Eccrine Porocarcinoma: New Insights and a Systematic

Review of the Literature

Azadeh Nazemi, MS,* Shauna Higgins, MD,* Reyna Swift, MD,* Gino In, MD, MPH,*†
Kimberly Miller, PhD, MPH,*‡ and Ashley Wysong, MD, MS*x

BACKGROUND Eccrine porocarcinoma (EPC) is a rare cutaneous neoplasm with high potential for morbidity
and mortality. Due to its rarity, there is a paucity of data profiling diagnosis, work-up, and management.

OBJECTIVE To consolidate reported information on demographics, diagnostics, clinical behavior, treatment

modalities, and patient outcomes in EPC to provide a reference tool to optimize diagnosis and management.

METHODS A comprehensive PubMed search was performed from 1963 to November 2017 using PRISMA
guidelines. This yielded 155 articles detailing 206 cases of porocarcinoma.

RESULTS Eccrine porocarcinoma most often presents in elderly patients on the head and neck or lower
limbs. Metastatic disease at presentation is not uncommon (22%). Primary tumor location is significantly
correlated with presence of metastasis (p = .038). The most common treatment is excision followed by Mohs
micrographic surgery (MMS), although the outcomes after MMS were superior to those after surgical excision.

CONCLUSION This systematic review of individual patient data reveals that all patients should have a his-
tological diagnosis with imaging considered for high-risk cases. Primary tumor location should also be con-
sidered in diagnostic and therapeutic decision-making. Although wide local excision (WLE) is currently the
first-line treatment, MMS is becoming increasingly used, with evidence indicating improved outcomes as
compared to those seen with WLE.

The authors have indicated no significant interest with commercial supporters.

ccrine porocarcinoma (EPC) was first described

E by Pinkus and Mehregan in 1963, at which time it
was called epidermotropic eccrine carcinoma to reflect
its histological epidermotropism and pagetoid
diffusion.1 The term eccrine porocarcinoma was
coined in 1969 by Mishima and Morioka.2 Eccrine
porocarcinoma is generally rare, representing 0.005%
to 0.01% of all cutaneous tumors; however, it is the
most frequently encountered malignant eccrine gland
tumor.3–5 Eccrine sweat glands consist of secretory
coils in the lower dermis or subcutis that secrete
electrolytes and water into a thin duct terminating in
the acrosyringium, the intraepidermal ductal portion
opening directly to the surface of the skin. Poromas are
benign sweat gland tumors that arise from the
acrosyringium. Triggers for poroma development
include electron beam therapy and chronic radiation
dermatitis.6,7 It has been noted that up to 18% to 50%
of porocarcinomas degenerate from initially benign
poromas.8,9 Factors hypothesized to play a role in this
transformation include chronic light exposure and
immunosuppression.10
Eccrine porocarcinomas are histologically diverse;
however, common characteristics on hematoxylin &
eosin (H&E) staining include nuclear atypia, increased
mitotic activity, and necrosis. Polygonal tumor cells
may also be seen clustering to form multiple intra-
epidermal nests of varying sizes with regular squa-
mous or dyskeratotic cells. Often, epidermal
acanthosis and hyperkeratosis are present. Tumor
borders may be “infiltrative,” “pagetoid,” or

Departments of *Dermatology, †Medical Oncology, ‡Preventative Medicine, and xOtolaryngology-Head and Neck
Surgery, Keck Medicine of USC, Los Angeles, California

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· ·
ISSN: 1076-0512 Dermatol Surg 2018;0:1–15 DOI: 10.1097/DSS.0000000000001566

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 POROCARCINOMA: A SYSTEMATIC REVIEW

