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CASE REPORT Pigmented epithelioid melanocytoma: Report of a case and review of 173 cases in the literature Pai-Shan Cheng 1 , Shih-Sung Chuang 2 , Tseng-Tong Kuo 3 , Feng-Jie Lai 1 , * 1 Department of Dermatology, Chi Mei Medical Center, Tainan, Taiwan 2 Department of Pathology, Chi Mei Medical Center, Tainan and Taipei Medical University, Taipei, Taiwan 3 Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan article info Article history: Received: Dec 27, 2010 Revised: Apr 7, 2011 Accepted: Jun 1, 2011 Keywords: animal-type melanoma cellular blue nevus epithelioid blue nevus malignant melanoma pigmented epithelioid melanocytoma abstract Pigmented epithelioid melanocytoma (PEM), or animal-type melanoma, is an unusual variant of mela- noma which has been reported to have indolent behavior and a relatively good prognosis. We report a 12-year-old girl with PEM on the third nger web of her right hand. Histopathologically, it was composed of heavily pigmented dermal epithelioid and spindled melanocytic tumor cells. A sentinel lymph node biopsy was negative, and no recurrence was noted 1 yearlater. We reviewed 173 previously published cases of PEM or so-called animal-type melanoma in the literature. Among the 173 cases and our case, extremities were the most common sites of occurrence (52/129, 40.3%), and most of the depth of invasions were Clark level IV and V [76/114 (66.7%) and 33/114 (28.9%), respectively]. Lymph nodes metastasis was noted in 39/89 (43.8%) of the cases being investigated. Only two cases died of the disease with visceral metastasis. Thus, a more advanced level of invasion and the presence of lymph node metastasis did not imply a denitely malignant clinical course, because spreading beyond lymph nodes was rare (5/174, 2.9%). However, long-term follow-up with more cases and further research are needed to fully delineate the true biological nature of this pigmented melanocytic tumor. Copyright Ó 2012, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. Introduction Pigmented epithelioid melanocytoma (PEM) is an unusual variant of malignant melanoma with relatively indolent behavior. It was rst described by Dick, 1 who noted its predilection for gray horses and named it as equine-type melanomain 1832. In 1925, Darier 2 rst described this kind of tumor in humans, and introduced the name melanosarcoma.Since then, a small number of cases have been reported, and the name animal-type melanomahas been used. In 2004, Zembowicz 3 gave it the name pigmented epithe- lioid melanocytomawhich was proposed to include animal-type melanoma and epithelioid blue nevus as malignant and benign ends of this histological spectrum. In 2006, Antony 4 named it as pigment synthesizing melanoma.He thought that the name PEM implied a benign tumor according to the nomenclature system. However, PEM is still the most common name being used in current literature. PEM is extremely rare. It affects all age groups. The prognosis is relatively good compared to conventional malignant melanoma. However, there are only limited cases and results of long-term follow-up, and the role of sentinel lymph node biopsy (SLNB) in the disease is still controversial. We reviewed 173 cases in the literature, regarding the results of gender ratio, location, Clarks level of invasion, and outcomes such as lymph node metastasis, distant metastasis and follow-up. Case presentation A 12-year-old Taiwanese girl (Fitzpatrick type IV) presented with an asymptomatic brown-black nodule, measuring 0.4 0.4 cm, on the third nger web of her right hand (Figure 1A). The nodule had been observed for 3e4 years, increasing in size in a few months. There was no past history or family history of malignant melanoma, blue nevus or Carney complex. No palpable lymph node or history of frequent sun exposure was noted. The lesion was excised and histology showed predominant formation of heavily pigmented epithelioid cells (Figures 1B and 1C) and some spindled cells with large nuclei, prominent nucleoli (Figure 1D) and rare mitosis (Figure 1D, arrow). Ulceration was also noted (Figure 1B). Neither * Corresponding author. Feng-Jie Lai, Department of Dermatology, Chi Mei Medical Center, No. 901, Zhonghua Road, Yongkang District, Tainan City 710, Taiwan. Tel.: þ886 6 281 2811x57109; fax: þ886 6 220 3706. E-mail address: [email protected] (F.-J. Lai). Contents lists available at SciVerse ScienceDirect Dermatologica Sinica journal homepage: http://www.derm-sinica.com 1027-8117/$ e see front matter Copyright Ó 2012, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. All rights reserved. doi:10.1016/j.dsi.2011.09.011 DERMATOLOGICA SINICA 30 (2012) 57e61

