20 - 109 - APPENDAGE TUMORS OF THE SKIN Flashcards

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1
Q

ductal differentiation is most commonly observed in what type of appendage tumor

A

Eccrine Tumors

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2
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A
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3
Q

Eccrine Tumors

A
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4
Q

Apocrine Tumors

A
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5
Q

Follicular Tumors

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6
Q

Sebaceous Tumors

A
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7
Q

Genetic Syndromes Associated with Appendage Neoplasms

A
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8
Q

Eccrine Appendage Neoplasms

A
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9
Q
A

Hidradenoma is a solitary, indistinct dermal nodule

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10
Q
A

B, The tumor is a circumscribed, nonencapsulated, solid and cystic dermal neoplasm.

C, Hidradenoma is composed of clear cells and cells with ductal and glandular differentiation.

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11
Q
A

Eccrine poroma is an erythematous dermal nodule with an epidermal rim that often occurs on acral surfaces.

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12
Q
A
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13
Q
A

A, Cylindroma is a smooth, flesh-colored papule, often presenting on the head and neck

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14
Q
A

B, The tumor is a dermally based proliferation of islands of basaloid epithelium, arranged in a pattern similar to that seen in a jigsaw puzzle.

C, An eosinophilic zone of basement membrane material separates the interlocking nests of tumor cells. Within the tumor nests, central cells have vesicular nuclei and eosinophilic cytoplasm, while peripheral cells have round basophilic nuclei with scant cytoplasm.

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15
Q
A

Spiradenoma is a flesh-colored nodule

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16
Q
A

B, It is usually a multinodular nonencapsulated dermal tumor wherein the basaloid nodules resemble blue balls in the dermis.

C, The central tumor cells have vesicular nuclei and eosinophilic cytoplasm, and the peripheral cells have smaller, round, basophilic nuclei with scant cytoplasm.

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17
Q
A

Syringoma presents as multiple, small, flesh-colored papules, often in a periorbital distribution

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18
Q
A

B, The tumor is composed of a dermal proliferation of cords and nests of tumor cells in the upper dermis.

C, Small ductal structures are present in a sclerotic stroma forming tadpoleshaped and comma-shaped epithelial structures.

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19
Q

Summary of Immunohistochemical Stains in the Distinction between Squamous Cell Carcinoma and Select Appendage Neoplasms

A
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20
Q
A

A, Microcystic adnexal carcinoma is an infiltrative tumor composed of cords and cysts of varying sizes and shapes.

B, The tumor extends through the dermis to involve the subcutaneous fat.

C, Perineural invasion is often seen.

D, The tumor cells may be positive for CK7.

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21
Q
A
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22
Q

Apocrine Appendage Neoplasms

A
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23
Q
A

Hidrocystoma is a translucent papule, frequently seen in a periorbital distribution.

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24
Q
A
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25
Q
A

A, Syringocystadenoma papilliferum can present as a crusted verrucous plaque on the scalp.

B, Serous drainage is not an uncommon finding.

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26
Q
A

C, The tumor occurs as an invagination of papillomatous projections in the epidermis.

D, On the left side of this image a papillary projection is seen, composed of an inner layer of columnar epithelium with decapitation secretion and an outer layer of myoepithelial cells.

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27
Q
A

A, Hidradenoma papilliferum occurs as a well-circumscribed solid and cystic dermally based nodule, with interconnected glands that form labyrinthlike arrangements of tumor cells.

B, The projections are composed of cuboidal and columnar epithelium with decapitation secretion and a basal layer of myoepithelial cells.

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28
Q
A

A, Adenoid cystic carcinoma is composed of dermal islands of basaloid cells.

B, The tumor cells have a characteristic cribriform arrangement, with punched out pseudocysts filled with mucin.

C, The tumor cells usually stain positively for CD117 (cKIT).

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29
Q
A

A, Trichofolliculoma is characterized by numerous small hair follicles radiating from a central pore.

B, The follicles vary in stages of differentiation.

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30
Q

Classification of Follicular Appendage Neoplasms

A
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31
Q
A

Trichoblastoma present as red to flesh-colored dermal nodules, often on the face.

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32
Q
A

B, The tumor is a circumscribed dermal nodule composed predominantly of basaloid cells.

