97 97 97 97
The Korean Journal of Pathology 2010; 44: 97-100
An endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare skin tumor that
most commonly occurs on the eyelids of elderly women. This tumor is morphologically analo-
gous to endocrine ductal carcinoma in situ and solid papillary carcinoma of the breast. We de-
scribe one case of a 51-year-old male with an EMPSGC co-existing with mucinous carcinoma
of the eyelid. The tumor was composed of dilated ducts with a smooth border and was partial-
ly filled with a papillary proliferation. Tumor cells were uniform, small-to-medium in size, and
oval-to-polygonal with light eosinophilic cytoplasm. Nuclei were bland with diffusely stippled
chromatin and inconspicuous nucleoli. Tumor cells expressed chromogranin, synaptophysin,
estrogen and progesterone receptors, cytokeratin 7, and epithelial membrane antigen.
Key Words : Sweat gland neoplasms; Adenocarcinoma, mucinous; Carcinoma, neuroendocrine
Yong Kyu Kim1
Sang Hwa Shim
A Case of Endocrine Mucin-Producing Sweat Gland Carcinoma
Co-existing with Mucinous Carcinoma
- A Case Report -
An endocrine mucin-producing sweat gland carcinoma (EMPS-
GC) is a rare, low-grade sweat gland carcinoma that is morpho-
logically analogous to an endocrine ductal carcinoma in situ (E-
DCIS)/solid papillary carcinoma of the breast.1An EMPSGC is
characterized histologically by mucin production, low nuclear
grade, and neuroendocrine differentiation. Twenty-three cases of
EMPSGCs have been reported in the literature.1-9We describe
one case of this under-recognized lesion.
A 51-year-old male presented with a small flesh-colored nod-
uleon the left lower eyelid, which had been growing slowly for
2 years. Incisional biopsy was performed. Gross examination found
that the specimen was a pale, soft, ovoid nodule, measuring 0.8
cm in diameter. Histological examination of the specimen showed
a well-defined tumor. The tumor was composed of a large pool
of mucin, which was loculated by thin fibrous septa and contained
small, irregular floating epithelial islands (Fig. 1A). Tumor cells
were organized into short cords or small nests within the epithe-
lial islands; some of these had branching and cribriform patterns.
Tumor cells were oval-to-cuboidal with a moderate amount of
eosinophilic cytoplasm (Fig. 1B). Nuclei were centrally located
with mild pleomorphism and diffusely stippled chromatin. These
findings were consistent with mucinous carcinoma.
The patient underwent wide excision two months later. Gross
examination showed that the specimen was ellipsoid, and mea-
sured 1×0.7×0.3 cm. Histological examination showed that
the specimen was a dermal tumor with upward extension along
the eccrine duct to the overlying epidermis (Fig. 2A). The tumor
was composed of dilated ducts with a smooth border and was
partially filled with a papillary proliferation. Tumor cells were
uniform, small-to-medium in size, and oval-to-polygonal with
light eosinophilic cytoplasm (Fig. 2B). Nuclei were bland with
diffusely stippled chromatin and inconspicuous nucleoli. Some
of the tumor cells lining the fibrovascular cores of the papillary
projections were elongated. Extracellular mucin was observed
Sunhee Chang, M.D.
Department of Pathology, InJe University Ilsan Paik
Hospital, 2240 Daewha-dong, Ilsanseo-gu, Goyang
Departments of Pathology and 1Plastic
Surgery, Inje University Ilsan Paik
Hospital, Goyang, Korea
Received : December 11, 2008
Accepted : June 2, 2009
Sunhee Chang Sang Hwa Shim Mee Joo, et al.
in the duct and adjacent dermis. Results from immunohisto-
chemical stainings for chromogranin and synaptophysin showed
a diffuse, positive reaction in an area of the EMPSGC and a focal
positive reaction in an area of mucinous carcinoma (Fig. 3A, B).
Both areas were positive for estrogen receptor, progesterone recep-
tor, epithelial membrane antigen, and cytokeratin 7 (Fig. 3C,
D). The myoepithelial cells were positive for p63.
An EMPSGC is a low-grade adnexal sweat gland carcinoma.
