Trilateral retinoblastoma
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| * Systemic chemotherapy followed by surgery and myeloablative chemotherapy with stem cell rescue
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| * Systemic chemotherapy followed by surgery and radiation therapy
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| |Extracranial metastatic retinoblastoma
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| * Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue and radiation therapy
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| |Progressive or recurrent extraocular retinoblastoma
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| * Systemic chemotherapy and radiation therapy for orbital disease
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| * Systemic chemotherapy followed by myeloablative chemotherapy with stem cell rescue, and radiation therapy for extraorbital disease
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| |}
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| {| border="3"
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| |+ Intraocular classifications of retinoblastoma and their features
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| ! !! International Intraocular Retinoblastoma Classification (IIRC) !! Intraocular Classification of Retinoblastoma (ICRB)
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| |-
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| ! Group A
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| (very low
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| risk)
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| |Small intraretinal tumors away from foveola and optic nerve<br>3mm or smaller in the greatest dimension, confined to the retina<br>Located further than 3 mm from the foveola and 1.5 mm from the optic disc
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| | Tumors ≤ 3 mm (in basal dimension or thickness)
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| |-
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| !Group B
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| (low risk)
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| |Tumors confined to the retina<br>Not in the group A<br>Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
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| |Tumors > 3 mm (in basal dimension or thickness) or<br>Macular location (≤ 3 mm to foveola)<br>Juxtapapillary location (≤ 1.5 mm to disc)<br>Additional subretinal fluid (≤3 mm from margin)
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| |-
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| ! Group C
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| (moderate
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| risk)
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| |Local disease with minimal subretinal or vitreous seeding with following characteristics:<br>Discrete<br>Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina<br>Local fine vitreous seeding may be present close to the discrete tumor<br>Local subretinal seeding less than 3 mm (2 DD) from the tumor
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| |Tumor with:<br>Subretinal seeds ≤ 3 mm from tumor<br>Vitreous seeds ≤ 3 mm from tumor<br>Both subretinal and vitreous seeds ≤ 3 mm from retinoblastoma
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| |-
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| !Group D
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| (high risk)
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| |Diffuse tumor with significant vitreous or subretinal seeding<br>Maybe massive or diffuse<br>Subretinal fluid present or past without seeding, involving up to total retinal detachment<br>The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses<br>Diffuse subretinal seeding may include subretinal plaques or tumor nodules
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| |Tumor with:<br>Subretinal seeds > 3 mm from tumor<br>Vitreous seeds > 3 mm from tumor<br>Both subretinal and vitreous seeds > 3 mm from retinoblastoma
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| |-
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| !Group E
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| (very high
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| risk)
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| |Presence of any one or more of the following poor prognosis features<br>Tumor touching the lens<br>Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment<br>Diffuse infiltrating retinoblastoma<br>Neovascular glaucoma<br>Opaque media from hemorrhage<br>Tumor necrosis with aseptic orbital cellulitis<br>Phthisis bulbi
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| |Extensive tumor filling >50% globe or with<br>Neovascular glaucoma<br>Opaque media from hemorrhage in the anterior chamber, vitreous or subretinal space<br>Invasion of the post-laminar optic nerve<br>choroid (>2 mm), sclera, orbit, anterior chamber
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| |}
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| {| style="border: 0px; font-size: 90%; margin: 3px; width: 800px" align=center
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| |valign=top|
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| |+
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| ! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Groups}}
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| ! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Features}}
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
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| :Group A
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Small intraretinal tumors away from foveola and optic nerve
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| **3mm or smaller in the greatest dimension, confined to retina
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| **Located further than 3 mm from the foveola and 1.5 mm from the optic disc.
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
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| :Group B
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Tumors confined to the retina.
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| **Not in the group A
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| **Tumor-associated subretinal fluid less than 3 mm from the tumor with no subretinal seeding.
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC;font-All remaining discrete tumors confined to the retina.weight: bold" |
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| :Group C
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Local disease with minimal subretinal or vitreous seeding with following caracteristics:
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| **Discrete
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| **Subretinal fluid, present or past, without seeding involving up to one-fourth of the retina
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| **Local fine vitreous seeding may be present close to the discrete tumor
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| **Local subretinal seeding less than 3 mm (2 DD) from the tumor
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
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| :Group D
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Diffuse tumor with significant vitreous or subretinal seeding
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| **May be massive or diffuse
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| **Subretinal fluid present or past without seeding, involving up to total retinal detachment
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| **The diffuse or massive vitreous disease may include “greasy” seeds or avascular tumor masses
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| **Diffuse subretinal seeding may include subretinal plaques or tumor nodules
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| |-
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| | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" |
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| :Group E
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| | style="padding: 5px 5px; background: #F5F5F5;" |
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| *Presence of any one or more of the following poor prognosis features
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| **Tumor touching the lens
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| **Tumor anterior to anterior vitreous face involving the ciliary body or anterior segment
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| **Diffuse infiltrating retinoblastoma
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| **Neovascular glaucoma
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| **Opaque media from hemorrhage
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| **Tumor necrosis with aseptic orbital cellulitis
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| **Phthisis bulbi
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| |}
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| {|
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| ! colspan="2" style="background:#DCDCDC;" align="center" + |Diagnostic algorithm for Infantile onset glyogen storage disease type II
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| *On [[microscopic]] [[histopathological]] analysis, [[carotid body]] [[tumor]] composed of:
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| **The chief or [[paraganglionic]] cells composing the predominant part of the [[tumor]] and contain [[eosinophilic]] granular materials and oval or round nuclei.<ref name="PatetsiosGable2002">{{cite journal|last1=Patetsios|first1=Peter|last2=Gable|first2=Dennis R.|last3=Garrett|first3=Wilson V.|last4=Lamont|first4=Jeffrey P.|last5=Kuhn|first5=Joseph A.|last6=Shutze|first6=William P.|last7=Kourlis|first7=Harry|last8=Grimsley|first8=Bradley|last9=Pearl|first9=Gregory J.|last10=Smith|first10=Bertram L.|last11=Talkington|first11=C.M.|last12=Thompson|first12=Jesse E.|title=Management of Carotid Body Paragangliomas and Review of a 30-year Experience|journal=Annals of Vascular Surgery|volume=16|issue=3|year=2002|pages=331–338|issn=08905096|doi=10.1007/s10016-001-0106-8}}</ref>
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| **The supporting or sustentacular cells responsible for the [[chemoreceptor]] activity of the [[carotid body]]
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| *The characteristic finding of this [[tumor]] is:
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| :*Chief cells Arranged in distinctive pattern called [[cell]] balls (zellballen)
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| :*Separated by fibrovascular stroma and surrounded by [[sustentacular]] cells
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| :*The [[cytoplasm]] is [[pale]] and diffuse with occasional presence of the [[eosinophilic]] [[granules]].<ref>{{cite book | last = Bibbo | first = Marluce | title = Comprehensive cytopathology | publisher = Saunders/Elsevier | location = Philadelphia, PA | year = 2008 | isbn = 978-1-4160-4208-2 }}</ref>
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| :*The nuclei are round to spindle shape.
