PMID- 10077147
OWN - NLM
STAT- MEDLINE
DCOM- 19990331
LR  - 20190708
IS  - 0020-7136 (Print)
IS  - 0020-7136 (Linking)
VI  - 81
IP  - 1
DP  - 1999 Mar 31
TI  - First cytogenetic study of a recurrent familial chordoma of the clivus.
PG  - 24-30
AB  - Two recurrences of a familial clivus chordoma, arisen from a patient who 
      developed the primary tumor at age of 8 years, were investigated by cytogenetic 
      and the fluorescence in situ hybridization (FISH) approach. Of the patient's 3 
      daughters, 2 developed, respectively, a clivus chordoma and an astrocytoma in 
      infancy, a familial aggregation highly suggestive of a genetic background. After 
      a 31-year hiatus, 2 tumor recurrences, developed over 17 months, were removed 
      surgically. Both were hypo- or nearly diploid, and had a pronounced karyotypic 
      heterogeneity with clonal and non-clonal rearrangements affecting several 
      chromosomes. The same rearrangement, a dic(1;9)(p36.1;p21), was shared in both 
      tumor specimens and, in 90% of the cells, chromosome 1p appeared to be involved 
      in unbalanced translocations with different chromosomes, leading to variable 
      losses of 1p. Previous cytogenetic data concerning chordoma are limited to 10 
      sporadic tumors with an abnormal karyotype; although no tumor-specific 
      rearrangements have been identified, chromosome 1p appears to be involved 
      frequently.
FAU - Dalpra, L
AU  - Dalpra L
AD  - Department of Biology and Genetics, Medical Faculty, University of Milan, Italy. 
      dalpra@imiucca.csi.unimi.it
FAU - Malgara, R
AU  - Malgara R
FAU - Miozzo, M
AU  - Miozzo M
FAU - Riva, P
AU  - Riva P
FAU - Volonte, M
AU  - Volonte M
FAU - Larizza, L
AU  - Larizza L
FAU - Fuhrman Conti, A M
AU  - Fuhrman Conti AM
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Int J Cancer
JT  - International journal of cancer
JID - 0042124
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Chordoma/*genetics/pathology
MH  - Cranial Fossa, Posterior
MH  - Female
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Karyotyping
MH  - Male
MH  - Neoplasm Recurrence, Local/*genetics/pathology
MH  - Skull Base Neoplasms/*genetics/pathology
EDAT- 1999/03/17 03:04
MHDA- 2000/06/20 09:00
CRDT- 1999/03/17 03:04
PHST- 1999/03/17 03:04 [pubmed]
PHST- 2000/06/20 09:00 [medline]
PHST- 1999/03/17 03:04 [entrez]
AID - 10.1002/(SICI)1097-0215(19990331)81:1<24::AID-IJC5>3.0.CO;2-O [pii]
AID - 10.1002/(sici)1097-0215(19990331)81:1<24::aid-ijc5>3.0.co;2-o [doi]
PST - ppublish
SO  - Int J Cancer. 1999 Mar 31;81(1):24-30. doi: 
      10.1002/(sici)1097-0215(19990331)81:1<24::aid-ijc5>3.0.co;2-o.