The chief complaint was a slow-growing, skin-colored solid, so-
metimescystic nodule.1An EMPSGC is found in adults’ between
the ages of 48 and 84 years, with a median age of 71 years. It is
four times more common in females than in males. Approximate-
ly two-thirds of lesions occur on the eyelid,1,4-6,8however, it has
also been observed on the cheek in proximity to the eyelid, scalp,
labium major, and forehead.1-3,9The lesion described in the pre-
sentcase was observed on the eyelid of a 51-year-old male.
Histologically, an EMPSGC is characterized by solid, papil-
lary, and cystic growth patterns, uniform medium-sized cells
with neuroendocrine differentiation, intracellular and extracel-
lular mucin, and a low nuclear grade.1,6Papillary architecture
with hyalinized fibrovascular core can be identified. Small cysts,
clefts, and variably developed, compressed, papillary structures
may be observed even in predominantly solid nodules. Tumor
cells are oval, polygonal, or, rarely, elongated or columnar with
moderately abundant, fine, lightly eosinophilic cytoplasm. Rare
cells contain mucin filled intracytoplasmic vacuoles and may re-
semble signet ring cells. Small pools of extracellular mucin can
also be found in the clefts, however, mucin is never abundant.
Fig. 1. (A) Epithelial islands float in mucoid material compartmentalized by delicate fibrous septa. Tumor cells are arranged in short cords
or small nests, some of which had lumens. (B) Tumor cells are small-to-medium in size with oval-to-round nuclei and eosinophilic cytoplasm.
Fig. 2. (A) The tumor consists of dilated ducts with smooth border and partly filled with papillary proliferation. Some foci of extracellular
mucin are present. (B) The dilated duct is lined by atypical epithelial cells and myoepithelial cells. The epithelial cells are uniform, small-
to-medium in size, and oval-to-polygonal with eosinophilic cytoplasm. The nuclei are bland with diffusely stippled chromatin.
Endocrine Mucin-producing Sweat Gland Carcinoma
Focal presence of moderate nuclear pleomorphism and hyper-
chromasia may be observed. Mitotic activity is low but always
Various markers of endocrine differentiation have been used
by different groups.1-8However, there is no common marker
across all cases. Neuroendocrine markers may be expressed focal-
ly; therefore, in a small specimen, lack of expression of neuroen-
docrine markers does not exclude a diagnosis of EMPSGC. Ex-
pression of chromogranin and synaptophysin was confirmed in
the present case. Expression of estrogen and progesterone recep-
tors was positive in all cases assessed.1,3-7Expression of estrogen
and progesterone receptors was confirmed in the present case.
EMPSGC has been suggested as a precursor of cutaneous muci-
nous carcinoma. Morphologic and immunohistochemical resem-
blanceof breast E-DCIS/solid papillary carcinoma to an EMPS-
GC has been well-described.6,10This variant of breast carcinoma
frequently co-exists with mucinous carcinoma of the breast. A
subset of mucinous carcinoma in the breast shows the expression
of neuroendocrine markers, argyrophilia with Grimelius stain-
ing, or the presence of neurosecretory-type granules on electron
microscopy.3,7Zembowicz et al.1reported that 50% of EMPS-
GCs display an area of mucinous carcinoma. Co-existence of muci-
nouscarcinoma with an EMPSGC led us to speculate on associ-
ation between the two lesions. Cytologic features of both muci-
nouscarcinomas and EMPSGCs are identical. As determined by
positivity to neuroendocrine markers, endocrine differentiation
is seen in both mucinous carcinomas and in EMPSGCs.5In the
present case, the EMPSGC co-existed with a mucinous carcino-
ma. Both tumors showed similar cytologic features and expres-
sion of neuroendocrine markers (Fig. 3A, B). Thus, based on this
common thread of spatial relationship, morphologic features, and
immunophenotype, an EMPSGC is apparently a precursor of a
cutaneous mucinous carcinoma.