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DERMATOLOGICA SINICA 30 (2012) 57e61

Contents lists available

Dermatologica Sinica

journal homepage: http: / /www.derm-sinica.com

CASE REPORT

Pigmented epithelioid melanocytoma: Report of a case and review of 173 cases inthe literature

Pai-Shan Cheng 1, Shih-Sung Chuang 2, Tseng-Tong Kuo 3, Feng-Jie Lai 1,*1Department of Dermatology, Chi Mei Medical Center, Tainan, Taiwan2Department of Pathology, Chi Mei Medical Center, Tainan and Taipei Medical University, Taipei, Taiwan3Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan

a r t i c l e i n f o

Article history:Received: Dec 27, 2010Revised: Apr 7, 2011Accepted: Jun 1, 2011

Keywords:animal-type melanomacellular blue nevusepithelioid blue nevusmalignant melanomapigmented epithelioid melanocytoma

* Corresponding author. Feng-Jie Lai, DepartmenMedical Center, No. 901, Zhonghua Road, YongkanTaiwan. Tel.: þ886 6 281 2811x57109; fax: þ886 6 22

E-mail address: [email protected] (F.-J. Lai).

1027-8117/$ e see front matter Copyright � 2012, Tadoi:10.1016/j.dsi.2011.09.011

a b s t r a c t

Pigmented epithelioid melanocytoma (PEM), or animal-type melanoma, is an unusual variant of mela-noma which has been reported to have indolent behavior and a relatively good prognosis. We reporta 12-year-old girl with PEM on the third finger web of her right hand. Histopathologically, it wascomposed of heavily pigmented dermal epithelioid and spindled melanocytic tumor cells. A sentinellymph node biopsy was negative, and no recurrence was noted 1 year later. We reviewed 173 previouslypublished cases of PEM or so-called animal-type melanoma in the literature. Among the 173 cases andour case, extremities were the most common sites of occurrence (52/129, 40.3%), and most of the depthof invasions were Clark level IV and V [76/114 (66.7%) and 33/114 (28.9%), respectively]. Lymph nodesmetastasis was noted in 39/89 (43.8%) of the cases being investigated. Only two cases died of the diseasewith visceral metastasis. Thus, a more advanced level of invasion and the presence of lymph nodemetastasis did not imply a definitely malignant clinical course, because spreading beyond lymph nodeswas rare (5/174, 2.9%). However, long-term follow-up with more cases and further research are needed tofully delineate the true biological nature of this pigmented melanocytic tumor.

Copyright � 2012, Taiwanese Dermatological Association.Published by Elsevier Taiwan LLC. All rights reserved.

Introduction

Pigmented epithelioid melanocytoma (PEM) is an unusual variantof malignant melanoma with relatively indolent behavior. It wasfirst described by Dick,1 who noted its predilection for gray horsesand named it as “equine-type melanoma” in 1832. In 1925, Darier2

first described this kind of tumor in humans, and introduced thename “melanosarcoma.” Since then, a small number of cases havebeen reported, and the name “animal-type melanoma” has beenused. In 2004, Zembowicz3 gave it the name “pigmented epithe-lioid melanocytoma” which was proposed to include animal-typemelanoma and epithelioid blue nevus as malignant and benignends of this histological spectrum. In 2006, Antony4 named it as“pigment synthesizing melanoma.” He thought that the name PEMimplied a benign tumor according to the nomenclature system.However, PEM is still themost common name being used in currentliterature.

t of Dermatology, Chi Meig District, Tainan City 710,0 3706.

iwanese Dermatological Associatio

PEM is extremely rare. It affects all age groups. The prognosis isrelatively good compared to conventional malignant melanoma.However, there are only limited cases and results of long-termfollow-up, and the role of sentinel lymph node biopsy (SLNB) inthe disease is still controversial. We reviewed 173 cases in theliterature, regarding the results of gender ratio, location, Clark’slevel of invasion, and outcomes such as lymph node metastasis,distant metastasis and follow-up.

