Comparison in treatments of nonleukemic granulocytic sarcoma: report of two cases and a review of 72 cases in the literature

Background: The purpose of this study was to reveal the clinical characteristics of nonleukemic granulocytic sarcoma (GS) and an association between the therapeutic regimens and the nonleukemic period.

Method: Clinical records of 2 patients reported here and 72 patients gathered using a literature search on Medline from other institutions were analyzed. The patients consisted of 57 patients who preceded acute nonlymphoblastic leukemia (ANLL) and 17 patients who did not develop ANLL. These patients were divided into 3 groups by therapeutic regimens; Group I included 12 patients who received only biopsy or surgical resection of the tumor, Group II was 20 patients who received local irradiation for the tumor, and Group III consisted of 42 patients who received systemic chemotherapy. The nonleukemic periods between these groups were compared. In Group III, the period in the patients who were treated with chemotherapy given to ANLL was compared with that in the patients who received chemotherapy used for malignant lymphoproliferative disorders (MLPDs).

Results: Thirty-five patients (47%) initially were misdiagnosed, and the disease was most often malignant lymphoma. Preferential sites of GS were the small intestine, mediastinum, epidural site, uterus, and ovary, which often are difficult for the detection and diagnosis in addition to the skin and lymph nodes known commonly. The nonleukemic period after the diagnosis of GS was significantly longer in Group III than in the other groups (median, 12 months in Group III vs. 3 and 6 months in Groups I and II, respectively). The aggressive chemotherapy given to ANLL led to a longer nonleukemic period than the chemotherapy used for MLPDs.

Conclusions: To reduce the risk of subsequent ANLL in patients with nonleukemic GS, it is important that accurate histologic diagnosis is established initially for GS and that all isolated cases of GS, even those that appear to be cured by resection or irradiation of the tumor, are treated with intensive chemotherapy similar to that used to treat ANLL during the nonleukemic period as soon as possible.