Difference between revisions of "Epithelioid sarcoma differential diagnosis"

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(Differentiating Epithelioid sarcoma from other Diseases)
 
(3 intermediate revisions by the same user not shown)
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* Clear cell sarcoma
 
* Clear cell sarcoma
 
* Vascular sarcoma
 
* Vascular sarcoma
 +
 +
 +
{| class="wikitable"
 +
|+
 +
!
 +
!Keratin
 +
!CD34
 +
!Cytoplasm
 +
!In-sutu Kerataniztion
 +
!Location
 +
!Age group
 +
!H/O of epithelial injury
 +
!CA125
 +
!CK5/6
 +
!GCDFP15, S100, actin
 +
!Necrosis
 +
!Calcification
 +
!Hemangiopericytomatous vessels
 +
|-
 +
|Epitheloid Sarcoma
 +
| Positive
 +
| Positive
 +
|Sharply defined
 +
|Negative
 +
|Hand and wrist
 +
|Under 40 years
 +
|No
 +
|Positive
 +
|Rare, Local
 +
|Negative
 +
|Extensive
 +
|Infrequent
 +
|Negative
 +
|-
 +
|Palisaded Necrotic Granuloma
 +
|Neagtive
 +
|Negative
 +
|Indistinct
 +
|
 +
|Uncommon
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|-
 +
|Squamous Carcinoma
 +
|
 +
|Negative
 +
|
 +
|Positive
 +
|
 +
|>40
 +
|Positive
 +
|Negative in cutaneous SCC
 +
 +
Positive everywhere
 +
|Extensive
 +
|
 +
|
 +
|
 +
|
 +
|-
 +
|Sweat Gland Carcinoma
 +
|
 +
|Negative
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|Positive
 +
|
 +
|
 +
|
 +
|-
 +
|Monophasic Synovial Sarcoma
 +
|
 +
|Negative
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|Negative
 +
|
 +
|
 +
|Focal
 +
|Frequent
 +
|Positive
 +
|-
 +
|Melanoma
 +
|Negative
 +
|Negative
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|Negative
 +
|
 +
|Positive
 +
|
 +
|
 +
|
 +
|-
 +
|Epithelioid Hemangioendothelioma
 +
|Negative
 +
|
 +
|
 +
|
 +
|Uncommon on extremities
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|-
 +
|Sclerosing Epithelioid Fibrosarcoma
 +
|Negative
 +
|
 +
|Cords of cells in hylainzed stroma
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|-
 +
|Ischemic Fasciitis
 +
|Negative
 +
|
 +
|Basophilic or amphophilic
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|
 +
|}
 +
 +
{| class="wikitable"
 +
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Disease
 +
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |History/demography
 +
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptoms
 +
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Physical examination
 +
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diagnosis
 +
|-
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Palpable mass
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass tenderness
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Genetics
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
 +
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histology
 +
|-
 +
!Epitheloid Sarcoma
 +
!
 +
* Epithelioid sarcoma is a very rare disease.
 +
* The incidence of epithelioid sarcoma increases between ages of 10 and 39 and the average age at presentation is 27 years.
 +
* Males are more commonly affected with epithelioid sarcoma than females. Epithelioid sarcoma of the upper extremity usually affects individuals of the Caucasian race.
 +
!+
 +
!-
 +
!Mainly cutaneous manifestations
 +
!-
 +
!
 +
* Irregular (or nodular) lesions below the skin surface
 +
* Lesion with the appearance of a badly-healed wound
 +
!
 +
* Loss of INI1 ocated on the long arm of chromosome 22 (22q11.2).
 +
!
 +
* On T2/fluid sequences, the mass is typically hyperintense with areas of central necrosis and surrounding edema.
 +
* There is usually heterogeneous enhancement, often with areas of necrosis.
 +
* A characteristic finding, is propagation along fascial planes, tendon sheaths and nerves.
 +
* Radiography and CT may reveal a soft-tissue mass with speckled calcifications.
 +
* There may be adjacent osseous remodeling; however, cortical destruction and invasion are rare.
 +
!
 +
* Tissue biopsy is the diagnostic modality of choice for epithelioid sarcoma.
 +
 +
:* White nodules with infiltrating margins
 +
:* Epithelial cells well blended with fusiform cells with intracytoplasmic vacuoles
 +
:* "Pseudogranulomatous" proliferation of cells around acellular necrotic debris
 +
:* Hyalinized collagen and necrotic debris in the central zone
 +
:* Calcification in the necrotic zones
 +
:* Multinuclear giant cells in the tumors
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Rhabdomyosarcoma]]<ref name="pmid24966711">{{cite journal| author=Egas-Bejar D, Huh WW| title=Rhabdomyosarcoma in adolescent and young adult patients: current perspectives. | journal=Adolesc Health Med Ther | year= 2014 | volume= 5 | issue=  | pages= 115-25 | pmid=24966711 | doi=10.2147/AHMT.S44582 | pmc=4069040 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24966711  }}</ref><ref name="pmid27955730">{{cite journal| author=Dasgupta R, Fuchs J, Rodeberg D| title=Rhabdomyosarcoma. | journal=Semin Pediatr Surg | year= 2016 | volume= 25 | issue= 5 | pages= 276-283 | pmid=27955730 | doi=10.1053/j.sempedsurg.2016.09.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27955730  }}</ref><ref name="pmid18442956">{{cite journal| author=Park K, van Rijn R, McHugh K| title=The role of radiology in paediatric soft tissue sarcomas. | journal=Cancer Imaging | year= 2008 | volume= 8 | issue=  | pages= 102-15 | pmid=18442956 | doi=10.1102/1470-7330.2008.0014 | pmc=2365455 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18442956  }}</ref><ref name="pmid26349418">{{cite journal| author=Shern JF, Yohe ME, Khan J| title=Pediatric Rhabdomyosarcoma. | journal=Crit Rev Oncog | year= 2015 | volume= 20 | issue= 3-4 | pages= 227-43 | pmid=26349418 | doi= | pmc=5486973 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26349418  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
