Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5797_Библиотеки_им_академика_М_И_Перельмана.pdf
ab
5 Skin Tumors
143
whole layer of the skin, or even the muscles
and bones. Sometimes in-transit and satellite
metastases appear.
• It should be mainly differentiated from melanocytic nevus, hemangioma, and cSCC.
5.3.5 Extramammary Paget’s
Disease
5.3.5.1 Clinical Manifestation
andPathology
Extramammary Paget’s disease (EMPD), also
known as eczematoid carcinoma, is an uncommon skin malignancy. Its histological origin is
controversial, and mainly has the following theories. (1) Apocrine sweat gland theory: It is based
on the fact that the disease mainly occurs in the
apocrine sweat gland site, which is the mainstream
theory of the pathogenesis of the disease at present. (2) Migration theory: It is mainly based on
the fact that EMPD may be associated with
malignant tumors in its adjacent sites, such as
skin appendages cancer and visceral organs
tumors. (3) Evolutionary theory of pluripotential
germinative cell: It is mainly based on the presence of ectopic EMPD, so some studies supposed
that pluripotent stem cells are the origin of Paget
cells.
In the Western populations, vulva is the most
common site for EMPD.In the Eastern populations, on the other hand, EMPD occurs mostly in
middle-aged and elderly men and is common in
areas of apocrine sweat gland distributed such as
the perineum, scrotum, penis, and groin. A few
EMPD are ectopic, which can be observed in the
axilla, chest, eyelids, auricle, etc.
EMPD clinically presents as a red eczematoid
patch covered with scales and crusts on the surface, accompanied by pigmentation or hypopigmentation. The margin is slightly elevated. There
may be exudates and erosion in the center of the
lesion. With the progression of the disease, the
lesion gradually develops into verrucous plaques
or nodules (Fig.5.66).
Patients with EMPD conned to the epidermis
have an excellent prognosis. The disease develops slowly and is often misdiagnosed in the early
Fig. 5.66 Visual appearance of extramammary Paget’s
disease. (a) Visual observation shows eczematoid erythema in the perineum, with pigmentation and scab
(arrows). (b) Visual observation shows verrucous nodules
in the perineum with exuduates on the surface (arrows)
144
L.-H. Guo et al.
stage so as to be delayed in treatment.
Approximately 20% of the lesions that are diagnosed as EMPD have already invaded the dermis,
and some even have developed lymph node and
distant metastases.
The thickness of lesion, the layers of involvement, the invasion of skin appendages, and the
presence of lymph node metastasis are important
factors in evaluating the disease.
Histopathologically, EMPD is typically characterized by thickened stratum spinosum and the
presence of single or nests of Paget cells in the
epidermis.
Paget cells are divided into two types. (1)
Classical type (type A): The cells are large and
round, with translucent cytoplasm, hyperchromatic nuclei, which were round and centered,
and mitotic gures. (2) Signet ring type (type B):
The cytoplasm contains a large amount of mucus.
The hyperchromatic nuclei are semilunar and
squeezed by the cytoplasm to one side in a ring
shape. Immunohistochemically, CK7 is usually
positive and CK20 is negative in primary EMPD
tumor cells, and the expression of GCDFP-15 is
specic. CK7 and CK20 are usually negative in
secondary EMPD tumor cells.
5.3.5.2 Ultrasound Manifestation
Gray-Scale Ultrasound
The lesion usually shows a crawling hypoechoic
area in the epidermis. The surface is mostly at
and has abnormal keratinization, with various
degrees of posterior acoustic shadowing. In the
early stage, the bottom of the lesion is usually
at. With the progression of the disease, it can
break through the dermo-epidermal junction to
invade the dermis, appendages, and even the subcutaneous tissue. Lymph node metastasis may
occur in the late stage.
Because the extent of the disease is often
larger than that of the transducer, the technique of
extended eld of view can be conducted. On
high-frequency ultrasound, if there is a
“pseudopodia- like” hypoechogenicity extending
to the deep tissue at the bottom of the lesion, the
invasion of skin appendages should be
considered.
Color Doppler Ultrasound
Most lesions show rich blood ow signals
(Figs.5.67 and 5.68).
5.3.5.3 Dierential Diagnosis
BD
The location and appearance of the lesion can be
helpful in differentiation. BD is mostly located in
the face and trunk, showing local reddish-brown
patches, with scales on the surface. EMPD, on
the other hand, is mostly located in the perineum
and shows eczematoid erythema, which is more
extensive than BD.On gray-scale ultrasound, BD
appears as a hypoechoic lesion conned to the
epidermis, with thick linear hyperechogenicity of
the surface due to abnormal keratinization. While
EMPD can involve the whole layers of the skin,
and the degree of abnormal keratinization on the
surface is milder than Bowen’s disease. Compared
with BD, EMPD shows more rich internal blood
ow signals.