“pushing.” Infiltrative EPCs are characterized by
a poorly defined lower margin with malignant cell
clusters infiltrating the dermis; pagetoid EPCs have
intraepidermal spread of tumor cells mimicking Paget
disease; pushing EPCs have a distinct dermal lining
around the polypoid tumor.152 Immunohistochemical
stains used for the diagnosis of EPC include carci-
noembryonic antigen (CEA), cytokeratin (CK) (pan-
cytokeratin and CK5/6), epithelial membrane antigen
(EMA), p53, and p63.11 Staining for p63 may be of
particular utility because positive p63 indicates a pri-
mary cutaneous neoplasm.11 CD117 has been repor-
ted to be highly effective in identifying EPC and recent
evidence has indicated that CD117 may be helpful in
distinguishing EPC from squamous cell carcinoma.12
To date, current literature reports that EPC most com-
monly develops on the lower extremities (44%), trunk
(24%), or head and neck region (24%) and is most
prevalent in adults aged 50 to 80 years.4,13 There are
conflicting reports regarding sex predominance.4,13–15
The neoplasm may have morphologically diverse clinical
presentations that include papules, nodules, and pla-
ques.16 Treatment is necessary due to its aggressive
nature. Approximately 20% of patients experience local
recurrence and 20% experience metastatic disease after
treatment. In patients with lymph node involvement,
mortality rates are approximately 67%.16 The survival
period for patients with distant metastases is reported to
be 5 to 24 months.17
Given the high potential for morbidity and mortality
in EPC, prompt and effective treatment is of para-
mount importance. Many therapeutic approaches
with varying results have been reported with no clear
standard.18 This review aims to consolidate existing
data regarding the diagnosis and management of EPC
to elucidate trends and to create a reference for clini-
cians when they encounter this rare, poorly charac-
terized neoplasm.

Figure 1. PRISMA flow diagram. It summarizes the steps involved in the systematic review of porocarcinoma. From: Moher
D, Liberati A, Tetzlaff J, Altman DG, The PRISMA Group. Preferred Reporting Items for Systematic Reviews and Meta-
Analyses: The PRISMA Statement. PLoS Med 2009;6:e1000097. doi:10.1371/journal.pmed1000097.

2 DERMATOLOGIC SURGERY

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 NAZEMI ET AL

Methods among the sexes with 52.9% (109/206) of reported cases

occurring in males and 47.1% (97/206) in females. Most
Search Strategy cases did not report ethnicity (61.2%, 126/206). Of
the 80 cases that did, Caucasian (62.5%, 50/80) and
This review was performed using the Preferred
Japanese (16.3%, 13/80) were most prevalent, with other
Reporting Items for Systematic Reviews and Meta-
ethnicities including African American, Korean, Chinese,
Analyses (PRISMA-IPD) guidelines. All reported
Hispanic, and Filipino (Table 1).2–40,41–80,81–120,121–153
cases of porocarcinoma from 1963 to November
2017 were identified using PubMed. Search terms
Histopathologic Characteristics
included: “eccrine porocarcinoma” (MeSH Terms),
OR “eccrine” AND “porocarcinoma,” OR “eccrine All cases of EPC were diagnosed using histology. Of
porocarcinoma,” OR “porocarcinoma.” The initial the 206 cases, the most common histological markers
search yielded 335 results. Of these, 144 were used included EMA (100% positive, 51/51), periodic
excluded because they lacked quantitative data on acid–Schiff (PAS) (100% positive, 30/30), Ki67
individual patients. An additional 18 were non- (100% positive, 6/6), CK AE1/AE3 (100% positive,
English with no full-text translation available and 22/22), p63 (100% positive, 7/7), CEA (95% positive,
20 were not accessible through the extensive aca- 58/61), p53 (87.5% positive, 7/8), CK7 (78.9% pos-
demic library network used for this review, and were itive, 15/19), and S100 (38.1% positive, 8/21). Other
thus excluded (Figure 1). The remaining 153 articles stains expected to be negative included CK20 (0%
yielded 206 cases included in this review. Patient positive, 0/10), synaptophysin (0% positive, 0/4), and
demographics, tumor characteristics, work-up, and TTF-1 (0% positive, 0/4) (Table 2).
treatment modalities were recorded. Outcomes
included local recurrence, lymph node metastasis,
distant metastasis, and survival (patients being alive
without disease [AWOD], alive with disease [AWD], TABLE 1. Demographic Results
or death).
Porocarcinoma
Diagnosis
Data Analysis
Characteristic N = 206
Relevant individual patient data were extracted Age, y
Mean (SD) 63.6 (16.6)
from the included 153 articles. Data were consoli-
Median (IQR) 65 (54–77)
dated and subsequently summarized with descrip-
Sex
tive statistics. A Pearson chi-square test was used to Male, N (%) 109 (52.9)
compare frequencies and determine statistical sig- Female, N (%) 97 (47.1)
nificance of differences in primary tumor site as it Ethnicity
relates to presence of any metastatic disease. Fisher African American, N (% of 80 8 (10.0)
reported)
exact test was used to compare frequencies and
Asian, N (% of 80 reported) 3 (3.8)
determine the statistical significance of differences in Caucasian, N (% of 80 50 (62.5)
primary tumor site and presence of distant metas- reported)
tasis. All data analysis was performed in Stata Chinese, N (% of 80 reported) 2 (2.5)
Filipino, N (% of 80 reported) 1 (1.3)
(Version 12.1; StataCorp, College Station, TX).
Hispanic, N (% of 80 1 (1.3)
reported)
Japanese, N (% of 80 13 (16.3)
Results reported)
Korean, N (% of 80 reported) 2 (2.5)
Demographics Not reported, N (% of 206 126 (61.2)
total)
The median age at presentation was 65 (interquartile
IQR, interquartile range.
range 54–77). Incidence was approximately equal

