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Juvenile myelomonocytic leukemia: a surprising cause of peri-orbital tumor and squint

  • Anselm Chi-wai Lee
  • Seo Wei Leo
Letter to the Editor
  • 22 Downloads

Dear Editor,

Juvenile myelomonocytic leukemia (JMML) is a rare hemic malignancy in children. It typically presents with fever, skin lesions, hepatosplenomegaly, and hyperleukocytosis with signs of marrow failure [1]. Distracting extramedullary disease may potentially delay diagnosis. Ocular involvement has been rarely reported in JMML, either at disease progression after diagnosis [2] or at relapse after stem cell transplantation [3]. Our patient is unique as she presented primarily with ocular manifestations while hematologic changes were subtle.

A 19-month-old girl with no stigmata of neurofibromatosis presented with bilateral proptosis, left abducens nerve palsy, and prolonged fever for 2 weeks. There was no external swelling. No significant lymphadenopathy or hepatosplenomegaly was found and there were no other neurologic deficits. Computed tomography showed soft tissue swelling infiltrating the orbits on both sides with intra-orbital and intracranial, extradural masses (Fig. 1a)....

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Study involving animals or human subjects

Not applicable.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors. As such, in our institution, approval by the institutional review board is not needed. On similar terms, informed consent other than medical management is not required.

References

  1. 1.
    Sakashita A, Matsuda K, Koike K (2016) Diagnosis and treatment of juvenile myelomonocytic leukemia. Pediatr Int 58(8):681–690CrossRefGoogle Scholar
  2. 2.
    Nambu M, Shimizu K, Ito S, Ohta S (1999) A case of juvenile myelomonocytic leukemia with ocular infiltration. Ann Hematol 78(12):568–570CrossRefGoogle Scholar
  3. 3.
    Chang GC, Moshfeghi DM, Alcorn DM (2006) Choroidal infiltration in juvenile myelomonocytic leukemia. Br J Ophthalmol 90(8):1067CrossRefGoogle Scholar
  4. 4.
    Edison MN, O’Dell MC, Letter HP, Scherer K, Williams JL (2017) Juvenile myelomonocytic leukemia presenting as bilateral breast masses. Pediatr Radiol 47(1):104–107CrossRefGoogle Scholar
  5. 5.
    Urs L, Stevens L, Kahwash SB (2008) Leukemia presenting as solid tumors: report of four pediatric cases and review of the literature. Pediatr Dev Pathol 11(5):370–376CrossRefGoogle Scholar
  6. 6.
    Imamura T, Matsuo S, Yoshihara T, Chiyonobu T, Mori K, Ishida H, Nishimura Y, Kasubuchi Y, Naya M, Morimoto A, Hibi S, Imashuku S (2004) Granulocytic sarcoma presenting with severe adenopathy (cervical lymph nodes, tonsils, and adenoids) in a child with juvenile myelomonocytic leukemia and successful treatment with allogeneic bone marrow transplantation. Int J Hematol 80(2):186–189CrossRefGoogle Scholar
  7. 7.
    Gustafsson B, Hellebostad M, Ifversen M, Sander B, Hasle H (2011) Acute respiratory failure in 3 children with juvenile myelomonocytic leukemia. J Pediatr Hematol Oncol 33(8):e363–e367CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Children’s Haematology and Cancer CentreMount Elizabeth HospitalSingaporeSingapore
  2. 2.Dr Leo Adult & Paediatric Eye Specialist Pte LtdMount Elizabeth Medical CentreSingaporeSingapore

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