A 53-year-old female was referred to our department with complaints of a slight dry cough and enlarged pulmonary nodules [computed tomography (CT) value: 15 Hounsfield units (HU)] with slight peripheral enhancement, which were under close monitoring after cytoreductive surgery for a BOT.
Seven years prior, she had suffered from progressive abdominal distention. Abdominal contrast-enhanced CT (Figures 1A,B) and a thorough check-up revealed a giant pelvic-abdominal cyst with an uneven density (7 HU) with peripheral and internal septal enhancement. The giant cyst extended from the pelvic cavity to the level of right renal pelvis, measuring 20 x 40 cm, with ascites. Meanwhile, chest CT showed multiple bilateral cystic nodules without obvious enhancement (Figures 2A-C). The patient's serum carbohydrate antigen 125 (CA-125) and CA-19-9 levels were increased to 69.15 U/ml (0-35 U/ml) and 369.4 U/ml (0-37 U/ml), respectively. Other serum tumor markers [carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP), neuron-specific enolase (NSE), and cytokeratin 19 fragments] were within normal ranges. For final diagnosis of the mass in the abdomen, exploratory laparotomy was planned. Before laparotomy, to determine the nature of the nodules in the lungs and the detailed staging of the mass, the patient underwent percutaneous biopsy, and the histopathological results indicated only fibrosis and degeneration of the lung tissue but no evidence of metastasis from the ovary. Repeated percutaneous biopsy for another nodule was performed to avoid a missed diagnosis of metastasis, and the histopathology confirmed the previous results showing clear cystic fluid but not metastasis. Subsequent laparotomy revealed a cystic mass with a smooth surface and an intact capsule on the right ovary and ascites. Intraoperative frozen section revealed an ovarian mucinous borderline cystadenoma (MBOT). Exfoliative cytological examination revealed no evidence of implantation in the ascites. Thus, staging surgery (bilateral salpingo-oophorectomy (BSO), hysterectomy, appendectomy with omentectomy, and the adjacent lymphadenectomy including para-aortic lymph nodes, and pelvic lymph nodes) was performed. The final pathology confirmed the diagnosis of ovarian mucinous borderline cystadenoma, intestinal type, with the intestinal epithelium rich in goblet cells and the stratification of cells showing light to moderate atypia in a papillary structure (Figures 1C,D) with no involvement of the left ovary, omentum, and bilateral adjacent lymph nodes. Thus, the FIGO stage at diagnosis was incomplete stage due to the obscure diagnosis of the lung nodules. After resection of the tumor, the serum CA-125 and CA-19-9 levels of the patient declined to normal values of 27.5 and 18.09 U/ml, respectively, which is consistent with the reported correlation between preoperative CA-125 levels and increasing FIGO stages of BOTs. Then, the patient was followed closely for the pulmonary nodules on CT scans (Figures 2D-F) and for possible asymptomatic recurrence of the BOT and malignant transformation arising from the BOT.
During the following 7 years, CA-125 was within the normal range after the risk-reducing cytoreductive surgery, and the size and morphology of the pulmonary nodules were stable.
For this admission in our department at the end of the seventh year during the monitoring of the disease, the patient presented with cough and clear sputum without hemoptysis or any other symptoms. Chest CT showed a new irregular nodule at the dorsal segment of the right inferior lobe, and a cavity was observed in the largest original pulmonary nodule (Figures 2G-O). The patient's serum CA-125 and CEA levels were elevated to 68.9 and 66.50 U/mL (normal range 0 ~6 U/ml), respectively. Abdominal CT scan revealed no obvious abnormality or ascites. Thus, transbronchial lung biopsy (TBLB) targeting the new consolidation was performed. The histopathology result indicated papillary mucinous adenocarcinoma, while immunostaining suggested malignant transformation arising from the BOT [Cytokeratin(CK)(+), P63(-), CK5/6(-), Napsin-A(-), Transcription Factor-1(TTF-1)(-), Cluster of Differentiation(CD)56(-), Ki-67(15%), CA-125(focal+), Estrogen receptor (ER)(-), paired box gene 8(PAX8)(-), CK7(+), CK20(-), Caudal type homeobox transcription Factor 2(CDX2)(-), and P16(-)] (Figures 3A-F). Breast cancer susceptibility gene (BRCA) mutation was not observed in the tissue of the lung biopsy. After four cycles of chemotherapy [AUC (area under the curve): 5 mg/ml/min of carboplatin and 65 mg/m2 of docetaxel per cycle] with 3-week intervals were completed, the pulmonary solid metastasis progressed with the development of pleural effusion and pleura thickening (Figures 2P-R). Ultrasonic-guided transcutaneous pleural biopsy revealed metastatic adenocarcinoma (Figures 3G-J). The patient passed away 4 months later.