Case presentation

A 12-year-old Taiwanese girl (Fitzpatrick type IV) presentedwith anasymptomatic brown-black nodule, measuring 0.4� 0.4 cm, on thethird finger web of her right hand (Figure 1A). The nodule had beenobserved for 3e4 years, increasing in size in a few months. Therewas no past history or family history of malignant melanoma, bluenevus or Carney complex. No palpable lymph node or history offrequent sun exposure was noted. The lesion was excised andhistology showed predominant formation of heavily pigmentedepithelioid cells (Figures 1B and 1C) and some spindled cells withlarge nuclei, prominent nucleoli (Figure 1D) and rare mitosis(Figure 1D, arrow). Ulceration was also noted (Figure 1B). Neither

n. Published by Elsevier Taiwan LLC. All rights reserved.

Figure 1 (A) A 0.4� 0.4 cm, brown-black nodule on the third fingerweb of the right hand; (B) the scanning view of the section showed an ulcerated and heavily pigmented lesion (H&E,40�); (C) the tumor is composed of predominantly heavily pigmented polygonal epithelioid cells with large nuclei and prominent nucleoli. Heavily pigmented spindled cells constitutethe minor component (H&E, 400�); (D) the neoplastic cells demonstrate round nuclei with prominent nucleoli. Mitosis is rare, but could be found in the section (arrow) (melaninbleached H&E, 400�); (E) focal vascular invasion (arrow) at the junction of deep dermis and subcutis (melanin bleached H&E, 200); (F) the tumor cells were positive for S-100 protein(200�); (G) the tumor cells were negative for HMB-45 (200); (H) the tumor cells were negative forMelan-A (200�); (I) immunohistochemical stain of the specimenwith Ki-67 revealedsome positive immunoreactivity of the tumor cells with low proliferative index (6%; 400�); (J) One year after wide excisionwith full-thickness skin graft, therewas no local recurrence.However, due to the disfigured lesion, the patient wore a glove at all times, and a dividing line of fair-colored and tan-colored skin on the forearm was noted (arrow).

P.-S. Cheng et al. / Dermatologica Sinica 30 (2012) 57e6158

P.-S. Cheng et al. / Dermatologica Sinica 30 (2012) 57e61 59

junctional nests nor epidermal spread was found. However, focalvascular invasion was seen at the junction of deep dermis andsubcutis (Figure 1E, arrow). Tumor cells were positive for S-100protein (Figure 1F), but negative for HMB-45 (Figure 1G) andMelan-A (Figure 1H). The Ki-67 proliferative index was low(approximately 6%, Figure 1I). A pigmented epithelioid melanocy-toma, so-called animal-type melanoma, was diagnosed. The depthof invasionwas classified as Clark level V, with maximum thicknessof 3.8 mm. Wide excision with sentinel lymph node sampling andfull-thickness skin graft was performed at another hospital. Noresidual tumor was found, and the sentinel lymph node wasnegative. Positron emission tomography scan was also negative.The patient was followed for 1 year, and neither local recurrencenor metastasis occurred (Figure 1J).

Discussion

PEM is a distinctive clinicopathological variant of melanocytictumor of unknown malignant potential. It is characterized by itsunique feature of indolent behavior compared to conventionalmelanoma. While Zembowicz et al.3 reported an equal sex predi-lection for younger individuals without ethnic predominance,Antony et al.4 reported that middle-aged to old adults havea predilection for the disease. Extremities were the most commonsites of occurrence, followed by trunk and head and neck.3,4

Histopathologically, these lesions often have a wedge-shapedconfiguration and are composed of heavily pigmented dermalmelanocytic tumor cells with a mixture of epithelioid and spindledcells. Mitosis can be seen, but is rare. The histopathological differ-ential diagnoses of PEM include cellular blue nevus, malignant bluenevus, Spitz nevus, deep penetrating nevus and epithelioid bluenevus. The most specific differentiating feature between PEM andcellular blue nevus is the presence of abundant epithelioid cells inPEM.5 However, it is challenging to differentiate between PEM andmalignant blue nevus, since they both have atypical epithelioidcells. The presence of significant nuclear pleomorphism, hyper-chromatism, prominent eosinophilic nucleoli, brisk mitotic activity,atypical mitoses and tumor necrosis are important features formalignant blue nevus. Besides, malignant blue nevus is classicallydefined as malignant melanoma arising within a preexisting bluenevus or the site of prior biopsy/excision of a blue nevus.5 Spitznevus usually presents with maturation and prominent demarca-tion, which can be distinguished from PEM.4 Deep penetratingnevus is a distinctive deeply pigmented lesion showing overlappingfeatures with blue nevus and Spitz nevus.6 Histologically, deeppenetrating nevus shows a typical wedge-shaped, deeply pig-mented dermal tumor with a junctional component. Tumor cellsare arranged in nests or bundles and have a short spindle-shapedor, less commonly, round morphology. In addition, a thin rim ofsustentacular cells is present around the edges of many nests. Onecharacteristic feature of deep penetrating nevus, is the extension ofmelanocytes along the path of adnexal structures, blood vesselsand nerve bundles, which is absent in PEM.

