Small-cell undifferentiated (neuroendocrine) carcinoma of the cecum in a child with common variable immunodeficiency.
OBJECTIVE
Patients with primary or secondary immunodeficiencies are known to be at increased risk for the development of malignancies, predominantly of lymphoreticular origin.


METHODS
We here describe a patient with infant-onset hypogammaglobulinemia due to a common variable immunodeficiency. At the age of 16 a small-cell undifferentiated (neuroendocrine) carcinoma of the cecum (SCUNC) was diagnosed.


RESULTS
Neurohormonal analysis showed normal values.


CONCLUSIONS
To our knowledge this is the first report in childhood of SCUNC of the gastrointestinal tract, which has also previously not been associated with primary immunodeficiency.
Department of Pediatrics, Sophia Children's Hospital/Erasmus University, Rotterdam, The Netherlands.
N C
NC
de Bruin
R
R
de Groot
J C
JC
den Hollander
S I
SI
Agoston
J J
JJ
van Dongen
H J
HJ
Neijens
D018288:Carcinoma, Small Cell / Q000150:complications* / Q000473:pathology; D002430:Cecal Neoplasms / Q000150:complications*; D002454:Cell Differentiation / Q000502:physiology; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male