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intestinal perineurioma

Defintion: subtype of benign peripheral nerve sheath tumor.

Synopsis

- Age: median age, 51 years; range, 35-72 years
- intramucosal perineuriomas (small sessile polyps)
- submucosal masses
- uniform bland spindle cells having ovoid to elongated nuclei and pale indistinct cytoplasm
- no cytologic atypia, pleomorphism, or mitotic activity
- fine collagenous stroma
- irregular borders with the adjacent lamina propria and entrapped colonic crypts
- +/- hyperplastic changes in the adjacent or entrapped epithelium.
- collagenous stroma or myxoid stroma
- spindle cells with tapered nuclei and elongated bipolar cytoplasmic processes

Immunochemistry

- positive for epithelial membrane antigen (EMA+)
- CD34+
- +/- claudin-1 and 2 of 10 expressed
- negative for

  • S-100 protein
  • glial fibrillary acidic protein
  • neurofilament protein
  • smooth muscle actin
  • desmin
  • caldesmon
  • KIT
  • pan-keratin

Ultrastructure

- spindle cells with long bipolar cytoplasmic processes
- prominent pinocytotic vesicles
- cells surrounded by discontinuous basal lamina

See also

- benign peripheral nerve sheath tumors
- digestive fusiform cell tumors (spindle cell tumors)

References

- Hornick JL, Fletcher CD. Intestinal perineuriomas: clinicopathologic definition of a new anatomic subset in a series of 10 cases. Am J Surg Pathol. 2005 Jul;29(7):859-65. PMID: #15958849#