Mycosis fungoides natural history, complications and prognosis: Difference between revisions

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{{Mycosis fungoides}}
{{Mycosis fungoides}}
{{CMG}}; {{AE}} {{AS}}, {{S.G.}}
{{CMG}}; {{AE}} {{S.G.}}
==Overview==
==Overview==
If left untreated, cutaneous T cell lymphoma may progress to develop cutaneous patches, plaque, and tumors. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.
If left untreated, [[cutaneous T cell lymphoma|mycosis fungoides]] may progress to develop [[cutaneous]] [[Patched|patches]], [[plaque]], and [[Tumor|tumors]]. Depending on the extent of the [[lymphoma]] at the time of [[diagnosis]], the [[prognosis]] may vary.
==Natural History==
==Natural History==
* Mycosis fungoides is initially an indolent lymphoma that may later develop peripheral lymphadenopathy and can finally progress to widespread visceral involvement.<ref name="radio">Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 20, 2016</ref>
*The [[Symptom|symptoms]] of mycosis fungoides usually develop as non-specific, [[Indolent ulcer|indolent]] inflammation such as etopic [[dermatitis]], nonspecific [[Chronic (medical)|chronic]] [[dermatitis]], or [[parapsoriasis]] ( large-[[plaque]] [[parapsoriasis]]).<ref name="QuaglinoPimpinelli20123">{{cite journal|last1=Quaglino|first1=Pietro|last2=Pimpinelli|first2=Nicola|last3=Berti|first3=Emilio|last4=Calzavara-Pinton|first4=Piergiacomo|last5=Alfonso Lombardo|first5=Giuseppe|last6=Rupoli|first6=Serena|last7=Alaibac|first7=Mauro|last8=Bottoni|first8=Ugo|last9=Carbone|first9=Angelo|last10=Fava|first10=Paolo|last11=Fimiani|first11=Michele|last12=Mamusa|first12=Angela Maria|last13=Titli|first13=Stefano|last14=Zinzani|first14=Pier Luigi|last15=Bernengo|first15=Maria Grazia|title=Time course, clinical pathways, and long-term hazards risk trends of disease progression in patients with classic mycosis fungoides|journal=Cancer|volume=118|issue=23|year=2012|pages=5830–5839|issn=0008543X|doi=10.1002/cncr.27627}}</ref>
* Cutaneous T cell lymphoma is usually initially seen by dermatologists with patients presenting with skin lesions such as erythematous patches or plaque.
*The majority of [[Patient|patients]] have early stage (77.9% : IA stage IA 38.8%, stage IB  39.1% ), and [[Patient|patients]] with stage IIB diagnosed in 5.5% and stage III observed in 6.6%. The majority of patients of mycosis fungoides are not diagnosed and end stage not be observed.<ref name="QuaglinoPimpinelli20123" />
* Patients often have a history of several years of eczematous or dermatitic skin lesions before the diagnosis is finally established.
*Mycosis fungoides in early stage is characterized by non-specific [[dermatitis]] or consistent [[Patched|patches]] observed on the lower [[trunk]] and [[buttocks]].<ref name="QuaglinoPimpinelli20123" />
* The skin lesions then progress from the patch stage to the [[plaque]] stage to cutaneous tumors.
* Mycosis fungoides initiates as an [[Indolent ulcer|indolent]] [[lymphoma]] that may later develop peripheral [[lymphadenopathy]] and may finally progress to widespread visceral involvement.<ref name="radio">Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 20, 2016</ref>
* [[Patient|Patients]] often have a history of several years of [[Eczema|eczematous]] or [[dermatitis]] [[skin]] [[Lesion|lesions]] before the diagnosis is finally established.<ref name="QuaglinoPimpinelli20123" />
* The [[skin]] [[Lesion|lesions]] then progress from the patch stage to the [[plaque]] stage to [[cutaneous]] [[Tumor|tumors]].<ref name="QuaglinoPimpinelli20123" />
 
