SUCCESSFUL TREATMENT OF PRIMARY EXTRAMEDULLARY LEUKEMIA (EML) OF THE UTERUS WITH RADICAL SURGERY, CHEMOTHERAPY, AUTOLOGOUS BONE-MARROW TRANSPLANTATION (BMT) AND PROPHYLACTIC LOCAL IRRADIATION

Extramedullary myeloid cell tumors are rare manifestations of acute no nlymphocytic leukemia (ANLL), While many advances in diagnosis have be en made, dilemmas remain concerning the treatment of this disease, In primary extramedullary leukemia (EML) most reports agree upon a local therapy followed by systemic chemotherapy such as is used for ANLL, Ho wever, further prophylactic local or systemic therapy with stem cell s upport remains controversial, A 20-year-old patient was diagnosed as h aving granulocytic sarcoma (GS) of the uterus without evidence of ANLL in 1991, After resection of the tumor at the uterine cervix and chemo therapy with daunorubicin 50 mg/m(2) (days 1-3) and cytosine-arabinosi de 200 mg/m(2) (days 1-7) in September 1991, complete remission was ac hieved, In October 1991 cytosine-arabinoside 1000 mg/m(2) every 12 h f rom day 1 to day 6 and amsacrine 200 mg from day 5 to day 7 were given as consolidation, Two years later relapse occurred in the adnexae, Af ter radical hysterectomy, the same induction and consolidation chemoth erapy was administrated, Subsequently, cytoxane 60 mg/m(2) and fractio nated total body irradiation (6 x 200 cGy) were given as conditioning and the previously cryopreserved bone marrow was reinfused, Finally, a fter hematopoietic engraftment, prophylactic local irradiation (4500 c Gy) to the pelvis was given resulting in a disease-free long-term surv ival of more than 36 months after relapse, Although this experience is confined to one patient, it may contribute to the design of prospecti ve therapeutic studies in patients with primary EML.