C, The basaloid epithelioid structures may resemble basal cell carcinoma; however, the characteristic mucin-containing clefts separating basal cell carcinoma from the stroma are not a feature of trichoblastoma.

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33
Q
A
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34
Q
A

A, Trichoepithelioma is a well-circumscribed, superficial, dermal tumor.

B, The tumor is composed of basaloid germinative epithelial cells that form papillary mesenchymal bodies, which are composed of a cup-shaped arrangement of basaloid epithelium with a central cellular fibroblastic core.

C, In tumors with a predominance of basaloid cells the differential diagnosis includes basal cell carcinoma. D, CK20+ Merkel cells are often present.

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35
Q
A

A, Fibrofolliculoma is composed of pilar and stromal elements.

B, Irregularly shaped follicles with delicate fronds extend into a densely fibrotic perifollicular stroma.

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36
Q
A

Pilomatricoma is a circumscribed dermal nodule

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37
Q
A

(B) peripheral nucleated basaloid cells and central eosinophilic shadow cells (ghost cells).

C, The basaloid cells have round nuclei resembling pilar matrical cells, whereas shadow cells are composed of eosinophilic cellular material with a faint outline of nuclear membrane.

D, In some cases basaloid cells are inconspicuous and there is prominent calcification, in which event, the tumor also has been known as calcifying epithelioma of Malherbe.

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38
Q
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39
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40
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41
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42
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43
Q
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44
Q
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45
Q

A 55-year-old woman sought consult due to a solitary, flesh colored papule surrounded by an epidermal rim on the palm (acral skin). Biopsy will theoretically show:

a. well-circumscribed endophytic proliferations of cuboidal cells with basophilic round nuclei and eosinophilic cytoplasm with extension from the epidermis into the dermis.
b. islands of basaloid cells arranged in a “jigsaw puzzle”–like arrangement in the dermis surrounded by eosinophilic basement membrane material
c. multinodular, unencapsulated dermal neoplasm composed of basaloid cells arranged in cords in a trabecular pattern
d. ductal structures classically have a “tadpole”-shaped or “comma”-shaped tail

A

A

Page 1824. Lesion is a poroma. B: cylindroma; C: spiradenoma D. Syringoma

46
Q

islands of basaloid cells arranged in a “jigsaw puzzle”–like arrangement in the dermis surrounded by eosinophilic basement membrane material

A

cylindroma

47
Q

well-circumscribed endophytic proliferations of cuboidal cells with basophilic round nuclei and eosinophilic cytoplasm with extension from the epidermis into the dermis

A

poroma

48
Q

multinodular, unencapsulated dermal neoplasm composed of basaloid cells arranged in cords in a trabecular pattern

A

spiradenoma

Two cell types are seen in spiradenomas:

(a) small basaloid cells with hyperchromatic nuclei and scant cytoplasm located at the periphery of the nodules, and

(b) larger basaloid cells with vesicular nuclei and pale cytoplasm located in the center of the nodules

49
Q

ductal structures classically have a “tadpole”-shaped or “comma”-shaped tail

A

Syringoma

50
Q

Which syndrome presents with multiple cylindromas?

a. Down syndrome
b. Brooke-Spiegler Syndrome
c. Goltz-Gorlin Syndrome
d. Birt-Hogg-Dube Syndrome

A

B

Page 1825. A. Syringoma,

C. hydrocystoma.

D. Trichodiscoma *Brooke-Spiegler Syndrome may also present with multiple spiradenoma, cyndrocarcinoma, spiradenocarcinoma, trichoepithelioma

51
Q

The following are histological differential diagnosis of microcystic adnexal carcinoma except:

a. morpheaform basal cell carcinoma
b. desmoplastic trichoepithelioma
c. syringoma
d. spiradenocarcinoma

A

D

52
Q

What is the most commonly affected site of trichofolliculoma?

a. scalp
b. cheeks
c. nose
d. trunk

A

C

53
Q

Multiple trichoepitheliomas are seen in the following except

a. Brooke-Spiegler Syndrome
b. Rombo Syndrome
c. Bazex-Dupre-Christol Syndrome
d. Gardner Syndrome

A

D

Gardner Syndrome: Pilomatricoma

54
Q

Birt-Hogg-Dube syndrome have an increased risk of the following except?

