CMT/DSS: Intraneural perineurioma: a systematic review with illustrative cases

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Arch Pathol Lab Med. 2007 Sep;131(9):1382-92.

Intraneural perineurioma: a systematic review with illustrative

cases.

Boyanton BL Jr, JK, Shenaq SM, Hicks MJ, Bhattacharjee MB.

Department of Clinical Pathology, Beaumont Hospital, 3601 W

Thirteen Mile Rd, Royal Oak, MI 48073, USA.

CONTEXT: Intraneural perineurioma may be confused with other " onion

bulb " Schwann cell entities (localized hypertrophic neuropathy,

reactive/demyelinating processes, or inherited polyneuropathies of

Charcot-Marie-Tooth/Dejerine Sottas) due to similar clinical,

radiologic, and histologic features. Perineurial and Schwann cells

can only be differentiated by ultrastructure and

immunohistochemsitry.

OBJECTIVE: To identify and summarize the clinicopathologic features

of true cases of intraneural perineurioma from the English language

literature.

DATA SOURCES: A systematic review was performed on definitive

intraneural perineuriomas identified through Medline. Baylor College

of Medicine-affiliated hospitals' anatomic pathology databases

yielded 2 illustrative intraneural perineurioma cases. STUDY

SELECTION: Intraneural perineurioma inclusion criteria consisted of

characteristic histology and confirmation of perineurial cell

lineage by either immunohistochemistry (epithelial membrane antigen

positive, S100 protein negative) and/or ultrastructural analysis

(thin cytoplasmic processes with an incomplete basal lamina, poorly

formed tight junctions, and pinocytotic vesicles).

DATA EXTRACTION: Clinicopathologic data were extracted from all

identified articles, with subsequent statistical analysis of the

following parameters: age, sex, race, tumor location, tumor size,

duration of symptoms prior to diagnosis, treatment modalities and

outcomes measures, follow-up assessment for tumor recurrence and

metastasis, clinical features (history of trauma, motor/sensory

abnormalities, clinical/family history), and diagnostic workup

(routine histology, immunohistochemistry, ultrastructural analysis,

and molecular/cytogenetic characteristics).

CONCLUSIONS: Intraneural perineurioma is a neoplastic proliferation

of perineurial cells with unique immunohistochemistry and

ultrastructural features, and it is distinct from other onion bulb

Schwann cell-derived entities. Despite harboring molecular

abnormalities of the long arm of chromosome 22, intraneural

perineurioma has not been associated with neurofibromatosis.

Intraneural perineurioma is a benign peripheral nerve sheath tumor

that does not recur or metastasize.