Mycosis fungoides classification: Difference between revisions

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__NOTOC__
__NOTOC__
{{Cutaneous T cell lymphoma}}
{{Mycosis fungoides}}
{{CMG}}; {{AE}} {{AS}}
{{CMG}}; {{AE}} {{S.G.}}


==Overview==
==Overview==
There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes. Cutaneous T cell lymphoma may be classified into several subtypes based on WHO-EORTC classification.
There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes. Cutaneous T cell lymphoma may be classified into several subtypes based on WHO-EORTC classification.


method 1], [classification
==Classification==
==Classification==
According to world Health Organization (WHO) and European Organization for Research and Treatment of Cancer (EORTC) classification, cutaneous T-cell lymphomas may be classified into the following types:<ref name="Andrews">{{cite book |author=James, William D.; Berger, Timothy G.|title=Andrews' Diseases of the Skin: clinical Dermatology |publisher=Saunders Elsevier |location= |year=2006 |pages= |isbn=0-7216-2921-0 |oclc= |doi=|accessdate=|display-authors=etal}}</ref>
According to [[world Health Organization]] ([[WHO]]) and European Organization for Research and Treatment of Cancer (EORTC) classification, mycosis fungoides may be classified into the following types:<ref name="Matutes2018">{{cite journal|last1=Matutes|first1=E.|title=The 2017 WHO update on mature T- and natural killer (NK) cell neoplasms|journal=International Journal of Laboratory Hematology|volume=40|year=2018|pages=97–103|issn=17515521|doi=10.1111/ijlh.12817}}</ref><ref name="Sundram2018">{{cite journal|last1=Sundram|first1=Uma|title=Cutaneous Lymphoproliferative Disorders|journal=Advances In Anatomic Pathology|year=2018|pages=1|issn=1072-4109|doi=10.1097/PAP.0000000000000208}}</ref>
 


*Mycosis fungoides
*Mycosis fungoides
*Mycosis fungoides variants and subtypes
*Mycosis fungoides variants and subtypes
**Folliculotropic mycosis fungoides
**Folliculotropic mycosis fungoides
**Pagetoid reticulosis
**[[Pagetoid reticulosis]]
**Granulomatous slack skin
**[[Granulomatous]] [[Slackia|slack]] [[skin]]
*Sezary syndrome
*Adult T cell leukemia/lymphoma
*Primary cutaneous CD30+ lymphoproliferative disorders
**Primary cutaneous anaplastic large cell lymphoma
**Lymphomatoid papulosis
*Subcutaneous panniculitis-like T cell lymphoma
*Extranodal NK/T cell lymphoma, nasal type
*Primary cutaneous peripheral T cell lymphoma, rare subtypes
**Primary cutaneous gamma-delta T cell lymphoma
**Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma (provisional)
**Primary cutaneous CD4+ small/medium-sized pleomorphic T cell lymphoproliferative disorder (provisional)
**Primary cutaneous acral CD8+ T cell lymphoma (provisional)
*Primary cutaneous peripheral T cell lymphoma, not otherwise specified
 
 
 
:* [[Pagetoid reticulosis]]
:* [[Sézary syndrome]]
:* Granulomatous slack skin
:* [[Lymphomatoid papulosis]]
:* [[Pityriasis lichenoides chronica]]
:* [[Pityriasis lichenoides et varioliformis acuta]]
:* CD30+ cutaneous T-cell lymphoma
:* Secondary cutaneous CD30+ large cell lymphoma
:* Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
:* Pleomorphic T-cell lymphoma
:* Lennert lymphoma
:* Subcutaneous T-cell lymphoma
:* Angiocentric lymphoma
:* Blastic NK-cell lymphoma
 
Based on the organ involvement, cutaneous T cell lymphoma may be classified into:<ref name="canadiancancer">Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016</ref>
* [[Mycosis fungoides]] (MF)
* [[Sézary syndrome]] (SS) 


