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Extranodal natural killer/t-cell lymphoma, nasal type, in a patient with a constitutional 11q terminal deletion
Sujata Gaitonde1, Anoja Attele, Syed A Abutalib
1Department of Pathology, University of Illinois at Chicago, Chicago, Ill 60612, USA. sgaitond@uic.edu
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.
Aim:
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.
Case Report:
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.
Conclusion:
Patients with 11q terminal deletion disorder who reach adulthood may be predisposed to develop neoplasias by virtue of the constitutional deletion.