==Complications==
==Complications==
*Common complications of Cutaneous T cell lymphoma include:
*Common complications of mycosis fungoides include:<ref name="pmid28832009">{{cite journal |vauthors=Cengiz FP, Emiroğlu N, Onsun N |title=Frequency and Risk Factors for Secondary Malignancies in Patients with Mycosis Fungoides |journal=Turk J Haematol |volume=34 |issue=4 |pages=378–379 |date=December 2017 |pmid=28832009 |pmc=5774354 |doi=10.4274/tjh.2017.0234 |url=}}</ref><ref name="LawTeicher2003">{{cite journal|last1=Law|first1=Meng|last2=Teicher|first2=Noah|last3=Zagzag|first3=David|last4=Knopp|first4=Edmond A.|title=Dynamic contrast enhanced perfusion MRI in mycosis fungoides|journal=Journal of Magnetic Resonance Imaging|volume=18|issue=3|year=2003|pages=364–367|issn=1053-1807|doi=10.1002/jmri.10361}}</ref><ref name="BassunerMiranda2016">{{cite journal|last1=Bassuner|first1=Juri|last2=Miranda|first2=Roberto N.|last3=Emge|first3=Drew A.|last4=DiCicco|first4=Beau A.|last5=Lewis|first5=Daniel J.|last6=Duvic|first6=Madeleine|title=Mycosis Fungoides of the Oral Cavity: Fungating Tumor Successfully Treated with Electron Beam Radiation and Maintenance Bexarotene|journal=Case Reports in Dermatological Medicine|volume=2016|year=2016|pages=1–7|issn=2090-6463|doi=10.1155/2016/5857935}}</ref><ref name="pmid6640491">{{cite journal |vauthors=Price NM, Hoppe RT, Deneau DG |title=Ointment-based mechlorethamine treatment for mycosis fungoides |journal=Cancer |volume=52 |issue=12 |pages=2214–9 |date=December 1983 |pmid=6640491 |doi= |url=}}</ref><ref name="pmid3722479">{{cite journal |vauthors=Abel EA, Sendagorta E, Hoppe RT |title=Cutaneous malignancies and metastatic squamous cell carcinoma following topical therapies for mycosis fungoides |journal=J. Am. Acad. Dermatol. |volume=14 |issue=6 |pages=1029–38 |date=June 1986 |pmid=3722479 |doi= |url=}}</ref>
**Mycosis Fungoides increased risk of secondary malignancies such as primary malignancy, especially Hodgkin lymphoma, chronic leukemia, and lung cancer.<ref name="pmid28832009">{{cite journal |vauthors=Cengiz FP, Emiroğlu N, Onsun N |title=Frequency and Risk Factors for Secondary Malignancies in Patients with Mycosis Fungoides |journal=Turk J Haematol |volume=34 |issue=4 |pages=378–379 |date=December 2017 |pmid=28832009 |pmc=5774354 |doi=10.4274/tjh.2017.0234 |url=}}</ref>
**Mycosis Fungoides increases risk of secondary [[malignancies]] such as primary [[malignancy]], especially [[Hodgkin's lymphoma|Hodgkin lymphoma]], [[Chronic (medical)|chronic]] [[leukemia]], and [[lung]] [[cancer]].
**[Complication 2]
**[[Neurologic]] involvement (intracerebral mycosis fungoides)
**[Complication 3]
**[[Oral]] involvement
**[[Hypersensitivity]] reaction with [[topical]] HN2 [[therapy]]  
**[[Hypopigmentation]] or [[hyperpigmentation]] of treated areas


==Prognosis==
==Prognosis==
* Cutaneous T cell lymphoma is usually a slow-growing (indolent) lymphoma.<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>
* Cutaneous T cell lymphoma is usually a slow-growing (indolent) [[lymphoma]].<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>
* The prognosis for people with cutaneous T cell lymphoma is based on the extent of disease and how the person responds to treatment.  
* The [[prognosis]] for people with [[cutaneous T cell lymphoma]] is based on the extent of [[disease]] and how the person responds to treatment.  
* Although more advanced stages of cutaneous T cell lymphoma may not be cured, the lymphoma can still be controlled with treatment.
* Although more advanced stages of [[cutaneous T cell lymphoma]] may not be cured, the [[Lymphomas|lymphoma]] can still be controlled with treatment
===Favorable prognosis===
===Favorable prognosis===
* Early stage disease
* Early stage [[disease]]
* Lymphoma is confined to the skin
* [[Lymphoma]] is confined to the [[skin]]
===Unfavorable prognosis===
===Unfavorable prognosis===
* More advanced disease
* More advanced [[disease]]
* Lymphoma has spread to lymph nodes
* [[Lymphoma]] has spread to [[Lymph node|lymph nodes]]  
* Lymphoma has spread to other organs
* [[Lymphoma]] has spread to other [[Organ (anatomy)|organs]]
 
