Skin Cancer 3 Flashcards

1
Q

What are 75% of most cutaneous lymphomas?

A

T cell

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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

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3
Q

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

A
  • Sezary syndrome

- Mycosis fungoides

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4
Q

What is the underlying molecular pathogenesis of CTCL?

A
  • unknown

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

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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
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6
Q

What is mycosis fungoides?

A

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

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7
Q

What does diagnosis of cutaneous T cell lymphoma require?

A
  • Indolent clinical course

* Diagnosis requires skin biopsy

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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
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9
Q

What is the progression of mycosis fungiodes?

A

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

years to decades

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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
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11
Q

How is the early patch stage of mycosis fungoides characterised?

A

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

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12
Q

What is the pathogenesis of mycosis fungoides?

A

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

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13
Q

What are the molecular events in mycosis fungoides?

A

unidentified

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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
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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
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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
What can kaposi sarcoma look like?
* May be endemic or related to immunosuppression | * Cutaneous lesions can vary from pink patches to dark violet plaques, nodules or polyps
26
What is the treatment of kaposi sarcoma?
1. chemotherapy (vincristine, doxorubicin, etoposide, bleomycin) 2. and / or radiation is favoured over surgery
27
What is merkel cell carcinoma?
malignant proliferation of highly anaplastic cells which share structural and immunohistochemical features with various neuroectodermally derived cells, including Merkel cells
28
What is merkel cell carcinoma associated with?
80% are associated with polyomavirus
29
What is an aetiological factor for merkel cell carcinoma?
UV exposure
30
Where is merkel cell carcinoma?
predilection for the head and neck region of older adults
31
What does merkel cell carcinoma look like?
- solitary, rapidly growing nodule - pink-red to violaceous, firm, dome shaped - Ulceration can occur
32
Is merkel cell carcinoma aggressive?
* Aggressive, malignant behaviour | * >40% develop advanced disease
33
What is the treatment of merkel cell carcinoma?
1. Treated with surgery, radiation therapy 2. anti-PD1 (Pembrolizumab) / anti-PDL1 (Avelumab) - checkpoint inhibitors when metastasis present