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 POROCARCINOMA: A SYSTEMATIC REVIEW

Ten cases reported data on tumor depth on pre-
sentation, with a range of 1.8 to 29 mm. Nine cases
reported mitotic index, with a range of 4 to 50 per
high-power field. No trends existed to designate
a specific tumor depth or mitotic index as high-risk.
Clinical Presentation
Primary tumor location of the head and neck (32%,
66/206) and lower limbs (33%, 68/206) were most
common. These were followed by involvement of the
trunk (14.1%, 29/206) and genitalia/buttocks
(11.2%, 23/206) (Table 3). Upper limb involvement
was least common (9.7%, 20/206) (Figure 2). Of the
66 head and neck tumors, a predominance was on the
scalp (21.2% [14/66] or 6.8% [14/206] overall),
whereas of the 68 lower extremity tumors, the
majority were on the leg (40.6% [23/68] or 11.2%
[23/206] overall).
At the time of diagnosis, 22.3% (46/206) patients had
metastatic disease. These included regional lymph

TABLE 2. Special Immunohistochemical Stains Used in Eccrine Porocarcinoma

Stain Positive Negative % Positivity (n)

Epithelial membrane antigen (EMA) 51 0 100% (51)
Periodic acid–Schiff (PAS) 30 0 100% (30)
Cytokeratin (CK) AE1/AE3 13 0 100% (13)
Antigen Ki67 6 0 100% (6)
p63 7 0 100% (7)
Cytokeratin (CK) 19 4 0 100% (4)
Cytokeratin (CK) 5/6 3 0 100% (3)
CA 19–9 antigen 2 0 100% (2)
Cytokeratin (CK) antibody KL1 2 0 100% (2)
Cytokeratin (CK) 116 2 0 100% (2)
MIB1 antibody 2 0 100% (2)
Mucicarmine 2 0 100% (2)
Cytokeratin 34beta(b)E12 1 0 100% (1)
Angiotensin converting enzyme (ACE) 1 0 100% (1)
CA 15–3 antigen 1 0 100% (1)
Cytokeratin (CK) 14 1 0 100% (1)
Cytokeratin (CK) 8 1 0 100% (1)
Podaplanin (D2-40) 1 0 100% (1)
p16 1 0 100% (1)
Carcinoembryonic antigen (CEA) 57 3 95% (60)
p53 7 1 87.5% (8)
Cytokeratin (CK) 7 15 4 78.9% (19)
Anti-cytokeratin (CAM 5.2) 3 2 60% (5)
Anti-EpCAM antibody (BerEP4) 1 1 50% (2)
S100 Protein 8 13 38.1% (21)
Cluster of differentiation (CD) 15 1 2 33.3% (3)
Cytokeratin (CK) 20 0 10 0% (10)
Synaptophysin 0 4 0% (4)
Thyroid transcription factor (TTF)-1 0 4 0% (4)
Human epidermal growth factor receptor (HER)2/neu 0 3 0% (3)
Chromogranin A 0 2 0% (2)
Cytokeratin (CK) 88 0 1 0% (1)
Anti-MUC1 antibody (HMFG1) 0 1 0% (1)
Mammaglobin 0 1 0% (1)
Non-specific esterase (NSE) 0 1 0% (1)
Prostate specific antigen (PSA) 0 1 0% (1)

