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Amelanotic primary dermal melanoma with V600E BRAF mutation lable at ScienceDirect DERMATOLOGICA SINICA xxx (2016) 1e2 Contents lists avai Dermatologica Sinica journal homepage http / /www derm sin ic[.]

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Amelanotic primary dermal melanoma with V600E BRAF mutation

Dear Editor,

We herein report the case of a Japanese patient who presented

with amelanotic primary dermal melanoma (PDM) with V600E

BRAF mutation The lesion developed at the site of previous laser

treatment

A 52-year-old Japanese female noticed a red papule on her right

cheek sometime in 1997 The lesion was allegedly treated with laser

ablation by a local doctor without histological diagnosis Sixteen

years later, she visited us with complaints of a tumor, which

appeared at the same region about half a year ago Physical

examina-tion revealed an erythematous induraexamina-tion (Figure 1A) Laboratory

in-vestigations were all within normal limits Histopathologically, the

cutaneous biopsy specimen revealed aggregates of nests composed

of round to ovoid cells with swollen nucleus, prominent nucleolus,

and rather abundant eosinophilic cytoplasm Mitoticfigures were

sparse, but an atypical one was recognized No melanization was

seen within the tumor cells (Figure 1B) Atypical melanocytes were

not found in the epidermis with multiple tissue sections

Immuno-histochemical analysis were positive for tyrosinase (Figure 1C) and

S-100 protein but negative for Melan-A, HMB-45, MITF, AE1/AE3,

CK20, and CD68 BRAF p.V600E mutation was detected by

immuno-histochemical analysis in dermal tumor cells (Figure 1D) The Ki-67

index was 18.8% The EWSR1eATF1 fusion was undetected in blood

plasma and tumor usingfluorescence in situ hybridization method

Imaging examinations, including computed tomography, magnetic

resonance imaging, and fluorine-18-fluorodeoxyglucose positron

emission tomography/computed tomography scan, revealed no

pri-mary or metastatic lesion We performed wide excision with a

margin of 1 cm from the induration and sentinel lymph node biopsy

on the right neck Histopathologically, variously sized nests were

scattered from the superficial dermis down to the subcutaneous

tis-sue (Figure 1E) No component of benign intradermal nevus was

detected in any of the sections Metastasis was not detected in the

four lymph nodes excised The patient has been followed up for 30

months without recurrence or metastasis

Differential diagnoses included epithelial tumors, perivascular

epithelioid cell tumor, nodular melanoma, atypical Spitz nevus,

cutaneous clear cell sarcoma, and metastatic carcinomas

Histo-pathologically, tumor nests composed of round to ovoid cells

were localized from the dermis to subcutaneous tissue and no atyp-ical cell was found in the epidermis Immunohistochematyp-ical analysis was positive for tyrosinase and S-100 protein but repeatedly nega-tive for epithelial markers Nodular melanoma usually grows quite quickly and has poor prognosis The Ki-67 index was reported to be 7.02e12.98% in atypical Spitz nevus and 23.99e49.67% in malig-nant melanoma.1In our case, 18.8% index is higher than atypical Spitz nevus but lower than conventional malignant melanoma Cutaneous clear cell sarcoma was excluded based on the histologi-cal features and the absence of the EWSR1eATF1 fusion gene.2No primary melanoma has been found at any other sites with exten-sive investigations Therefore, we are able to diagnose our case as amelanotic PDM

PDM is considered a rare subtype of solitary melanoma confined

to the dermis and/or subcutaneous tissue with no known separate primary melanoma and negative metastaticfindings on work-up.3

PDM shows a much better prognosis compared with similarly staged cutaneous melanoma, and 5-year survival rate is as high

as 80e100% An associated benign intradermal nevus is histopath-ologically detected in 29% (14/49) of PDM cases,3which was not found in our case

Our case is probably thefirst amelanotic type of PDM reported

in English literature No melanization was found with negative immunohistochemical results for Melan-A, HMB-45, and MITF; however, additional staining for tyrosinase eventually led to the diagnosis of melanoma While tyrosinase antibody is rarely used

in Japan, it is used in the form of a pan-melanoma antibody cocktail including Melan-A and HMB-45.4

In our case, the tumor was not likely to be a recurrence of malig-nant melanoma, because the laser ablation was done 16 years ago The possibility of melanoma development subsequent to laser treatment of histologically diagnosed benign lesions was reported.5

It was also reported that sublethal laser damage could increase p16 expression, and BRAF mutation is detected in most benign melano-cytic nevi as well, not just in melanoma.6,7The association of the incidence of BRAF V600E mutation and clinical subtypes was not statistically significant, which showed that BRAF mutation was detected in 37.7% of superficial spreading melanoma, 30.2% of nodular melanoma, 17.0% of lentigo maligna, and 15.1% of acral or mucosal melanomas.8Although histological examination was not done before the laser treatment and we could not find dermal component of benign nevus in the resected tumor, laser treatment

in our case might still possibly induce malignant transformation of dermal nevus cells harboring BRAF mutation

Conflicts of interest: The authors declare that they have no financial or

non-financial conflicts of interest related to the subject matter or materials discussed

in this article.

