Claudins
The claudins are a family of over
twenty proteins which are components of the tight junction (Latin claudere,
to close). They are found with other components including occludin and
junctional adhesion molecule, and interact with scaffold proteins, ZO-1,
ZO-2 and ZO-3. The claudins constitutes a variable component, with specific
claudins being associated with specific tissues.
Immunohistochemical expression
A number
of tumours which form the differential from perineuromas are
negative
for Claudin-1:
|
Claudin-1 |
|
perineuroma |
11/12(staining
is membranous: >50% of cells in 7/12 cases, 25-50% of cells
in 3/12 cases, <25% of cells in 1/12 cases)1,
22/76(soft
tissue perineuromas: 14 cases staining of more than 50% of cells,
3 cases staining of 26-50% of cells, 4 cases staining of 5-25%
of cells, 1 case staining of less than 5% of cells.)2 |
|
0/71 |
low-grade fibromyxoid
sarcoma |
0/81 |
desmoplastic
fibroblastoma |
0/31 |
fibromatosis |
0/71 |
|
6/9(a
significant number of cells, mainly peripheral, with long cytoplasmic
processes, thought to be perineural in origin were positive.)1 |
|
3/5(capsular
cells in three cases and perivascular cells in one case were positive)1 |
| |
Claudin-3; lung and liver epithelium1
Claudin-5; endothelium1
Claudin-6 is one of the earliest
molecules to be expressed in stem cells committed to epithelial differentiation
with expression almost entirely restricted to embryonal/fetal life.
It has been proposed as a marker for atypical teratoid/rhabdoid
tumours but probably lacks both sensitivity and specificity. The significant
staining is said to be membranous6.
| |
CNS tumours |
atypical teratoid/rhabdoid tumour |
7/74,
17/595,
12/316
|
|
| Germinoma |
2/26
|
| PNET |
1/26
|
| Meningioma |
1/106
|
| Medulloblastoma |
1/246
|
| Choroid plexus tumour |
0/11(10/11
showed apical staining)6
|
| Pilocytic astrocytoma |
0/216
|
| Astrocytoma (grade II/III) |
0/146
|
| Ganglioglioma |
0/136
|
| Glioblastoma |
0/126
|
| Ependymal tumours |
0/126
|
| Dysembryoplastic neuroepithelial tumours |
0/86
|
| Oligodendrogliomas |
0/86
|
| Craniopharyngioma |
0/46
|
| Central neurocytoma |
0/26
|
| non-CNS tumours |
Malignant rhabdoid tumour |
7/166
|
| Wilms tumour |
5/106
|
| Hepatoblastoma |
1/96
|
| Alveolar rhabdomyosarcoma |
0/96
|
| Embryonal rhabdomyosarcoma |
0/106
|
| Synovial sarcoma |
0/106
|
| Ewings sarcoma |
0/96
|
| Neuroblastoma |
0/106
|
Diagnostic utility
Claudin-1, with EMA
(positive) and S-100 (negative) in
the diagnosis of perineuroma.
Claudin 18 in combination with annexin
8 has potential for differentiating benign pancreatic epithelium
from infiltrating ductal carcinoma of the pancreas3.
References
1 Folpe,
A.L., Billings, S.D., McKenney, J.K., Walsh, S.V., Nusrat, A. and Weiss,
S.W. Expression of claudin-1, a recently described tight junction-associated
protein, distinguishes soft tissue perineurioma from potential mimics.
Am J Surg Pathol 2002;26:1620-6.
2 Hornick
JL,Fletcher CD. Soft tissue perineurioma: clinicopathologic analysis of
81 cases including those with atypical histologic features. Am J Surg
Pathol 2005; 29:845-58
3 Karanjawala
ZE, Illei PB, Ashfaq R, et al. New markers of pancreatic cancer identified
through differential gene expression analyses: claudin 18 and annexin
A8. Am J Surg Pathol 2008; 32:188-96
4 Birks
DK, Kleinschmidt-DeMasters BK, Donson AM, Barton VN, McNatt SA, Foreman
NK, et al. Claudin 6 is a positive marker for atypical teratoid/rhabdoid
tumors. Brain Pathol. 2010 Jan;20(1):140-50.
5 Antonelli
M, Hasselblatt M, Haberler C, Di Giannatale A, Garre ML, Donofrio V, et
al. Claudin-6 is of limited sensitivity and specificity for the diagnosis
of atypical teratoid/rhabdoid tumors. Brain Pathol. 2011 Sep;21(5):558-63.
6 Sullivan
LM, Yankovich T, Le P, Martinez D, Santi M, Biegel JA, et al. Claudin-6
is a nonspecific marker for malignant rhabdoid and other pediatric tumors.
Am J Surg Pathol. 2012 Jan;36(1):73-80.
This page last
revised 6.3.2012.
©SMUHT/PW
Bishop