The clinicopathologic findings in nine patients with ovarian
Sertoli-Leydig cell tumor with retiform pattern are described. The patients ranged in age from 11 months to 23 years; and seven patients were 12 years of age or younger. The most frequent presenting sign was the finding of an abdominal mass. This was associated with
pain in five patients. In three patients the
pain was severe due to torsion, causing an acute abdominal emergency. Slight
virilization was observed in one patient only. Two patients had elevated serum alphafetoprotein (AFP), which correlated well with disease activity. The remaining patients had normal serum AFP. All the
tumors were unilateral. At
laparotomy the
tumor was intact in six patients and ruptured in three. The
tumors ranged from 8 to 22 cm, were round or oval, and cystic or solid and cystic. Eight
tumors were in FIGO Stage I, and one was associated with abdominal
metastases and was Stage III. Histologically, the retiform component varied from moderate to predominant in eight of the nine cases. In two
tumors a heterologous component composed of striated muscle was also present. Three patients developed
metastases. Two of the patients died 11 months and 2 years after diagnosis and the third patient was lost to follow-up with evidence of disease 2 years after diagnosis. The remaining six patients were well and disease-free for periods of 8 months to 6 years. The majority of these
tumors were misinterpreted as serous
papillary cystadenocarcinoma or
endodermal sinus tumor, which are more
malignant neoplasms requiring different
therapy. This further underlines the importance of recognizing this histopathologic entity.