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Primary malignant peripheral nerve sheath tumour of the heart
Author(s) -
Patrick Pauwels,
Paola Dal Cin,
Raf Sciot,
Martin Lammens,
Olaf C. Penn,
E. van Nes,
W.S. Kwee,
Herman Van den Berghe
Publication year - 1999
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1046/j.1365-2559.1999.00572.x
Subject(s) - synovial sarcoma , pathology , vimentin , malignant peripheral nerve sheath tumor , cytokeratin , sarcoma , immunohistochemistry , differential diagnosis , s100 protein , medicine , biology
Aims Malignant peripheral nerve sheath tumour (MPNST) is an unusual sarcoma of the heart and should be differentiated from other spindle cell sarcomas. Methods and results We combined morphological, immunohistochemical and cytogenetic techniques in the differential diagnosis, particularly from monophasic synovial sarcoma. The tumour consisted of dense fascicles, alternating with hypocellular, myxoid zones. There was a marked herniation of tumour cells into the vascular lumen. Immunohistochemically, the tumour cells expressed vimentin and focally S100 protein. There was no expression of cytokeratin or epithelial membrane antigen (EMA). Although we found a complex karyotype, the finding of involvement of 17q11, the loss of chromosome 22, and the absence of a t(X;18)(p11.2;q11.2), which characterizes synovial sarcoma, allowed us to the final diagnosis of malignant peripheral nerve sheath tumour. Conclusion MPNST of the heart can be diagnosed by combining morphological and immunohistochemical date. Cytogenetics can be useful in reaching a correct diagnosis, also by helping to rule out monophasic synovial sarcoma.