Case 1
A 57-year-old woman was admitted to our clinic with an abdominal mass that had appeared 2 weeks earlier, but she did not experience symptoms of abdominal pain and distension. She had been postmenopausal for 3 years and had no vaginal bleeding after menopause. Vaginal and abdominal examinations confirmed the presence of a cystic mass, with a diameter of 6-7 cm and no pressing pain. Vaginal ultrasonography revealed a mass 9.6*8.2*5.2 cm in diameter that was thickly encapsulated with a liquid and irregular mass in the left ovarian region. The interior of the cyst contained several septa, with medium to strong echo bulges on the septa, the largest of which was 1.8*1.1 cm in size. A slight blood flow signal could be seen on the septum, and a liquid area with a maximum depth of 4.6 cm could be seen in the pelvic cavity. Before surgery, the levels of serum tumor markers were within normal limits.
During the laparotomy, an irregular, multilocular, cystic mass 8*10 cm in diameter was found in the right ovarian region, and no metastatic lesions were observed. Total abdominal hysterectomy (TAH) plus bilateral adnexectomy was performed. The pathologic diagnosis of the mass was a well-differentiated sarcoma of the right ovary (fibrostromal sarcoma or fibrosarcoma). The immunohistochemical results were as follows: Vimentin (+), SMA (+), PR (±), ER (-), CD10 (-), Caldesmon (-), Melan (-), α-inhibin (-).
The tumor was categorized as an International Federation of Gynecology and Obstetrics (FIGO) stage I C. The patient was given 2 cycles of systemic chemotherapy consisting of Cisplatin + Vincristine + Bleomycin (PVB) at 3-week intervals. After chemotherapy, the patient was followed-up regularly, and no recurrence was seen 15 years after surgery.
Case 2
A 41-year-old female was admitted with an abdominal mass that had appeared 2 months earlier. This patient reported no additional complaints, such as abdominal pain or distension. She had normal menstruation and no abnormal vaginal bleeding. Upon abdominopelvic examination, a firm, unmovable mass 5*6 cm in diameter without tenderness was detected on the posterior left side of the uterus. Vaginal ultrasonography revealed a heterogeneous, left ovarian mass with a clear boundary and a diameter of 6.1*5.5*4.6 cm. No signs of metastasis, lymph node enlargement or ascites were observed. No obvious blood flow signal was detected. The levels of serum tumor markers were within normal limits with the exception of a slightly elevated carcinoma antigen (CA) 125 level (40.4 U/mL). By magnetic resonance imaging (MRI), a heterogeneous mass 48*62*60 mm in diameter that was closely related to the uterus was detected on the posterior left side of the uterus. The possibility of subserosal fibroids was considered, whereas an adnexal tumor was not considered.
During laparoscopic exploration, we found a solid mass 7*6 cm in diameter with a clear boundary; the mass originated in the left ovary, and no peritoneal dissemination was observed. The right ovary appeared full and the uterus appeared normal. Ovarian cystectomy, sampling of the right ovary and intraperitoneal washing were performed during surgery. After the mass was cut open, solid granular materials were present. The intraoperative pathological diagnosis was left ovarian follicular fibroma. However, a week later, the final pathologic diagnosis was fibrosarcoma of the left ovary, and the mitotic counts were evaluated >10 times in 10 high-power fields (HPFs). The immunohistochemical analysis showed AE1/AE3 (-), Calretinin (-), Ki-67 (index 5%), p53 (-), α-inhibin (-). After surgery, the serum CA125 level was 27.1 U/ml.
This case was diagnosed as FIGO stage I A. The patient refused chemotherapy and remained disease-free with normal ovarian function during the 7-year follow-up.
Case 3
A 76-year-old woman was admitted with abdominal pain and fever. She had been postmenopausal for 28 years without any vaginal bleeding after the onset of menopause. Fourteen years ago, the patient underwent an exploratory laparotomy due to acute abdomen at another hospital. During the operation, 2800 ml of intraabdominal blood clots and non-clotted blood was removed, and a cauliflower-like tumor 3*3*2 cm in diameter with active bleeding was seen around the left tubal umbrella. Complete hysterectomy and bilateral salpingo-oophorectomy and omentectomy were performed. The postoperative pathology report described a diffuse granular follicular cell tumor of the left ovary with invasion of the left fallopian tube. The clinical FIGO stage was II C. After surgery, she received 3 cycles of systemic chemotherapy consisting of Cisplatin + Cyclophosphamide (PC), after which the patient did not return for follow-up.
