Small-cell undifferentiated (neuroendocrine) carcinoma of the cecum in a child with common variable immunodeficiency.

PURPOSE: Patients with primary or secondary immunodeficiencies are known to be at increased risk for the development of malignancies, predominantly of lymphoreticular origin.

PATIENTS AND

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.