Epithelioid blue nevus, which is associated with the Carneycomplex, but also occurs sporadically, is described as beinghistopathologically indistinguishable from PEM, and the relation-ship between these two entities is still not entirely clear.3e5

Zembowicz et al.3 proposed the term PEM to compose of animal-type melanoma and epithelioid blue nevus as malignant andbenign ends of this histological spectrum. Murali et al.7 suggestedthat a subset of epithelioid blue nevus remained and was distinctfrom PEM by less cell density and less pigmentation. Zembowiczet al.3 mentioned that ulceration was the only feature morecommon in PEM than epithelioid blue nevus of Carney complex. Inour case, the tumor was highly cellular, heavily pigmented and

showed cellular atypia with ulceration, and, in our opinion, wasbest considered as PEM.

Molecular testing of histologically ambiguous primary cuta-neous melanocytic tumors by using techniques such as compara-tive genomic hybridization or fluorescence in situ hybridization(FISH) may assist in establishing a diagnosis of melanoma ifmultiple chromosomal aberrations are identified.8 However, therewas still a limited result in the field of PEM. There were only twocases of congenital PEM being studied by FISH, and one had focalFISH positivity.9 This study revealed that there were still limits inthe technique for the diagnosis of PEM. Thus, a definitive diagnosisfor puzzling lesions such as PEM should not rely on molecular dataalone. The associations between clinical, histopathological and FISHdata are needed.9 Another molecular study showed a loss ofexpression of protein kinase A regulatory subunit 1a (R1a coded bythe PRKAR1A gene) in 28 of 34 (82%) PEMs and in 8 of 8 Carneycomplex-associated epithelioid blue nevus, whereas R1a wasexpressed in 291 of 292 various benign and malignant non-PEMmelanocytic lesions and in 5 of 5 equine melanomas.10 Theresults of these studies suggested that PEM and Carney complex-associated epithelioid blue nevus are closely related neoplasms.Although being a morphological mimic, PEM is a distinct melano-cytic tumor that differs in molecular pathogenesis from cellularblue nevi, malignant blue nevi, Spitz nevi, deep penetrating nevi,melanomas of various types and equine melanomas.

We reviewed 17 literature publications from the year 1999 to2010 and identified 173 previously reported cases (Table 1). Amongthe cases with detailed data (including our case), there were 72(55.8%) females and 57 (44.2%) males. There were four cases ofcongenital PEM. The most common sites of occurrence were theextremities (52/129; 40.3%), followed by trunk (36/129; 27.9%) andhead and neck (36/129; 27.9%). Most of the tumors at the time ofdiagnosis involved Clark’s level IV (76/114; 66.7%) or Clark’s level V(33/114; 28.9%). Among the 174 cases, 39 cases (22.4%) had lymphnode metastasis; only 5 cases (2.9%) had spread beyond lymphnodes. The most common site of visceral metastasis was liver (4/5,80%). Two cases (1.1%) died of the disease due to visceral metastasisduring follow-up (Table 2). Although presenting at deep levels, PEMhas better prognosis than conventional melanoma.

The true nature of PEM remains controversial. Many consider ita tumor of uncertain malignant potential or possibly a low-grademalignancy. Although it has a tendency to have deep invasionand even spread to regional lymph nodes, distant metastasis is rare.Even if it recurs or metastasizes, it has a better prognosis comparedto conventional malignant melanoma with the same depth ofinvasion.3e5,11 Ludgate et al.11 and Zembowicz et al.3 reported SLNBpositive rates of 47% (8/17) and 46% (11/24), respectively. However,in contrast to these two studies, Orlandi et al.12 and Scolyer et al.13