In addition, EMPD should especially be differentiated from Paget-like BD, and the two entities show similar ultrasound features.
Immunohistochemical examination is helpful to
differentiate the two entities, the former tumor
cells are often positive for CK7, and the latter
tumor cells are negative for CK7 and CEA.
MM
The location and appearance of the lesion are
helpful in differentiating the two entities. MM
lesions are mostly located at the extremity and
face, and often black. However, EMPD is mostly
located in the perineum and shows eczematoid
erythema. On gray-scale ultrasound, malignant
melanoma appears irregular or nodular shape
with a tendency to invade deeply, while EMPD is
crawling.
5.3.5.4 Diagnosis Clues
1. EMPD occurs mostly in middle-aged and
elderly people, and are mostly located in the
perineum.
2. In visual appearance, the lesions appear as
eczematoid erythemas, verrucous patches, or
nodules.
cd
ab
5 Skin Tumors
a b
145
Fig. 5.67 Paget’s disease in the perineum. Female,
67years of age. (a) Visual observation shows eczematoid
erythema with an area of pigmentation and erosion in the
scrotum (arrows) (diameter: approximately 30.0 mm).
(b) Gray-scale ultrasound shows a regular, crawling, and
ill-dened hypoechoic lesion (arrows) located in the epidermis and dermis (thickness: 0.8~1.2mm). The surface
shows ne linear hyperechogenicity. The bottom of the
lesion (yellow dotted line) reaches the junction of dermis/
Fig. 5.68 Paget’s disease with different invasion extent.
(a) Male, 82years of age. Gray-scale ultrasound shows a
regular, crawling, ill-dened hypoechoic lesion (arrows)
located in the epidermis and dermis (thickness:
0.8~ 1.6mm). The surface appears as ne linear hyperechogenicity. The bottom is ill-dened but regular. The
lesion was homogeneous (Frequency: 34MHz). (b) Male,
subcutaneous tissue (red dotted line). The surrounding
subcutaneous tissue is thickened, with increased echogenicity. The lesions are homogeneous (Frequency:
50MHz). (c) The ultrasound features are the same as that
of the lesion described above (Frequency: 22 MHz).
(d) Histopathology (HE staining, panoramic scan) shows
nests of Paget cells [Image cited from Chen ST etal., J
Ultrasound Med, 2019, 38 (12): 3229–3237]
79 years of age. Gray-scale ultrasound shows a regular,
protrusive, ill-dened hypoechoic lesion (arrows) in the
epidermis and dermis (thickness: 2.7~5.0mm). The surface appears as ne linear hyperechogenicity. The bottom
is ill-dened with the pseudopodia extending to the depth
(yellow dotted line). The lesion is homogeneous in echo
texture (Frequency: 34MHz)
146
L.-H. Guo et al.
3. Gray-scale ultrasound shows a crawling
hypoechoic lesion that involves the whole layers of the skin, with “pseudopodia-like”
hypoechogenicity at the bottom of some
lesions, suggesting the invasion of skin
appendages.
4. Color Doppler ultrasound shows rich blood
ow signals inside the lesion.
5.3.5.5 Clinical Signicance
1. High-frequency ultrasound plays a very
important role in assessing the thickness of
the lesion, the layers of involvement, the invasion of appendages, and the presence of lymph
node metastasis in EMPD.
Our previous study retrospectively analyzed
the features of ultrasound biomicroscopy and
high-frequency ultrasound in 17 patients with
EMPD conrmed pathologically. The results
indicated that ultrasound biomicroscopy showed
the layers of involvement more clearly than highfrequency ultrasound (100% vs. 29.4%). However, high-frequency ultrasound showed rich
blood ow signals in 11 lesions (64.7%), and
inguinal lymph node metastasis was found in one
lesion.
Ultrasound biomicroscopy provides detailed
morphological information, while high- frequency
ultrasound is prone to show lymph node metastasis. The combination of ultrasound biomicroscopy and high-frequency ultrasound can provide
key information for the diagnosis of EMPD.