a. renal tumors
b. heart disease
c. lung cysts
d. recurrent pneumothoraces

A

B

55
Q

A well-circumscribed dermal nodule with peripheral, nucleated basaloid cells and central, enucleated eosinophilic “ghost” cells is seen in

a. Trichoblastoma
b. Trichoepithelioma
b. Pilomatricoma
d. Trichilemomma

A

Pilomatricoma

56
Q

Dilated pore shows differentiation toward the ___________.

a. Germinative cells
b. Follicular infundibulum
c. Isthmus
d. Mesenchyme

A

b. Follicular infundibulum

Dilated pore shows infundibular differentiation, rather than isthmic differentiation with eosinophilic or clear cytoplasm seen in pilar sheath acanthoma.

57
Q

Lesions associated with Muir-Torre Syndrome except

A. Sebaceous hyperplasia

B. Sebaceous adenoma

C. Sebaceoma

D. Sebaceous carcinoma

A

A. Sebaceous hyperplasia

58
Q

All are true in describing AK, except:

a. The overall risk of progression to invasive SCC is 1-5%
b. Risk factors include fair skin, age, cumulative UV radiation exposure, immunosuppression, prior history of non-melanoma skin cancer.
c. Workers with a long history of outdoor occupation, such as farmers, construction workers, or fishermen, have an up to 2.5-fold increased risk compared to indoor workers.
d. The prevalence increases with age in both sexes.

However, men are more likely to develop AK than women.

A

ANSWER: A. Page. 1857. The overall risk of progression to invasive SCC is estimated as 5% to 10%.

59
Q

A. Erythematous AK

B. Hypertrophic AK

  1. has a propensity for dorsal hands, arms, and scalp.
  2. It is usually more easily felt than seen
  3. most often found against a background of photodamaged skin
A

B, A, A

P.1858 The typical AK lesion presents most commonly as a 2to 6-mm, erythematous, flat, rough, gritty or scaly papule (Fig. 110-2). It is usually more easily felt than seen. They are most often found against a background of photodamaged skin

60
Q

enumerate benign eccrine tumors

A

“Cylinder and Spiral HiPS”

■ Cylindroma

■ Spiradenoma

■ Hidradenoma

■ Poroma

■ Syringoma

61
Q

enumerate benign apocrine tumors

A

“HAS HTN”

■ Hidrocystoma

■ Apocrine mixed tumor

■ Syringocystadenoma papilliferum

■ Hidradenoma papilliferum

■ Tubular adenoma

■ Nipple adenoma

62
Q

Adnexal tumor with PANFOLLICULAR differentiation

A

Trichofolliculoma

63
Q

Adnexal tumor with FOLLICULAR GERMINATIVE AND FOLLICULAR STROMAL CELLS differentiation

A

■ Trichoblastoma

■ Trichoepithelioma

■ Desmoplastic trichoepithelioma

64
Q

Adnexal tumor with FOLLICULAR MESENCHYMAL differentiation

A

■ Trichodiscoma (fibrofolliculoma)

65
Q

Adnexal tumor with MATRICAL differentiation

A

Pilomatricoma (pilomatrixoma)

66
Q

Adnexal tumor with Bulb/stem outer root sheath differentiation

A

■ Trichilemmoma

67
Q

Adnexal tumor with Isthmic outer root sheath differentiation

A

■ Tumor of follicular isthmus

■ Pilar sheath acanthoma

■ Proliferating pilar tumor

68
Q

Adnexal tumor with infundibular differentiation

A

■ Trichoadenoma

■ Dilated pore (of Winer)

69
Q

What appendage neoplasms are associated with Brooke-Spiegler

A

Cylindroma, Spiradenoma, Cylindrocarcinoma/ Spiradenocarcinoma, trichoepitheliomas (included in the text, not in the table)

70
Q

What appendage neoplasms are associated with Multiple familial trichoepitheliomas