* Most common [[soft tissue]] [[cancer]] among children and adolescents
 +
* The third most common extracranial [[solid]] tumors
 +
* Two-third of all cases happen under 6 years old
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Skin]] changes
 +
* [[Respiratory]] difficulties
 +
* Vomitting
 +
*[[Hematuria]]
 +
| style="background:#F5F5F5;" align="left" |  +/-
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Fever]]
 +
* Erythmatous [[skin]]
 +
*[[Proptosis]]
 +
* Ophtalmoplasia
 +
* Dysconjugate gaze
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Loss of heterozygosity]] of 11p15.
 +
 +
[[Mutations]] in:
 +
 +
*[[TP53]]
 +
*[[NRAS]]
 +
*[[KRAS]]
 +
*[[HRAS]]
 +
* PIK3CA
 +
* CTNNB1
 +
* FGFR4
 +
*[[Translocations]] in [[PAX3]] or [[PAX7]] [[genes]] with [[FOXO1]]
 +
| style="background:#F5F5F5;" align="left" |[[CT scan]]:
 +
 +
*[[Soft tissue]] [[density]]
 +
* Enhancement with [[contrast]]
 +
* [[Bone]] destruction
 +
 +
[[Ultrasound]]:
 +
* Well-defined and irregular mass
 +
* Low to medium [[echogenicity]]
 +
 +
[[MRI]]:
 +
 +
*[[T1]]:
 +
** Low to intermediate intensity
 +
**[[Hemorrhage]] areas are present in [[alveolar]] [[rhabdomyosarcoma]]
 +
*[[MRI|T2]]:
 +
** Hyperintense
 +
** Prominent flow voids are present in extremity [[lesions]] of [[rhabdomyosarcoma]]
 +
* T1 C+(Gd):
 +
** Enhancement
 +
 +
{| class="wikitable"
 +
|
 +
|}
 +
| style="background:#F5F5F5;" align="left" |
 +
* An [[appearance]] of round [[blue]] [[cell]] [[tumors]]
 +
*[[Myogenesis]] pathway has various types of [[differentiation]]
 +
* [[Positive]] [[Immunohistochemistry|immunohistochemical]] results for:
 +
**[[myoglobin]]
 +
**[[actin]]
 +
**[[desmin]]
 +
**[[Myogenin]]
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Wilms' tumor|Wilms tumor]]<ref name="pmid61529362">{{cite journal |vauthors=Hartman DS, Sanders RC |title=Wilms' tumor versus neuroblastoma: usefulness of ultrasound in differentiation |journal=J Ultrasound Med |volume=1 |issue=3 |pages=117–22 |date=April 1982 |pmid=6152936 |doi= |url=}}</ref><ref name="pmid30036602">{{cite journal |vauthors=De Campo JF |title=Ultrasound of Wilms' tumor |journal=Pediatr Radiol |volume=16 |issue=1 |pages=21–4 |date=1986 |pmid=3003660 |doi= |url=}}</ref><ref name="pmid4080660">{{cite journal |vauthors=Cahan LD |title=Failure of encephalo-duro-arterio-synangiosis procedure in moyamoya disease |journal=Pediatr Neurosci |volume=12 |issue=1 |pages=58–62 |date=1985 |pmid=4080660 |doi= |url=}}</ref><ref name="pmid10985142">{{cite journal| author=Coppes MJ, Pritchard-Jones K| title=Principles of Wilms' tumor biology. | journal=Urol Clin North Am | year= 2000 | volume= 27 | issue= 3 | pages= 423-33, viii | pmid=10985142 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10985142  }}</ref><ref name="pmid22789581">{{cite journal| author=Davidoff AM| title=Wilms tumor. | journal=Adv Pediatr | year= 2012 | volume= 59 | issue= 1 | pages= 247-67 | pmid=22789581 | doi=10.1016/j.yapd.2012.04.001 | pmc=3589819 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22789581  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
* Also called [[nephroblastoma]]
 +
* The most common childhood [[abdominal]] [[malignancy]]
 +
*[[Average]] [[age]] of 3.5 years old
 +
|<nowiki>+</nowiki>
 +
|  +
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Hematuria]]
 +
* [[Respiratory]] [[symptoms]] ( due to [[lung]] [[metastases]])
 +
|<nowiki>+/-</nowiki>
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Fever]]
 +
*[[Hypertension]]/ [[hypotension]]
 +
| style="background:#F5F5F5;" align="left" |Present [[mutations]] of:
 +
 +
*[[WT1]]
 +
*[[P53]]
 +
* FWT1
 +
* FWT2 11p15.5 [[loci]]
 +
| style="background:#F5F5F5;" align="left" |[[Ultrasound]]:
 +
 +
*The best initial [[Diagnostic study of choice|diagnostic study]].
 +
*Distinguish [[tumor]] [[mass]] from other causes of renal [[swelling]] like [[hydronephrosis]].
 +
*[[Doppler ultrasonography]] can help to detect [[invasion]] of [[renal vein]] and [[Inferior vena cava|IVC]] by the [[tumor]].
 +
[[CT scan]]:
 +
*[[Heterogeneous]] [[soft-tissue]] [[density]] [[mass]]
 +
* Areas of [[calcification]] and [[fat]] [[density]] regions
 +
*[[Lymph node]] [[metastasis]]
 +
*Surrounding [[organs]] [[invasion]]
 +
*[[Thrombus]] in [[renal vein]] or [[inferior vena cava]]
 +
| style="background:#F5F5F5;" align="left" |
 +
*Arises from mesodermal [[precursors]] of the [[renal]] [[parenchyma]]
 +
*Well-circumscribed/ macrolobulated [[lesion]]
 +
*[[Hemorrhage]]/ [[central]] [[necrosis]] may be present
 +
*It is comprised of 3 types of [[Cells (biology)|cells]]:
 +
**[[Stromal]]
 +
**[[Epithelium|Epithelial]]
 +
**[[Blastema|Blastemal]]
 +
*The [[stroma]] may include:
 +
**[[Striated muscle|Striated]] [[muscle]] [[cartilage]]
 +
**[[bone]]
 +
**[[Adipose tissue|Fat tissue]]
 +
**[[Fibrous connective tissue|Fibrous tissue.]]
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Ewing sarcoma]]<ref name="pmid12560386">{{cite journal| author=Burchill SA| title=Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities. | journal=J Clin Pathol | year= 2003 | volume= 56 | issue= 2 | pages= 96-102 | pmid=12560386 | doi= | pmc=1769883 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12560386  }}</ref><ref name="pmid8443760">{{cite journal| author=Maygarden SJ, Askin FB, Siegal GP, Gilula LA, Schoppe J, Foulkes M et al.| title=Ewing sarcoma of bone in infants and toddlers. A clinicopathologic report from the Intergroup Ewing's Study. | journal=Cancer | year= 1993 | volume= 71 | issue= 6 | pages= 2109-18 | pmid=8443760 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8443760  }}</ref><ref name="pmid8988217">{{cite journal| author=Panicek DM, Gatsonis C, Rosenthal DI, Seeger LL, Huvos AG, Moore SG et al.| title=CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: Report of the Radiology Diagnostic Oncology Group. | journal=Radiology | year= 1997 | volume= 202 | issue= 1 | pages= 237-46 | pmid=8988217 | doi=10.1148/radiology.202.1.8988217 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8988217  }}</ref><ref name="pmid29977059">{{cite journal| author=Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H et al.| title=Ewing sarcoma. | journal=Nat Rev Dis Primers | year= 2018 | volume= 4 | issue= 1 | pages= 5 | pmid=29977059 | doi=10.1038/s41572-018-0003-x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29977059  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