{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
Line 61: Line 27:
* May appear as a scaly, pink patches on the skin
* May appear as a scaly, pink patches on the skin
* Signs can progress to the development of skin tumors in more advanced cases
* Signs can progress to the development of skin tumors in more advanced cases
|}
==Staging==
* The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.<ref name="canadiancancer">Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016</ref>
* Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:<ref name="TrautingerEder2017">{{cite journal|last1=Trautinger|first1=Franz|last2=Eder|first2=Johanna|last3=Assaf|first3=Chalid|last4=Bagot|first4=Martine|last5=Cozzio|first5=Antonio|last6=Dummer|first6=Reinhard|last7=Gniadecki|first7=Robert|last8=Klemke|first8=Claus-Detlev|last9=Ortiz-Romero|first9=Pablo L.|last10=Papadavid|first10=Evangelia|last11=Pimpinelli|first11=Nicola|last12=Quaglino|first12=Pietro|last13=Ranki|first13=Annamari|last14=Scarisbrick|first14=Julia|last15=Stadler|first15=Rudolf|last16=Väkevä|first16=Liisa|last17=Vermeer|first17=Maarten H.|last18=Whittaker|first18=Sean|last19=Willemze|first19=Rein|last20=Knobler|first20=Robert|title=European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – Update 2017|journal=European Journal of Cancer|volume=77|year=2017|pages=57–74|issn=09598049|doi=10.1016/j.ejca.2017.02.027}}</ref><ref name="pmid17540844">{{cite journal |vauthors=Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, Zackheim H, Duvic M, Estrach T, Lamberg S, Wood G, Dummer R, Ranki A, Burg G, Heald P, Pittelkow M, Bernengo MG, Sterry W, Laroche L, Trautinger F, Whittaker S |title=Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC) |journal=Blood |volume=110 |issue=6 |pages=1713–22 |date=September 2007 |pmid=17540844 |doi=10.1182/blood-2007-03-055749 |url=}}</ref>
{| class="wikitable"
|+
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Staging for mycosis fungoides and Sezary syndrome
|-
! colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;" |'''[[Skin]] (T)'''
|-
| align="center" style="background:#ADD8E6;" |T1
|Limited patches, [[Papule|papules]], and/or [[Plaque|plaques]] covering <10% of the [[skin]] [[Surface area|surface]]. May further stratify into T1a ([[Patched|patch]] only) versus T1b ([[plaque]]  [[Patched|patch]])
|-
| align="center" style="background:#ADD8E6;" |T2
|Patches, [[Papule|papules]], [[or]] [[Plaque|plaques]] covering 10% of the [[skin]] [[Surface area|surface]]. May further stratify into T2a (patch only) versus T2b ([[plaque]]  patch).
|-
|-
| style="text-align: center; padding: 5px 5px; background: #F5F5F5;" | '''Sézary syndrome''' (SS)
| align="center" style="background:#ADD8E6;" |T3
| style="padding: 5px 5px; background: #F5F5F5;" |  
|One or more [[Tumor|tumours]] (1-cm diameter)
* Often the more aggressive form of cutaneous T cell lymphoma
|-
* An advanced form of mycosis fungoides
| align="center" style="background:#ADD8E6;" |T4
* Affects skin, blood, and lymph nodes
|Confluence of [[erythema]] covering 80% [[body surface area]]
:* Large areas of the skin are affected
|-
:* Skin is often red, itchy, painful, and peeling
! colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;" |'''Node (N)'''
:* Skin tumors can also appear
|-
:* Entire body can be affected, also known as "red man syndrome"
| align="center" style="background:#ADD8E6;" |N0
|No [[Clinical|clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; [[biopsy]] not required
|-
| align="center" style="background:#ADD8E6;" |N1
|Clinically [[abnormal]] [[Lymph node|lymph nodes]]; [[histopathology]] Dutch grade 1 or [[NCI]] LN0-2
|-
| align="center" style="background:#ADD8E6;" |N1a
|[[Clone]] negative
|-
| align="center" style="background:#ADD8E6;" |N1b
|[[Clone]] posetive
|-
| align="center" style="background:#ADD8E6;" |N2
|[[Clinical|Clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; [[histopathology]] Dutch grade 2 or [[NCI]] LN3
|-
| align="center" style="background:#ADD8E6;" |N2a
|[[Clone]] negatove
|-
| align="center" style="background:#ADD8E6;" |N2b
|[[Clone]] posetive
|-
| align="center" style="background:#ADD8E6;" |N3
|[[Clinical|Clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; [[histopathology]] Dutch grades 3e4 or [[NCI]] LN4; [[clone]] positive or negative
|-
| align="center" style="background:#ADD8E6;" |NX
|[[Clinical|Clinically]] [[abnormal]] [[T-cell lymphoma classification|peripheral]] [[Lymph node|lymph nodes]]; no [[histologic]] confirmation
|-
! colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;" |[[Visceral|'''Visceral''']] ('''M''')
|-
| align="center" style="background:#ADD8E6;" |M0
|No [[visceral]] [[Organ (anatomy)|organ]] involvement
|-
| align="center" style="background:#ADD8E6;" |M1
|[[Visceral]] involvement (must have [[pathology]] confirmation and [[Organ (anatomy)|organ]] involved should be specified)
|-
! colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;" |[[Blood|'''Blood''']] '''(B)'''
|-
| align="center" style="background:#ADD8E6;" |B0
|0 Absence of significant [[blood]] involvement: 5% of [[T-cell lymphoma classification|peripheral]] [[blood]] [[Lymphocyte|lymphocytes]] are atypical (Sezary) [[Cell (biology)|cell]]<nowiki/>s B0a [[Clone]] negative B0b [[Clone (cell biology)|Clone]] positive
|-
| align="center" style="background:#ADD8E6;" |B1
|Low [[blood]] [[Tumor|tumour]] burden: >5% of [[T-cell lymphoma classification|peripheral]] [[blood]] [[Lymphocyte|lymphocytes]] are atypical (Sezary) cells but does not meet the [[criteria]] of B2 B1a Clone negative B1b [[Clone (cell biology)|Clone]] positive
|-
| align="center" style="background:#ADD8E6;" |B2
|High [[blood]] tumour burden: 1000/mL Sezary [[Cell (biology)|cells]] with positive clone
|}
|}


