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Intraocular classifications of retinoblastoma and their features
International Intraocular Retinoblastoma Classification (IIRC) Intraocular Classification of Retinoblastoma (ICRB)
Group A

(very low risk)

Cell 2 Cell 3
Group B

(low risk)

Cell B Cell C
Group C

(moderate risk)

Cell B Cell C
Group D

(high risk)

Cell B Cell C
Group E

(very high risk)

Cell B Cell C









  • Chief cells Arranged in distinctive pattern called cell balls (zellballen)
  • Separated by fibrovascular stroma and surrounded by sustentacular cells
  • The cytoplasm is pale and diffuse with occasional presence of the eosinophilic granules.[2]
  • The nuclei are round to spindle shape.
Diagnostic algorithm for Infantile onset glyogen storage disease type II
Features on Gross Pathology Image
Characteristic findings of carotid body tumor, include:[3]
    • Well-circumscribed with psudocapsule
    • The size of the tumor varies greatly and it may be as large as 10 cm
    • The cutting surface is solid with a smooth, rubbery texture||
Contributed by Paweł Kuźniar in wikimedia.commons
 
 
 
 
 
 
 
 
 
 
 
Patient with carotid body tumor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
History, Physical examination, and evaluation of cnotralateral side
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Patients with age < 50 years
Patients with multiple paraganglioma
Patients with a positive family history
 
 
 
The rest of the patients
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
SDHD genetic testing
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Presence of SDHD mutation
 
 
 
 
Absence of SDHD mutation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
SDHC and SDHB genetic testing
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Presence of SDHC/B mutation
 
 
 
Absence of SDHC/B mutation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
All the relatives should be evaluated for the presence of paragnaglioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
whole-body 18F-dihydroxyphenylalanine (F-DOPA) positron emission tomography to assess the presence of other paragangliomas
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Presence of other paraganglioma
 
 
 
Absence of other paraganglioma
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
24-hour urine catecholamines and MRI for biochemical screening
 
 
 
surveillance screening every 5 years
  1. Patetsios, Peter; Gable, Dennis R.; Garrett, Wilson V.; Lamont, Jeffrey P.; Kuhn, Joseph A.; Shutze, William P.; Kourlis, Harry; Grimsley, Bradley; Pearl, Gregory J.; Smith, Bertram L.; Talkington, C.M.; Thompson, Jesse E. (2002). "Management of Carotid Body Paragangliomas and Review of a 30-year Experience". Annals of Vascular Surgery. 16 (3): 331–338. doi:10.1007/s10016-001-0106-8. ISSN 0890-5096.
  2. Bibbo, Marluce (2008). Comprehensive cytopathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-4208-2.
  3. 3.0 3.1 Wieneke, Jacqueline A.; Smith, Alice (2009). "Paraganglioma: Carotid Body Tumor". Head and Neck Pathology. 3 (4): 303–306. doi:10.1007/s12105-009-0130-5. ISSN 1936-055X.