* Include [[ewing sarcoma]], askin [[tumor]], and peripheral  [[Neuroectodermal tumor primitive|neuroectodermal tumors primitive]]
 +
* The second most common [[childhood]] [[malignant]] primary [[bone]] [[tumors]]
 +
* Usually arises in the long [[bones]] of the [[extremities]]
 +
* Common [[age]] between 10-20 years old
 +
|<nowiki>+</nowiki>
 +
| style="background:#F5F5F5;" align="left" |  +
 +
|
 +
* [[Weight loss]]
 +
* [[Fatigue]]
 +
|<nowiki>+</nowiki>
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Fever]]
 +
*[[Pathological|Pathologic]] [[fractures]]
 +
* [[Petechiae]]/ [[purpura]]
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Reciprocal translocation]] between [[chromosomes]] 11 and 22
 +
| style="background:#F5F5F5;" align="left" |[[Radiographic]] of region:
 +
 +
* Poorly marginated destructive [[lesion]]
 +
* Permeative or "moth-eaten" [[appearance]]
 +
 +
[[CT scan]]:
 +
*[[Cortical area|Cortical]] destruction
 +
* Demonstrate [[soft tissue]] [[disease]]
 +
 +
[[MRI]]:
 +
 +
* Considered as a preferred [[Diagnostic study of choice|diagnostic study]]
 +
* Better shows [[tumor]] size/ [[intraosseous]]/extraosseous extent
 +
| style="background:#F5F5F5;" align="left" |
 +
* Small/ round/ [[blue]] cell [[tumors]]
 +
* May be [[undifferentiated]] or differentiated
 +
* Regular sized [[Primitive (integral)|primitive]] appearing [[Cells (biology)|cells]]
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Pediatric]] [[neuroblastoma]] <ref name="pmid12110723">{{cite journal| author=Lonergan GJ, Schwab CM, Suarez ES, Carlson CL| title=Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. | journal=Radiographics | year= 2002 | volume= 22 | issue= 4 | pages= 911-34 | pmid=12110723 | doi=10.1148/radiographics.22.4.g02jl15911 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12110723  }}</ref><ref name="pmid12580370">{{cite journal| author=Golden CB, Feusner JH| title=Malignant abdominal masses in children: quick guide to evaluation and diagnosis. | journal=Pediatr Clin North Am | year= 2002 | volume= 49 | issue= 6 | pages= 1369-92, viii | pmid=12580370 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12580370  }}</ref><ref name="pmid2403727">{{cite journal| author=Angstman KB, Miser JS, Franz WB| title=Neuroblastoma. | journal=Am Fam Physician | year= 1990 | volume= 41 | issue= 1 | pages= 238-44 | pmid=2403727 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2403727  }}</ref><ref name="pmid6493702">{{cite journal| author=Musarella MA, Chan HS, DeBoer G, Gallie BL| title=Ocular involvement in neuroblastoma: prognostic implications. | journal=Ophthalmology | year= 1984 | volume= 91 | issue= 8 | pages= 936-40 | pmid=6493702 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6493702  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
* Most common extracranial [[solid]] [[tumor]] of [[infancy]]
 +
* Arising from  [[pluripotent]] [[sympathetic]] [[Cells (biology)|cells]]
 +
 +
[[Age]] distribution:
 +
 +
* < 1 years old ( 40%)
 +
* 1-2 years old (35%)
 +
* > 2 years old (25%)
 +
| style="background:#F5F5F5;" align="left" |
 +
+ ([[Abdominal]])
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Constipation]]
 +
* [[Weakness]]
 +
* [[Diarrhea]]
 +
| style="background:#F5F5F5;" align="left" |
 +
+([[Abdominal]])
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Proptosis]]
 +
* Periorbital [[ecchymosis]]
 +
* [[Horner syndrome]]
 +
*[[Opsoclonus myoclonus syndrome]]
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Chromosome]] 1p [[Deletion (genetics)|deletion]]
 +
* [[N-myc-interactor|N-myc]] [[amplification]]
 +
| style="background:#F5F5F5;" align="left" |[[CT scan]]:
 +
 +
*[[Heterogeneous]] [[mass]]
 +
*[[Calcification]]
 +
*[[Necrotic]] areas
 +
[[MRI]]:
 +
*[[T1]]:
 +
**[[heterogeneous]] [[mass]]
 +
*[[Magnetic resonance imaging|T2]]:
 +
**[[Heterogeneous]]/ hyperintense
 +
**[[Cystic]]/ [[necrotic]] areas
 +
*C+ (Gd):
 +
**[[Heterogeneous]] [[mass]]
 +
| style="background:#F5F5F5;" align="left" |
 +
* Well defined/ infiltrative [[mass]]
 +
* Homer [[Wright stain|wright]] rosettes
 +
* [[Secretion]] of [[vanillylmandelic acid]] (VMA) and [[homovanillic acid]] (HVA)
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Pediatric]] [[pheochromocytoma]]<ref name="pmid23345359">{{cite journal| author=Leung K, Stamm M, Raja A, Low G| title=Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging. | journal=AJR Am J Roentgenol | year= 2013 | volume= 200 | issue= 2 | pages= 370-8 | pmid=23345359 | doi=10.2214/AJR.12.9126 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23345359  }}</ref><ref name="pmid1988766">{{cite journal| author=Stein PP, Black HR| title=A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience. | journal=Medicine (Baltimore) | year= 1991 | volume= 70 | issue= 1 | pages= 46-66 | pmid=1988766 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1988766  }}</ref><ref name="pmid17876523">{{cite journal| author=Bravo EL| title=Pheochromocytoma: new concepts and future trends. | journal=Kidney Int | year= 1991 | volume= 40 | issue= 3 | pages= 544-56 | pmid=1787652 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1787652  }}</ref><ref name="pmid1787652">{{cite journal| author=Bravo EL| title=Pheochromocytoma: new concepts and future trends. | journal=Kidney Int | year= 1991 | volume= 40 | issue= 3 | pages= 544-56 | pmid=1787652 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1787652  }} </ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[Rare]] [[catecholamine]]-secreting [[tumor]]
