Schwannoma
Schwannoma
Updated: 08/07/2020
© Jun Wang, MD, PhD
General features
- Benign nerve sheath tumor arising from differentiated Schwann cells
- Most commonly 20 - 50 years old
- Majority are sporadic, may be associated with neurofibromatosis II (NF2) or Carney complex
Clinical presentations
- Mass with abnormal sensation
- Commonly in limbs, especially upper limbs, head and neck
- Deep tumor commonly in posterior mediastinum or retroperitoneum
Pathogenesis
- Abnormal activation of PI3K, RAS and RAC due to merlin inactivation
Genetic abnormality
- Loss of function mutation of merlin at 22q12
Morphological features
- Encapsulated biphasic nerve sheath tumor
- Cellular component(Antoni A) that palisades (Verocay bodies), and myxoid component (Antoni B)
Marker
- Positive for S100
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