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Case of pediatric acute promyelocytic leukemia presenting as extramedullary tumor of the mandible. | LitMetric

Case of pediatric acute promyelocytic leukemia presenting as extramedullary tumor of the mandible.

Head Neck

Department of Oral and Maxillofacial Surgery, Faculty of Medicine, Saga University, Saga, Japan.

Published: October 2013

AI Article Synopsis

  • Acute promyelocytic leukemia (APL) is a rare but malignant form of leukemia usually caused by a specific genetic fusion; it's more common in adults than children.
  • A 1-year-old boy was diagnosed with APL after presenting with a mass in the jaw, where imaging suggested it could be a serious tumor.
  • Treatment with tretinoin was successful, and there have been no signs of recurrence after one year.

Article Abstract

Background: Acute promyelocytic leukemia (APL) is a malignant subtype of acute myeloid leukemia caused by the PML-retinoic acid receptor (RAR)α fusion gene. APL may be discovered in adulthood and diagnosed after spontaneous gingival bleeding or difficulty in hemostasis after oral surgery such as tooth extraction. However, APL is extremely rare in children.

Methods And Results: A 1-year-old boy presented with a mass on the mentum of the mandible. The marked periosteal reaction was seen on CT and MRI, leading to strong suspicion of a malignant bone-derived tumor such as a sarcoma. Chromosome banding by fluorescence in situ hybridization (FISH) showed PML-RARα, confirming the diagnosis of APL. Treatment with tretinoin was immediately initiated. No signs of recurrence have been noted 1 year after treatment.

Conclusions: We report herein a rare case involving an infant with APL who presented with an extramedullary tumor of the mandible, whom we treated with good results.

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Source
http://dx.doi.org/10.1002/hed.23163DOI Listing

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