 +
* Occur in both children and adults
 +
*[[Average]] [[age]] of 11 years old
 +
* Associated with  [[neurofibromatosis]], [[von Hippel-Lindau disease]], [[tuberous sclerosis]], [[Sturge-Weber syndrome|sturge-weber syndrome]], and [[multiple endocrine neoplasia]] ([[Multiple endocrine neoplasia|MEN]]) [[syndromes]]
 +
| style="background:#F5F5F5;" align="left" |<nowiki>-</nowiki>
 +
| style="background:#F5F5F5;" align="left" |<nowiki>+/-</nowiki>
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Headache]]
 +
* [[Sweating]]
 +
*[[Weakness]]
 +
*[[Convulsion]]
 +
| style="background:#F5F5F5;" align="left" |<nowiki>-</nowiki>
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Hypertension]]
 +
* [[Tachycardia]]
 +
* [[Pallor]] [[face]]
 +
|[[Genetic]] [[mutation]] in:
 +
 +
*[[NF1]]
 +
*[[RET gene|RET]]
 +
*[[VHL]]
 +
*[[SDHD]]
 +
*[[SDHC]]
 +
*[[EGLN1]]
 +
*[[EGLN2]]
 +
*[[KIF1B]]
 +
*[[SDHAF2]]
 +
*[[TMEM127]]
 +
*[[SDHA]]
 +
*[[IDH1]]
 +
*[[SDHB]]
 +
*[[MAX (gene)|MAX]]
 +
* HIF2A
 +
*[[FH]]
 +
| style="background:#F5F5F5;" align="left" |[[Ultrasound]]:
 +
 +
* Different [[appearance]] from [[solid]] to mixed [[cystic]] or [[solid]] to [[cystic]]
 +
 +
[[CT scan]]:
 +
* Large and heterogenous
 +
*[[Calcification]]
 +
*[[Necrosis]]
 +
*[[Cystic]] changes
 +
[[MRI]] (in extra [[adrenal]] [[tumors]]):
 +
*[[T1]]:
 +
** Heterogenous enhancement
 +
** Hypointense
 +
*[[MRI|T2]]:
 +
** Hyperintense
 +
*T1 C+ (Gd):
 +
** Heterogenous enhancement
 +
| style="background:#F5F5F5;" align="left" |
 +
* Zellballen pattern on [[microscopy]]
 +
* Well-defined clusters
 +
*[[Eosinophilic]] [[cytoplasm]]
 +
 +
Positive stains for:
 +
 +
*[[Chromogranin]] for zellballlen [[Cells (biology)|cells]]
 +
* Neurospecific [[enolase]] markers for [[neuronal]] [[Cells (biology)|cells]]
 +
*[[S-100 protein|S-100]] [[protein]] for [[Sustentacular cell|sustentacular]] [[Cells (biology)|cells]]
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Pediatric]] [[osteosarcoma]]<ref name="pmid8000997">{{cite journal| author=Dorfman HD, Czerniak B| title=Bone cancers. | journal=Cancer | year= 1995 | volume= 75 | issue= 1 Suppl | pages= 203-10 | pmid=8000997 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8000997  }}</ref><ref name="pmid21071381">{{cite journal| author=Yarmish G, Klein MJ, Landa J, Lefkowitz RA, Hwang S| title=Imaging characteristics of primary osteosarcoma: nonconventional subtypes. | journal=Radiographics | year= 2010 | volume= 30 | issue= 6 | pages= 1653-72 | pmid=21071381 | doi=10.1148/rg.306105524 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21071381  }}</ref><ref name="pmid1884549">{{cite journal| author=Araki N, Uchida A, Kimura T, Yoshikawa H, Aoki Y, Ueda T et al.| title=Involvement of the retinoblastoma gene in primary osteosarcomas and other bone and soft-tissue tumors. | journal=Clin Orthop Relat Res | year= 1991 | volume=  | issue= 270 | pages= 271-7 | pmid=1884549 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1884549  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
* The second most common primary [[bone]] [[tumor]]
 +
* The third most common [[tumor]] among adolescents
 +
* Can be primary or secondary
 +
* Primary [[osteosarcoma]] occurs in [[age]] of 10-20 years old
 +
* Secondary [[osteosarcoma]] occurs in older [[patients]] and is secondary to [[paget disease]] and [[bone]] infarcts
 +
* Accompanied with positive history of [[trauma]]
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Soft tissue]] [[swelling]]
 +
* [[Fracture]]
 +
*[[Night Sweats|Night]] [[sweating]]
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[Mass]] [[swelling]]
 +
*[[Fever]]
 +
*[[Arthritis]]
 +
* Decreased [[joint]] [[range of motion]]
 +
*[[Lymphadenopathy]]
 +
| style="background:#F5F5F5;" align="left" |
 +
* Alteration in [[retinoblastoma]] [[gene]] ([[Rb]])
 +
| style="background:#F5F5F5;" align="left" |[[Radiography]]:
 +
 +
*[[Osteolytic metasteses|Osteolytic]]/ [[Osteoblastic osteosarcoma|osteoblastic]] feature
 +
* [[Periosteum]] reaction
 +
*[[Calcification]] or [[ossification]]
 +
 +
[[CT scan]]:
 +
* Primary [[lesion]] and [[chest]] [[CT]] are required
 +
* Demonstrate [[tumor]] location and [[extension]]
 +
 +
[[MRI]]:
 +
 +
* Exact assessment of [[tumor]] [[extension]]
 +
* Involving [[joint]] to [[joint]] findings
 +
| style="background:#F5F5F5;" align="left" |
 +
* Contain various [[cellular]] [[pleomorphism]] and [[mitoses]]
 +
* Poorly [[Trabecular bone|trabecular]] [[bone]] formation
 +
*[[Fibrocystic Disease|Fibrocystic]] and chondroblastic features