An EMPSGC shares some clinical features with mucinous car-
Fig. 3. On immunohistochemical staining for chromogranin, the mucinous carcinoma shows focal positive reaction (A), while the endocrine
mucin-producing sweat gland carcinoma (EMPSGC) shows a diffuse positive reaction (B). The mucinous carcinoma (C) and the EMPS-
GC (D) show a positive reaction for estrogen receptor.
cinomaof the skin. A mucinous carcinoma typically presents as Download full-text
a slow-growing, soft, sometimes cyst-like, and solitary mass. As
with an EMPSGC, the periocular/eyelid region and cheek are
its most common sites.3,11,12Also, like patients with EMPSGCs,
patients with mucinous carcinomas are typically between 50 and
70 years of age; furthermore, mucinous carcinomas are more com-
monin women. Mucinous carcinomas most often display indo-
Mucinous carcinomas can be easily recognized and distingui-
shed from EMPSGCs by the presence of mucinous pools with
floating tumor cells.
EMPSGCs can be distinguished from a hidradenoma by the
absence of ductal differentiation, clear cellular change, squamoid
differentiation, and epidermal attachment. Presence of mucin
production and neuroendocrine nuclear appearance are helpful
in differentiation of EMPSGCs from apocrine adenomas and ap-
Follow-up data from previous series have supported the notion
that even in cases associated with mucinous carcinomas, the prog-
nosis for EMPSGCs is favorable. However, two cases have been
reported on recurrence at 18 months and 3 years after complete
excision.5,6Therefore, close clinical follow-up is critical for patients
1. Zembowicz A, Garcia CF, Tannous ZS, Mihm MC, Koerner F, Pilch
BZ. Endocrine mucin-producing sweat gland carcinoma: twelve new
cases suggest that it is a precursor of some invasive mucinous car-
cinomas. Am J Surg Pathol 2005; 29: 1330-9.
2. Banerjee SS, Eyden BP. Neuroendocrine differentiation in eccrine
carcinoma. Histopathology 1996; 29: 389-90.
3. Bellezza G, Sidoni A, Bucciarelli E. Primary mucinous carcinoma of
the skin. Am J Dermatopathol 2000; 22: 166-70.
4. Bulliard C, Murali R, Maloof A, Adams S. Endocrine mucin-produc-
ing sweat gland carcinoma: report of a case and review of the liter-
ature. J Cutan Pathol 2006; 33: 812-6.
5. Emanuel PO, de Vinck D, Waldorf HA, Phelps RG. Recurrent endo-
crine mucin-producing sweat gland carcinoma. Ann Diagn Pathol
2007; 11: 448-52.
6. Flieder A, Koerner FC, Pilch BZ, Maluf HM. Endocrine mucin-pro-
ducing sweat gland carcinoma: a cutaneous neoplasm analogous
to solid papillary carcinoma of breast. Am J Surg Pathol 1997; 21:
7. Hanby AM, McKee P, Jeffery M, et al.Primary mucinous carcinomas
of the skin express TFF1, TFF3, estrogen receptor, and progesterone
receptors. Am J Surg Pathol 1998; 22: 1125-31.
8. Mehta S, Thiagalingam S, Zembowicz A, Hatton MP. Endocrine
mucin-producing sweat gland carcinoma of the eyelid. Ophthal Plast
Reconstr Surg 2008; 24: 164-5.
9. Rahilly MA, Beattie GJ, Lessells AM. Mucinous eccrine carcinoma
of the vulva with neuroendocrine differentiation. Histopathology
1995; 27: 82-6.
10. Tsang WY, Chan JK. Endocrine ductal carcinoma in situ (E-DCIS)
of the breast: a form of low-grade DCIS with distinctive clinicopatho-
logicand biologic characteristics. Am J Surg Pathol 1996; 20: 921-43.
11. Snow SN, Reizner GT. Mucinous eccrine carcinoma of the eyelid.
Cancer 1992; 70: 2099-104.
12. Kazakov DV, Suster S, LeBoit PE, et al. Mucinous carcinoma of the
skin, primary, and secondary: a clinicopathologic study of 63 cases
with emphasis on the morphologic spectrum of primary cutaneous
forms: homologies with mucinous lesions in the breast. Am J Surg
Pathol 2005; 29: 764-82.
Sunhee Chang Sang Hwa Shim Mee Joo, et al.