PMID- 7785653
OWN - NLM
STAT- MEDLINE
DCOM- 19950714
LR  - 20220331
IS  - 0002-9173 (Print)
IS  - 0002-9173 (Linking)
VI  - 103
IP  - 6
DP  - 1995 Jun
TI  - Intraneural perineurioma. A clonal neoplasm associated with abnormalities of 
      chromosome 22.
PG  - 696-704
AB  - The nature of perineurioma, variably termed "localized hypertrophic neuropathy," 
      "intraneural neurofibroma," and "hypertrophic interstitial neuritis" has long 
      been an issue of contention. Most authors consider it a neoplasm, but some a 
      reactive process. Eight clinically and morphologically typical perineuriomas were 
      studied by histologic, immunohistochemical and ultrastructural methods. One 
      perineurioma was subject to tissue culture and cytogenetic study and another to 
      fluorescence in situ hybridization (FISH) analysis. The patients, 3 males and 5 
      females, ranged in age from 11 to 38 years. All tumors were intraneural, and 
      involved extremities (2 sciatic, 1 median, 1 femoral, 1 peroneal, 1 brachial 
      plexus, 1 ulnar, and 1 radial). Neurologic symptoms, motor in all cases and 
      sensory in 4, were present from 1 month to 7 years (mean 1.2 years). Fusiform, 
      segmental nerve enlargement was clinically apparent in only two patients, but was 
      evident on MRI in five of eight patients. Lesion length ranged from 3.5 to 30 cm, 
      the largest involving the sciatic nerve from the obturator foramen to the knee. 
      One lesion involved two nerve roots, but no association with a phakomatosis was 
      noted. Treatment consisted of biopsy in six cases and resection in two cases. 
      Histologically, pseudo-onion bulbs composed of epithelial membrane 
      antigen-reactive, S-100 protein-negative perineurial cells surrounded myelinated 
      or nonmyelinated nerve fibers. Many were accompanied by their S-100 
      protein-positive Schwann sheaths. Some whorls lacked a central axon. A single 
      mitosis was noted in one case. The MIB-1 antigen labelling index ranged from 4% 
      to 17%. Staining for p53 antigen in six cases showed no (2 of 6), rare (2 of 6), 
      or scattered (2 of 6) immunoreactive nuclei. Cytogenetic analysis in one case 
      demonstrated a chromosomally abnormal clone. Each of 16 metaphases was abnormal; 
      the tumor cells appeared to be homozygously deficient for the region 22q11.2qter. 
      In another case, 53% of interphase nuclei showed three FISH signals with a 
      chromosome 14/22 probe, thus suggesting either monosomy for the centromere of 
      chromosome 14 or that of chromosome 22.(ABSTRACT TRUNCATED AT 400 WORDS)
FAU - Emory, T S
AU  - Emory TS
AD  - Department of Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.
FAU - Scheithauer, B W
AU  - Scheithauer BW
FAU - Hirose, T
AU  - Hirose T
FAU - Wood, M
AU  - Wood M
FAU - Onofrio, B M
AU  - Onofrio BM
FAU - Jenkins, R B
AU  - Jenkins RB
LA  - eng
PT  - Journal Article
PL  - England
TA  - Am J Clin Pathol
JT  - American journal of clinical pathology
JID - 0370470
RN  - 0 (Molecular Probes)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Base Sequence
MH  - Cell Cycle
MH  - Child
MH  - *Chromosome Aberrations
MH  - *Chromosome Disorders
MH  - *Chromosomes, Human, Pair 22
MH  - Female
MH  - Histocytochemistry
MH  - Humans
MH  - Immunohistochemistry
MH  - In Situ Hybridization, Fluorescence
MH  - Male
MH  - Microscopy, Electron
MH  - Molecular Probes/genetics
MH  - Molecular Sequence Data
MH  - Nervous System Neoplasms/*genetics/metabolism/pathology
MH  - Neuroma/*genetics/metabolism/pathology
EDAT- 1995/06/01 00:00
MHDA- 1995/06/01 00:01
CRDT- 1995/06/01 00:00
PHST- 1995/06/01 00:00 [pubmed]
PHST- 1995/06/01 00:01 [medline]
PHST- 1995/06/01 00:00 [entrez]
AID - 10.1093/ajcp/103.6.696 [doi]
PST - ppublish
SO  - Am J Clin Pathol. 1995 Jun;103(6):696-704. doi: 10.1093/ajcp/103.6.696.