Cytogenetic analysis of a malignant triton tumor and a malignant peripheral nerve sheath tumor and a review of the literature

• Published in: Cancer genetics and cytogenetics Vol. 91; no. 1; pp. 8 - 12
• Main Authors: McComb, Erin N., McComb, Rodney D., DeBoer, Joanne M., Neff, James R., Bridge, Julia A.
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01-10-1996; Elsevier Science
• Subjects: Adolescent; Adult; Biological and medical sciences; Chromosome Aberrations - genetics; Chromosome Disorders; Female; Humans; Karyotyping; Medical sciences; Nerve Sheath Neoplasms - genetics; Neurilemmoma - genetics; Neurofibrosarcoma - genetics; Neurology; Peripheral Nervous System Neoplasms - genetics; Peroneal Nerve; Tumors of the nervous system. Phacomatoses; Chromosomal aberration; Human; Skin disease; Nervous system diseases; Phacomatosis; Neurosarcoma; Partial deletion; Exploration; Malignant tumor; Neurofibromatosis Recklinghausen; Genetic disease; Case study; Abnormal chromosome E17; Adolescent; Abnormal chromosome A1; Abnormal chromosome G22; Cytogenetics; Abnormal chromosome; Genetics; Adult; Neurofibrosarcoma; Comparative study
• ISSN: 0165-4608; 1873-4456
• DOI: 10.1016/S0165-4608(96)00125-2

Cytogenetic analyses of malignant peripheral nerve sheath tumors (MPNST) and malignant triton tumors (MTT) are few to date. Two separate triton tumor specimens from one patient and a MPNST specimen from another patient, both with peripheral neurofibromatosis (NF-1, von Recklinghausen disease), showed complex near-triploid complements and partial deletion of the short arm of chromosome 1. Notably, a structural abnormality of chromosome 17 was detected in the MPNST, and loss of chromosome 22 was detected in the MTT. The genes for peripheral neurofibromatosis (NF-1) and central neurofibromatosis (NF-2) have been mapped to these two chromosomes respectively.