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Skin-Melanocytic tumors
Paraganglioma-like dermal melanocytic tumor
Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Revised: 6 July 2009, last major update November 2008
Copyright: (c) 2005-2009, PathologyOutlines.com, Inc.
Definition
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● First described in 2004 (AJSP 2004;28:1579)
Epidemiology
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● Very rare (8 of 30,000 consultation cases in one series)
● 75% females, mean age 35 years (range 18-53 years)
Case reports
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● 60 year old man (Cases J 2008;1:48)
Clinical description
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● Dermal nodule, mean 1 cm
Micro description
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● Nested growth pattern similar to paraganglioma, composed of clear to amphophilic oval cells separated by delicate fibrous strands
● No/mild nuclear atypia
● 1-4 MF/10 HPF
● No melanin
● Normal epidermis, no junctional activity
Micro images
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Well demarcated cellular nodule Neoplasm composed of organoid and nested groups
in dermis with normal epidermis of large epithelioid cells separated by delicate fibrous
strands and prominent blood vessels, epithelioid cells
have somewhat clear cytoplasm, large nuclei with
prominent red nucleoli
S100+
Positive stains
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● S100 (100%), HMB45 (100%), Microphthalmia transcription factor (100%), MelanA (50%)
Negative stains
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● Keratin, smooth muscle actin
Molecular / cytogenetics
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● No t(12;22) of clear cell sarcoma
Differential Diagnoses
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● Clear cell sarcoma - centered in fascia and tendons, rare in dermis, high grade with prominent nucleoli and scattered tumor giant cells, t(12;22)+
● Melanoma - high grade, no nesting pattern
End of Skin-Melanocytic Tumors > Paraganglioma-like dermal melanocytic tumor
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