2. Among various factors related to the clinical
risk of EMPD, the pathological invasion level
is the most pivotal factor leading to poor prognosis. Therefore, according to the layers of
involvement, EMPD is divided into 2 groups
as follows: Lesions in situ in the epidermis
and lesions invade into the dermis or subcutaneous tissue. Our previous study found that
tumor growth pattern and inltration depth
were signicantly different between the two
groups, which could be used to assess the
invasion extent of EMPD.With a cutoff value
of 1.55mm for inltration depth, the AUROC
was 0.833 (Table5.7).
3. In addition, high-frequency ultrasound can
also be used to monitor the effect of disease
treatment. Skin defects, penile reconstruction,
and functional effects after the surgery of
EMPD are challenges to cure this disease.
Photodynamic treatment (PDT) is performed
by injecting patients with photosensitizers,
which specically accumulate in tumor tissue
over a period of time. Then a specic wavelength of light is used to irradiate the skin
lesions, so as to stimulate the photosensitizer
to produce reactive oxygen species, thereby
tumor cells are killed and the effect of treatment is achieved. PDT can be used as an
important treatment for EMPD. In Shanghai
Skin Disease Hospital, the treatment of recurrent EMPD by non-surgical methods uses a
therapy combining 5-aminolevulinic acid
photodynamic treatment (ALA-PDT) with
holmium laser. After 9 sessions of treatment,
the abnormal echogenicity in the original
lesion area disappears on gray-scale ultrasound, and the process from presence to
absence of the lesion is recorded.
Key Points
• EMPD, also known as eczematoid carcinoma,
is a rare cutaneous malignancy. It is common
in middle-aged and elderly people, and often
located in the perineum.
• Gray-scale ultrasound shows a crawling
hypoechoic lesion that can involve the whole
Table 5.7 High-frequency ultrasound features of EMPD of different pathological invasion levels
a
Lesion inltration depth
Tumor growth pattern (localized/invasive) 38 (90.5%)/4 (9.5%) 6 (33.3%)/12 (66.7%) 0.000
IE group: lesions in situ in the epidermis
ID group: lesions invade into the dermis or subcutaneous tissue
a
Data are presented as median (25th, 75th)
(mm) 1.1 (0.6~1.5) 2.2 (1.6~3.9) 0.000
IE Group (n=42) ID Group (n=18) P value
5 Skin Tumors
147
layer of the skin, with “pseudopodia-like”
hypoechogenicity at the bottom of some
lesions, suggesting the invasion of skin
appendages. Rich blood ow signals are
observed inside the lesion.
• It should be mainly differentiated from BD
and MM.
• Ultrasound can be used to assess the thickness
of the lesion, the layers of involvement, the
invasion of appendages, and the presence of
lymph node metastasis in EMPD.
5.3.6 Dermatobrosarcoma
Protuberans
5.3.6.1 Clinical Manifestation
andPathology
Dermatobrosarcoma protuberans (DFSP) originates from spindle cell in the dermis and is a
cutaneous broblastic neoplasm with a low
degree of malignancies. DFSP often occurs in the
trunk, followed by the extremities, and rare in the
head and neck. The lesions grow slowly and
mostly present as painless subcutaneous nodules
with a tendency to inltrate, mostly involving the
dermis and subcutaneous tissues.
Some lesions invade upwards into the epider-
mis or downwards into deeper tissue. When the
epidermis is involved, the lesion appears as a
brownish-red, reddish nodule with a slightly elevated surface and occasional ulceration and exudates. When the epidermis is not involved, most
patients are asymptomatic except having palpable masses, which are easily misdiagnosed.
The etiology of DFSP is unknown. DFSP may
occur congenitally, and some may relate to a history of surgery, trauma, local injection of drugs,
or insect bites.
The main treatment of the disease is surgery,
with high rates of recurrence (20%~50%) and
the average time for local recurrence is 32months.
If the surgical margin is negative, the patient has
a good prognosis, with 2-year and 5-year survival
rates of 97% and 92%, respectively. The metastasis rate of this disease is low, less than 5%, and
occurs on the basis of multiple recurrences.
5.3.6.2 Ultrasound Manifestation
Gray-Scale Ultrasound
Most lesions show oval or lobulated, ill-dened
mixed echogenic areas in the dermis and subcutaneous tissues, which are parallel to the skin. The
disease specically invades adipose tissue, that
is, hypoechogenic pseudopodial-like protrusions
extending into peripheral hyperechoic adipose
tissue, which can form a characteristic of “whirlpool sign.” The lesion is heterogeneous in echo
texture, and banded hyperechogenicity and
hypoechogenicity are observed inside, which is
associated with mucinous degeneration of tumor
cells and hyalinization of brous tissue. The
lesion is rm, and the compression of transducer
has no signicant effect on the morphology of the
lesion.
