This is a 30-32 kD cell surface glycoprotein encoded by a pseudoautosomal gene located on the X and Y chromosomes.
Antibodies are effective on paraffin-embedded tissue5.
positivity takes the form of strong membrane staining.
normal tissues:
cortical thymocytes
pancreatic islet cells
urothelium
some squamous cells
columnar epithelial cells
fibroblasts
endothelial cells2
granulosa/Sertoli cells
T-cell ALL: one T-cell acute lymphoblastic leukaemia6 and seven of 11 T-lymphoblastic lymphomas6. All 8 B-lymphoblastic lymphomas were negative6.
Ewing's sarcoma / PNETs: 13 of 15 Ewing's sarcomas and 14 of 15 PNETS 6
islet cell tumours
carcinoids
rhabdomyosarcoma: 4 of 14 embryonal rhabdomyosarcomas using 12E76 . All 10 alveolar rhabdomyosarcomas were negative6.
nephroblastoma
leiomyosarcoma: 6 of 47 cases1
synovial sarcomas: 41 of 85 cases1
haemangiopericytomas: 21 of 33 cases1
malignant fibrous histiocytoma: 9 of 24 cases1
mesenchymal chondrosarcoma
rarely mesotheliomas: 1 of 10 cases1
uterine tumours with sex cord differentiation (1/23, 7/74)
Leydig cell tumour of testis and occasional Sertoli cell tumour of testis
PNET / Ewing's sarcoma
References
1Ordonez NG. Localized (solitary) fibrous tumor of the pleura. Adv Anat Pathol 2000;7:327-340.
©SMUHT/PW Bishop