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

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==Complications==
==Complications==
*Common complications of mycosis fungoides 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 increases risk of secondary [[malignancies]] such as primary [[malignancy]], especially [[Hodgkin's lymphoma|Hodgkin lymphoma]], [[Chronic (medical)|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]].
**[[Neurologic]] involvement (intracerebral mycosis fungoides)<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>
**[[Neurologic]] involvement (intracerebral mycosis fungoides)
**[[Oral]] involvement<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>
**[[Oral]] involvement
**[[Hypersensitivity]] reaction with [[topical]] HN2 [[therapy]] <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>
**[[Hypersensitivity]] reaction with [[topical]] HN2 [[therapy]]
**[[Hypopigmentation]] or [[hyperpigmentation]] of treated areas<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>
**[[Hypopigmentation]] or [[hyperpigmentation]] of treated areas


==Prognosis==
==Prognosis==

Revision as of 18:17, 6 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


Staging

  • The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.[8]
  • Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:[9][10]
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:[9][11]

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. 1.0 1.1 1.2 1.3 1.4 1.5 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. 8.0 8.1 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
  9. 9.0 9.1 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.
  10. 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.
  11. 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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