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Pediatric]] [[liposarcoma]]<ref name="pmid6861094">{{cite journal| author=Shmookler BM, Enzinger FM| title=Liposarcoma occurring in children. An analysis of 17 cases and review of the literature. | journal=Cancer | year= 1983 | volume= 52 | issue= 3 | pages= 567-74 | pmid=6861094 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6861094  }}</ref><ref name="pmid9386667">{{cite journal| author=Marcus KC, Grier HE, Shamberger RC, Gebhardt MC, Perez-Atayde A, Silver B et al.| title=Childhood soft tissue sarcoma: a 20-year experience. | journal=J Pediatr | year= 1997 | volume= 131 | issue= 4 | pages= 603-7 | pmid=9386667 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9386667  }}</ref><ref name="pmid16160117">{{cite journal| author=Murphey MD, Arcara LK, Fanburg-Smith J| title=From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation. | journal=Radiographics | year= 2005 | volume= 25 | issue= 5 | pages= 1371-95 | pmid=16160117 | doi=10.1148/rg.255055106 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16160117  }}</ref><ref name="pmid17372913">{{cite journal| author=Italiano A, Cardot N, Dupré F, Monticelli I, Keslair F, Piche M et al.| title=Gains and complex rearrangements of the 12q13-15 chromosomal region in ordinary lipomas: the "missing link" between lipomas and liposarcomas? | journal=Int J Cancer | year= 2007 | volume= 121 | issue= 2 | pages= 308-15 | pmid=17372913 | doi=10.1002/ijc.22685 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17372913  }}</ref>
 +
|
 +
* Considered as a nonrhabdomyosarcoma [[soft tissue]] [[sarcomas]]
 +
* One of the least frequent [[tumors]] during  [[childhood]]
 +
* Rarely seen in adolescents and [[age]] of < 8 years old
 +
* [[Average]] [[age]] is 50 years among adults
 +
* Occur mostly in [[lower extremities]],  [[retroperitoneal]] region, and [[shoulder]]
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |  +/-
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Weight loss]]
 +
* [[Fatigue]]
 +
| style="background:#F5F5F5;" align="left" |  -
 +
| style="background:#F5F5F5;" align="left" |
 +
* N/A
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[Amplification]] of 12q13–15 region in  [[MDM2]] and [[CDK4]] [[genes]]
 +
*[[Translocations|Translocation]] of  t (12;16) (q13;p11.2) in myxoid [[liposarcoma]]
 +
| style="background:#F5F5F5;" align="left" |[[CT scan]]:
 +
 +
* Inhomogenous fatty structure
 +
* [[Tumor]] [[mineralization]]
 +
*[[Cortical bone|Cortical]] [[bone]] erosion
 +
*[[Calcification]]
 +
* Infiltration to [[mediastinum]]
 +
[[MRI]]:
 +
*[[Adipose]] content [[mass]]
 +
* Thin [[Irregular bone|irregular]] [[septa]]
 +
*[[Hemorrhage]]
 +
*[[Necrosis]] areas
 +
| style="background:#F5F5F5;" align="left" |Divided into following subtypes:
 +
 +
* Well-differentiated
 +
* Dedifferentiated, Myxoid/ round [[cell]]
 +
*[[Pleomorphic]]
 +
 +
Common findings:
 +
 +
* Lipoblasts presence
 +
*[[Cytoplasmic]] [[lipid]] [[vacuoles]]
 +
*[[Chromatin]] spikes
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Pediatric]] [[acute myelocytic leukemia]]<ref name="pmid18064533">{{cite journal| author=Yamamoto JF, Goodman MT| title=Patterns of leukemia incidence in the United States by subtype and demographic characteristics, 1997-2002. | journal=Cancer Causes Control | year= 2008 | volume= 19 | issue= 4 | pages= 379-90 | pmid=18064533 | doi=10.1007/s10552-007-9097-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18064533  }}</ref><ref name="pmid23634996">{{cite journal| author=Cancer Genome Atlas Research Network. Ley TJ, Miller C, Ding L, Raphael BJ, Mungall AJ et al.| title=Genomic and epigenomic landscapes of adult de novo acute myeloid leukemia. | journal=N Engl J Med | year= 2013 | volume= 368 | issue= 22 | pages= 2059-74 | pmid=23634996 | doi=10.1056/NEJMoa1301689 | pmc=3767041 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23634996  }}</ref><ref name="pmid3864727">{{cite journal| author=Islam A, Catovsky D, Goldman JM, Galton DA| title=Bone marrow biopsy changes in acute myeloid leukaemia. I: Observations before chemotherapy. | journal=Histopathology | year= 1985 | volume= 9 | issue= 9 | pages= 939-57 | pmid=3864727 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3864727  }}</ref><ref name="pmid17587881">{{cite journal| author=Orazi A| title=Histopathology in the diagnosis and classification of acute myeloid leukemia, myelodysplastic syndromes, and myelodysplastic/myeloproliferative diseases. | journal=Pathobiology | year= 2007 | volume= 74 | issue= 2 | pages= 97-114 | pmid=17587881 | doi=10.1159/000101709 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17587881  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
* Replacement of [[normal]] [[Bone marrow cells|bone marrow]] [[Cells (biology)|cells]] with [[abnormal]] [[Cells (biology)|cells]]
 +
*[[Myeloblasts|Myeloblast]] is [[malignant]] [[cell]]
 +
* Wide [[Distribution (pharmacology)|distribution]] among [[childhood]] to adults
 +
*[[Survival rate]] of 60%
 +
* Common in [[down syndrome]]
 +
| style="background:#F5F5F5;" align="left" |  +/- ( [[Abdominal]] [[mass]], [[mediastinal]] [[mass]])
 +
| style="background:#F5F5F5;" align="left" |  + ([[bone]] [[pain]], [[joint]] [[pain]])
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Bleeding]]
 +
* [[Infectious]]
 +
|<nowiki>+/-</nowiki>
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Lymphadenopathy]]
 +
* [[Hepatosplenomegaly]]
 +
* [[Bruising]]
 +
* [[Petechiae]]
 +
* [[Pallor]] [[face]]
 +
*[[Anemia]]
 +
* [[Fever]]
 +
| style="background:#F5F5F5;" align="left" |[[Genetic]] [[translocations]] include:
 +
 +
*t (8;21)
 +
*t (3;21)
 +
*t (15;17)
 +
| style="background:#F5F5F5;" align="left" |[[Radiography]]:
 +
 +
*[[Chest]] [[radiography]]:
 +
**[[Diagnosis]] of [[mediastinal]] [[mass]]
 +
*[[Extremities]] [[radiography]]:
 +
**[[Metaphyseal]] [[bands]]
 +
**[[Lytic]] [[lesions]]
 +
** New [[Periosteal reaction|periosteal]] [[bone]] formation
 +
**[[Pathological|Pathologic]] [[fractures]]
 +
[[CT scan]]/ [[MRI]]:
 +
* Thickening/ [[edema]] of the [[bowel]] wall in presence of [[abdominal]] [[pain]] or [[bowel]] [[infection]]
 +
* Detection of early [[sinusitis]]
 +
*[[Intracranial hemorrhage|Intracranial]] [[hemorrhage]] in presence of [[neurological]] [[symptoms]]
 +
 +