{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
The staging of [[Sezary syndrome]] is based on the [[clinical]] stages:<ref name="TrautingerEder2017" /><ref name="JawedMyskowski2014">{{cite journal|last1=Jawed|first1=Sarah I.|last2=Myskowski|first2=Patricia L.|last3=Horwitz|first3=Steven|last4=Moskowitz|first4=Alison|last5=Querfeld|first5=Christiane|title=Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)|journal=Journal of the American Academy of Dermatology|volume=70|issue=2|year=2014|pages=205.e1–205.e16|issn=01909622|doi=10.1016/j.jaad.2013.07.049}}</ref>
|+ '''Cutaneous T cell lymphoma classification<ref name="seer.cancer">Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016</ref>'''
 
! style="background: #4479BA;; color:#FFF;" | Name
{| class="wikitable"
! style="background: #4479BA;; color:#FFF;" | Description
|+
! colspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" !colspan="3" |clinical stages
|-
| align="center" style="background:#4479BA;" |Stage
! colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;" |T
! colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;" |N
! colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;" |M
! colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;" |B
! colspan="1" style="background: #B0C4DE; color:#FFFFFF; text-align: center;" |DDS
|-
| align="center" style="background:#ADD8E6;" |IA
|1
|0
|0
|0/1
|98
|-
| align="center" style="background:#ADD8E6;" |IB
|2
|0
|0
|0/1
|89
|-
| align="center" style="background:#ADD8E6;" |IIA
|1.2
|1.2
|0
|0/1
|89
|-
| align="center" style="background:#ADD8E6;" |IIB
|3
|0-2
|0
|0/1
|56
|-
| align="center" style="background:#ADD8E6;" |IIIA
|4
|0-2
|0
|0
|54
|-
| align="center" style="background:#ADD8E6;" |IIIB
|4
|0-2
|0
|1
|48
|-
| align="center" style="background:#ADD8E6;" |IVA1
|1-4
|0-2
|0
|2
|41
|-
|-
| style="text-align: center; padding: 5px 5px; background: #F5F5F5;" | '''Primary or cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma''' 
| align="center" style="background:#ADD8E6;" |IVA2
| style="padding: 5px 5px; background: #F5F5F5;" |  
|1-4
* Characterized by localized or disseminated eruptive papules, nodules with tumors showing central ulceration, and necrosis or by superficial hyperkeratotic patches and plaques
|3
* Dissemination to other visceral sites (lung, testis, CNS, and oral mucosa) 
|0
* Lymph nodes are seldom affected
|0-2
* Aggressive clinical course with median survival rate of 32 months
|23
 
|-
|-
| style="text-align: center; padding: 5px 5px; background: #F5F5F5;" | ''' Primary cutaneous CD4-positive small/medium T-cell lymphoma''' 
| align="center" style="background:#ADD8E6;" |IVB
| style="padding: 5px 5px; background: #F5F5F5;" |  
|1-4
* Clinical presentation is usually a solitary plaque or nodule, commonly on the face, neck, or upper trunk
|0-3
* The involvement of lower extremities is rare
|1
* Cutaneous patches are generally absent
|0-2
|18
|}
|}
*[5-year [[disease]] free [[survival]] (DSS)]
* [[Cancer]] has spread to other [[Organ (anatomy)|organs]] in the [[Human body|body]], including the [[blood]] and [[bone marrow]]
* [[Lymph node|Lymph nodes]] may be enlarged and may contain [[cancer]]


==References==
==References==

Latest revision as of 13:12, 7 February 2019

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

Overview

There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes. Cutaneous T cell lymphoma may be classified into several subtypes based on WHO-EORTC classification.

