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B. Sebaceous gland tumors

Sebaceous carcinoma
An orangey nodule occurs, most frequently on the Meibom Clinical images are available in
glands in the palpebra sebaceous glands and less frequently in the hardcopy only.
skin (Fig. 22.17). Histopathologically, the tumor cell nest contains
atypical clear sebaceous cells. In the autosomal dominantly inher-
ited Muir-Torre syndrome, multiple benign or malignant seba- Fig. 22.17 Sebaceous carcinoma in the Mei-
ceous tumors occur, often accompanied by visceral malignancies. bom gland.

C. Sweat gland tumors

1. Mammary Paget’s disease

Outline
● Infiltrative eczema-like erythema or erosion occurs in the
Clinical images are available in hardcopy only.
nipples and at their periphery.
● It occurs most commonly in the opening of the lactiferous

ducts of middle-aged women. It is a carcinoma in situ

that derives from the lactiferous duct epithelia. It corre-
sponds to breast cancer.
● A tumor does not form in most cases.
● It is not itchy, nor does it respond to steroids. Mammary Fig. 22.18 Mammary Paget’s disease.
Infiltrative erythema is present on the nipple. The
Paget’s disease can be distinguished from eczema by treatment for mammary Paget’s disease is gener-
these characteristics. ally the same as for breast cancer.
● The treatments are the same as those for breast cancer.

Clinical features
A plaque with clearly circumscribed erythema, infiltration and
crusts appears on the nipple and areola (Fig. 22.18). The lesion is
slightly firm and palpable, and usually unilateral. Middle-aged
women are most frequently affected. Bilateral mammary Paget’s
disease and mammary Paget’s disease in men are extremely rare.
22
Mammary Paget’s disease accounts for 1% to 4% of all breast
cancer cases. The symptoms progress gradually with each year.
As they progress, a palpable tumor forms in the breast and metas-
tasizes to a regional lymph node (mainly the axillary lymph node).

Pathogenesis
Mammary Paget’s disease is thought to originate from cancer
in the excretory duct cells of the mammary glands (intraductal
carcinoma) or carcinoma from epidermal keratinocytes.

Pathology
Large, clear Paget’s cells replace wall cells in the ducts and
 402 22 Malignant Skin Tumors and Melanomas

glands. The Paget’s cells may also proliferate in cavities in the

lactiferous ducts and mammary glands. Although the skin lesion
does not clinically appear to be severe, Paget’s cells infiltrate lac-
tiferous ducts and mammary glands more extensively than is
clinically obvious. With further progression, Paget’s cells infil-
trate into the dermis. Immunostaining is positive for CEA.

Differential diagnosis
Chronic breast eczema, tinea corporis, and basal cell carcino-
ma should be distinguished from mammary Paget’s disease.
Intractable eczematous lesions on the breast that do not respond
to topical agents should be suspected of being mammary Paget’s
disease.

Treatment
The treatments are the same as those for breast cancer. Mastec-
tomy and regional lymph node dissection are the basic treatments.

2. Extramammary Paget’s disease

Outline
● This is Paget’s disease on areas other than the breasts.

Clinical images are available in Clinical images are available in

Clinical images are available in hardcopy only.
hardcopy only. hardcopy only.

a b c d e f a g b h c i d j e ak f bl g cm h dn i eo j fp k gq l hr m

22

Clinical images are available in Clinical images are available in

Clinical images are available in hardcopy only.
hardcopy only. hardcopy only.

c d e a f b g c h d ai e bj f ck g dl h em i fn j go k hp l iq m jr nk ol pm
Fig. 22.19 Extramammary Paget’s disease.
a: Sharply demarcated erythematous plaques. b: Mix of hypopigmented macules and erythematous plaques. c: Paget cells present in
hypopigmented macules around the anus. d, e: Extramammary Paget’s disease on the labia majora of an elderly woman. f: Extra-
mammary Paget’s disease on the axillary fossa.
 Malignant skin tumors / C. Sweat gland tumors 403

The elderly are affected.

● Sharply margined eczematous erythema and erosion
occur.
● It is thought to be intraepidermal cancer that originates

from the apocrine glands. The genitalia, anal region and

axillary fossae are most frequently involved.
● It occasionally destroys the basement membranes and
Clinical images are available in hardcopy only.

progresses to invasive carcinoma.

