An extremely rare case of large cell variant of ovarian small cell car
cinoma is described. A 34-year-old woman (gravida 1, para 1) had a uni
lateral ovarian mass measuring 17 cm in greatest diameter and a metast
atic lesion in the omentum. Microscopically, the tumor showed a diffus
e arrangement of large, closely packed epithelial cells with abundant
eosinophilic cytoplasm and large nuclei with prominent nucleoli. The t
umor cells also were arranged in follicle-like and trabecular structur
es and cords. Immunohistochemically, many tumor cells were diffusely p
ositive for epithelial membrane antigen and some cells contained cytok
eratin CAM5.2, vimentin, neurofilament, neuron-specific enolase, or al
pha-1 antitrypsin. However, no specific lineage was detected. The tumo
r was aneuploid by flow cytometry. The patient received chemotherapy p
ostoperatively. However, the patient showed metastases in the inguinal
and retroperitoneal lymph nodes. The serum calcium level, which was n
ot measured preoperatively, was mildly elevated postoperatively. The p
atient was well with no evidence of disease 17 months after diagnosis.
This tumor must be distinguished from other primary or metastatic 'un
differentiated' neoplasms, especially ovarian small cell carcinoma of
pulmonary type and granulosa cell tumor.