Primary primitive neuroectodermal tumor of the ovary

Abstract

Primitive neuroectodermal tumor of the ovary is a rare and aggressive small round
cell soft tissue sarcoma of neural origin that is usually associated with high morbidity
and mortality. Immunohistochemistry is a useful adjunct in the differential diagnosis.
We describe a case of a primitive neuroectodermal tumor of the ovary in a 21-yearold nulliparous patient who reported pain and increased abdominal circumference.
Ultrasonography showed a solid-cystic heterogeneous tumor apparently originating
from the left adnexa. Magnetic resonance imaging confirmed the presence of a
tumor extending into the left iliac fossa without local organ involvement or regional
or distant metastases. Tumor markers were all within the normal range. During
laparotomy, a left ovarian tumor was observed with a normal right ovary. Left
salpingo-oophorectomy was performed due the size of the tumor, right ovarian
wedge resection, pelvic lymphadenectomy and omentectomy. Histopathologic
examination revealed tumor composed of sheets of round cells. The tumor cells were
positive for chromogranin A, synaptophysin, vimentin and neuron-specific enolase,
which confirmed the diagnosis of a primitive neuroendocrine tumor of the left
ovary originating from immature cystic teratoma. The patient refused postoperative
chemotherapy.