Acute lymphoblastic leukemia secondary to chemoradiotherapy for perivascular epithelioid cell tumor of uterus

Acute lymphoblastic leukemia (ALL), a primary hematologic malignancy that is especially common in childhood, occurs relatively rarely as a secondary malignant neoplasm. Available data indicate that ALL often follows chemoradiotherapy for soft tissue sarcoma. Perivascular epithelioid tumor (PEComa), a primitive mesenchymal tissue origin, can be classified as a soft tissue sarcoma. An 11-year-old girl was diagnosed with ALL secondary to chemoradiotherapy (vincristine, ifosfamide, and anthracycline) and radiotherapy comprising 45 Gy to the whole pelvis for PEComa. ALL, FAB L2, and immunophenotypically pro-B developed 16 months after the final chemotherapy treatment. Moreover, a cytogenetic study of lymphoblasts showed t(1;11)(p32;q23). Herein, the authors report a case of secondary ALL that might be related to a previously used intercalating DNA topoisomerase II inhibitor (anthracycline) for a very rare sarcoma, PEComa.