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Flashcards in Skin Cancer 3 Deck (33)
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1
Q

What are 75% of most cutaneous lymphomas?

A

T cell

2
Q

What are cutaneous T cell lymphoma?

A
  1. Heterogenous group of neoplasms of skin-homing T-cells

2. show considerable variation in clinical presentation, histological appearance, immunophenotype and prognosis

3
Q

What are the most common subtypes of cutaneous T cell lymphoma?

A
  • Sezary syndrome

- Mycosis fungoides

4
Q

What is the underlying molecular pathogenesis of CTCL?

A
  • unknown

- inactivation of genes controlling cell cycle and apoptosis has been identified

5
Q

What is the epidemiology of T cell lymphoma?

A
  • Mycosis fungoides 0.4/100,000
  • Typically older adults (median age of diagnosis 55-60)
  • Sézary syndrome is rare - <5% of all CTCL
6
Q

What is mycosis fungoides?

A

Common variant of primary CTCL and accounts for 50% of all primary cutaneous lymphoma

7
Q

What does diagnosis of cutaneous T cell lymphoma require?

A
  • Indolent clinical course

* Diagnosis requires skin biopsy

8
Q

How long can diagnosis of mycosis fungiodes take?

A
  • Diagnosis may take years as skin lesions may be present that are neither clinically nor histologically diagnostic for many years
  • Atypical T-cell infiltrates may also be found in lymphomatoid drug eruptions
  • Patches or plaques
9
Q

What is the progression of mycosis fungiodes?

A

patch stage → plaque stage → (finally) tumour stage disease

years to decades

10
Q

What is the duration of mycosis fungoides?

A
  • Generally many years of nonspecific eczematous or psoriasiform skin lesions
  • Median duration of onset of skin lesions to diagnosis of MF is 4-6 years, but may vary from several months to more than 5 decades
11
Q

How is the early patch stage of mycosis fungoides characterised?

A

variably sized erythematous, finely scaling lesions which may be mildly pruritic

12
Q

What is the pathogenesis of mycosis fungoides?

A

stepwise accumulation of genetic abnormalities → clonal proliferation → malignant transformation → progressive and widely disseminated disease

13
Q

What are the molecular events in mycosis fungoides?

A

unidentified

14
Q

What are the genetic abnormalities of Mycosis fungoides?

A
  • P53, CDKN2A, PTEN, STAT3 identified in advanced MF, but not early
    e. g. likely secondary genetic events
  • Persistent antigenic stimulation plays a crucial role in various lymphomas but no antigens known in MF
15
Q

What evaluation and investigation is needed in mycosis fungoides?

A
  1. Type and extent of skin lesions
  2. Presence of palpable lymph nodes
  3. Skin biopsies
  4. Complete blood counts and serum chemistries
16
Q

How do you treat the mycosis fungoides plaque/patch stage?

A
  • topical corticosteroids
  • phototherapy
  • radiotherapy
17
Q

When do you give systemic chemotherapy in mycosis fungoides?

A

-advanced stage when there is nodal or visceral involvement or in patients with rapidly progressive tumours unresponsive to less aggressive therapies
•Brentuximab vedotin (anti-30)

18
Q

What are the 10 year survival rates in mycosis fungoides?

A
  1. > 95% in limited patch / plaque disease
  2. 85% in generalised patch / plaque disease
  3. 42% in tumour stage disease
  4. 20% in those with histological lymph node involvement
19
Q

What are the differential diagnosis for mycosis fungoides?

A
  • psoriasis
  • eczema (discoid)
  • parapsoriasis
20
Q

What is the triad in sezary syndrome?

A
  1. Erythroderma
  2. Generalised lymphadenopathy
  3. Presence of neoplastic T-cells (Sézary cells) in the skin, lymph nodes and peripheral blood
21
Q

What is sezary syndrome?

A

cutaneous T cell lymphoma

22
Q

What is the criteria for diangosis of sezary syndrome?

A
  1. Demonstration of a T-cell clone in peripheral blood (eventhough skin) by molecular or cytogenetic methods
  2. Demonstration of immunophenotypical abnormalities an expanded CD4+ T-cell population – resulting in a CD4/ CD8 ratio of greater than 10 and / or aberrant expression of pan-T-cell antigens)
  3. An absolute Sézary cell count of at least 1000 cells per microlitre
23
Q

What is the treatment of sezary syndrome?

A
  1. Systemic treatment is required
  2. Extracorporeal photophoresis
  3. Skin-directed therapies like PUVA or potent topical corticosteroids may be used as adjuvant therapy
24
Q

What is kaposi sarcoma?

A
  • HHV8

* Multifocal systemic disease

25
Q

What can kaposi sarcoma look like?

A
  • May be endemic or related to immunosuppression

* Cutaneous lesions can vary from pink patches to dark violet plaques, nodules or polyps

26
Q

What is the treatment of kaposi sarcoma?

A
  1. chemotherapy (vincristine, doxorubicin, etoposide, bleomycin)
  2. and / or radiation is favoured over surgery
27
Q

What is merkel cell carcinoma?

A

malignant proliferation of highly anaplastic cells which share structural and immunohistochemical features with various neuroectodermally derived cells, including Merkel cells

28
Q

What is merkel cell carcinoma associated with?

A

80% are associated with polyomavirus

29
Q

What is an aetiological factor for merkel cell carcinoma?

A

UV exposure

30
Q

Where is merkel cell carcinoma?

A

predilection for the head and neck region of older adults

31
Q

What does merkel cell carcinoma look like?

A
  • solitary, rapidly growing nodule
  • pink-red to violaceous, firm, dome shaped
  • Ulceration can occur
32
Q

Is merkel cell carcinoma aggressive?

A
  • Aggressive, malignant behaviour

* >40% develop advanced disease

33
Q

What is the treatment of merkel cell carcinoma?

A
  1. Treated with surgery, radiation therapy
  2. anti-PD1 (Pembrolizumab) / anti-PDL1 (Avelumab)
    - checkpoint inhibitors when metastasis present