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 NAZEMI ET AL

TABLE 3. Anatomic Location of Primary Lesion

All Cases All Cases All Cases

Head and neck, N (%) 66 (32) Trunk, N (%) 29 (14.7) Lower limb, N (%) 68 (33)
Cheek 2 Abdomen 10 Ankle 2
Ear 8 Back 8 Calf 1
Eyelid 8 Breast 2 Foot 14
Face 4 Chest 5 Heel 3
Forehead 3 Crus 1 Knee 2
Head 2 Flank 2 Leg 23
Lip 1 Thorax 1 Sole 2
Mandible 1 Thigh 16
Neck 4 Upper limb, N (%) 20 (7.4) Tibia 2
Nose 3 Arm 10 Toe 3
Occiput 3 Axilla 2 Genitalia/buttocks, N (%) 23 (11.2)
Parietal 1 Finger 2 Buttocks 5
Parieto-occipital 1 Hand 3 Inguinal 2
Preauricular 1 Palm 1 Labium 5
Retroauricular 3 Shoulder 2 Mons pubis 1
Scalp 14 Perianal 3
Temple 2 Prepuce 1
Temporal 3 Scrotum 6
Temporomandibular 1
Temporoparietal 1

node metastasis (17%, 35/206), distant metastasis
(3.9%, 8/206), and locoregional cutaneous metastasis
(1.5%, 3/206). Statistically significant differences
were found between site of primary lesion and pres-
ence of any metastatic disease (p = .038). Lymph node
metastasis on presentation was associated with pri-
mary lesions on the lower limb (20.5%, 14 of 68
lower-limb tumors), trunk (24.1%, 7 of 29 trunk
tumors), genitalia/buttocks (30.4%, 7 of 23 genitalia/
buttocks tumors), upper limb (15%, 3 of 20 upper-
limb tumors), and head/neck (6.1%, 4 of 66 head/neck
tumors). Thus, across all primary tumor sites, head
and neck tumors seem to have the lowest incidence of
lymph node metastasis at time of presentation.

Figure 2. Anatomic location of primary EPC lesion. EPC, eccrine porocarcinoma.

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 POROCARCINOMA: A SYSTEMATIC REVIEW

Statistically significant differences were found
between site of primary lesion and presence of distant
metastatic disease (p < .0001). Distant metastasis was
associated with primary lesions on the genitalia/but-
tocks (13%, 3 of 23 genitalia/buttocks tumors), upper
limb (10%, 2 of 20 upper limb tumors), head/neck
(3%, 2 of 66 head/neck tumors), and lower limb
(1.5%, 1 of 68 lower-limb tumors) (Table 4).
Diagnostics
Radiology was used at diagnosis in approximately
half of reported cases (41.3%, 85/206). The most
common imaging techniques were computed
tomography (CT) (43.5% [37/85] or 18% [37/206]
of all porocarcinomas), magnetic resonance imaging
(MRI) (21.1% [18/85] or 8.7% [18/206]), positron
emission tomography/computed tomography (PET/
CT) (16.5% [14/85] or 6.8% [14/206]), X-ray
(11.8% [10/85] or 4.9% [10/206]), and ultrasound
(8.2% [7/85] or 3.4% [7/206]) (Table 5). Twenty-one
cases of occult lymph node metastasis, or 60% (21/
35) of total lymph node metastases, were diagnosed
with imaging on presentation. Of these, 62% used CT
(13/21), 23.8% PET/CT (5/21), 9.5% MRI (2/21),
and 4.8% ultrasound (1/21). Fourteen cases of dis-
tant metastasis (on presentation and after treatment)
were diagnosed with imaging, of which 50% used
PET/CT (7/14), 35.7% CT (5/14), 7.1% MRI (1/14),
and 7.1% X-ray (1/14).
Palpable lymphadenopathy was noted at presentation
in 6.3% cases (13/206) and fine-needle aspiration
(FNA) was performed with 100% positivity. Sentinel
lymph node biopsy (SLNB) was performed in 16 cases
of suspected lymph node involvement (45.7% [16/35]
of lymph node metastases or 7.8% [16/206] of all
porocarcinomas). The majority of SLNBs revealed
metastases (81.3%, 13/16), whereas the remaining 3
were negative (18.7%, 3/16). Of the positive SLNBs,
primary tumor location was noted as head and neck in
30.8% (4/13), trunk in 23.1% (3/13), upper limbs in
15.4% (2/13), lower limbs in 23.1% (3/13), and gen-
italia/buttocks in 33.3% (1/13) of cases. Of the nega-
tive SLNBs, primary tumor locations were noted to be
the head and neck in 66.7% (2/3) and the lower
extremities in 33.3% (1/3) of cases. Sentinel lymph
node biopsies were most common in the head/neck
region (37.5%, 6/16), trunk (25%, 4/16), and lower
limbs (25%, 4/16), followed by upper limbs (6.3%,
1/16) and genitalia (6.3%, 1/16).
Treatment
The most common treatments for the 160 primary
tumors without metastasis were wide local excision

