Bilateral parotid gland enlargement as a presenting manifestation of relapsed pediatric acute myeloid leukemia

Parotid gland involvement is a rare presentation of Acute Myeloid Leukemia (AML). We report a 10-year-old girl of acute myelomonocytic leukemia with normal cytogenetics and positivity for inversion 16, who after completion of first consolidation of the BFM 2004 AML protocol, presented with bilateral parotid gland enlargement. Bone marrow examination was suggestive of a relapse. Fine needle aspiration of the parotid gland showed presence of myeloblasts. Patient was given FLAG-IDA chemotherapy, with which the parotid swellings rapidly regressed and she achieved a remission and was planned for allogenic bone marrow transplantation. Acute myelomonocytic and monoblastic leukemias are known to be associated with tissue infiltration. However, exocrine gland involvement such as parotid enlargement is rare and is associated with a poor prognosis as was seen in our patient who despite having inversion 16 positivity had poor disease outcome.