title: Extranodal natural killer/t-cell lymphoma, nasal type, in a patient with a constitutional 11q terminal deletion disorder.
abstract: BACKGROUND
  Most cases of constitutional 11q terminal deletion disorder are children. Malignancy is a potential concern, as these children reach adulthood. However, since the majority of patients are young, their risk of developing malignancy in adulthood is essentially unknown.
  
  
  OBJECTIVE
  To report the first hematologic malignancy [extranodal natural killer (NK)/T-cell lymphoma, nasal type] arising in the trachea of a patient with constitutional 11q terminal deletion disorder. Our patient is the oldest patient reported in the literature. It is of note that this cytogenetic abnormality has not been described as a recurring abnormality in extranodal NK/T-cell lymphoma.
  
  
  METHODS
  A 36-year-old male with congenital psychomotor retardation was transferred to our hospital. Pathologic evaluation was diagnostic of extranodal NK/T-cell lymphoma, nasal type. Staging marrow was negative for lymphoma, but cytogenetic analysis revealed a constitutional deletion of chromosome 11 at band q23 [46,XY,del(11)(q23)(c)]. This abnormality was present in a subsequent bone marrow specimen, along with an acquired abnormality, namely an extra copy of part of the long arm of chromosome 1 translocated to the short arm of chromosome 14.
  
  
  CONCLUSIONS
  Patients with 11q terminal deletion disorder who reach adulthood may be predisposed to develop neoplasias by virtue of the constitutional deletion.
authors.affiliation: Department of Pathology, University of Illinois at Chicago, Chicago, Ill 60612, USA. sgaitond@uic.edu
authors.forename: Sujata
authors.initials: S
authors.lastname: Gaitonde
authors.forename: Anoja
authors.initials: A
authors.lastname: Attele
authors.forename: Syed A
authors.initials: SA
authors.lastname: Abutalib
authors.forename: Damiano
authors.initials: D
authors.lastname: Rondelli
authors.forename: Valerie
authors.initials: V
authors.lastname: Lindgren
mesh_terms: D000328:Adult; D001707:Biopsy, Needle; D002869:Chromosome Aberrations; D002872:Chromosome Deletion; D002880:Chromosomes, Human, Pair 11; D020732:Cytogenetic Analysis; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D007694:Killer Cells, Natural / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics* / Q000473:pathology*; D008297:Male; D009297:Nasal Mucosa / Q000473:pathology; D009669:Nose Neoplasms / Q000235:genetics* / Q000473:pathology*; D012720:Severity of Illness Index