[[Radionuclide test|Radionuclide]] [[imaging]]:
 +
*[[Detection theory|Detection]] of [[occult]] [[infection]]
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[Hyperplastic]] [[bone marrow]] with [[leukemia]] [[Cells (biology)|cells]] replacement
 +
*[[Megaloblastic Anemias|Megaloblastic]] feature
 +
* Decrease in [[normal]] [[hematopoietic cell]]
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Pediatric]] [[acute lymphoblastic leukemia]]<ref name="pmid25184049">{{cite journal| author=Zuckerman T, Rowe JM| title=Pathogenesis and prognostication in acute lymphoblastic leukemia. | journal=F1000Prime Rep | year= 2014 | volume= 6 | issue=  | pages= 59 | pmid=25184049 | doi=10.12703/P6-59 | pmc=4108947 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25184049  }}</ref><ref name="pmid18358930">{{cite journal| author=Pui CH, Robison LL, Look AT| title=Acute lymphoblastic leukaemia. | journal=Lancet | year= 2008 | volume= 371 | issue= 9617 | pages= 1030-43 | pmid=18358930 | doi=10.1016/S0140-6736(08)60457-2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18358930  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
* The most common [[malignancy]] among children
 +
* Few cases may associated with [[down syndrome]], wiskott-  aldrich syndrome, and [[ataxia-telangiectasia]]
 +
* Peak [[age]] of 2-5 years old
 +
* Previous history of [[cancer]]/ [[drug]] [[Exposure (photography)|exposure]]
 +
*[[Bone marrow]] replaced with [[malignant]] [[lymphoblasts]]
 +
| style="background:#F5F5F5;" align="left" |
 +
+/-( Extramedullary [[mass]] in  [[abdomen]]/ [[head]]/ [[neck]])
 +
| style="background:#F5F5F5;" align="left" |  +/- ([[Musculoskeletal]] [[pain]])
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Weakness]]
 +
* [[Fatigue]]
 +
* [[Weight loss]]
 +
* [[Bleeding]]
 +
| style="background:#F5F5F5;" align="left" |  -
 +
| style="background:#F5F5F5;" align="left" |
 +
* [[Fever]]
 +
* [[Lymphadenopathy]]
 +
* [[Hepatosplenomegaly]]
 +
* [[Pallor]]
 +
* [[Papilledema]]
 +
*[[Meningism|Nuchal rigidity]]
 +
*[[Cranial nerve palsy]]
 +
*[[Dyspnea]]
 +
| style="background:#F5F5F5;" align="left" |[[Chromosomal]] [[translocations]]:
 +
 +
* t (9;22)
 +
* t (12;21)
 +
* t (5;14)
 +
* t (1;19)
 +
| style="background:#F5F5F5;" align="left" |[[Radiography]]:
 +
[[Chest]] [[x ray]]:
 +
*[[Nodular]] [[mass]]
 +
*[[Central]] [[lymphadenopathy]]
 +
[[Bone]] [[x ray]]:
 +
* Radiolucent [[metaphyseal]] [[bands]]
 +
* Coarse trabeculation
 +
*[[Periosteal reaction]]
 +
*[[Osteopenia]]
 +
 +
[[Brain]] [[MRI]]:
 +
 +
*[[Leukoencephalopathy]]
 +
*[[Glial cell]] [[hyperplasia]]
 +
*[[Meningitis]]
 +
| style="background:#F5F5F5;" align="left" |Divided into 3 subgroups:
 +
L1:
 +
 +
* Small [[lymphoblast]] [[Cells (biology)|cells]]
 +
* Scant [[cytoplasm]]
 +
* Invisible [[nucleoli]]
 +
 +
L2:
 +
 +
* Larger [[lymphoblast]] [[Cells (biology)|cells]]
 +
* Abundant [[cytoplasm]]
 +
* Prominent [[nucleoli]]
 +
 +
L3:
 +
 +
* Large [[lymphoblast]] [[Cells (biology)|cells]]
 +
* Deep [[cytoplasmic]] [[basophilia]]
 +
* Similar to [[Burkitt lymphoma]]
 +
|-
 +
! style="background:#DCDCDC;" align="center" |[[Pediatric]] [[Non-Hodgkin lymphoma|non-hodgkin]] [[lymphoma]]<ref name="pmid23297126">{{cite journal| author=Green MR, Gentles AJ, Nair RV, Irish JM, Kihira S, Liu CL et al.| title=Hierarchy in somatic mutations arising during genomic evolution and progression of follicular lymphoma. | journal=Blood | year= 2013 | volume= 121 | issue= 9 | pages= 1604-11 | pmid=23297126 | doi=10.1182/blood-2012-09-457283 | pmc=3587323 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23297126  }}</ref><ref name="pmid26174528">{{cite journal| author=Sandlund JT| title=Non-Hodgkin Lymphoma in Children. | journal=Curr Hematol Malig Rep | year= 2015 | volume= 10 | issue= 3 | pages= 237-43 | pmid=26174528 | doi=10.1007/s11899-015-0277-y | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26174528  }}</ref><ref name="pmid25655608">{{cite journal| author=El-Galaly TC, Hutchings M| title=Imaging of non-Hodgkin lymphomas: diagnosis and response-adapted strategies. | journal=Cancer Treat Res | year= 2015 | volume= 165 | issue=  | pages= 125-46 | pmid=25655608 | doi=10.1007/978-3-319-13150-4_5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25655608  }}</ref>
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[Cancer]] derives from [[lymphocytes]]
 +
* Diverse [[age]] of incidence
 +
* Associated with [[autoimmune disorders]], previous [[cancer]] [[therapy]], and [[infection]]
 +
| style="background:#F5F5F5;" align="left" |  +
 +
| style="background:#F5F5F5;" align="left" |  -
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[Lymph node]] [[swelling]]
 +
*[[Weight loss]]
 +
*[[Anorexia]]
 +
*[[Abdominal]] [[pain]]
 +
 +
*[[Nausea]]/ [[vomiting]]
 +
| style="background:#F5F5F5;" align="left" |  + ([[Chest]] [[tenderness]])
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[Fever]]
 +
*[[Hepatosplenomegaly]]
 +
*[[Lymphadenopathy]]
 +
*[[Seizure]]
 +
*[[Petechiae]]
 +
| style="background:#F5F5F5;" align="left" |
 +
*[[MLL2]]
 +
*[[MEF2B]]
 +
*[[EZH2]]
 +
*[[EP300]]
 +
*[[KMT2D]]
 +
*[[CDKN2A]]
 +
| style="background:#F5F5F5;" align="left" |[[Radiography]]:
 +
 +
*[[Chest]] [[x ray]]:
 +
**[[Central]] [[lymphadenopathy]]
 +
**[[Pleural effusion]]
 +
**[[Pericardial effusion]]
 +
 +
[[CT scan]]:
 +
 +
* Presence of enlarged [[lymph node]] in [[chest]], [[abdomen]], and [[pelvis]]
 +
 +
[[Ultrasound]]:
 +
 +
*[[Hepatosplenomegaly]]
 +
| style="background:#F5F5F5;" align="left" |[[Histology]] findings of [[Non-Hodgkin lymphoma|non-hodgkin]] [[lymphoma]] depend on:
 +
 +
*[[Cell]] [[differentiation]]
 +
*[[Cell]] [[Lineage (evolution)|lineage]]
 +
* Location of [[cell]] [[origin]]
 +
|}
  
