Markers and Immunoprofile of Peripheral Nerve and Nerve Sheath Tumors

, Hans Guski2 and Glen Kristiansen3



(1)
Carl-Thiem-Klinikum, Institut für Pathologie, Cottbus, Germany

(2)
Vivantes Klinikum Neukölln, Institut für Pathologie, Berlin, Germany

(3)
Universität Bonn, UKB, Institut für Pathologie, Bonn, Germany

 




Diagnostic Antibody Panel for Peripheral Nerve and Nerve Sheath Tumors

S100, CD56, PGP 9.5, myelin basic protein, glial fibrillary acidic protein (GFAP), and neurofilaments
























Myelin basic protein

Expression pattern: cytoplasmic

Main diagnostic use

Expression in other tumors

Expression in normal cells

Neurogenic sarcoma, neuroma, neurofibroma, ganglioneuroma

Granular cell tumor

Cells of white matter of central and peripheral nervous systems

Positive control: brain tissue


Diagnostic Approach

Myelin basic protein (MBP) is a major component of the myelin sheath produced by oligodendrocytes and Schwann cells. It is localized in myelin surrounding nerve fibers in both the central and the peripheral nervous systems and takes a part in formation and stabilization of neuronal structures. Antibodies to MBP are used as a marker for neuroma, neurofibroma, and neurogenic sarcoma but are negative in other spindle cell tumors.
























Neurofilaments

Expression pattern: cytoplasmic

Main diagnostic use

Expression in other tumors

Expression in normal cells

Medulloblastoma, retinoblastoma, neuroblastoma, ganglioglioma, paraganglioma, neurofibroma

Merkel cell tumor, pancreatic endocrine neoplasms, carcinoid, small cell carcinoma, parathyroid tumors, pheochromocytoma

Neuronal cells

Positive control: brain tissue


Diagnostic Approach

Neurofilaments are intermediate filament proteins, heteropolymers composed of four subunits (light, medium, high, and internexin or peripherin). They are the main cytoskeletal element in nerve axons and dendrites of both central and peripheral nervous systems providing neuronal structural support and regulate the axon diameter and the transmission of electrical impulses. Neurofilaments are good markers for tumors derived from neurons and ganglion cells and label tumors with neuronal differentiation.


Diagnostic Pitfalls

The expression of the neurofilaments is reported in rare cases of non-neurogenic tumors such as rhabdomyosarcoma and epithelioid sarcoma and rare carcinoma types.

















Protein gene product 9.5 (PGP 9.5)

Expression pattern: cytoplasmic

Main diagnostic use

Expression in other tumors

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Dec 25, 2017 | Posted by in ONCOLOGY | Comments Off on Markers and Immunoprofile of Peripheral Nerve and Nerve Sheath Tumors
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