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FITZPATRICK'S DERMATOLOGY 9TH EDITION (SHAMMY) > 20 - 111 - BASAL CELL CARCINOMA AND BASAL NEVUS SYNDROME > Flashcards

20 - 111 - BASAL CELL CARCINOMA AND BASAL NEVUS SYNDROME Flashcards

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1
Q

All statements regarding BCC are true, except:

a. Basal cell carcinoma (BCC) is the most common cancer in humans.
b. Men are affected slightly more often than are women.
c. BCC is rare in dark skin
d. BCC develops on sun-exposed skin in older adults, most commonly on the head and hands.

A

ANSWER:D p.1884. BCC develops on sun-exposed skin in older adults, most commonly on the head and neck.

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2
Q

a. Nodular BCC
b. Pigmented BCC
c. Superficial BCC
d. Morpheaform BCC
2. The melanocytes are interspersed between tumor cells and contain numerous melanin granules in their cytoplasm and dendrites.
3. most common clinical subtype of BCC
3. This histologic subtype is encountered most often on the trunk and extremities but may also appear on the head and neck.
4. consists of strands of tumor cells embedded within a dense fibrous stroma.
5. appears as an often well-demarcated erythematous patch

A

B, A, C, D, C, p. 1886-1887

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3
Q

classically presents as a pink papule, usually on the lower back

a. Basosquamous carcinoma
b. Superficial BCC
c. Sclerosing infiltrative BCC
d. Fibroepithelioma of pinkus

A

ANSWER: D. P. 1888 FEP classically presents as a pink papule, usually on the lower back. It may be difficult to distinguish from an acrochordon or skin tag.

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4
Q

it is the treatment of first choice for primary facial BCCs with an aggressive histopathological subtype and for recurrent facial BCCs

a. Moh’s micrographic surgery
b. Standard excision
c. Curettage and dessication
d. 5-Fluorouracil

A

ANSWER: A. Based on the fact that MMS provides the lowest recurrence. it is the treatment of first choice for primary facial BCCs with an aggressive histopathological sub- type and for recurrent facial BCCs

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5
Q

Approved by the FDA in 2012 for patients with locally advanced or metastatic BCC.

A

Vismodegib was approved by the FDA in 2012 for patients with locally advanced or metastatic BCC.

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6
Q

All are most characteristic abnormalities in BCNS, except:

a. Medulloblastomas or BCCs
b. Pits of the palms and soles,
c. PTCH gene mutation
d. odontogenic cysts of the jaw.

A

ANSWER: C. P.1891 In BCNS, the three most characteristic abnormalities are tumors such as medulloblastomas or BCCs, pits of the palms and soles, and odontogenic cysts of the jaw.

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7
Q

All are major criteria in BCNS except:

a. Medulloblastoma
b. Plantar pits
c. First-degree relative with BCNS
d. Splayed ribs

A

ANSWER:A. P.1895 table 111-4.

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8
Q
A
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9
Q

T/F. All BCCs have mutations activating the Hedgehog signaling pathway.

A

True

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10
Q

Risk factors for BCC

A
  • ultraviolet radiation (UVR) exposure
  • light hair and eye color
  • northern European ancestry
  • inability to tan
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11
Q

Common mutations that activate the aberrant Hedgehog signaling pathway

A
  • loss of PTCH1 or Suppressor of Fused (SUFU) and activation of Smoothened (SMO)
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12
Q

commonly seen features of BCC

A

translucency, ulceration, telangiectasias, and the presence of a rolled border

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13
Q

Most common type of BCC

A
  1. Nodular
  2. Superficial
  3. Morpheaform
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14
Q

Nodular and morpheaform are most commonly found where?

A

head and necks

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15
Q

Superficial BCC is most often found where?

A

Trunk

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16
Q

Most Common Genes Mutated in Sporadic Basal Cell Carcinoma

A
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17
Q

Most common gene mutated in BCC

A

PTCH1

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18
Q

Enumerate the BCC subtypes

A
  1. Nodular BCC
  2. Pigmented BCC
  3. Superficial BCC
  4. Morpheaform/ Sclerosing/ Infiltrative BCC
  5. Basosquamous Carcinoma
  6. Fibroepithelioma of Pinkus
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19
Q

subtype of nodular BCC that exhibits increased melanization

A

Pigmented BCC

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20
Q

The lesions of this type of BCC may have an ivorywhite appearance and may resemble a scar

A

MORPHEAFORM (SCLEROSING, INFILTRATIVE BASAL CELL CARCINOMA)

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21
Q

Characterized histologically by strands of tumor cells embedded within a dense fibrous stroma

A

MORPHEAFORM (SCLEROSING, INFILTRATIVE BASAL CELL CARCINOMA)

22
Q
A

Nodular BCC

23
Q
A

Pigmented BCC

24
Q
A

Superficial BCC

25
Q
A

Morpheaform BCC

26
Q

these treatments appear to be the most effective for BCC

A

Surgery and radiotherapy

27
Q

gold standard for treating BCC

A

removal of the tumor with clear margins

28
Q

What type of BCC will you consider nonsurgical treatment?

