Sixteen patients presenting with granulocytic sarcoma without evidence of acute leukemia were seen and diagnosed at The University of Texas M.D. Anderson Hospital and Tumor Institute at Houston from 1962 to 1985. Seven of them (44%) did not develop acute leukemia. Of these seven, four are alive with no evidence of disease 3.5 to 16 years after initial presentation; the remaining three patients died of their disease within 2 to 8 months of presentation. Two of 16 patients were diagnosed within the last 15 months and do not have adequate follow-up. The seven remaining patients developed acute leukemia within 1 week to 13 months of the diagnosis of granulocytic sarcoma. Six of them died 5 weeks to 16 months after diagnosis; one patient has been in complete remission for 8 years. Twelve of these 16 cases (75%) were initially misdiagnosed, most frequently as large cell lymphoma. The remaining four cases were correctly diagnosed as granulocytic sarcoma. The naphthol-ASD-chloroacetate esterase stain was required to make the correct diagnosis in all cases. Contrary to findings in other series, granulocytic sarcoma arising in nonleukemic patients does not necessarily progress to acute leukemia. At least four of 16 (25%) patients in this series did not develop acute leukemia during the 3.5 to 16 years they have been followed. No prognostic factors were identified in this series to predict which patients would develop acute leukemia and which ones would not.

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Myxoinflammatory fibroblastic sarcoma (MIFS) is a low-grade malignant neoplasm for which limited genetic information, including a t(1;10)(p22;q24) and amplification of chromosome 3 material, is available. To further characterize these aberrations, we have investigated eight soft tissue sarcomas diagnosed as MIFS, haemosiderotic fibrolipomatous tumour (HFT), myxoid spindle cell/pleomorphic sarcoma with MIFS features, and inflammatory malignant fibrous histiocytoma/undifferentiated pleomorphic sarcoma with prominent inflammation (IMFH) harbouring a t(1;10) or variants thereof and/or ring chromosomes with possible involvement of chromosome 3. Using chromosome banding, fluorescence in situ hybridization, array-based comparative genomic hybridization, global gene expression, and real-time quantitative PCR analyses, we identified the breakpoint regions on chromosomes 1 and 10, demonstrated and delineated the commonly amplified region on chromosome 3, and assessed the consequences of these alterations for gene expression. The breakpoints in the t(1;10) mapped to TGFBR3 in 1p22 and in or near MGEA5 in 10q24, resulting in transcriptional up-regulation of NPM3 and particularly FGF8, two consecutive genes located close to MGEA5. The ring chromosomes contained a commonly amplified 1.44 Mb region in 3p11-12, which was associated with increased expression of VGLL3 and CHMP2B. The identified genetic aberrations were not confined to MIFS; an identical t(1;10) was also found in a case of HFT and the amplicon in 3p was seen in an IMFH.

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