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Ph RadOnc Residency PBRROE2-3 Review 2021 > Primary Cutaneous Lymphomas > Flashcards

Primary Cutaneous Lymphomas Flashcards

1
Q

Primary Cutaneous Lymphoma

What is the most common primary cutaneous lymphoma?

A

CTCL (cutaneous T-cell lymphoma)

In the United States, 71% of the 3,884 cases of primary cutaneous
lymphoma diagnosed during 2001 to 2005 were CTCL. Similarly, 78% of
primary cutaneous lymphoma diagnoses recorded in the Dutch and Austrian
Cutaneous Lymphoma Group registry over 1986 to 2002 and 85% of diagnoses
in the Central Cutaneous Lymphoma Registry of the German Society of
Dermatology over 1999 to 2004 were CTCL.

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2
Q

Primary Cutaneous Lymphoma
CTCL

What is the most common type of CTCL serving as the archetype?

A

MF (mycosis fungoides)

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3
Q

Primary Cutaneous Lymphoma
CTCL-MF

MF is a disease of skin-homing CD8+ cytotoxic T-cells.

TRUE or FALSE?

A

False.

CD4+ T-helper

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4
Q

Primary Cutaneous Lymphoma
CTCL-MF

MF is more common in men or women?

A

Men (1.6 to 2.0 : 1)

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5
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is the range median age at diagnosis?

A

55 to 60

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6
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is the most common symptom associated with MF?

A

Pruritus, either diffuse or localized to areas of involved skin

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7
Q

Primary Cutaneous Lymphoma
CTCL-MF

Identify the phase of the classic MF:

small number of red, scaled,
macular or patchlike lesions develop in sun-shielded areas of the skin such as the
trunk, pelvis, and extremities

these may regress, followed by development of new lesions

A

premycotic phase

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8
Q

Primary Cutaneous Lymphoma
CTCL-MF

Identify the phase of the classic MF:

durable lesions, persistent cutaneous lesions without induration or significant elevation above the
surrounding uninvolved skin

A

patch phase

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9
Q

Primary Cutaneous Lymphoma
CTCL-MF

Identify the phase of the classic MF:

As the lesions
become more densely infiltrated by both malignant and reactive lymphocytes,
they evolve into lesions with thickened and raised borders

A

plaque phase

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10
Q

Primary Cutaneous Lymphoma
CTCL-MF

Identify the phase of the classic MF:

Development of any solid or nodular lesion ≥1 cm in diameter with evidence of deep
infiltration in the skin and/or vertical growth.

A

tumor phase

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11
Q

Primary Cutaneous Lymphoma
CTCL-MF

Biopsies of premycotic lesions
are often diagnostic because of an increased deposition of malignant lymphocytes in the
skin.

TRUE or FALSE?

A

False.

Biopsies of premycotic lesions
are rarely diagnostic because of a paucity of malignant lymphocytes in the
lesion

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12
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is the disease characterized by CTCL (typically MF-erythroderma) with identified T-cells in the peripheral blood also known as leukemic CTCL?

A

Sézary syndrome

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13
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is B0 in characterization of tumor burden in the peripheral blood?

A

≤5% atypical or Sezary cells

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14
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is B1 in characterization of tumor burden in the peripheral blood?

A

> 5% atypical cells but doesn’t meet B2 criteria

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15
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is B2 in characterization of tumor burden in the peripheral blood?

A

presence of a dominant T-cell clone + ≥1000 Sezary cells/mm

increased CD3+ or CD4+ T cells with CD4:CD8 ratios >10, or increased quantities of abnormal T cells.

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16
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is a less common but pathognomonic histologic finding associated with MF?

A

Pautrier microabscess

Langerhans cell surrounded by atypical T cells in the epidermis

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17
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is the treatment for patients with stage IA MF present with “minimal”
disease, defined as a solitary lesion or two to three MF lesions clustered
sufficiently close to one another?

A

Local superficial irradiation

18
Q

Primary Cutaneous Lymphoma
CTCL-MF

In local superficial irradiation, where do you set the 80% isodose line?

A

deep border of the dermis, usually 4.5 mm depth

19
Q

Primary Cutaneous Lymphoma
CTCL-MF

In delivering TSEBT, what is the distance of the patient from the linear accelerator head?

A

3-8 m from the source

20
Q

Primary Cutaneous Lymphoma
CTCL-MF

This device is used to scatter the incident electron beam and contributes to an improved surface dose in TSEBT.

A

Polycarbonate screen.

21
Q

Primary Cutaneous Lymphoma
CTCL-MF

Describe the treatment “cycle” in TSEBT.

A

1 cycle over 2 days.
2 Gy per cycle.
2 cycles per week.