 
==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).
|-
| align="center" style="background:#ADD8E6;" |T3
|One or more [[Tumor|tumours]] (1-cm diameter)
|-
| align="center" style="background:#ADD8E6;" |T4
|Confluence of [[erythema]] covering 80% [[body surface area]]
|-
! colspan="3" style="background: #B0C4DE; color: #FFFFFF; text-align: center;" |'''Node (N)'''
|-
| 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
|}
 
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>


{| class="wikitable"
|+
! colspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" !colspan="3" |clinical stages
|-
| align="center" style="background:#6495ED;" |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
|-
| align="center" style="background:#ADD8E6;" |IVA2
|1-4
|3
|0
|0-2
|23
|-
| align="center" style="background:#ADD8E6;" |IVB
|1-4
|0-3
|1
|0-2
|18
|}


*[5-year [[disease]] free [[survival]] (DSS)]
* Cancer has spread to other organs in the body, including the blood and bone marrow
* Lymph nodes may be enlarged and may contain cancer
|}


==References==
==References==

Latest revision as of 13:20, 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

If left untreated, mycosis fungoides may progress to develop cutaneous patches, plaque, and tumors. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.

Natural History

Complications

Prognosis

Favorable prognosis

Unfavorable prognosis


References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 Quaglino, Pietro; Pimpinelli, Nicola; Berti, Emilio; Calzavara-Pinton, Piergiacomo; Alfonso Lombardo, Giuseppe; Rupoli, Serena; Alaibac, Mauro; Bottoni, Ugo; Carbone, Angelo; Fava, Paolo; Fimiani, Michele; Mamusa, Angela Maria; Titli, Stefano; Zinzani, Pier Luigi; Bernengo, Maria Grazia (2012). "Time course, clinical pathways, and long-term hazards risk trends of disease progression in patients with classic mycosis fungoides". Cancer. 118 (23): 5830–5839. doi:10.1002/cncr.27627. ISSN 0008-543X.
  2. ↑ Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 20, 2016
  3. ↑ Cengiz FP, Emiroğlu N, Onsun N (December 2017). "Frequency and Risk Factors for Secondary Malignancies in Patients with Mycosis Fungoides". Turk J Haematol. 34 (4): 378–379. doi:10.4274/tjh.2017.0234. PMC 5774354. PMID 28832009.
  4. ↑ Law, Meng; Teicher, Noah; Zagzag, David; Knopp, Edmond A. (2003). "Dynamic contrast enhanced perfusion MRI in mycosis fungoides". Journal of Magnetic Resonance Imaging. 18 (3): 364–367. doi:10.1002/jmri.10361. ISSN 1053-1807.
  5. ↑ Bassuner, Juri; Miranda, Roberto N.; Emge, Drew A.; DiCicco, Beau A.; Lewis, Daniel J.; Duvic, Madeleine (2016). "Mycosis Fungoides of the Oral Cavity: Fungating Tumor Successfully Treated with Electron Beam Radiation and Maintenance Bexarotene". Case Reports in Dermatological Medicine. 2016: 1–7. doi:10.1155/2016/5857935. ISSN 2090-6463.
  6. ↑ Price NM, Hoppe RT, Deneau DG (December 1983). "Ointment-based mechlorethamine treatment for mycosis fungoides". Cancer. 52 (12): 2214–9. PMID 6640491.
  7. ↑ Abel EA, Sendagorta E, Hoppe RT (June 1986). "Cutaneous malignancies and metastatic squamous cell carcinoma following topical therapies for mycosis fungoides". J. Am. Acad. Dermatol. 14 (6): 1029–38. PMID 3722479.
  8. ↑ 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


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