Eccrine Porocarcinoma Presenting with Unusual Clinical Manifestations: A Case Report and Review of the Literature

Article (PDF Available)inAnnals of Dermatology 23 Suppl 1(Suppl 1):S79-83 · September 2011with17 Reads
DOI: 10.5021/ad.2011.23.S1.S79 · Source: PubMed
Abstract
Eccrine porocarcinoma is a rare neoplasm that originates from the intraepidermal ductal portion of the eccrine sweat gland, and it usually occurs on the pre-existing lesion of benign eccine poroma. Its occurrence is more common in females and elderly persons. We present a case of a 44-year-old man who suffered from eccrine porocarcinoma, which developed on the right scrotum and pelvic area with metastases to the lung, adrenal gland, esophagus, ureter, and distant lymph nodes. Here we report on a unique case of eccrine porocarcinoma that developed primarily on the scrotum, which is an uncommon site, and showed rapid metastasis to the internal organs.

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Vol. 23, Suppl. 1, 2011 S79
Received June 30, 2010, Revised October 4, 2010, Accepted for
publication October 4, 2010
Corresponding author: Woo-Young Sim, M.D., Department of Der-
matology, Kyunghee University Hospital at Gangdong, 149 Sangil-
dong, Gangdong-gu, Seoul 134-727, Korea. Tel: 82-2-440-7329, Fax:
82-2-440-7336, E-mail: bellotte@hanmail.net
T
his is an Open Access article distributed under the terms of the
Creative Commons Attribution Non-Commercial License (http:/
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creativecommons.org/licenses/by-nc/3.0) which permits unrestricted
non-commercial use, distribution, and reproduction in any medium,
provided the original work is properly cited.
Ann Dermatol Vol. 23, Suppl. 1, 2011 http://dx.doi.org/10.5021/ad.2011.23.S1.S79
CASE REPORT
Eccrine Porocarcinoma Presenting with Unusual Clinical
Manifestations: A Case Report and Review of the
Literature
Chang-Min Choi, M.D., Hee-Ryung Cho, M.D., Bark-Lynn Lew, M.D., Woo-Young Sim, M.D.
Department of Dermatology, Kyunghee University Hospital at Gangdong, Seoul, Korea
Eccrine porocarcinoma is a rare neoplasm that originates
from the intraepidermal ductal portion of the eccrine sweat
gland, and it usually occurs on the pre-existing lesion of
benign eccine poroma. Its occurrence is more common in
females and elderly persons. We present a case of a
44-year-old man who suffered from eccrine porocarcinoma,
which developed on the right scrotum and pelvic area with
metastases to the lung, adrenal gland, esophagus, ureter, and
distant lymph nodes. Here we report on a unique case of
eccrine porocarcinoma that developed primarily on the
scrotum, which is an uncommon site, and showed rapid
metastasis to the internal organs. (Ann Dermatol 23(S1) S79
S83, 2011)
-Keywords-
Atypical clinical manifestations, Eccrine porocarcinoma,
Neoplasm metastasis, Scrotum
INTRODUCTION
Eccrine porocarcinoma (EPC) is a rare cutaneous malig-
nancy that arises from the intraepidermal ductal portion of
the eccrine sweat gland. Since it was first described by
Pinkus and Mehregan
1
. in 1963, hundreds of such cases
have been reported in the literature. It occurs in both
sexes, with a female predominance, and it is often seen
during the sixth to eighth decades of life. It may arise de
novo; however, it usually develops on the longstanding
site of eccrine poroma. The lower extremities are the most
common representing site, followed by the trunk, head,
and upper limbs
2
. Twelve cases have been reported in
Korea: one case showed metastasis to regional lymph
nodes from the right thigh
3
, while 11 cases were confined
to a local cutaneous area. However, a case of multiple
metastases to distant organs has not yet been reported.
Here we present a case of EPC that developed primarily
on the scrotum, which is an uncommon site, and it
showed metastasis to the internal organs in a relatively
young man, and we briefly review previous reports in the
Korean literature.
CASE REPORT
A 44-year-old man presented with painful skin lesions on
the right scrotum and pelvic area, which had been present
for the previous 7 months. The scrotal skin lesions were
small macules and papules at the beginning; however, the
lesions had shown rapid enlargement and spread to the
pelvic area. His past medical history was unremarkable,
except that he was a heavy drinker. On physical exa-
mination, more than half of the right scrotum was firm and
ulcerated (the area measured 6.0×7.0 cm in diameter),
and it was replaced by hyperemic granulation tissue with
a purulent discharge (Fig. 1A, B). Erythematous nodules
measuring 1.2×1.0 cm were also observed on the right
pelvic area (Fig. 1C). His right leg was enlarged, compared
to the left one, indicating lymphodema. The red cell count
was 4.79×10
6
and the white cell count was 9.9×10
3
with
CM Choi, et al
S80 Ann Dermatol
Fig. 1. (A, B) A firm and ulcerated right scrotum covered with hyperemic granulation tissue. (C) Multiple 1.2×1.0 cm-sized, well-
demarcated nodules on the right pelvic area.
