Relapse of childhood acute lymphoblastic leukemia in the lacrimal gland

✍ Scribed by Johnston, Donna L.

• Publisher: John Wiley and Sons
• Year: 2003
• Tongue: English
• Weight: 103 KB
• Volume: 40
• Category: Article
• ISSN: 0098-1532
• DOI: 10.1002/mpo.10203

✦ Synopsis

1972 [5]. Cytogenetic studies have revealed a reciprocal t(9;22)(q22;q12) in the myxoid variant that is characteristic of this tumor and confirm that EMC is a unique entity [6]. Myxoid chondrosarcoma has a broad morphologic spectrum, recently reviewed by Meis-Kindblom et al. [3]. Although, EMC has been classified as a low-grade sarcoma, and typically has a protracted clinical course, the large series of Meis-Kindlom et al. [3] suggests a less sanguine prognosis. To our knowledge, EMC has been described as a secondary malignancy in only one other patient; a child with Hodgkin disease who received mediastinal irradiation as part of therapy [7].

The treatment of EMC is problematic as it has the propensity to recur locally if not completely excised and usually does not respond to chemotherapy [8]. It appears that complete surgical excision for primary and recurrent tumors and metastases, when possible, is the mainstay of therapy. Our patient has undergone two local resections and is currently disease free, but the follow-up period has been relatively short.