Classification

According to world Health Organization (WHO) and European Organization for Research and Treatment of Cancer (EORTC) classification, mycosis fungoides may be classified into the following types:[1][2]


Cutaneous T cell lymphoma classification[3]
Name Description
Mycosis fungoides (MF)
  • Most common form of cutaneous T cell lymphoma
  • Starts in the skin in areas of the body not usually exposed to the sun
  • May appear as a scaly, pink patches on the skin
  • Signs can progress to the development of skin tumors in more advanced cases

Staging

  • The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.[3]
  • Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:[4][5]
Staging for mycosis fungoides and Sezary syndrome
Skin (T)
T1 Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) versus T1b (plaque patch)
T2 Patches, papules, or plaques covering 10% of the skin surface. May further stratify into T2a (patch only) versus T2b (plaque patch).
T3 One or more tumours (1-cm diameter)
T4 Confluence of erythema covering 80% body surface area
Node (N)
N0 No clinically abnormal peripheral lymph nodes; biopsy not required
N1 Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2
N1a Clone negative
N1b Clone posetive
N2 Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3
N2a Clone negatove
N2b Clone posetive
N3 Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3e4 or NCI LN4; clone positive or negative
NX Clinically abnormal peripheral lymph nodes; no histologic confirmation
Visceral (M)
M0 No visceral organ involvement
M1 Visceral involvement (must have pathology confirmation and organ involved should be specified)
Blood (B)
B0 0 Absence of significant blood involvement: 5% of peripheral blood lymphocytes are atypical (Sezary) cells B0a Clone negative B0b Clone positive
B1 Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Sezary) cells but does not meet the criteria of B2 B1a Clone negative B1b Clone positive
B2 High blood tumour burden: 1000/mL Sezary cells with positive clone

The staging of Sezary syndrome is based on the clinical stages:[4][6]

clinical stages
Stage T N M B DDS
IA 1 0 0 0/1 98
IB 2 0 0 0/1 89
IIA 1.2 1.2 0 0/1 89
IIB 3 0-2 0 0/1 56
IIIA 4 0-2 0 0 54
IIIB 4 0-2 0 1 48
IVA1 1-4 0-2 0 2 41
IVA2 1-4 3 0 0-2 23
IVB 1-4 0-3 1 0-2 18

References

  1. ↑ Matutes, E. (2018). "The 2017 WHO update on mature T- and natural killer (NK) cell neoplasms". International Journal of Laboratory Hematology. 40: 97–103. doi:10.1111/ijlh.12817. ISSN 1751-5521.
  2. ↑ Sundram, Uma (2018). "Cutaneous Lymphoproliferative Disorders". Advances In Anatomic Pathology: 1. doi:10.1097/PAP.0000000000000208. ISSN 1072-4109.
  3. ↑ 3.0 3.1 Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016
  4. ↑ 4.0 4.1 Trautinger, Franz; Eder, Johanna; Assaf, Chalid; Bagot, Martine; Cozzio, Antonio; Dummer, Reinhard; Gniadecki, Robert; Klemke, Claus-Detlev; Ortiz-Romero, Pablo L.; Papadavid, Evangelia; Pimpinelli, Nicola; Quaglino, Pietro; Ranki, Annamari; Scarisbrick, Julia; Stadler, Rudolf; Väkevä, Liisa; Vermeer, Maarten H.; Whittaker, Sean; Willemze, Rein; Knobler, Robert (2017). "European Organisation for Research and Treatment of Cancer consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome – Update 2017". European Journal of Cancer. 77: 57–74. doi:10.1016/j.ejca.2017.02.027. ISSN 0959-8049.
  5. ↑ Olsen E, Vonderheid E, Pimpinelli N, Willemze R, Kim Y, Knobler R, Zackheim H, Duvic M, Estrach T, Lamberg S, Wood G, Dummer R, Ranki A, Burg G, Heald P, Pittelkow M, Bernengo MG, Sterry W, Laroche L, Trautinger F, Whittaker S (September 2007). "Revisions to the staging and classification of mycosis fungoides and Sezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the cutaneous lymphoma task force of the European Organization of Research and Treatment of Cancer (EORTC)". Blood. 110 (6): 1713–22. doi:10.1182/blood-2007-03-055749. PMID 17540844.
  6. ↑ Jawed, Sarah I.; Myskowski, Patricia L.; Horwitz, Steven; Moskowitz, Alison; Querfeld, Christiane (2014). "Primary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome)". Journal of the American Academy of Dermatology. 70 (2): 205.e1–205.e16. doi:10.1016/j.jaad.2013.07.049. ISSN 0190-9622.


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