Color Doppler Ultrasound
Blood ow signals can be observed in the lesion,
and the degree of vascularization varies from rare
to rich, usually without nourishing vessels
(Figs.5.69 and 5.70).
5.3.6.3 Dierential Diagnosis
DFSP appears to be benign lesion on ultrasound
and is often misdiagnosed, so it needs to be differentiated from benign soft tissue tumors.
Lipoma
Lipomas appear as hyperechoic, isoechoic, or
hypoechoic lesions in the subcutaneous fat layer
on ultrasound. DFSP needs to be differentiated
from hypoechoic lipomas. Both are palpable subcutaneous nodules, lipoma is generally skincolored, while DFSP appears brownish-red or
reddish when invading the epidermis.
On ultrasound, both can show banded hyperechoic septa inside. However, the hypoechoic
lipoma appears regular and well-dened with
capsule, which does not invade the surrounding
tissue, and shows no blood ow signals inside.
DFSP is ill-dened without capsule. The characteristic hypoechoic pseudopodia-like protrusions
extend to adipose tissue in the periphery, forming
a typical characteristic of “whirlpool sign,” indi-
148
ab
L.-H. Guo et al.
cd
Fig. 5.69 Dermatofbrosarcoma protuberans. A 12-yearold girl underwent tumor resection in her right forearm
6years ago. (a) Visual observation shows two bean-sized
subcutaneous nodules at the surgical site, beside an incision (arrows). (b) Gray-scale ultrasound shows an irregular hypoechoic lesion (arrows) in the subcutaneous tissue,
with multiple hypoechoic pseudopodia-like protrusions in
the periphery (size: 28.0 mm × 26.0 mm; thickness:
cating the invasion of surrounding fat layer, and
blood ow signals can be detected inside.
Keloid
This disease refers to exuberant scarring due to
reactive proliferations produced by an abnormal
healing process after skin trauma. Clinically, it
appears as a red and elevated nodule with a
smooth surface. While DFSP may present as a
subcutaneous nodule and also as a reddish elevated nodule with a smooth surface. On grayscale ultrasound, keloid shows signicantly
thickened dermis with plaque-like hypoechogenicity. The lesion has no space-occupying
effect. However, DFSP shows the pseudopodialike protrusions extending to adipose tissue in the
10mm). The lesion is heterogeneous, and banded hyperechogenicity and hypoechogenicity are observed inside
(Frequency: 34 MHz). (c) Gray-scale ultrasound shows
the pseudopodia- like protrusions extend into the peripheral adipose tissue (arrows) (Frequency: 34 MHz).
(d) Color Doppler ultrasound shows rare blood ow signals in the periphery and interior of the lesion (arrows)
(Frequency: 22MHz)
periphery, forming a typical characteristic of
“whirlpool sign”.
Nodular Panniculitis
Both of the diseases locate in the subcutaneous
adipose tissue. In contrast to DFSP, most nodular
panniculitis is accompanied by pain. The ultrasound features of the two diseases are similar.
Nodular panniculitis is characterized by increased
echogenicity and thickened subcutaneous adipose tissue, with patchy hyperechoic and irregular hypoechoic areas in the lesion. The lesion is
irregular and ill- dened with no space-occupying
effect. However, DFSP is irregular and involves
subcutaneous tissues mostly, with characteristic
hypoechoic pseudopodia- like protrusions extend-
cd
5 Skin Tumors
a b
149
e
Fig. 5.70 Dermatofbrosarcoma protuberans. Male, 30
years of age. (a) Visual observation shows a subcutaneous
nodule in the abdominal wall (size: 25.0 mm × 20.0 mm),
and a reddish nodule in the periphery with an elevated surface (arrows). (b) Gray- scale ultrasound shows an oval and
well-dened hypoechoic lesion (arrows) in the subcutaneous tissue (size: 24.4mm×20.2mm; thickness: 11.2mm).
The lesion is heterogeneous, and banded hyperechogenicity and hypoechogenicity are observed inside
(Frequency: 22MHz). (c) Characteristic hypoechoic pseudopodia-like protrusions (△) are presented in the periphery of the lesion, emanating from the lesion (*) and
extending to adipose tissue to form the “whirlpool sign”.