A

Multiple trichoepitheliomas, Cylindroma

71
Q

What appendage neoplasms are associated with Rombo Syndrome

A

Multiple trichoepitheliomas

72
Q

What appendage neoplasms are associated with Bazex-Dupré-Christol

A

Multiple trichoepitheliomas

73
Q

What appendage neoplasms are associated with Schöpf-Schulz-Passarge

A

Multiple hidrocystomas

74
Q

What appendage neoplasms are associated with Goltz-Gorlin

A

Multiple hidrocystomas

75
Q

What appendage neoplasms are associated with Birt-Hogg-Dubé

A

Trichodiscoma/fibrofolliculoma

76
Q

What appendage neoplasms are associated with Gardner

A

Pilomatricoma

77
Q

What appendage neoplasms are associated with Myotonic muscular dystrophy

A

Pilomatricoma

78
Q

What appendage neoplasms are associated with Rubinstein-Taybi

A

Pilomatricoma

79
Q

What appendage neoplasms are associated with Cowden

A

Trichilemmoma (with mucosal involvement)

80
Q

What appendage neoplasms are associated with Keratitis, ichthyosis, and deafness (KID)

A

Proliferating pilar tumor

81
Q

What appendage neoplasms are associated with Muir-Torre

A

Sebaceous adenoma (especially at sites other than head and neck), sebaceoma, sebaceous carcinoma

82
Q

What do you call the spiraled, intraepidermal portion of the eccrine duct?

A

Acrosyringium

83
Q

Syringomas have been reported in association with what syndrome?

A

Down syndrome

84
Q

dermal islands of basaloid cells arranged in a cribriform pattern with “punched-out” pseudocysts filled with mucin

A

ADENOID CYSTIC CARCINOMA

85
Q

most common appendage tumor in children

A

Pilomatricoma

86
Q

What are the 2 layers of syringocystadenoma papilliferum?

A
  1. Inner Layer: Columnar to cuboidal epithelium with decapitation secretion; (+) CK7, EMA, CEA
  2. Basal/ Myoepithelial cells

(+) Plasma cells

86
Q

well-circumscribed solid and cystic dermally based nodule, with interconnected glands that form labyrinthlike arrangements of tumor cells.

A

Hidradenoma papilliferum

86
Q

dermal islands of basaloid cells arranged in a cribriform pattern with “punched-out” pseudocysts filled with mucin

The tumor cells usually stain positive for what stains

A

Adenoid cystic carcinoma

CK7, CD117 (cKIT), CK15, and SOX-10

87
Q

benign follicular hamartoma that shows differentiation toward** all portions of the hair follicle**

A

TRICHOFOLLICULOMA

88
Q

central dilated or cystic infundibulum and secondary hair follicles radiating into the dermis

A

Trichofolliculoma

89
Q

Papillary mesenchymal bodies are a characteristic finding of these tumors

A

TRICHOEPITHELIOMA AND DESMOPLASTIC TRICHOEPITHELIOMA

also trichoblastoma

90
Q

Multiple trichodiscomas/fibrofolliculomas are manifestations of what syndrome?

A

Birt-Hogg-Dubé syndrome, an autosomal dominant syndrome caused by mutations in the folliculin (FLCN) gene on chromosome 17

91
Q

What syndromes are pilomatricoma associated to?

A

Gardner syndrome
Myotonic muscular dystrophy
Rubinstein-Taybi syndrome

92
Q

well-circumscribed dermal nodule with peripheral, nucleated basaloid cells and central, enucleated eosinophilic “ghost” cells

A

Pilomatricoma

93
Q

Trichilemmoma is associated with what syndrome?

A

Cowden Syndrome

  • autosomal dominant syndrome caused by mutations in the tumor-suppressor gene PTEN
  • have hamartomas affecting multiple organ systems, including the skin, breast, thyroid, endometrium, and GI tract, and have an increased risk of malignancy in those affected organs, especially the breast
94
Q

Tumor of follicular infundibulum displays what type of differentiation?

A

follicular isthmic differentiation

Page 1847: Despite its name, tumor of follicular infundibulum displays follicular isthmic differentiation, rather than infundibular, and its pathogenesis is still unclear.

95
Q

Sebaceous adenoma (especially at sites other than head and neck), sebaceoma, sebaceous carcinoma are associated with what syndrome?

A

Muir-Torre syndrome

Sebaceous Adenoma > Sebaceoma

96
Q

Sebaceous cells (>50%) > Basaloid Cells

A

Sebaceous Adenoma

97
Q

Basaloid Cells > Sebaceous Cells

A

Sebaceoma