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On microscopic histopathological analysis, carotid body tumor composed of:
- The chief or paraganglionic cells composing the predominant part of the tumor and contain eosinophilic granular materials and oval or round nuclei.^[1]
- The supporting or sustentacular cells responsible for the chemoreceptor activity of the carotid body
The characteristic finding of this tumor is:

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.

The tumor is highly vascular.
Although there is no well-accepted histologic criteria for the diagnosis of malignant tumors, worrisome histologic features include:^[3]
- Necrosis
- Extensive vascular or capsular invasion
- Increased mitotic activity
- Atypical mitotic figures

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

↑ 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.
↑ Bibbo, Marluce (2008). Comprehensive cytopathology. Philadelphia, PA: Saunders/Elsevier. ISBN 978-1-4160-4208-2.
↑ ^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.

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+{|
+! colspan="2" style="background:#DCDCDC;" align="center" + |Diagnostic algorithm for Infantile onset glyogen storage disease type II
 |-
-| colspan="2" style="background:#7d7d7d; color: #FFFFFF;" + |<small>'''Adapted from GENETICS IN MEDICINE'''<ref name="pmid16702877">{{cite journal| author=ACMG Work Group on Management of Pompe Disease. Kishnani PS, Steiner RD, Bali D, Berger K, Byrne BJ et al.| title=Pompe disease diagnosis and management guideline. | journal=Genet Med | year= 2006 | volume= 8 | issue= 5 | pages= 267-88 | pmid=16702877 | doi=10.109701.gim.0000218152.87434.f3 | pmc=3110959 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16702877  }} </ref></small>
-|}