Fig. 2. (A) Well-defined tumor nests composed of polygonal or cuboidal cells invade the dermis and subcutaneous fat layer. Note
the connection between the epidermis and tumor (H&E stain, ×40). (B) This high-grade tumor show area of necrosis (H&E stain,
×200). (C) The malignant cells have hyperchromatic, vesicular, and atypical nuclei with frequent mitoses. Ductal structures are present
(H&E stain, ×400).
64% neutrophils. The erythrocyte sedimentation rate was
34 mm/h (normal range: 015 mm/h) and the C-reactive
protein level was 3.19 mg/dl (normal range: 0.00.5 mg/dl).
Other hematological and biochemical tests, including α
-feto-protein (AFP), carcino-embryonic antigen (CEA), car-
bohydrate antigen (CA) 125, CA 19-9, and prostate
specific antigen (PSA) were within normal limits. Multiple
biopsy specimens showed that well- defined tumor nests
composed of polygonal or cuboidal cells had invaded the
dermis and subcutaneous fat layer. In addition, malignant
cells demonstrated conspicuous atypia, mitoses, and
necrosis. Nuclei of tumor cells were pleomorphic and ve-
sicular on high magnification. Ductal structures were also
observed (Fig. 2). On immunoperoxidase staining, EMA
and cytokeratin (CK) 7 were positive in tumor nests and
the cells were negative for S-100 protein, CK-20, thyroid
transcription factor-1 (TTF-1), and CEA (Fig. 3). Histo-
pathologic and immunoperoxidase findings were con-
sistent with EPC. Computed tomography (CT) and positron
emission tomography (PET) revealed metastases to both
lungs, the right adrenal gland, right ureter, esophagus, and
multiple lymph nodes (LN); both inguinal LNs, both
external iliac LNs, the interaorto- caval LN, the paraaortic
LN, and the cervical LN (Fig. 4). Combination treatment with
5-fluorouracil 1,000 mg/m
2
and cisplatin 75 mg/m
2
was
initiated. Unfortunately, following 2 cycles, he fell victim to
sepsis, and died.
DISCUSSION
EPC is a rare skin appendage tumor, with the main
incidence occurring in patients more than 60 years old.
The lower limbs are the most common site (44%), fol-
lowed by the trunk (24%), head (18%), upper limbs (11%),
Eccrine Porocarcinoma with Unusual Clinical Manifestations
Vol. 23, Suppl. 1, 2011
S81
Fig. 3. Immunoperoxidase stain shows positivity for (A) EMA and (B) CK-7, and negativity for (C) CEA.
Fig. 4. PET CT scans (A, B). Extensive metastasis involving the cervical, left supraclavicular, mediastinal, abdominopelvic and inguinal
lymph nodes. Metastatic lesions on the (C) right lung (D) and right adrenal gland on the CT scans (arrow).
and neck (3%)
2
. The scrotum is an extremely uncommon
primary location in that only 2 such cases have been
reported in the international medical literature
4,5
. EPC
tends to be localized; however, it sometimes spreads to
regional lymph nodes or adjacent skin, and it rarely
metastasizes to distant organs or distant lymph nodes.
Distant visceral metastases to the lung, retroperitoneum,
long bones, breast, liver, mediastinum, urinary bladder,
and ovary have been reported
4,6
. Clinical features of EPCs
reported in Korea to date are summarized in Table 1
3,7-16
.
CM Choi, et al
S82 Ann Dermatol
Table 1. Summary of reported cases of eccrine porocarcinoma in Korean literature
Author Sex Age Duration Site Size Metastasis Treatment
Kim et al.
3
F 86 10 years Right thigh 5.0×4.0 cm Regional LN Excisionradiation
J
ung et al.
7
F 72 5 years Left buttock 4.5×4.5 cm Excision
Lee et al.
8
M 69 30 years Back 2 cm None
Han et al.
9
F 71 5 years Scalp 4.0×3.8 cm Excision
Yoon et al.
10
M 57 3 years Right thumb 1.7 cm Excision
Kim et al.
11
F 37 4 years Ant.helix of right ear 1.0×1.0 cm Excision
Lee et al.
12
F 84 1.25 year Left eyelid 0.8×0.8 cm Excision
Shin et al.
13
F8256 years Scalp 2.0×2.0 cm Mohs surgery
Oh et al.
14
M 55 7 years Left sole 1.0×1.0 cm Excision
Oh et al.
14
M 54 2 years Back 1.0×1.0 cm Excision
Park et al.
15
M 75 3 months Suprapubic area 2.0×2.0 cm Excision
Choi et al.
16
F 71 4 years Right palm 3.5×2.5 cm Excision
Present case M 44 7 months Right scrotum 6.0×7.0 cm Chemotherapy
According to Shaw et al.
17
, all 27 cases of EPC arose from
a benign eccrine poroma (BEP); however, Robson et al.
2
reported occurrence of EPC in only 18% of cases of BEP.
In our view, skin manifestations of our patient developed
de novo because there was no preexisting lesion on the
scrotum as well as on the pelvic area. In addition, EPC that
arises from BEP usually shows very slow progression. This
patient was relatively young and his disease showed an
aggressive clinical course; the tumor developed primarily
on the scrotum and showed rapid metastasis to multiple
internal organs.