 
==References==
 
==References==

Latest revision as of 04:37, 21 September 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

Epithelioid sarcoma must be differentiated from synovial sarcoma, wart, ganglion cysts, rhabdomyosarcoma, and clear cell sarcoma.

Differentiating Epithelioid sarcoma from other Diseases


Keratin CD34 Cytoplasm In-sutu Kerataniztion Location Age group H/O of epithelial injury CA125 CK5/6 GCDFP15, S100, actin Necrosis Calcification Hemangiopericytomatous vessels
Epitheloid Sarcoma Positive Positive Sharply defined Negative Hand and wrist Under 40 years No Positive Rare, Local Negative Extensive Infrequent Negative
Palisaded Necrotic Granuloma Neagtive Negative Indistinct Uncommon
Squamous Carcinoma Negative Positive >40 Positive Negative in cutaneous SCC

Positive everywhere

Extensive
Sweat Gland Carcinoma Negative Positive
Monophasic Synovial Sarcoma Negative Negative Focal Frequent Positive
Melanoma Negative Negative Negative Positive
Epithelioid Hemangioendothelioma Negative Uncommon on extremities
Sclerosing Epithelioid Fibrosarcoma Negative Cords of cells in hylainzed stroma
Ischemic Fasciitis Negative Basophilic or amphophilic
Disease History/demography Symptoms Physical examination Diagnosis
Palpable mass Pain Others Mass tenderness Others Genetics Imaging Histology
Epitheloid Sarcoma
  • Epithelioid sarcoma is a very rare disease.
  • The incidence of epithelioid sarcoma increases between ages of 10 and 39 and the average age at presentation is 27 years.
  • Males are more commonly affected with epithelioid sarcoma than females. Epithelioid sarcoma of the upper extremity usually affects individuals of the Caucasian race.
+ - Mainly cutaneous manifestations -
  • Irregular (or nodular) lesions below the skin surface
  • Lesion with the appearance of a badly-healed wound
  • Loss of INI1 ocated on the long arm of chromosome 22 (22q11.2).
  • On T2/fluid sequences, the mass is typically hyperintense with areas of central necrosis and surrounding edema.
  • There is usually heterogeneous enhancement, often with areas of necrosis.
  • A characteristic finding, is propagation along fascial planes, tendon sheaths and nerves.
  • Radiography and CT may reveal a soft-tissue mass with speckled calcifications.
  • There may be adjacent osseous remodeling; however, cortical destruction and invasion are rare.
  • Tissue biopsy is the diagnostic modality of choice for epithelioid sarcoma.
  • White nodules with infiltrating margins
  • Epithelial cells well blended with fusiform cells with intracytoplasmic vacuoles
  • "Pseudogranulomatous" proliferation of cells around acellular necrotic debris
  • Hyalinized collagen and necrotic debris in the central zone
  • Calcification in the necrotic zones
  • Multinuclear giant cells in the tumors
Rhabdomyosarcoma[2][3][4][5]
  • Most common soft tissue cancer among children and adolescents
  • The third most common extracranial solid tumors
  • Two-third of all cases happen under 6 years old
+ + +/-

Mutations in:

CT scan:

Ultrasound:

MRI:

Wilms tumor[6][7][8][9][10] + + +/- Present mutations of: Ultrasound:

CT scan:

Ewing sarcoma[11][12][13][14] + + + Radiographic of region:

CT scan:

MRI:

Pediatric neuroblastoma [15][16][17][18]

Age distribution:

  • < 1 years old ( 40%)
  • 1-2 years old (35%)
  • > 2 years old (25%)

+ (Abdominal)

+

+(Abdominal)

CT scan:

MRI:

Pediatric pheochromocytoma[19][20][21][22] - +/- - Genetic mutation in: Ultrasound:

CT scan:

MRI (in extra adrenal tumors):

  • T1:
    • Heterogenous enhancement
    • Hypointense
  • T2:
    • Hyperintense
  • T1 C+ (Gd):
    • Heterogenous enhancement

Positive stains for:

Pediatric osteosarcoma[23][24][25] + + + Radiography:

CT scan:

MRI:

Pediatric liposarcoma[26][27][28][29] + +/- -
  • N/A
CT scan:

MRI:

Divided into following subtypes:

Common findings:

Pediatric acute myelocytic leukemia[30][31][32][33] +/- ( Abdominal mass, mediastinal mass) + (bone pain, joint pain) +/- Genetic translocations include:
  • t (8;21)
  • t (3;21)
  • t (15;17)
Radiography:

CT scan/ MRI:

Radionuclide imaging:

Pediatric acute lymphoblastic leukemia[34][35]

+/-( Extramedullary mass in abdomen/ head/ neck)

+/- (Musculoskeletal pain) - Chromosomal translocations:
  • t (9;22)
  • t (12;21)
  • t (5;14)
  • t (1;19)
Radiography:

Chest x ray:

Bone x ray:

Brain MRI:

Divided into 3 subgroups:

L1:

L2:

L3:

Pediatric non-hodgkin lymphoma[36][37][38] + - + (Chest tenderness) Radiography:

CT scan:

Ultrasound:

Histology findings of non-hodgkin lymphoma depend on:

