We report the cytogenetic findings in a case of mixed germ cell-sex co
rd-stromal tumor of the ovary in a 5-month-old girl. Monosomy 22 was o
bserved as the sole karyotypic abnormality. This result was confirmed
by comparative genomic hybridization, which revealed no additional chr
omosomal imbalances. This is the first observation of a chromosomal ab
erration in a mixed germ cell-sex cord-stromal tumor of the ovary. Mon
osomy 22 has been previously observed in granulosa cell tumors of the
ovary. This could suggest a common pathogenetic pathway for both types
of tumors. (C) 1997 Wiley-Liss, Inc.