Hepatoid carcinoma of the ovary without staining for alpha-fetoprotein
A 51-year-old woman presented to the department of internal medicine with left lower quadrant pain and hematochezia for a month. Colonoscopy revealed hyperemic mass with spontaneous bleeding at about 20 cm above the anal verge which almost obstructs the lumen. A computed tomography (CT) scan of the abdomen showed 12.5×7.8×11.2 cm sized large heterogeneous pelvic mass invading sigmoid colon and uterus (). There was peritoneal seeding and multiple metastatic lymphadenopathy along the bilateral iliac chain and retroperitoneal space up to level of left renal vein but the liver was normal in size and texture. Laboratory analysis revealed normal renal and liver function. The level of CA-125 was elevated to 37.5 U/mL but carcinoembryonic antigen (CEA) and CA-19-9 was normal. She was transferred to the department of Gynecology to rule out ovarian malignancy and positron emission tomography (PET) scan was done. PET scan showed large mass with heterogeneous FDG uptake in the pelvic cavity and multiple hypermetabolic seeding nodules, suggesting peritoneal seeding. The left supraclavicular lymphadenoapathy was seen and fine needle aspiration biopsy revealed metastatic carcinoma. She underwent an explorative laparotomy with total abdominal hysterectomy, bilateral salpingo-oophorectomy, appendectomy and tumorectomy including sigmoid colon with Hartmann's operation. The white-pink, granular appearance 9×8×6 cm sized cancerous mass with necrosis was found posterior to uterus which invades sigmoid colon. Frozen biopsy of the pelvic mass showed poorly differentiated carcinoma but the cell type was unknown because of severe inflammation. Bilateral pelvic lymph nodes were enlarged on palpation however, dissection was not performed. Histologically, the tumor was composed of solid sheets or aggregates of uniform cells with moderate or abundant eosinophilic cytoplasm, distinct cell borders, and centrally located nuclei with prominent nucleoli (). On immunohistochemical staining, p53, p16 was positive; hepatocyte paraffin-1, caudal type homeobox-2, Wilms Tumor-1, estrogen receptor and AFP was negative (). The tumor was relatively homongeneous, with no evidence of conventional germ cell tumor or ovarian surface epithelial tumor morphology. It was consistent with hepatoid carcinoma of the right ovary. After the surgery the CA-125 increased from 37.5 to 43.2 U/mL. But the level of serum AFP was 2.2 ng/mL (normal range, 0.0 to 10.0 ng/mL). Chemotherapy was started with an ovarian cancer regimen of paclitaxel 175 mg/m2 and carboplatin with an area under the curve of 5 every 3 weeks. Serum CA-125 returned to normal range after the first cycle of chemotherapy and remained within normal range (24 to 27 U/mL) during three cycles of chemotherapy. A CT scan after the three cycles of chemotherpy showed progression of disease with 4.3 cm recurrent peritoneal carcinomatosis in pelvic cavity and extensive metastatic lymphadenopathy with necrotic change in retroperitoneum, bilateral iliac chain and retrocrural area. Second line treatment with docetaxel was given three cycles but the disease progressed rapidly and ileus developed due to seeding mass in the abdominal cavity and pulmonary effusion newly developed. The patient expired 6 months after the initial diagnosis.