reported no positive SLNB in 7 and 5 patients with PEM, respec-tively (Table 1). Among the 174 cases we reviewed, the positive rateof lymph node metastasis either confirmed by SLNB or completelymph node dissection (CLND) was 43.8% (39/89), accounting for22.4% of the total number of cases. The variation in these resultsmay be due to the small number of cases in previous reports, andthe criteria for performing a SLNB/CLND not being clearly estab-lished. Thus, the positive rate in cases undergoing lymph nodessampling or dissection may be over-estimated due to deeper tumorcell invasion or a more progressive clinical course. Regardless of therelatively high rate of lymph node metastasis, there were only 2.9%of cases whose disease had spread beyond the lymph nodes(Table 2). Thus, whether SLNB or CLND is needed for all cases ofPEM is still controversial. Some suggested that PEM may not needto bemanaged as aggressively as conventional melanoma, due to itsindolent behavior,14 while others recommended SLNB as a “diag-nostic procedure” in an attempt to better recognize the biological

Table 1 Previous reports of pigmented epithelioid melanocytoma/animal-type melanoma and our patient.

Author Year No. ofcases

Sex Location Clark level Outcome Follow-up

Ludgate et al.11 2010 22 M: 13F: 9

Head & neck: 6Trunk: 6Extremities: 9Genitalia: 1

I: 1III: 2IV: 16V: 3

SLNB (þ): 8/17 (47%)Recurrence: 4 cases (18%)(2 had distant metastasis, and 1 died ofvisceral metastasis 2.5 y following theinitial diagnosis.)

Median: 17.6 mo

Battistella et al.9 2010 2 M: 1F: 1

Head & neck: 1Extremity: 1

V: 1Another N/A

Congenital PEM.SLNB was not performed in both cases.

26 mo and 15 mo, respectively

Yun et al.16 2010 1 F:1 Head & neck: 1 V: 1 Congenital PEM. Excision was performed at5 mo, regional LN metastasis at 7 mo andthe patient underwent 8 courses ofCVD-BIO biochemotherapy*. Tumor-freefor 20 mo after excision.

20 mo

Orlandi et al.12 2009 7 M: 2F: 5

Head & neck: 3Trunk: 3Extremity: 1

IV: 1V: 6

SLNB (þ): 0/7 (0%)One died of cardiac failure without evidenceof disease progression.

3 y (4/7) and 5 y (3/7)

Mandal et al.15 2009 26 M: 9F: 17

Head & neck: 7Trunk: 6Extremities: 12Genitalia: 1

III: 2IV: 19V: 5

SLNB or CLND (þ): 8/20 (40%)Alive and free of disease during follow-up.

Median : 67 mo (range 39e216 mo)

Ito et al.17 2009 2 F: 2 Trunk: 2 V: 2 SLNB was not performed in both cases.Alive and free of disease during follow-up.

22 mo and 16 mo, respectively

Vezzoni et al.18 2008 1 F: 1 Trunk :1 IV: 1 SLNB (-).Alive and free of disease during follow-up

N/A

Sabah et al.19 2007 1 F: 1 Extremity: 1 V: 1 Local recurrence occurred 9 y after previoussimple excision. SLNB(-)Alive and free of disease during follow-up.

46 mo

Antony et al.4 2006 14 M: 8F: 6

Head & neck: 5Trunk: 3Extremities: 6

Only mentionedthe Breslow level,no Clark level.

SLNB or CLND(þ): 5Distant metastasis (liver): 1Local recurrence: 3No recurrence or metastasis: 6No death during follow-up.

Median 5 y (range 1e17 y)

Lu et al.20 2006 1 F: 1 Head & neck: 1 IV: 1 SLNB was not performed.Alive and free of disease during follow-up.

3 mo

Ward et al.21 2006 1 M: 1 Trunk: 1 IV: 1 SLNB (-).Alive and free of disease during follow-up.

6 mo

Howard et al.22 2005 2 M: 1F: 1

Head & neck: 1Extremity: 1

IV: 2 CLND (þ): 1, with radiation therapy andintravenous alfa-interferon therapy.Alive and free of disease during follow-up.

The first case was followed upfor 60 mo; another one was N/A.

Zemboxicz et al.3 2004 40(with 41lesions)

M: 18F: 22

Head & neck: 10Trunk: 11Extremities: 18Genitalia: 2

IV: 30V: 11

SLNB (þ): 11/24 (46%)1 case: congenital PEM with hepaticmetastasis discovered at presentation, iswell during the 3-y follow-up.No death during follow-up.