Six positions per cycle
3 positions per day
(anterior and 2 posterior obliques, then posterior and 2 anterior obliques)

22
Q

Primary Cutaneous Lymphoma
CTCL-MF

What are the primary targets for TSEBT?
(EORTC)

A

epidermis, adnexal structures, and dermis

23
Q

Primary Cutaneous Lymphoma
CTCL-MF

What should be the doses to 20 mm deep to the skin and the curved skin sites in relation to the prescribed skin dose in TSEBT?
(EORTC)

A

The 80% isodose line should extend to 4 mm below the skin surface, the dose 20 mm deep to the skin must be <20% of the maximum dose at the skin, and the dose at curved skin sites must not exceed 120% of the prescribed skin dose.

24
Q

Primary Cutaneous Lymphoma
CTCL-MF

In TSEBT (EORTC), the goal is to deliver a total dose of _______ Gy to a depth of 4 mm below the skin surface which translates to a truncal dose of 31 to 36 Gy.

A

26–28 Gy

25
Q

Primary Cutaneous Lymphoma
CTCL-MF

3 areas that need potential supplemental treatments after TSEBT

A

scalp
perineum
soles

26
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is the adjuvant treatment after TSEBT in patients with T2 disease?

A

PUVA or nitrogen mustard topical treatment

27
Q

Primary Cutaneous Lymphoma
CTCL-MF

When should supplemental boosts should be considered?

such boost treatment should be provided concomitantly with the initiation of TSEBT or prior to its initiation.

A

for patients with cutaneous tumors (T3)

The purpose of the boost is to
diminish the thickness of the lesion so that electrons from TSEBT can effectively
penetrate the entire lesion.

28
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is the palliative RT dose for MF?

A

low dose RT
starts at 10-12 Gy…
can be up to 30 Gy.

29
Q

Primary Cutaneous Lymphoma
CTCL-MF

What are the first-line treatment options for patients with stage IA, IB, IIA MF?

A
  • Expectant observation (IA)
  • Topical therapies
  • Phototherapy
  • TSEBT (consider adjuvant PUVA or nitrogen mustard)
30
Q

Primary Cutaneous Lymphoma
CTCL-MF

What are the treatment options for patients with relapsed/refractory stage IA, IB, IIA MF?

A

-Second course TSEBT
-Oral bexarotene
-IFN-α
-Low-dose MTX
-Vorinostat
-Denileukin diftitox
-Clinical trials
NOTE: Chemotherapy is
not recommended

31
Q

Primary Cutaneous Lymphoma
CTCL-MF

What are the first-line treatment options for patients with stage IIB MF?

A

-TSEBT (consider boosts to cutaneous tumors and adjuvant PUVA or nitrogen mustard

  • IFN-α
  • PUVA
32
Q

Primary Cutaneous Lymphoma
CTCL-MF

What are the first-line treatment options for patients with stage IIIA, IIIB, or SS MF?

A
  • TSEBT + ECP
  • ECP alone
  • IFN-α
  • PUVA + IFN-α
  • MTX
33
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is the role of TSEBT in stage IV MF?

A

Palliation

34
Q

Primary Cutaneous Lymphoma
CTCL-MF

What is an extremely rare variant of MF, with <100 cases reported in the literature.

It is characterized by cutaneous infiltration of a clonal population of malignant T cells coupled with a granulomatous infiltration.
The granulomatous infiltrate ultimately causes destruction of local elastin fibers and impairs the structural integrity and elasticity of the skin, which results in loose skin.

A

Granulomatous slack skin (GSS)

35
Q

Primary Cutaneous Lymphoma
CTCL-MF

What variant of MF is very slowly progressive and carries an excellent prognosis?

A

Woringer-Kolopp disease (pagetoid reticulosis)

36
Q

Primary Cutaneous Lymphoma
CBCL

What are three common types of CBCL?

A

PCMZL
PCFCL
PCLBCL-LT

37
Q

Primary Cutaneous Lymphoma
CBCL

t(14:18), as seen in systemic follicular lymphoma is also a characteristic finding in primary cutaneous follicle center lymphoma.

TRUE or FALSE?

A

FALSE

Importantly, the chromosomal translocation t(14:18) is infrequently seen in PCFCL, and therefore, detection of this translocation should prompt consideration of a nodal or systemic follicular lymphoma.

38
Q

Primary Cutaneous Lymphoma
CBCL

What type of CBCL has the highest rate of relapse, extracutaneous involvement, and worse OS?

A

PCLBCL-LT

39
Q

Primary Cutaneous Lymphoma
CBCL

As the nomenclature implies, PCLBCL, leg type is restricted to the development of violaceous cutaneous tumors over the lower extremities.

TRUE or FALSE?

A

False

40
Q

Primary Cutaneous Lymphoma
CBCL

PCMZL and PCFCL

definitive RT dose?

A

24 to 30 Gy

41
Q

Primary Cutaneous Lymphoma
CBCL

PCMZL and PCFCL

Margins for RT?

A

1 to 1.5 cm

42
Q

Primary Cutaneous Lymphoma
CBCL

PCLBCL-LT

Management strategies?

A

multiagent chemotherapy (R-CHOP or R-CHOP-like)

+ LSI

(rituximab for those unable to tolerate a multiagent course of chemotherapy).

Ph RadOnc Residency PBRROE2-3 Review 2021 (57 decks)

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