Primary orbital manifestation of acute myelogenous leukemia in the childhood
M. Jäger, K.-H. Emmerich, G. Lindemann
Case-report: In October 1999 a 4-year-old child was presented with a painless swelling of the right upper orbita that had been increasing in size for three weeks. The emergency admission was necessary because of additional diplopia. Further evaluation revealed a diarrhea accompanied with fever.
Diagnostic: B-scan ultrasound showed a non-infiltrative well-defined tumor at 12 o´clock near to the bulb and the lacrimal gland. The tumor was about 16 mm in size, had a smooth surface and a relatively homogenous structure. Additional MRI-scan was arranged which demonstrated an untypical enhancement with contrast medium with respect for a rhabdomyosarcoma. The examination of the peripheral blood revealed an anaemia and thrombocytopenia but no leucocytosis. The increased value of LDH hardened the suspect of a malignant disease with a high apoptosis rate of cells. Additional differential blood count showed a blast proportion of 50%. On the basis of these results we diagnosed an acute myelogenous leukemia.
Results: The orbital tumor occured as the primary manifestation of an acute leukemia. Chemotherapy was performed by the pediatric oncologists. One week after starting chemotherapy the swelling became smaller.
Conclusion: Leukemia is the most common malignancy in childhood. Primary orbital tumor manifestations are rare but may precede pathological findings in bone marrow or peripheral blood up to 15 months. For this reason correct early diagnosis is important, because chemotherapy may be more effective if initiated before the leukemic phase develops.
Eye-clinic, Klinikum Darmstadt, Heidelberger Landstr.379, D-64297 Darmstadt