(d) Color Doppler ultrasound shows rich blood ow signals in the periphery and interior of the lesion (arrows)
(Frequency: 22 MHz). (e) Histopathology (HE staining,
panoramic scan) shows mild irregular hyperplasia of the
epidermis. Diffuse proliferation of spindle cells is observed
in the dermis and can be deep to the subcutaneous tissue.
The tumor cells and collagen bers are interwoven with
storiform arrangement, involving the subcutaneous fat
layer and shuttling between the interlobular septa. There
are no obvious atypical cells and mitotic gures.
Pigmentation can be observed in some areas
150
L.-H. Guo et al.
ing to adipose tissue in the periphery, forming a
typical characteristic of “whirlpool sign”.
5.3.6.4 Diagnosis Clues
1. Clinically, DFSP presents as a palpable, painless, and skin-colored subcutaneous nodule.
When the lesion involves the epidermis, it
shows a brownish-red or reddish nodule with
a smooth surface and occasional ulceration
and exudates.
2. Gray-scale ultrasound shows a mixed echogenic lesion in the dermis and subcutaneous
tissue, with an oval or irregular shape. The
lesion is heterogeneous with banded hyperechogenicity and hypoechogenicity.
3. Characteristic hypoechogenic pseudopodiallike protrusions extend into peripheral hyperechoic adipose tissue, which can form a
characteristic of “whirlpool sign”.
Key Points
• DFSP is a cutaneous broblastic neoplasm
with a low degree of malignancies. It is prone
to recurring and has a low rate of metastasis.
• Gray-scale ultrasound shows a heterogeneous
mixed echogenic lesion in the dermis and subcutaneous tissue with banded hyperechogenicity and hypoechogenicity. Characteristic
hypoechogenic pseudopodial-like protrusions
extend into peripheral hyperechoic adipose
tissue, which can form a characteristic of
“whirlpool sign”.
• The lesions appear to be benign lesions on
ultrasound and are often misdiagnosed, which
should be mainly differentiated from lipoma,
keloid, and nodular panniculitis.
1963, when it was called epidermal endocrine
carcinoma. In 1969, Mishima and Morioka ofcially named it eccrine poroma. The disease is
rare, accounting for 0.005% to 0.01% of skin
tumors. About 50% of porocarcinomas occur in
prolonged eccrine poroma. The average time of
eccrine poroma developing into porocarcinoma
is reported to be about 8.5years in literature. The
disease often occurs in the elderly people, with
an equal incidence in men and women.
The etiology and pathogenesis are uncertain.
Porocarcinoma can develop from eccrine poroma
directly or occur adjacent to intraepidermal
eccrine poroma. The lesions are often located in
the head and neck and lower extremities. The
visual appearance is similar to that of eccrine
poroma, which appears as red, blue, or black
nodules, plaques, or ulcerative lesions. About
20% of this disease develops local metastasis and
10% develops distant metastasis, with a mortality
rate of 67% if distant metastasis occurs.
5.3.7.2 Ultrasound Manifestation
Gray-Scale Ultrasound
The lesion shows a well-dened, regular, or
irregular hypoechoic area involving the epidermis and dermis. The surface is elevated without
abnormal keratinization. The bottom of the lesion
is located in the dermis, and some extends downward with a cornu form. The lesion is homogeneous. Overall, there are no signicant
characteristic features.
Color Doppler Ultrasound
Rich blood ow signals are observed inside the
lesion (Fig.5.71).
5.3.7 Porocarcinoma
5.3.7.1 Clinical Manifestation
andPathology
Porocarcinoma, also known as malignant eccrine
poroma, is a malignant tumor originating from
the eccrine ducts in the epidermis. This disease
was rst reported by Pinkus and Mehregan in
5.3.7.3 Dierential Diagnosis
Eccrine Poroma
Porocarcinoma originates from the malignant
transformation of eccrine poroma. Eccrine
poroma is regular and well-dened on gray-scale
ultrasound. Color Doppler ultrasound shows rich
blood ow signals in both of them, which is dif-
5 Skin Tumors
a b
c d
151
Fig. 5.71 Porocarcinoma. Female, 83 years of age.