TABLE 4. Clinical Presentation at Diagnosis

On Presentation Distant Metastasis Lymph Node Metastasis Cutaneous Metastasis

Total (N) 8 35 3
Age, yr
Mean (SD) 57 (20) 66 (15) 72 (14)
By decades, N (%)
20–39 1 (12.5) 4 (11.4) —
40–59 3 (37.5) 10 (28.6) 1 (33.3)
60–79 4 (50.0) 15 (42.8) 1 (33.3)
80–99 — 6 (17.1) 1 (33.3)
Sex
Male, N (%) 6 (75) 16 (45.7) 1 (33.3)
Female, N (%) 2 (25) 19 (54.3) 2 (66.6)
Location
Head 2 (25%) 4 (11.4%) 1 (33.3%)
Trunk — 7 (20%) 1 (33.3%)
Upper limb 1 (12.5%) 3 (8.6%) —
Lower limb 4 (50%) 14 (40%) —
Genitalia 1 (12.5%) 7 (20%) 1 (33.3%)

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 NAZEMI ET AL

TABLE 5. Diagnostic Imaging and Results

Primary Mass Regional Metastasis Distant Metastasis No Visceral Involvement

Diagnostic Tool (N) N (%) N (%) N (%) N (%)
CT (37) 3 (8.1) 13 (35.1) 5 (13.5) 16 (43.2)
PET/CT (14) — 6 (42.9) 7 (50) 1 (7.1)
MRI (18) 9 (50) 1 (5.6) 1 (5.6) 7 (38.9)
X-ray (10) 2 (20) — 1 (10) 7 (70)
Ultrasound (7) 1 (14.3) 1 (14.3) — 5 (71.4)
FNA (13) — 13 (100) — —
SLNB (16) — 16 (81.3) — —

CT, computed tomography; FNA, fine-needle aspiration; MRI, magnetic resonance imaging; PET, positron emission tomography; SLNB,
sentinel lymph node biopsy.

(WLE) (55% [88/160] or 42.7% [88/206] of all
porocarcinomas), Mohs micrographic surgery (MMS)
(26.2% [42/160] or 20.4% [42/206]), and amputation
(2.5% [4/160] or 1.9% [4/206]) (Figures 3 and 4).
Treatments used at a rate of 0.6% (1/160 or 0.4%
[1/206]) included WLE with radiotherapy, WLE with
antibiotics, electrochemotherapy (ECT), oral cefazo-
lin, and diphencyprone. Twenty-one cases did not
report treatment data.
The treatments for the 35 patients presenting with
nodal metastasis were more varied. They included
WLE with lymphadenectomy (22.8% [8/35] or 3.8%
[8/206] of all porocarcinomas) and amputation with
lymphadenectomy (14.3% [5/35] or 2.4% [5/206]).
Wide local excision alone, WLE with chemotherapy,
and WLE with lymphadenectomy and radiotherapy
were each used at a rate of 11.4% (4/35) or 1.9%
(4/206). Other treatments include WLE with radio-
therapy (8.6% [3/35] or 1.5% [3/206]), WLE with
chemotherapy and radiotherapy (5.7% [2/35] or 1%
[2/206]), and radiotherapy (5.7% [2/35] or 1%
[2/206]). Treatments used at a rate of 2.9% (1/35 or
0.5% [1/206]) included amputation, WLE with
radiotherapy and chemotherapy, and ECT with
lymphadenectomy.

