Mycosis fungoides natural history, complications and prognosis
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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, 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.
Natural History
- The symptoms of mycosis fungoides usually develop as non-specific, indolent inflammation such as etopic dermatitis, nonspecific chronic dermatitis, or parapsoriasis ( large-plaque parapsoriasis).[1]
- The majority of patients have early stage (77.9% : IA stage IA 38.8%, stage IB 39.1% ), and patients with stage IIB diagnosed in 5.5% and stage III observed in 6.6%.[1]
- The majority of patients of mycosis fungoides are not diagnosed and end stage not be observed.[1]
- Mycosis fungoides in early stage is characterized by non-specific dermatitis or consistent patches observed on the lower trunk and buttocks.[1]
- Mycosis fungoides initiates as an indolent lymphoma that may later develop peripheral lymphadenopathy and may finally progress to widespread visceral involvement.[2]
- Patients often have a history of several years of eczematous or dermatitis skin lesions before the diagnosis is finally established.
- The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumors.
Complications
- Common complications of mycosis fungoides include:
- Mycosis Fungoides increases risk of secondary malignancies such as primary malignancy, especially Hodgkin lymphoma, chronic leukemia, and lung cancer.[3]
- Neurologic involvement (intracerebral mycosis fungoides)[4]
- Oral involvement[5]
- Hypersensitivity reaction with topical HN2 therapy [6]
- Hypopigmentation or hyperpigmentation of treated areas[7]
Prognosis
- Cutaneous T cell lymphoma is usually a slow-growing (indolent) lymphoma.[8]
- 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
Favorable prognosis
Unfavorable prognosis
- More advanced disease
- Lymphoma has spread to lymph nodes
- Lymphoma has spread to other organs
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 |
- [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
- ↑ 1.0 1.1 1.2 1.3 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.
- ↑ Mycosis fungoides. Radiopaedia.http://radiopaedia.org/articles/mycosis-fungoides Accessed on January 20, 2016
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Price NM, Hoppe RT, Deneau DG (December 1983). "Ointment-based mechlorethamine treatment for mycosis fungoides". Cancer. 52 (12): 2214–9. PMID 6640491.
- ↑ 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.
- ↑ 8.0 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
- ↑ 9.0 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.
- ↑ 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.
- ↑ 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.