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)

Gopalrao V Velagaleti, Markku Miettinen, Zoran Gatalica

Research output: Contribution to journalArticle

19 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)23-27
Number of pages5
JournalCancer Genetics and Cytogenetics
Volume149
Issue number1
DOIs
StatePublished - Feb 2004
Externally publishedYes

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Chromosomes, Human, Pair 1
Neurilemmoma
Chromosomes, Human, Pair 16
Neurofibromatoses
Chromosome Aberrations
Neoplasms
Chromosome Deletion
Chromosomes, Human, Pair 9
Chromosomes, Human, Pair 7
Trisomy
Sarcoma
Skeletal Muscle

ASJC Scopus subject areas

  • Cancer Research
  • Genetics
  • Molecular Biology

Cite this

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title = "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)",
abstract = "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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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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