20 - 111 - BASAL CELL CARCINOMA AND BASAL NEVUS SYNDROME

Question 1

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.

Answer 1

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

Question 2

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

Answer 2

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

Question 3

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

Answer 3

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.

Question 4

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

Answer 4

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

Question 5

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

Answer 5

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

Question 6

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.

Answer 6

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.

Question 7

All are major criteria in BCNS except:

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

Answer 7

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

Question 8

Question 9

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

Answer 9

True

Question 10

Risk factors for BCC

Answer 10

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

Question 11

Common mutations that activate the aberrant Hedgehog signaling pathway

Answer 11

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

Question 12

commonly seen features of BCC

Answer 12

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

Question 13

Most common type of BCC

Answer 13

1. Nodular
2. Superficial
3. Morpheaform

Question 14

Nodular and morpheaform are most commonly found where?

Answer 14

head and necks

Question 15

Superficial BCC is most often found where?

Answer 15

Trunk

Question 16

Most Common Genes Mutated in Sporadic Basal Cell Carcinoma

Answer 16

Question 17

Most common gene mutated in BCC

Answer 17

PTCH1

Question 18

Enumerate the BCC subtypes

Answer 18

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

Question 19

subtype of nodular BCC that exhibits increased melanization

Answer 19

Pigmented BCC

Question 20

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

Answer 20

MORPHEAFORM (SCLEROSING, INFILTRATIVE BASAL CELL CARCINOMA)

Question 21

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

Answer 21

MORPHEAFORM (SCLEROSING, INFILTRATIVE BASAL CELL CARCINOMA)

Question 22

Answer 22

Nodular BCC

Question 23

Answer 23

Pigmented BCC

Question 24

Answer 24

Superficial BCC

Question 25

Answer 25

Morpheaform BCC

Question 26

these treatments appear to be the most effective for BCC

Answer 26

Surgery and radiotherapy

Question 27

gold standard for treating BCC

Answer 27

removal of the tumor with clear margins

Question 28

What type of BCC will you consider nonsurgical treatment?

Answer 28

Question 29

What is the FDA-approved therapy for metastatic BCC?

Answer 29

Vismodegib

Question 30

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

Answer 30

Vismodegib, Sonidegib

Question 31

Question 32

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

Answer 32

MOHS MICROGRAPHIC SURGERY

Question 33

Question 34

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

Answer 34

MOHS MICROGRAPHIC SURGERY

Question 35

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

Answer 35

−50°C (−58°F)

Question 36

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

Answer 36

Imiquimod

Question 37

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

Answer 37

True

Question 38

Imiquimod is FDA approved only for what type of BCC?

Answer 38

Superficial BCC

Question 39

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

Answer 39

Radiation Therapy

Question 40

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

Answer 40

Locally advanced BCCs

Question 41

The most common sites of metastasis of BCC

Answer 41

regional lymph nodes, lungs, and bone

Question 42

What are the risk factors for metastasis

Answer 42

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

Question 43

The median survival period of patients with metastatic BCC

Answer 43

10 months

Question 44

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

Answer 44

Vismodegib

Question 45

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

Answer 45

40% to 50%

Question 46

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

Answer 46

- nicotinamide 500 mg BID - celecoxib (NSAID)

Question 47

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

Answer 47

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

Question 48

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

Answer 48

Autosomal Dominant

Question 49

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

Answer 49

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

Question 50

Major Criteria for BCCN

Answer 50

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

Question 51

Minor Criteria for BCCN

Answer 51

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

Question 52

most effective agent in patients with BCNS

Answer 52

oral vismodegib 150 mg/day

Question 53

pivotal abnormality in all BCCs

Answer 53

Malignant activation of the sonic hedgehog (SHH) signaling pathway

Question 54

majority of BCCs were found to have mutations in HH pathway genes, most commonly what?

Answer 54

PTCH1 (73%) ## Footnote SMO (20%), and SUFU (8%)

Question 55

**Sporadic** BCCs also commonly harbor mutations in what gene?

Answer 55

TP53

Question 56

often are the first detectable abnormality in BCNS

Answer 56

Jaw cysts

Question 57

presumably form from inappropriate SHH induction of dental epithelium and can recur often and be the most debilitating aspect of BCNS

Answer 57

Jaw cysts

Question 58

frequently the initial sign of BCNS.

Answer 58

childhood tumors (such as medulloblastoma)

Question 60

What is the typical latency period between time of UV damage and clinical onset of BCC?

Answer 60

20 - 50 yrs

Question 61

Why is BCC rare in individuals with dark skin?

Answer 61

because of the inherent photoprotection of melanin and melanosomal dispersion

Question 62

T/F: In the US, BCC is 75% of all NMSC and 25% of all cancers overall.

Answer 62

True

Question 63

What are the 4 risk factors for BCC formation?

Answer 63

UVR light hair and eye color northern European ancestry inability to tan

Question 64

Fill in the blanks: ___ type is/are commonly found in the head and neck, while ____ type is/are common in the trunk.

Answer 64

nodular & morpheaform superficial

Question 65

What type of treatment for BCC appear to have good cosmetic outcomes?

Answer 65

PDT

Question 66

What is the standard treatment of choice for BCC?

Answer 66

surgical excision

Question 67

T/F: Imiquimod is indicated for both superficial BCC and actinic keratosis, while 5-FU is approved for superficial BCC.

Answer 67

False imiquimod - superficial BCC 5-FU - AK and superficial BCC

Question 68

What are the risk factors for BCC metastasis?

Answer 68

morpheaform squamous metaplasia PNI

Question 69

What is the FDA approved drug for metastatic BCC that may lead to tumor resistance?

Answer 69

sonidegib

Question 70

T/F: BCNS is has incomplete penetrance and variable phenotypic expressivity.

Answer 70

complete penetrance

Question 71

Fill in the blanks: ___ and ___ accelerated BCC formation in BCNS patients.

Answer 71

sunlight and radiation

Question 72

Fill in the blanks: ___ is/are the first detectable abnormality, while ___ is/are frequently the initial sign of BCNS.

Answer 72

Jaw cysts medulloblastoma

Question 73

T/F: BCNS patients die earlier than sporadic BCC patients because of their disease.

Answer 73

False No long-scale survival studies to determine whether BCNS patients die earlier because of their disease

Question 74

Which drug is the most effective in preventing formation of new BCCs in BCNS patients?

Answer 74

vismodegib 150 mg/day

Question 75

T/F: Surgical approaches to BCC treatment should be used aggressively to BCCs in BCNS.

Answer 75

False Nonsurgical approaches

Question 76

T/F: BCNS patients have multiple BCCs which are locally aggressive and have the potential to metastasize.

Answer 76

False very rarely metastasize

Question 77

Fill in the blanks: Suspect BCNS in a patient with BCCs before age of ___, odontogenic keratocysts before age of ___, and with ___ or more palmar or plantar pits.

Answer 77

Question 78

What are the 3 most important aspects of management for BCNS?

Answer 78

frequent examination counselling about sun avoidance early treatment of small tumors