Figure 3. Distribution of various treatments. “All excisions” includes cases with and without adjunctive treatment. Treat-
ments comprising the “Other” group include oral cefazolin, diphencyprone, electrochemotherapy, and radiation with
chemotherapy. MMS, Mohs micrographic surgery.

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 POROCARCINOMA: A SYSTEMATIC REVIEW

Figure 4. Breakdown of treatments of porocarcinomas presenting with primary tumor only at diagnosis. Chemo, chemo-
therapy; radio, radiotherapy; lymph, lymphadenectomy; IFN, interferon therapy; photo, phototherapy.

The 8 patients presenting with distant metastatic
disease were predominantly treated with chemo-
therapy (including 5-fluorouracil, cisplatin, doxo-
rubicin, vincristine, and mitomycin) (62.5%, [5/
8]). Treatments used at a rate of 12.5% (1/8)
include chemotherapy with radiotherapy, WLE
with chemotherapy and radiotherapy, and WLE
with lymphadenectomy, chemotherapy, radio-
therapy, and interferon. Patients presenting with
cutaneous metastasis were treated with chemo-
therapy (66.7%, 2/3) or refused treatment
(33.3%, 1/3).
Clinical Outcomes
Patients presenting with primary tumor without
metastasis who were treated with MMS were AWOD
at a rate of 83% (35/42) at follow-up times ranging
from 2 to 206 months. A minority experienced lymph
node metastasis (2.4%, 1/42) after 24 months. Seven
percent of MMS cases (3/42) did not report patient
outcomes and 7.1% (3/42) died of other causes (Fig-
ure 5). Patients who received amputation, WLE with
radiation, ECT with lymphadenectomy, and diphen-
cyprone were all AWOD at follow-up. Data were not
reported on the patient treated with cefazolin.
Of the 35 patients with nodal metastasis on presentation,
those treated with WLE and lymphadenectomy (n = 8)

Of all primary tumor cases without metastasis on

presentation that were treated by WLE, 56.8% (50/
TABLE 6. Excision Margins and Time Until
88) were AWOD after a follow-up time ranging Recurrence
from 1 to 360 months. Seven patients experienced
lymph node metastasis after WLE (8%, 7/88) and 10 Excision
Margins N (%) Recurrence N (Time)
experienced distant metastasis (11.4%, 10/88).
Average times until lymph node and distant metas- 3 mm 1 (3.8)
4 mm 2 (7.7) 1 (9 mo)
tasis were 11 and 9 months, respectively. Twenty-
5 mm 2 (7.7)
two percent of WLE cases (20/88) did not report 10 mm 13 (50) 3 (2 wk, 8 mo, not
treatment results. Excision margins were reported in reported)
26 cases. A 10-mm margin was most common (50%, 20 mm 6 (23.1) 2 (6 mo, 8 mo)
13/26). Additional margins included 3, 4, 5, 20, 25, 25 mm 1 (3.8)
30 mm 1 (3.8) 1 (10 wk)
and 35 mm (Table 6).

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 NAZEMI ET AL

Figure 5. Outcomes curves depicting proportion of patients alive without disease over total patients at that follow-up time point.
Outcomes are depicted by treatments of primary tumors only (without lymph node or distant metastasis) on presentation (A) and
tumors with lymph node metastasis on presentation (B). Primary tumor treatments include excision (N = 45) and Mohs
micrographic surgery (N = 35). Lymph node metastasis treatments include WLE with lymphadenectomy (N = 6), amputation with
lymphadenectomy (N = 3), and WLE (N = 3). Survival time is limited by loss to follow-up. WLE, wide local excision; MMS, Mohs
micrographic surgery.

