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.