Four years later (10 years ago), the patient underwent a second exploratory laparotomy at another hospital due to abdominal pain and the presence of a pelvic mass. During the surgery, a solid-cystic mass 3 cm in diameter was observed between the sigmoid colon and the bottom of the left side of the bladder. In addition, a nodule 1 cm in diameter at the bottom of the left side of the bladder bottom and a nodule 0.5 cm in diameter on the surface of the sigmoid colon were seen. The tumor lesions were completely removed, and the postoperative pathological diagnosis was granulosa cell tumor. After surgery, she received 1 cycle of chemotherapy consisting of Taxol + Carboplatin. However, the patient was then lost to follow-up.
The next time, she was admitted for abdominal pain and fever that persisted for 1 day. Ultrasound revealed an irregular, cystic mass 10*8.6 cm in diameter with an unclear boundary in the pelvic cavity; the wall was uneven in its thickness, and the size of the cystic portion was approximately 7.21*6.34 cm. MRI revealed a double capsular structure 8*9*7 cm in diameter on the left side of the pelvic cavity. The mass had a thick wall with a relatively clear edge and was located close to the right bowel. The cephalic size of the lesion was approximately 4*2*3 cm, and the signal of the vesicle was slightly low. Vaginal and abdominal examinations confirmed the presence of a hypertonic, cystic mass with a diameter of 16 cm. The preoperative serum levels of CA125, Estradiol (E2), and Follicle Stimulating Hormone (FSH) were 15.5 U/ml, 25.8 pg/mL and 52.8 mIU/mL, respectively.
We performed exploratory laparotomy and secondary cytoreductive surgery. During the operation, a solid-cystic multilocular mass 10 cm in diameter was observed between the sigmoid colon and the left lateral wall of the bladder. After 600 ml of yellow intracapsular fluid was removed, the mass was completely excised. Then, we performed adhesion decompression, repair of the sigmoid colon, and partial ileotomy anastomosis. The tumor was then completely removed, and the pathologic diagnosis was ovarian fibrosarcoma. Immunohistochemical analysis showed the following: Melan-A (+), Vimentin (+), AE1/AE3 (+/-), CD99 (-), Calretinin (-), α-inhibin (-), Ki-67 index 10%.
After surgery, the patient experienced incomplete intestinal obstruction and received conservative treatment. The patient’s condition gradually improved and she fully recovered after 2 weeks. Since the patient was elderly, she did not receive adjuvant chemotherapy. No signs of recurrence or an increase in the serum E2 level have been observed more than 6 years after surgery.
Case 4
A 76-year-old woman was admitted with abdominal pain and an abdominal mass. She had been postmenopausal for 28 years and had no abnormal vaginal bleeding. Ultrasound revealed a heterogeneous, irregular, hypoechoic mass without a clear boundary that was located on the upper right-hand side of the uterus, with a diameter of 11.4*13.5*8.6 cm. Punctiform blood flow signals could be seen by Color Doppler Flow Imaging (CDFI). MRI revealed a large solid-cystic mass in the pelvic cavity, and we considered the possibility of malignant lesions in the accessory ovary, except mesenchymal tumors. Vaginal and abdominal examinations confirmed the presence of a hypertonic, cystic mass with a diameter of 12-15 cm and no pressing pain. Before surgery, the serum CA125 level was 593.3 U/ml.
Her medical history included sleep apnea syndrome for more than 10 years that necessitated supplementary positive pressure ventilation at night.
After a multidepartment consultation, surgical contraindications were eliminated, and we performed an exploratory laparotomy. During the operation, approximately 100-200 ml of bloody ascites was observed. A cystic-solid mass with a diameter of approximately 20 cm was seen below the incision. Cauliflower-like tumors with a rich blood supply could also be seen on the surface of the mass. The mass was widely adherent to the surrounding small intestine and colon, the surface of which contained cauliflower-like tumor lesions. The source of the mass and the metastatic tumors was found to be the left accessory ovary, and thus, the left adnexa was removed. The pathology report revealed a malignant spindle cell fibrosarcoma. The mitotic counts were evaluated >10 times/10 HPFs. Immunohistochemical analysis showed the following: CA125 (-), CD10 (partial+), Desmin (-), Ki-67 (index40%), SMA (+), S-100 (-), Vimentin (+), p53 (-), α-inhibin (-).