experienced distant metastasis (50% [4/8] or 11.4%
[4/35]) after an average of 6.5 months and additional
lymph node metastasis (25% [2/8] or 5.7% [2/35]) after
an average of 9.8 months. One death was reported due to
pneumonia (12.5% [1/8] or 3% [1/35]). One case did not
report results after treatment. The 5 patients treated with
amputation and lymphadenectomy were AWOD at
a rate of 40% (2/5 or 2/35 [5.7%]) or experienced distant
metastasis (20% [1/5] or 2.9% [1/35]) after 7 months.
Forty percent (2/5) did not report results. Outcomes of the
4 patients treated with WLE alone included lymph node
metastasis (25% [1/4] or 2.9% [1/35]) after 4 months or
patients were AWOD at 180 months (25% [1/4] or 2.9%
[1/35]). Two did not report results (50%). Outcomes of
the 4 patients treated with WLE with lymphadenectomy
and radiotherapy included distant metastasis (50% [2/4]
or 5.7% [2/35]) after an average of 4.5 months, patients
being AWOD at 12 months (25% [1/4] or 2.9% [1/35]),
and one was not reported. Outcomes of the 2 patients
treated with radiotherapy included distant metastasis
(50% [1/2] or 2.9% [1/35]) after 1 month. The other
result was not reported. All patients treated with WLE
plus chemotherapy, amputation, WLE with radiother-
apy, and ECT with lymphadenectomy were AWOD at
follow-up times ranging from 5 to 48 months. Data were
not reported on the patient treated with combination
WLE, chemotherapy, and phototherapy.
The 8 patients with distant metastasis at time of pre-
sentation experienced lymph node and distant metastasis
beyond what was seen at initial presentation (25% [2/8]
and 50% [4/8], respectively) and 2 deaths (25%) after
treatment. The 5 chemotherapy-treated patients experi-
enced lymph node metastasis (40% [2/5] or 25% [2/8])

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 POROCARCINOMA: A SYSTEMATIC REVIEW
after an average of 13 months, distant metastasis (40%
[2/5] or 25% [2/8]) after an average of 7.5 months, and
one death from sepsis (20% or 1/7 [14.3%]). Additional
distant metastases were seen after radiotherapy plus
chemotherapy, after WLE with chemotherapy plus
radiotherapy, and after combination of WLE, lympha-
denectomy, radiotherapy, and interferon (n = 1 for each).
Overall, follow-up times ranged from 1 month to 30 years.
The median follow-up time for patients AWOD was 12
months (range 1–360 months). Median follow-up time for
patients AWD was 10.5 months (range 1–18 months).
Discussion
Eccrine porocarcinoma is a rare malignant cutaneous
adnexal neoplasm with high potential for morbidity
and mortality. Given its rarity, no guidelines currently
exist for EPC diagnosis, work-up, or management.
This systematic review using PRISMA criteria quan-
titatively consolidated data on existing cases of EPC,
elucidating new and occasionally confirming known
patterns in demographics, diagnostics, and treatment.
In doing so, the authors identify several clinical pearls
that may be useful in this regard.
Demographic considerations seem to be consistent
with the current body of literature, with a mean age of
63.6 years at diagnosis, within the range of 50 to 80
reported in the literature, and approximately equal
incidence among males and females. Tumors were
found at higher rates in the head/neck region (32% vs
24%) and lower rates (33% vs 44%) on the lower
limbs compared with previous literature reports. The
combined overall metastatic rate of 22% on pre-
sentation is consistent with previous reports.
Histological appearance alone seems less useful because
the tumor cells can be confused with squamous, clear,
spindle, or Paget cells or melanocytes. Histologic stains
therefore become extremely useful to confirm the pri-
mary tumor diagnosis with EMA, PAS, CKs (AE1/AE3),
p63, and Ki67 positive in 100% of cases reported.
Radiology may be useful for surgical planning and
evaluation of regional or distant metastasis, includ-
ing the detection of occult metastases, with approxi-
10 DERMATOLOGIC SURGERY
© 2018 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
mately half of all patients receiving some diagnostic
imaging. Previous reports suggest that high-risk fea-
tures including increased tumor depth (>7 mm),
lymphovascular invasion, high number of mitoses
(>14 mitoses per 10 high power fields), and poorly
differentiated tumor may suggest the need for diag-
nostic imaging.139 Primary tumor location is also
found here to be significantly correlated with pres-
ence of any metastasis (p = .038) and distant metas-
tasis (p < .0001) and therefore should be considered
as well. Specifically, the authors’ data suggest that the
head/neck region may be responsible for a compara-
tively smaller proportion of lymph node metastasis
when compared with other primary tumor locations.
Ultrasound, CT, or MRI of the regional lymph node
basins should be considered for primary tumors on
the trunk, extremities, and genitalia. The data also
suggest an increased proportion of genitalia/buttocks
primary tumors eventuating in distant metastasis;
PET/CT scan might be considered in these patients.
Although further study is warranted in these areas,
this elucidates the importance of considering primary
tumor location in the decision to pursue diagnostic
radiology and also in overall diagnostic and thera-
peutic decision-making.
Sentinel lymph node biopsy should also be considered
if patients possess high-risk features but do not have
palpable lymphadenopathy, in which case FNA would
be performed. Sentinel lymph node biopsy was suc-
cessful in identifying occult lymph node metastasis
with an 81.3% positivity rate (18.7% negativity). One
hundred percent were positive on the trunk (4/4), 66%
were positive in the head and neck region (4/6), and
75% positive on the lower limbs (3/4), indicating that
a healthy index of suspicion should be maintained for
disease spread to these areas.
Wide local excision was the most common therapy
used in this systematic review; however, local recur-
rence rates were reported as 21% and patients who
underwent WLE account for 61% of patients who
developed subsequent metastasis. The surgical mar-
gins varied from 3 to 30 mm, and further prospective
studies on margins are necessary to make an assess-
ment in the context of patient outcomes and
recurrence.

