Background: Acute leukemia represents the main cause of neoplastic disease in pediatric age. The most frequent clinical presentation of acute lymphoblastic leukemia (ALL) are hepatomegaly and/or splenomegaly, lymphadenopathy, fever, anorexia, weight loss, asthenia and sweating. Although other forms of extramedullary involvement may exist, initial parotid infiltration is very rare.
Clinical Case: A previously healthy nine-year-old boy was admitted to the emergency department due to progressive bilateral pre-auricular swelling over the last 10 days, with no other associated systemic symptomatology. Bilateral exuberant and painless parotid swelling was observed, with hard consistency and adhesion to the deep planes, as well as submandibular adenopathy, the larger with 3cm, hard and painless, without other changes on clinical examination. Blood tests showed 27810/uL leukocytes [normal (N) 5000-15000/uL], 18080/uL lymphocytes (N 1500-7000/uL), hemoglobin 9,9g/dL (N 11,5-15,5g/dL), mean globular volume 80,2fL (N 72-84fL), mean corpuscular hemoglobin 26,9pg (N 25-33pg), platelets 111000/uL (N 150000-500000/uL), peripheral blood smear with atypical lymphocytes, erythrocyte sedimentation rate 9,2mm/h (N 0-10mm/h), LDH 567 IU/L (N
Conclusion: Bilateral parotid swelling constitutes a rare initial presentation form of acute leukemia. The present case underlines the importance of clinical suspicion of neoplastic disease in the presence of parotid swelling with atypical evolution and in the absence of associated inflammatory signs.