Due to her medical complications and older age, the patient refused adjuvant chemotherapy. One week later, she was discharged and returned home. The tumor relapsed 2 months later, and she died within 1 year of the first operation.
Case 5
A 54-year-old woman was admitted with fever and dull abdominal pain. She had been postmenopausal for 18 months and had no abnormal vaginal bleeding. A physical examination confirmed the presence of a cystic mass 10 cm in diameter on the right posterior of the uterus with clear boundaries, poor activity and no tenderness. The serum levels of CA125 and CA199 were 88.8 U/ml and 40.4 U/ml, respectively. Vaginal ultrasonography revealed a cystic mass full of fine spots that was located in the right ovarian area; the mass had clear boundaries, irregular low echo protrusions on the wall and no blood flow signals on CDFI. A solid mass with clear boundaries and a diameter of 7.3*5.9*6.5 cm was located below the former site of the cystic mass, and abundant arteriovenous blood flow signals could be seen inside and around the mass.
Her medical history included 30 years of dysmenorrhea and 18 years of endometriosis and adenomyosis.
We performed an exploratory laparotomy, and during the operation, a small amount of bloody ascites was observed. We observed that the size of the uterus was approximately equal to that at 6 weeks of pregnancy, and no obvious abnormality was seen in the left ovarian area. A cystic-solid mass with an approximate diameter of 10*15 cm was seen in the right ovarian area. After 600 ml of thin, chocolate-like intracapsular fluid was removed, we observed that the mass was densely adherent to the surrounding pelvic peritoneum and that the rectal pouch was completely closed. The source of the mass was found to be the left accessory ovary, and thus, the left adnexa was completely removed. After the mass was cut open, the contents appeared pale white, crisp and vortex-free and contained a chocolate-like liquid. The intraoperative rapid pathology report described a right ovarian spindle cell tumor with a large necrotic area, which was considered a sex cord-stromal tumor, but a malignant tumor was not excluded. After communicating with the patient's family, they wanted to wait for the final pathology results before proceeding with additional surgery. Total hysterectomy and bilateral adnexectomy were then performed.
However, 10 days later, the final pathologic diagnosis was fibrosarcoma of the right ovary, with extensive necrosis. The mitotic counts were evaluated >40 times/10 HPFs. Immunohistochemical analysis showed the following: CD31 (+), Ki-67 (+70%), SMA (+), AE1/AE3 (-), CD34 (-), CD117 (-), ER (-), PR (-), Desmin (-). No tumor cells were found during peritoneal washing.
After surgery, the serum levels of CA125 and CA19-9 were 109.0 U/ml and 5.6 U/ml, respectively. The patient was given 1 cycle of systemic chemotherapy consisting of Cisplatin + Epirubicin + Ifosfamide (PEI). After 1 course of chemotherapy, the serum levels of CA125 and CA19-9 were 24.9 U/ml and 4.3 U/ml, respectively.
We then performed a second laparoscopic exploration, adhesion decompression and partial removal of the pelvic mass 5 weeks after the first surgery and 3 weeks after chemotherapy. During the surgery, a hard, solid mass approximately 5 cm in diameter was palpable at the top of the stump and was located below the adhesion of the bladder and rectum. The surface of the mass was not visible. No obvious tumor lesions were seen on the pelvic peritoneal surfaces and visceral surfaces. Due to the tight adhesion, separation was extremely difficult, and the mass did not have a capsule or boundaries. Only 2/3 of the tumor was removed, and after the mass was opened, solid, brittle gray material was present.
The pathology report still described a fibrosarcoma. One week after the second surgery, the serum level of CA125 was 35.1 U/ml, and she received 1 cycle of chemotherapy consisting of Taxol + Carboplatin (TC). After 1 course of TC, the serum level of CA125 was 32.9 U/ml, after which the chemotherapy ended, and the patient died within 1 year after surgery.