A
29
Q

What is the FDA-approved therapy for metastatic BCC?

A

Vismodegib

30
Q

What are the FDA-approved therapy for locally advanced BCC?

A

Vismodegib, Sonidegib

31
Q
A
32
Q

treatment of choice for morpheaform, poorly delineated, incompletely removed, high-risk primary BCCs and recurrent BCCs

A

MOHS MICROGRAPHIC SURGERY

33
Q
A
34
Q

treatment of first choice for primary facial BCCs with an aggressive histopathological subtype and for recurrent facial BCCs

A

MOHS MICROGRAPHIC SURGERY

35
Q

Two freeze–thaw cycles with a tissue temperature of _________ are required to destroy BCC

A

−50°C (−58°F)

36
Q

Approved in 2004 by the U.S. Food and Drug Administration (FDA) for the topical treatment of biopsy-confirmed, small (<2 cm), primary superficial BCC

A

Imiquimod

37
Q

T/F. The more severe the local skin reaction (erythema, erosion, and crusting), the higher the clearance rate.

A

True

38
Q

Imiquimod is FDA approved only for what type of BCC?

A

Superficial BCC

39
Q

may be a very useful modality as adjunct treatment for BCC when margins are positive after excision or for extensive perineural or large nerve involvement

A

Radiation Therapy

40
Q

this term is applied to BCCs that cannot be adequately excised without significant functional impairment or tumors that cannot be treated with radiotherapy.

A

Locally advanced BCCs

41
Q

The most common sites of metastasis of BCC

A

regional lymph nodes, lungs, and bone

42
Q

What are the risk factors for metastasis

A

Aggressive histologic characteristics, including morpheaform features, squamous metaplasia, and perineural invasion

43
Q

The median survival period of patients with metastatic BCC

A

10 months

44
Q

approved by the FDA in 2012 for patients with locally advanced or metastatic BCC.

A

Vismodegib

45
Q

An estimated how many percent of patients with primary BCC will develop at least one or more further BCCs within 5 years?

A

40% to 50%

46
Q

Recent clinical trials have highlighted a potential role of these 2 drugs in producing some decrease in BCC risk

A
  • nicotinamide 500 mg BID
  • celecoxib (NSAID)
47
Q

Patients with basal cell nevus syndrome (BCNS, Gorlin syndrome) inherit an inactivating mutation in what gene?

A

PTCH1/ PATCHED1 gene (residing on the long arm of chromosome 9)

48
Q

mode of inheritance of basal cell nevus syndrome (BCNS, Gorlin syndrome)

A

Autosomal Dominant

49
Q

In BCNS, wha are the three most characteristic tumor abnormalities?

A
  1. Medulloblastoma or BCCs
  2. Pits of the palms and soles
  3. Odontogenic Cysts of the jaw
50
Q

Major Criteria for BCCN

A
  1. Basal cell carcinoma before age 20 yr
  2. Odontogenic keratocysts before age 15 yr
  3. Three or more palmar or plantar pits
  4. Bilamellar calcification of the falx cerebri (if younger than 20 yr)
  5. Fused, bifid, or markedly splayed ribs
  6. First-degree relative with BCNS
  7. PTCH gene mutation in normal tissue
51
Q

Minor Criteria for BCCN

A
  1. Macrocephaly determined after adjustment for height
  2. Congenital malformations: cleft lip or palate, frontal bossing, “coarse face,” moderate or severe hypertelorism
  3. Skeletal abnormalities: Sprengel deformity, marked pectus deformity, or marked syndactyly of the digits
  4. Radiologic abnormalities: bridging of the sella turcica; rib anomalies such as bifid or splayed ribs; vertebral anomalies such as hemivertebrae, fusion, or elongation of the vertebral bodies; modeling defects of the hands and feet; or flame-shaped lucencies of the hands or feet
  5. Ovarian fibroma
  6. Medulloblastoma
52
Q

most effective agent in patients with BCNS

A

oral vismodegib 150 mg/day

FITZPATRICK'S DERMATOLOGY 9TH EDITION (SHAMMY) (122 decks)

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