(a) Visual observation shows a hemispherical bump of
about 15.0mm in diameter in the right lower leg (arrows),
with a mild ulceration and a small number of exudates on
the surface. Dark red scaly patches are observed in the
periphery of the lesion. (b) Gray-scale ultrasound shows
an oval, well- dened, and homogeneous hypoechoic
lesion (arrows) in the epidermis and dermis (size:
14.8mm ×15.6mm; thickness: 6.7 mm). The surface is
elevated, with incomplete hyperechogenicity on the top of
the lesion (the epidermis is absent locally) (△)
(Frequency: 22 MHz). (c) Color Doppler ultrasound
shows rich blood ow signals inside the lesion (arrows),
with nourishing vessels observed at the bottom
(Frequency: 22MHz). (d) Histopathology (HE staining,
panoramic scanning) shows that the epidermis is locally
lost with ulceration. The lesion is connected to the epidermis and grows aggressively into the dermis. The tumor
cell clumps are irregular in shape, with multiple foci of
necrosis and duct-like differentiation. The tumor cells are
small cuboidal, with increased mitotic gures and a large
number of dyskeratotic cells. Collagen proliferation in the
interstitium is accompanied by deposition of mucin-like
material. Inammatory cell inltration, mainly plasma
cells, is observed
152
L.-H. Guo et al.
cult to differentiate. Some porocarcinomas are
also difcult to differentiate pathologically due to
the large cellular components of eccrine poroma.
If eccrine poroma grows rapidly in a short time
with an ill-dened boundary, the possibility of
malignant transformation should be considered.
cSCC
The gray-scale ultrasound features of the two
entities are similar, which show an ill-dened
boundary and the tendency to inltrate deep tissues. However, the invasion of SCC is deeper and
wider, and the lesion usually shows more signicant abnormal keratinization on the surface.
In addition, the appearance of the lesion can
provide a diagnostic clue for differentiation.
Typical SCC is prone to form central ulcerations,
and the margin may be cauliower-like irregular
elevations, with a large size and no pigmentation
generally. However, porocarcinoma is relatively
small in size and appears as a red, blue, or black
lesion, some with ulcerations on the surface.
5.3.7.4 Diagnosis Clues
1. The visual appearance of porocarcinoma is
similar to that of eccrine poroma, mostly
showing red, blue, or black nodules, some
with ulcerations on the surface.
2. Porocarcinoma is rare, and previous literatures
indicated that ultrasound can be used to evaluate the presence of lymph node metastasis.
However, to the best of the authors’ knowledge,
there is no literature reporting the ultrasound
features of porocarcinoma. The authors have
summarized the ultrasound features of 5 porocarcinomas conrmed by pathology in Shanghai
Skin Disease Hospital. The ultrasound features
are non-specic and mainly show ill-dened
hypoechoic lesions in the epidermis and dermis
with rich blood ow signals inside.
Key Points
• Porocarcinoma is rare and originates from the
eccrine ducts in the epidermis, mostly from
the malignant change of poroma. It often
occurs in the elderly people and located in the
head, neck, and lower extremities. Metastasis
may occur.
• Ultrasound shows an ill-dened hypoechoic
lesion in the epidermis and dermis with rich
blood ow signals inside, which is overall
non-specic.
• It should be mainly differentiated from eccrine
poroma and cSCC.
5.3.8 Sebaceous Gland Carcinoma
5.3.8.1 Clinical Manifestation
andPathology
Sebaceous gland carcinoma is a rare cutaneous
malignancy accounting for 0.2% to 4.6% of cutaneous malignancies. Sebaceous gland carcinoma
originates from the sebaceous glands of the eyelids, face, scalp, etc. Its etiology is uncertain.
Sebaceous gland carcinoma has been found to be
associated with smoke, exposure to organic compounds, local inammatory irritation, radiation
history around the eye, and partial gene mutations and deletions.
According to the location of the lesion, it is
divided into periocular sebaceous gland carcinoma and extraocular sebaceous gland carcinoma. Periocular sebaceous gland carcinoma is
common in the upper and lower eyelids and lacrimal caruncle, especially in the upper eyelids.
Periocular sebaceous gland carcinoma accounts
for about 3/4 of sebaceous gland carcinoma and
is the second common eyelid malignancy after
basal cell carcinoma in China, with poor differentiation. However, extraocular sebaceous gland
carcinoma is relatively rare, accounting for about
1/4 of sebaceous gland carcinomas, commonly in
the head and neck. The disease often occurs in
middle-aged and elderly people. Periocular sebaceous gland carcinoma often presents as diffuse
thick and stiff eyelid, or a single, yellow, and rm
nodule without pain. However, most lesions have
non-specic visual appearances and are often
misdiagnosed as cysts, benign tumors, or inammation of the eye. Sebaceous gland carcinoma is
prone to metastasize, easy to recur after surgical
resection, and has a poor prognosis with a mortality rate of 5% to 29%.
Histopathologically, it shows lobulated or
papillary growth. Basaloid cells are segmented