Malignant peripheral nerve sheath tumor with a t(X;18) - A synovial sarcoma variant?

Citation
R. Vang et al., Malignant peripheral nerve sheath tumor with a t(X;18) - A synovial sarcoma variant?, ARCH PATH L, 124(6), 2000, pp. 864
Citations number
10
Categorie Soggetti
Research/Laboratory Medicine & Medical Tecnology","Medical Research Diagnosis & Treatment
Journal title
ARCHIVES OF PATHOLOGY & LABORATORY MEDICINE
ISSN journal
00039985 → ACNP
Volume
124
Issue
6
Year of publication
2000
Database
ISI
SICI code
0003-9985(200006)124:6<864:MPNSTW>2.0.ZU;2-O
Abstract
We describe an ankle tumor arising in a 16-year-old girl. The tumor demonst rated histology typical of a malignant peripheral nerve sheath tumor (MPNST ), but exhibited a variant form of the (X;18) translocation associated with synovial sarcoma. Immunohistochemical stains were positive for vimentin, C D57, collagen type IV, and Bcl-2. Routine and molecular cytogenetic studies showed an unbalanced 3-way chromosomal translocation that involved chromos omes X, 18, and 1. Electron microscopic findings were noncontributory. This unusual tumor raises the following questions and possibilities: (1) As the t(X;18) suggests, could this tumor be a monophasic synovial sarcoma with t he histologic features of an MPNST? (2) Or, as the histology suggests, is t his tumor an MPNST that has a t(X;18)? (3) Finally, could MPNST histology, a t(X;18), and no defining immunohistochemical or electron microscopic feat ures represent an as yet unrecognized part of a spectrum that spans from sy novial sarcoma to MPNST or other spindle cell tumors?