Of note, when metastasis developed in patients treated
with excision alone, it generally occurred several
months before metastasis in those patients treated with
combined excision and adjuvant therapy. Thus,
patients should be monitored accordingly. Overall,
there is a paucity of data for adjuvant therapy in
porocarcinoma and further study is warranted.
Although WLE has historically been considered first-
line therapy, there is a temporal variation in manage-
ment with more recent literature citing MMS and
complete margin assessment as an excellent treatment
modality for the management of primary tumors. In
the authors’ review, MMS resulted in far fewer cases of
metastasis (2.4%, 1 of 42 patients treated with Mohs)
than excision (18%, 16 of 88 patients treated with
excision). Of the 28 cases of metastasis after treatment,
61% (17/28) occurred after WLE and 3.6% (1/28)
after MMS.
In summary, porocarcinoma is a rare cutaneous tumor
with high rates of extracutaneous spread. Accord-
ingly, it is imperative to appropriately diagnose and
treat EPC. The diagnosis should be confirmed using
immunohistochemistry because there are several his-
tologic mimics. Although the literature does not
definitively support diagnostic imaging and adjunctive
therapies, the presence of high-risk features should
precipitate consideration of imaging, nodal staging,
and potentially SLNB. The location of the primary
tumor should be considered in the decision to use these
modalities as well because it seems that primary lesions
on the upper and lower limbs, trunk, and genitalia/
buttocks have high risk of lymph node metastasis,
whereas head and neck tumors seem to have a com-
paratively lower risk. It also seems that primary lesions
on the genitalia/buttocks have a particularly increased
propensity for distant metastasis. Given the propensity
for nodal/distant metastases, EPC should be treated at
an academic center under a multimodality approach.
Novel systemic treatment options are needed and the
development of checkpoint inhibitors may offer
promise, but have not yet been evaluated. Although
WLE was reportedly the most common treatment
modality, more recently, there is increasing evidence
for MMS and complete margin assessment as the
preferred treatment of the primary tumor.
© 2018 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. Unauthorized reproduction of this article is prohibited.
NAZEMI ET AL
Despite the rarity of EPC, its high potential for mor-
bidity and mortality necessitates increased attention
from the scientific community. Although the authors’
review provides much-needed updates on EPC, further
prospective study is warranted. Limitations of this
study include the relatively small number of cases due
to the rarity of the disease. In addition, not all cases
were included because many studies excluded indi-
vidual patient data, precluding them from being used
in this analysis. Thus, conclusions made here should
continue to be reassessed and developed with contin-
ued reporting of cases. To the best of the authors’
knowledge, however, this study is the broadest review
assessing work-up, treatments, and outcomes of EPC
in individual patients to date.
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Address correspondence and reprint requests to: Shauna
Higgins, MD, Department of Dermatology, Keck Medicine
of USC, Los Angeles, CA 90017, or e-mail:
shaunahigginsmd@gmail.com
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