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| *The [[tumor]] is highly [[vascular]].
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| *Although there is no well-accepted [[histologic]] criteria for the [[diagnosis]] of [[malignant]] [[tumors]], worrisome [[histologic]] features include:<ref name="WienekeSmith2009">{{cite journal|last1=Wieneke|first1=Jacqueline A.|last2=Smith|first2=Alice|title=Paraganglioma: Carotid Body Tumor|journal=Head and Neck Pathology|volume=3|issue=4|year=2009|pages=303–306|issn=1936-055X|doi=10.1007/s12105-009-0130-5}}</ref>
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| **[[Necrosis]]
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| **Extensive [[vascular]] or capsular [[invasion]]
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| **Increased [[mitotic]] activity
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| **Atypical [[mitotic]] figures
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| {| {{table}} cellpadding="4" cellspacing="0" style="border:#c9c9c9 1px solid; margin: 1em 1em 1em 0; border-collapse: collapse;"
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| | align="center" style="background: #4479BA;" | {{fontcolor|#FFF|''' Features on Gross Pathology'''}}
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| | align="center" style="background: #4479BA;" | {{fontcolor|#FFF|'''Image'''}}
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| |Characteristic findings of [[carotid body]] [[tumor]], include:<ref name="WienekeSmith2009">{{cite journal|last1=Wieneke|first1=Jacqueline A.|last2=Smith|first2=Alice|title=Paraganglioma: Carotid Body Tumor|journal=Head and Neck Pathology|volume=3|issue=4|year=2009|pages=303–306|issn=1936-055X|doi=10.1007/s12105-009-0130-5}}</ref>
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| **Well-circumscribed with psudocapsule
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| **The size of the [[tumor]] varies greatly and it may be as large as 10 cm
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| **The cutting surface is solid with a smooth, rubbery texture||
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| |[[File:Carotid body tumor.jpg|thumb|300px|Contributed by Paweł Kuźniar in wikimedia.commons]]
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| {{Family tree/start}}
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| {{Family tree | | | | | | | | | | | | A01 | | | |A01= Patient with carotid body tumor}}
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| {{Family tree | | | | | | | | | | | | |!| | | | | }}
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| {{Family tree | | | | | | | | | | | | B01 | | | |B01= History, Physical examination, and evaluation of cnotralateral side}}
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| {{Family tree | | | | | | | | | |,|-|-|^|-|-|.| | }}
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| {{Family tree | | | | | | | | | C01 | | | | C02 |C01= Patients with age < 50 years<br>Patients with multiple paraganglioma<br>Patients with a positive family history| C02= The rest of the patients}}
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| {{Family tree | | | | | | | | | |!| | | | }}
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| {{Family tree | | | | | | | | | D01 | | | | | |D01= SDHD genetic testing}}
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| {{Family tree | | | | | |,|-|-|-|^|-|-|.| | }}
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| {{Family tree | | | | | E01 | | | | | E02 |E01= Presence of SDHD mutation |E02= Absence of SDHD mutation}}
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| {{Family tree | | | | | |!| | | | | | |!| | | | | }}
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| {{Family tree | | | | | |!| | | | | | F01 | | | |F01= SDHC and SDHB genetic testing}}
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| {{Family tree | | | | | |!| | | |,|-|-|^|-|-|.| | }}
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| {{Family tree | | | | | |!| | | G01 | | | | G02 |G01= Presence of SDHC/B mutation |G02= Absence of SDHC/B mutation}}
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| {{Family tree | | | | | |!| | | |!| | }}
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| {{familytree | | | | | | H02 |-|'| | | |H02=All the relatives should be evaluated for the presence of paragnaglioma}}
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| {{Family tree | | | | | | |!| | | | }}
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| {{Family tree | | | | | | I01 | | | |I01= whole-body 18F-dihydroxyphenylalanine (F-DOPA) positron emission tomography to assess the presence of other paragangliomas}}
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| {{Family tree | | | |,|-|-|^|-|-|.| |}}
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| {{Family tree | | | J01 | | | | J02 |J01= Presence of other paraganglioma |J02= Absence of other paraganglioma}}
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| {{Family tree | | | |!| | | | | |!| | | }}
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| {{Family tree | | | K01 | | | | K02 |K01= 24-hour urine catecholamines and MRI for biochemical screening|K02=surveillance screening every 5 years}}
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| {{Family tree/end}}
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