● Extensive resection and lymph node dissection are nec-

essary, if regional lymph node metastasis may occur.

Clinical features
Extramammary Paget’s disease occurs most commonly in the
elderly. A bright red infiltrative plaque resembling mammary Fig. 22.20 Invasive extramammary Paget’s
disease that had been left untreated for a
Paget’s disease appears (Fig. 22.19), most frequently on the geni- long period of time.
talia, less frequently on the perianal region, perineum, axillary A flat lesion elevated gradually, forming infiltra-
fossa or umbilical region. Itching is often present. The lesion tive nodules. The lesion destroyed the basal
membrane and infiltrated in the deep portions of
gradually spreads, with melanin deposition at the periphery in the dermis. Metastasis to the regional lymph node
some cases. Extramammary Paget’s disease occasionally was observed.
destroys the basement membranes and develops a palpable small
tumor in the lesion (Fig. 22.20). Regional lymph node metastasis
occurs in advanced cases; the prognosis is poor.

Pathogenesis
Extramammary Paget’s disease is thought to originate from
apocrine sweat gland cells.

Pathology
Large, bright, scattered or aggregated Paget’s cells are found
in the epidermis and sweat ducts (Fig. 22.21).

Differential diagnosis Fig. 22.21 Histopathology of extramammary

Eczema, candidiasis, genital tinea, Bowen’s disease, Hailey- Paget’s disease.
There are scattered Paget’s cells with large, clear
Hailey disease and pemphigus vegetans are distinguished from cytoplasm.
extramammary Paget’s disease. Diagnosis can be made by patho-
logical observation of Paget’s cells.

Treatment
The basic treatment is extensive surgical removal with a 1-to
22
3-cm margin including the peripheral normal skin.

3. Eccrine porocarcinoma
This is a malignant form of eccrine poroma (Chapter 21). A
red plaque or nodule, often ulcerative, occurs, most frequently on
the lower legs of the elderly (Fig. 22.22). In most cases, eccrine
porocarcinoma is clinically observed as a tumor that is mix of
eccrine poroma and eccrine porocarcinoma. It often metastasizes.
 404 22 Malignant Skin Tumors and Melanomas

4. Microcystic adnexal carcinoma (MAC)

Synonym: Syringoid eccrine carcinoma

A firm, discoidal, intradermal nodule 1 cm to 3 cm in diameter

occurs, most commonly around the mouth of persons of middle
Clinical images are available in hardcopy only. age and older. Some think it to be a sclerosing eccrine porocarci-
noma or a cancer derived from a hair follicle or apocrine sweat
gland. After extensive resection, the site should be examined
pathologically for any remaining lesions because of the high fre-
quency of local recurrence. For this reason, Moh’s microsurgery
is also effective. Distant metastasis rarely occurs.

Fig. 22.22 Eccrine porocarcinoma arising 5. Mucinous carcinoma of the skin

from eccrine poroma.
a: Eccrine porocarcinoma (malignant). b: Eccrine A nodule of 2 cm to 3 cm in diameter occurs, frequently on the
poroma (benign). eccrine secretory part of the face or scalp. The tumor is covered
by abundant mucin. The nuclei of tumor cells show slight atyp-
ism. Metastatic carcinoma of the skin with mucus production
should be distinguished from mucinous carcinoma of the skin.

D. Nervous system tumors

Merkel cell carcinoma

Clinical images are available in hardcopy only.

Outline
● Itis a skin cancer that originates from Merkel cells of the
epidermis. These cells are thought to be tactile receptor
cells.
● A highly malignant, domed red tumor forms on the face,
Fig. 22.23 Merkel cell carcinoma. head, neck or extremities of the elderly.
● Extensive resection, irradiation and chemotherapy are

the main treatments.

Clinical features
22 A firm, domed nodule varying in color from light pink to pur-
plish red and with a diameter of 1 cm to 3 cm occurs, most fre-
quently on the face of the elderly (Fig. 22.23).

Pathology
Deep-staining small cells arrange densely in a palisading pat-
tern, resembling the tumor cells of small-cell lung cancer (Fig.
22.24). Merkel cell carcinoma is characterized by dense-core
granules that resemble Merkel cells (Fig. 22.25). Immunohisto-
chemically, neuron specific enolase (NSE) and cytokeratin 20 are
positive in many cases.
Fig. 22.24 Histopathology of Merkel cell car-
cinoma.

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