Lester D. R. Thompson, MD

An intact surface epithelium is noted overlying a proliferation of spindled cells arranged in a vaguely storiform pattern with focal small whorls. Collections of inflammatory cells are present image.

There is a haphazard spindle cell proliferation showing a syncytial appearance. The cells show slightly spindled nuclei with cytoplasmic processes. The background stroma is slightly loose to myxoid.



  • Soft tissue perineurioma (STP)


  • Storiform perineurial fibroma

  • Perineurial cell tumor


  • Benign peripheral nerve sheath tumor, specifically of perineurial cell derivation that surrounds endoneurial connective tissue space of nerve fibers

    • Tumors are traditionally separated into intraneural, sclerosing, and soft tissue perineurioma

    • Perineurial cells can be seen in other tumors, such as neurofibroma and schwannoma



  • May be related to Schwann cells, fibroblasts, or arachnoid cap cells



  • Incidence

    • Exceedingly rare

    • Represents < 0.5% of peripheral nerve sheath tumors

  • Age

    • Wide age range: 2-85 years

    • Majority: 2nd-5th decades

      • Mean: 45 years

  • Gender

    • Slight female preponderance

      • Female > male (1.1-1.2:1)


  • Superficial subcutaneous soft tissue

    • Most common in soft tissues of lower and upper extremities

    • 2nd most common in trunk

    • Head and neck sites affected in ˜ 15% of all perineuriomas

  • Oral cavity is affected ˜ 4% of the time


  • Most patients present with solitary painless mass

  • May have syndrome/familial association

    • Neurofibromatosis type 2 (NF2)

    • Nevoid basal cell carcinoma (Gorlin) syndrome

    • Interestingly, both have meningioma in common

      • Perineurium may be derived from arachnoid cap cells


  • Surgical approaches

    • Excision is treatment of choice

      • Some advocate for wide excision to prevent recurrence


  • Local recurrence is uncommon (< 5% of cases)

    • May develop late

    • Only seen if originally incompletely excised

  • Metastases are not reported

  • Pleomorphic cells and infiltrative margins do not affect clinical outcome

Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Perineurioma

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