Mean: 32 mo

Scolyer et al.13 2004 45 N/A N/A N/A SLNB (þ): 0/5 (0%).CLND (þ): 1/1

N/A

Kazakov et al.23 2004 1 F: 1 Extremity: 1 IV: 1 SLNB (þ).Alive and free of disease during follow-up.

24 mo

Requena et al.24 2001 1 M: 1 Trunk: 1 V: 1 SLNB (þ), had chemotherapy withinterferon alfa-2b.Alive and free of disease during follow-up.

4 mo

Crowson et al.25 1999 6 M: 3F: 3

Head & neck: 1Trunk: 2Extremity: 1Genitalia: 1(another one:unknown)

IV: 4V: 1(another one:unknown)

1 died of the disease with metastases toregional LN, liver, and lungs.1 had regional LN metastasis, but was aliveand well.1 had local recurrence, but was lost tofollow-up.1 had chest mass, but had not yet beeninvestigated.2 were alive and free of disease duringfollow-up.

From 5 mo to 4 y

Our patient 2011 1 F:1 Extremity: 1 V: 1 Alive and free of disease during follow-up. 12 mo

CLND ¼ complete lymph node dissection; F ¼ female; LN ¼ lymph nodes; M ¼ male; N/A ¼ not available; SLNB ¼ sentinel lymph node biopsy.* Two 21-day cycles of chemotherapy and three cycles of interleukin-2 and interferon-alfa every 6 weeks.

P.-S. Cheng et al. / Dermatologica Sinica 30 (2012) 57e6160

potential of these tumors.3 Ludgate et al.11 recommended per-forming a SLNB in PEM that is greater than 1 mm in Breslow depthor 0.75e1 mm with other adverse features such as ulceration ora highmitotic rate. The longest follow-up to datewas reported aftera median follow-up period of 67 months (range from 39 to 216months) of 26 patients by Mandal et al.15 All of these patients werealive and free of disease during the follow-up period. These results

showed that the presence of lymph node metastases in PEM doesnot necessarily imply a malignant clinical course. Examination ofsentinel lymph nodes by lymphoscintigraphy and assessment byclinical examination and ultrasound for early detection of metas-tasis, rather than invasive SLNB, have been suggested.15

In summary, although there are still debates on the nomencla-ture of this entity, PEM is the most common name used currently; it

Table 2 Summary of 173 previously reported cases of pigmented epithelioidmelanocytoma/animal-type melanoma and our case.

Number of cases 174Gender Male: 57/129 (44.2%)

Female: 72/129 (55.8%)Locations of the lesions Extremities: 52/129 (40.3%)

Head & neck: 36/129 (27.9%)Trunk: 36/129 (27.9%)Genitalia: 5/129 (3.9%)

Clark level of the lesions at diagnosis I: 1 /114 (0.9%)II: 0 /114 (0%)III: 4 /114 (3.5%)IV: 76 /114 (66.7%)V: 33 /114 (28.9%)

Underwent sentinel lymph node biopsyor complete lymph node dissection

89

ePositive result 39 (43.8% of investigated cases);(22.4% of the total cases)

Spread beyond lymph nodes 5 (2.9%)*Follow-up (range 0 to 216 mo)eAlive and well 171 (98.3%)eDeath 3 (1.7%)

/Only 2 cases (1.1%) died ofthe diseasey

ATM ¼ animal-type melanoma; PEM ¼ pigmented epithelioid melanocytoma.* Three cases with hepatic metastasis, and one case with both hepatic and lung

metastasis. Another patient’s site of metastasis was not available.y One died of visceral metastasis (specific site was not available) 2.5 years after

the initial diagnosis, another died of hepatic and lung metastasis (the survival timewas not available).

P.-S. Cheng et al. / Dermatologica Sinica 30 (2012) 57e61 61

can be categorized as a borderline melanocytic tumor or a low-grade melanoma. PEM is characterized by a predominantly intra-dermal deeply pigmented melanocytic lesion, composed mainly ofepithelioid and spindled cells. Some cytologic atypia can be found,but mitotic activity is low. PEM may involve regional lymph nodes,but it has a limited ability to spread beyond the lymph nodes.Although it showed indolent behavior and an overall good prog-nosis in the 174 cases we reviewed, its potential for aggressivebehavior should not be ignored. Whether SLNB should be per-formed as a staging procedure in every case of PEM remainscontroversial. Longer periods of follow-up with more cases andfurther molecular studies for PEM are needed to fully delineate thetrue biological nature of this disease.

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