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Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other
Neurofibroma - Pacinian
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 31 October 2009, last major update - October 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Rare neurofibroma with proliferation of structures resembling Pacinian (Vater-Pacini) corpuscles
● Pacinian corpuscle is also called Vater-Pacini corpuscles (Wikipedia); is a mechanoreceptor in skin responsible for sensitivity to pain and pressure (Wikipedia)
Terminology
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● Initially described as Neuroma of Wagner-Meissner tactile corpuscles (Cancer 1951;4:879)
Epidemiology
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● Very rare
Sites
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● Fingers, hands and feet; also buttocks
● Buttock lesions may be associated with skeletal anomalies
Etiology
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● Some cases may be nerve sheath myxoma / neurothekeoma
Case reports
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● 4 year old child with hand tumor (J Hand Surg Am 1999;24:413)
● 24 year old woman with neurofibromatosis and hypertrophy of Pacinian corpuscles, but no neurofibroma (Am J Dermatopathol 2006;28:202)
● Multiple hairy tumors on buttocks (J Am Acad Dermatol 1988;18:416)
Micro description
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● Round or oval corpuscles with multiple concentric lamellae
● Lack the perfect structure of a Vater-Pacini corpuscle
Micro images
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A: mucinous nature is evident
B: tumor contains nerve fibers and delicate spindle cells focally arranged in parallel configurations similar to Pacinian corpuscles (C)
Source: AFIP Fascicle, 3rd series
Pacinian corpuscles
Differential Diagnosis
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● Pacinian neuroma / pacinioma / hyperplasia of pacinian corpuscles: rare, hamartomatous overgrowth, no neurofibroma component; often pain and tenderness at site; may be associated with trauma
● Nerve sheath myxoma / neurothekeoma: less resemblance to Vater-Pacini corpuscles; stromal mucin is present
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Neurofibroma > Pacinian
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