Contents lists available atScienceDirect Dermatologica Sinica

j o u r n a l h o m e p a g e : h t t p : / / w w w d e r m - s i n i c a c o m

DERMATOLOGICA SINICA xxx (2016) 1e2

http://dx.doi.org/10.1016/j.dsi.2016.10.001

1027-8117/Copyright © 2016, Taiwanese Dermatological Association Published by Elsevier Taiwan LLC This is an open access article under the CC BY-NC-ND license ( http:// creativecommons.org/licenses/by-nc-nd/4.0/ ).

Please cite this article in press as: Kabuto M, et al Amelanotic primary dermal melanoma with V600E BRAF mutation, Dermatologica Sinica (2016), http://dx.doi.org/10.1016/j.dsi.2016.10.001

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The authors thank Dr Masanobu Kumakiri for his help with

histo-pathological diagnosis and Ms Yuko Tsukamoto for her help with

immunohistochemical analysis

Miho Kabuto, Noriki Fujimoto*, Kazuya Teramura,

Takeshi Nakanishi

Department of Dermatology, Shiga University of Medical Science,

Setatsukinowa-cho, Otsu-shi, Shiga, Japan

Taku Fujimura

Department of Dermatology, Tohoku University Graduate School of Medicine,

Seiryo-machi 1-1, Aoba-ku, Sendai, Miyagi, Japan

Toshiaki Manabe

Shiga Medical Center Research for Adults, Research Institute,

Moriyama 5-4-30, Moriyama, Shiga, Japan

Toshihiro Tanaka

Department of Dermatology, Shiga University of Medical Science,

Setatsukinowa-cho, Otsu-shi, Shiga, Japan

* Corresponding author Department of Dermatology, Shiga University of

Medical Science, Setatsukinowa-cho, Otsu-shi, Shiga 520-2192, Japan.

E-mail address: noriki@belle.shiga-med.ac.jp (N Fujimoto).

References

1 Kapur P, Selim MA, Roy LC, et al Spitz nevi and atypical Spitz nevi/tumors: a his-tologic and immunohistochemical analysis Mod Pathol 2005;18:197e204

2 Yang L, Chen Y, Cui T, et al Identification of biomarkers to distinguish clear cell sarcoma from malignant melanoma Hum Pathol 2012;43:1463e70

3 Sidiropoulos M, Obregon R, Cooper C, et al Primary dermal melanoma: a unique subtype of melanoma to be distinguished from cutaneous metastatic melanoma:

a clinical, histologic, and gene expression-profiling study J Am Acad Dermatol 2014;71:1083e92

4 Orchard G Evaluation of melanocytic neoplasms: application of a pan-melanoma antibody cocktail Br J Biomed Sci 2002;59:196e202

5 Zipser MC, Mangana J, Oberholzer PA, et al Melanoma after laser therapy of pig-mented lesionsdcircumstances and outcome Eur J Dermatol 2010;20:334e8

6 Chan HH, Xiang L, Leung JC, et al In vitro study examining the effect of sub-lethal

QS 755 nm lasers on the expression of p16INK4a on melanoma cell lines Lasers Surg Med 2003;32:88e93

7 Kumar R, Angelini S, Snellman E, et al BRAF mutations are common somatic events in melanocytic nevi J Invest Dermatol 2004;122:342e8

8 Inumaru JS, Gordo KI, Fraga Junior AC, et al Analysis of the BRAF V600E muta-tion in primary cutaneous melanoma Genet Mol Res 2014;13:2840e8

Received: May 20, 2016 Revised: Sep 19, 2016 Accepted: Oct 19, 2016

Figure 1 (A) Initial clinical presentation An induration, which measures about 20 mm in diameter with a red nodule about 5 mm at the center, was observed on the patient’s right cheek (B) Histopathological findings in a cutaneous biopsy specimen from indurated lesion revealed nests of round to ovoid cells with swollen nucleus, eosinophilic cytoplasm, and

no melanization in the dermis [hematoxylin and eosin (HE); original magnification, 200] (C) Immunohistochemical staining for tyrosinase showed nests of round to ovoid cells with strong positive expression in the dermis (original magnification, 200) (D) Immunohistochemical staining for BRAF p.V600E (clone VE1, Spring Bioscience, Pleasanton, CA, USA) showed positive expression of tumor cells in the dermis (original magnification, 200) (E) Histopathological findings of the resected specimen demonstrated large or small nests from the superficial dermis to the subcutaneous layer (HE; original magnification, 1).

Correspondence / Dermatologica Sinica xxx (2016) 1e2 2

Please cite this article in press as: Kabuto M, et al Amelanotic primary dermal melanoma with V600E BRAF mutation, Dermatologica Sinica (2016), http://dx.doi.org/10.1016/j.dsi.2016.10.001

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