Paraganglioma-like Dermal Melanocytic Tumor
David Cassarino, MD, PhD
Cyril Fisher, MD, DSc, FRCPath
Key Facts
Terminology
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Paraganglioma-like dermal melanocytic tumor (PLDMT)
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Rare dermal-based, low-grade melanocytic tumor with histologic similarities to paraganglioma
Microscopic Pathology
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Typically composed of packets of oval to spindle-shaped cells surrounded by thin fibrous septa
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Uniform nuclei, small nucleoli, clear cytoplasm
Ancillary Tests
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Positive for S100; MART-1/Melan-A, and HMB-45 (variable)
Top Differential Diagnoses
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Clear cell/balloon cell melanoma
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Clear cell sarcoma
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Perivascular epithelioid cell tumor (PEComa)
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Primary dermal melanoma
TERMINOLOGY
Abbreviations
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Paraganglioma-like dermal melanocytic tumor (PLDMT)
Definitions
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Dermal-based low-grade melanocytic tumor with histologic similarities to paraganglioma
CLINICAL ISSUES
Epidemiology
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Incidence
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Very rare tumors; only 10 cases reported to date
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Age
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Typically occur in young adults
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Range: 18-53 years
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Mean: 35 years
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Gender
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More common in females
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Site
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Usually occur on extremities
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Mostly lower limb
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Thigh, knee, lower leg
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