References

  1. Epithelioid sarcoma. Sarcomahelp (2016). http://sarcomahelp.org/epithelioid-sarcoma.html Accessed on February 8, 2016
  2. Egas-Bejar D, Huh WW (2014). "Rhabdomyosarcoma in adolescent and young adult patients: current perspectives". Adolesc Health Med Ther. 5: 115–25. doi:10.2147/AHMT.S44582. PMC 4069040. PMID 24966711.
  3. Dasgupta R, Fuchs J, Rodeberg D (2016). "Rhabdomyosarcoma". Semin Pediatr Surg. 25 (5): 276–283. doi:10.1053/j.sempedsurg.2016.09.011. PMID 27955730.
  4. Park K, van Rijn R, McHugh K (2008). "The role of radiology in paediatric soft tissue sarcomas". Cancer Imaging. 8: 102–15. doi:10.1102/1470-7330.2008.0014. PMC 2365455. PMID 18442956.
  5. Shern JF, Yohe ME, Khan J (2015). "Pediatric Rhabdomyosarcoma". Crit Rev Oncog. 20 (3–4): 227–43. PMC 5486973. PMID 26349418.
  6. Hartman DS, Sanders RC (April 1982). "Wilms' tumor versus neuroblastoma: usefulness of ultrasound in differentiation". J Ultrasound Med. 1 (3): 117–22. PMID 6152936.
  7. De Campo JF (1986). "Ultrasound of Wilms' tumor". Pediatr Radiol. 16 (1): 21–4. PMID 3003660.
  8. Cahan LD (1985). "Failure of encephalo-duro-arterio-synangiosis procedure in moyamoya disease". Pediatr Neurosci. 12 (1): 58–62. PMID 4080660.
  9. Coppes MJ, Pritchard-Jones K (2000). "Principles of Wilms' tumor biology". Urol Clin North Am. 27 (3): 423–33, viii. PMID 10985142.
  10. Davidoff AM (2012). "Wilms tumor". Adv Pediatr. 59 (1): 247–67. doi:10.1016/j.yapd.2012.04.001. PMC 3589819. PMID 22789581.
  11. Burchill SA (2003). "Ewing's sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities". J Clin Pathol. 56 (2): 96–102. PMC 1769883. PMID 12560386.
  12. Maygarden SJ, Askin FB, Siegal GP, Gilula LA, Schoppe J, Foulkes M; et al. (1993). "Ewing sarcoma of bone in infants and toddlers. A clinicopathologic report from the Intergroup Ewing's Study". Cancer. 71 (6): 2109–18. PMID 8443760.
  13. Panicek DM, Gatsonis C, Rosenthal DI, Seeger LL, Huvos AG, Moore SG; et al. (1997). "CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: Report of the Radiology Diagnostic Oncology Group". Radiology. 202 (1): 237–46. doi:10.1148/radiology.202.1.8988217. PMID 8988217.
  14. Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H; et al. (2018). "Ewing sarcoma". Nat Rev Dis Primers. 4 (1): 5. doi:10.1038/s41572-018-0003-x. PMID 29977059.
  15. Lonergan GJ, Schwab CM, Suarez ES, Carlson CL (2002). "Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation". Radiographics. 22 (4): 911–34. doi:10.1148/radiographics.22.4.g02jl15911. PMID 12110723.
  16. Golden CB, Feusner JH (2002). "Malignant abdominal masses in children: quick guide to evaluation and diagnosis". Pediatr Clin North Am. 49 (6): 1369–92, viii. PMID 12580370.
  17. Angstman KB, Miser JS, Franz WB (1990). "Neuroblastoma". Am Fam Physician. 41 (1): 238–44. PMID 2403727.
  18. Musarella MA, Chan HS, DeBoer G, Gallie BL (1984). "Ocular involvement in neuroblastoma: prognostic implications". Ophthalmology. 91 (8): 936–40. PMID 6493702.
  19. Leung K, Stamm M, Raja A, Low G (2013). "Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging". AJR Am J Roentgenol. 200 (2): 370–8. doi:10.2214/AJR.12.9126. PMID 23345359.
  20. Stein PP, Black HR (1991). "A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience". Medicine (Baltimore). 70 (1): 46–66. PMID 1988766.
  21. Bravo EL (1991). "Pheochromocytoma: new concepts and future trends". Kidney Int. 40 (3): 544–56. PMID 1787652.
  22. Bravo EL (1991). "Pheochromocytoma: new concepts and future trends". Kidney Int. 40 (3): 544–56. PMID 1787652.
  23. Dorfman HD, Czerniak B (1995). "Bone cancers". Cancer. 75 (1 Suppl): 203–10. PMID 8000997.
  24. Yarmish G, Klein MJ, Landa J, Lefkowitz RA, Hwang S (2010). "Imaging characteristics of primary osteosarcoma: nonconventional subtypes". Radiographics. 30 (6): 1653–72. doi:10.1148/rg.306105524. PMID 21071381.
  25. Araki N, Uchida A, Kimura T, Yoshikawa H, Aoki Y, Ueda T; et al. (1991). "Involvement of the retinoblastoma gene in primary osteosarcomas and other bone and soft-tissue tumors". Clin Orthop Relat Res (270): 271–7. PMID 1884549.
  26. Shmookler BM, Enzinger FM (1983). "Liposarcoma occurring in children. An analysis of 17 cases and review of the literature". Cancer. 52 (3): 567–74. PMID 6861094.
  27. Marcus KC, Grier HE, Shamberger RC, Gebhardt MC, Perez-Atayde A, Silver B; et al. (1997). "Childhood soft tissue sarcoma: a 20-year experience". J Pediatr. 131 (4): 603–7. PMID 9386667.
  28. Murphey MD, Arcara LK, Fanburg-Smith J (2005). "From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation". Radiographics. 25 (5): 1371–95. doi:10.1148/rg.255055106. PMID 16160117.
  29. Italiano A, Cardot N, Dupré F, Monticelli I, Keslair F, Piche M; et al. (2007). "Gains and complex rearrangements of the 12q13-15 chromosomal region in ordinary lipomas: the "missing link" between lipomas and liposarcomas?". Int J Cancer. 121 (2): 308–15. doi:10.1002/ijc.22685. PMID 17372913.
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  31. Cancer Genome Atlas Research Network. Ley TJ, Miller C, Ding L, Raphael BJ, Mungall AJ; et al. (2013). "Genomic and epigenomic landscapes of adult de novo acute myeloid leukemia". N Engl J Med. 368 (22): 2059–74. doi:10.1056/NEJMoa1301689. PMC 3767041. PMID 23634996.
  32. Islam A, Catovsky D, Goldman JM, Galton DA (1985). "Bone marrow biopsy changes in acute myeloid leukaemia. I: Observations before chemotherapy". Histopathology. 9 (9): 939–57. PMID 3864727.
  33. Orazi A (2007). "Histopathology in the diagnosis and classification of acute myeloid leukemia, myelodysplastic syndromes, and myelodysplastic/myeloproliferative diseases". Pathobiology. 74 (2): 97–114. doi:10.1159/000101709. PMID 17587881.
  34. Zuckerman T, Rowe JM (2014). "Pathogenesis and prognostication in acute lymphoblastic leukemia". F1000Prime Rep. 6: 59. doi:10.12703/P6-59. PMC 4108947. PMID 25184049.
  35. Pui CH, Robison LL, Look AT (2008). "Acute lymphoblastic leukaemia". Lancet. 371 (9617): 1030–43. doi:10.1016/S0140-6736(08)60457-2. PMID 18358930.
  36. Green MR, Gentles AJ, Nair RV, Irish JM, Kihira S, Liu CL; et al. (2013). "Hierarchy in somatic mutations arising during genomic evolution and progression of follicular lymphoma". Blood. 121 (9): 1604–11. doi:10.1182/blood-2012-09-457283. PMC 3587323. PMID 23297126.
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  38. El-Galaly TC, Hutchings M (2015). "Imaging of non-Hodgkin lymphomas: diagnosis and response-adapted strategies". Cancer Treat Res. 165: 125–46. doi:10.1007/978-3-319-13150-4_5. PMID 25655608.

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