TY - JOUR
T1 - Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (malignant triton tumor) with balanced t(7;9)(q11.2;p24) and unbalanced translocation der(16)t(1;16)(q23;q13)
AU - Velagaleti, Gopalrao V.N.
AU - Miettinen, Markku
AU - Gatalica, Zoran
PY - 2004/2
Y1 - 2004/2
N2 - Malignant peripheral nerve sheath tumors (MPNST) with skeletal muscle differentiation are termed malignant triton tumors. A case of malignant triton tumor arising in a patient without signs of neurofibromatosis with two consistent chromosomal abnormalities is described. The first was of a balanced translocation between the long arm of chromosome 7 and the short arm of chromosome 9. The second was an unbalanced rearrangement between chromosomes 1 and 16, leading to partial trisomy for the long arm of chromosome 1 and partial monosomy for the long arm of chromosome 16. Review of previous reports on chromosomal abnormalities in malignant triton tumors revealed consistent abnormalities involving chromosome 1, regardless of the presence or absence of neurofibromatosis. This finding may relate to the observed poor prognostic outcome in this type of sarcoma. Also unique to our case is the translocation involving 7q and 9p, both regions may play a role in MPNST.
AB - Malignant peripheral nerve sheath tumors (MPNST) with skeletal muscle differentiation are termed malignant triton tumors. A case of malignant triton tumor arising in a patient without signs of neurofibromatosis with two consistent chromosomal abnormalities is described. The first was of a balanced translocation between the long arm of chromosome 7 and the short arm of chromosome 9. The second was an unbalanced rearrangement between chromosomes 1 and 16, leading to partial trisomy for the long arm of chromosome 1 and partial monosomy for the long arm of chromosome 16. Review of previous reports on chromosomal abnormalities in malignant triton tumors revealed consistent abnormalities involving chromosome 1, regardless of the presence or absence of neurofibromatosis. This finding may relate to the observed poor prognostic outcome in this type of sarcoma. Also unique to our case is the translocation involving 7q and 9p, both regions may play a role in MPNST.
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U2 - 10.1016/S0165-4608(03)00278-4
DO - 10.1016/S0165-4608(03)00278-4
M3 - Article
C2 - 15104279
AN - SCOPUS:0842324412
SN - 0165-4608
VL - 149
SP - 23
EP - 27
JO - Cancer Genetics and Cytogenetics
JF - Cancer Genetics and Cytogenetics
IS - 1
ER -