The histopathologic features of EPC are broad. Malignant
cells usually have large and hyperchromatic nuclei.
Nuclear atypia with frequent mitoses and necrosis are
characteristic. Cords and nests of polygonal tumor cells
penetrate to the adjacent dermis or extend into subcu-
taneous tissue. Histopathologically, metastatic adenocar-
cinoma, trabecular carcinoma, and Merkel cell carcinoma
should be included in the differential diagnosis. Distin-
guishing EPC from metastatic adenocarcinoma, and espe-
cially adenocarcinoma of a breast or lung origin, can be
difficult. However, most metastatic adenocarcinomas are
positive for EMA and CEA stains. Furthermore, a positive
reaction with CEA may be most helpful in differentiating
between primary and secondary lesions
18
. In the present
case, the negative reaction with CEA, the connection bet-
ween the epidermis and tumor, and the absence of
glandular structures are evidence for exclusion of the
diagnosis of metastatic adenocarcinoma. Trabecular carci-
noma and Merkel cell carcinoma also show findings com-
parable with EPC. Hyperchromatic, large, and irregular
nuclei arranged in cord or trabecular patterns are common
features of EPC and trabecular carcinoma; however, the
latter shows nuclear molding. On immunoperoxidase
studies, EMA and CK-7 are positive, and S-100 protein and
CK-20 are negative in EPC. CEA is negative in most EPC;
however, it can be positive in tumors containing well-
formed ducts
2
. However, trabecular carcinoma or Merkel
cell carcinomas express neuron-specific enolase (NSE) and
CK-20, and they do not express CK-7
19
. This present case
showed positivity for EMA and CK-7 and negativity for
S-100 protein, CK-20, and CEA. The negative result for
CEA might be due to the paucity of ductal structures in
this case.
Snow and Reizner
20
suggested local recurrence and
regional metastatic rates of approximately 20% and devel-
opment of distant metastases in 12% of cases. Similarly,
Robson et al.
2
observed that 11% of patients had metas-
tasis to distant organs; these patients have a high mortality
rate (64% in the series). They suggested that the prognosis
of EPC depends mainly on mitoses (more than 14 per high
power field), lympho-vascular invasion, and a tumor
depth of more than 7 mm. The present case was positive
for all of these poor prognostic factors, and this might
have induced the fatal clinical course.
We experienced a rather novel case of EPC on an uncom-
mon site and the patient presented with rapid multiple
metastases. Most EPCs are localized and a few of them are
metastatic. Therefore, it is essential to consider the possi-
bility of metastases of primary EPC in the process of
diagnostic evaluation of patients who are found to be
normal on a physical examination.
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    • "Poor prognostic factors are that in every growth area of the tumour, a maximum of 14 mitosis, lymphovascular invasion, tumour depth of >7mm, an infiltrative growth pattern in the tumour borders, multinodularity, ulceration and rapid growth [2]. In addition, a significant correlation has been determined between tumour size and prognosis [9]. In the current case, the tumour was of a depth to infiltrate some areas of muscle tissue. "
    Full-text · Article · Jan 2016 · Online Journal of Health and Allied Sciences
    • "neck, scalp, upper extremities, trunk and abdomen.[2] Scalp lesion can sometimes present with intracranial extension.[4] EPC usually metastasize through lymphatics to the regional lymph nodes.[5] There can be multiple cutaneous metastases or visceral metastases to lung, retroperitoneum, bones, breast, liver, mediastinum, urinary bladder, and ovary.[1] EPC arise denovo from the intraepidermal portion of eccrine duct or from pre-existing eccrine poroma. Robson et al reported occurrence of EPC in 18% of eccrine poroma.[6] This was supported by long clinical history of a nodular lesion with sudden increase in size. Symptoms like pain, itching and bleeding are in favour of malignant transf"
    [Show abstract] [Hide abstract] ABSTRACT: Eccrine porocarcinoma is a rare carcinoma arising from the intraepidermal duct of eccrine sweat glands and constitute about 0.005% of epithelial cutaneous neoplasms. Eccrine porocarcinoma is commonly seen in lower extremities, followed by head, scalp, upper extremities, trunk and abdomen. It can arise denovo or from pre-existing eccrine poroma in 18% of cases. We present a 45 years old female patient who presented with painful hard warty growth of size 3×2 cm with ulceration and an adjacent nodule measuring 1×1 cm in the lateral aspect of dorsum of left foot for the past 3 years with rapid increase in size for 3 months duration. Microscopically larger and smaller nodules showed features of eccrine porocarcinoma and eccrine poroma respectively. Immunohistochemistry for pancytokeratin showed strong membrane positivity, Ki-67 showed 40% nuclear positivity and CD 34 negative. The lesion was diagnosed microscopically as eccrine porocarcinoma associated with pre-existing eccrine poroma.
    Full-text · Article · Aug 2014
  • Full-text · Article · Jan 2013 · Online Journal of Health and Allied Sciences
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