Primary CD30/Ki-1 positive anaplastic large cell lymphoma of skeletal muscle with der(17)t(1;17)(q11;p11)

CD30/Ki-1 positive anaplastic large cell lymphoma (Ki-1 ALCL) frequently ex hibits extranodal disease and chromosomal t(2;5)(p23;q35). An Ii-year-old g irl presented with an intramuscular tu mor of the right upper arm. Tumors o f the chest wall, left arm and leg, hepatomegaly, pleural effusion, and enl arged lymph nodes then developed. The intramuscular tumor and pleural effus ion showed a diffuse infiltration of large atypical tells with abundant amp hophilic cytoplasms. The tumor cells were positive for CD30, CD2, CD45RO, a nd p80, but were negative for other T-cell, B-cell, and myeloid cell antige ns. She was diagnosed as having Ki-1 ALCL with a T-cell origin. Cytogenetic studies showed an abnormal karyotype including a der(17)t(1;17)(q11;p11). She received seven cycles of intensive chemotherapy followed by an autologo us peripheral blood stem cell transplantation, and has been in complete rem ission for more than two years. The primary involvement of skeletal muscle is quite uncommon in ALCL, and an abnormal karyotype including t(1;17)(q11: p11) has not been reported previously. Since a high frequency of aberration s of 1p36/1q12 or 17p13.3 was detected in sarcoma cells, the presence of su ppressor genes is suggestive in these sites. (C) 2000 Elsevier Science Inc. All rights reserved.