Case 1
A 57-year-old woman was admitted to our clinic with abdominal mass for 2 weeks and without abdominal pain or distension. She had been post-menopausal 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 without pressing pain. Vaginal ultrasonography demonstrated: a 9.6*8.2*5.2 cm in diameter, thickly capsulated, liquid and irregular mass in the left ovarian region; there were several septa inside the cyst, with medium strong echo bulges on the septa and largest size was 1.8*1.1 cm; Slight blood flow signal can be seen on the septum; liquid area with the maximum depth of 4.6 cm can be seen in the pelvic cavity Before surgery, serum tumor markers were within normal limits.
During the laparotomy, an 8*10 cm in diameter, irregular, multilocular, cystic mass was found in the right ovarian region, and no metastatic lesions were found. 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). Immunohistochemical results were Vimentin (+), SMA (+), PR (±), ER (-), CD10 (-), Caldeson (-), Melan (-), α-inhibin (-).
The tumor was labelled as Federation International Gynecology Obstetrics(FIGO) stage I C. The patient was given 2 cycles of systemic chemotherapy using Cisplatin + Vincristine + Bleomycin (PVB) at 3-weekly intervals. After the chemotherapy, the patient was under regular follow-up and no recurrence was seen in 15 years after the operation.
Case 2
A 41-year-old female was admitted with abdominal mass for 2 months. There were no additional complaints such as abdominal pain or distension. She had normal menstruation and no abnormal vaginal bleeding. Upon abdominopelvic examination, a 5*6 cm in diameter, firm, unmovable mass without tenderness was detected posterior left of uterus. Vaginal ultrasonography revealed a heterogeneous, left ovarian mass with clear boundary and a diameter of 6.1*5.5*4.6 cm. There were no signs of metastasis, lymph node enlargement or ascites. No obvious blood flow signal was detected. Serum tumor markers were within normal limits except slightly elevated carcinoma antigen 125 (40.4 U/mL). By magnetic resonance imaging (MRI), a 48*62*60 mm in diameter, heterogeneous mass closely related to uterus was detected posterior left of uterus, and the possibility of subserosal fibroids was considered, with exception of adnexal tumors.
When performing a laparoscopic exploration, we found a 7*6 cm in diameter, solid mass with clear boundary originated from the left ovary without any peritoneal dissemination. The right ovary appeared full and the uterus normal. Ovarian cystectomy, sampling of right ovary and intraperitoneal washing were performed during surgery. After cutting open the mass, solid granular materials were present. Intraoperative pathological diagnosis was left ovarian follicular fibroma. However, a week later the final pathologic diagnosis turned to be fibrosarcoma of the left ovary and the mitotic counts were evaluated > 10 times 10 high-power fields (HPFs). Immunohistochemical study showed AE1/AE3 (-), Calretinin (-), Ki-67 (index 5%), p53 (-), α-inhibin (-). After surgery, the serum level of CA125 was 27.1 U/ml.
This case was diagnosed as FIGO stage I A. The patient refused to receive chemotherapy and remained disease-free with normal ovarian functions in 7 years of follow-up.
Case 3
A 76-year-old woman was admitted with abdominal pain and fever. She had been post-menopausal for 28 years without any vaginal bleeding after menopause. Fourteen years ago, the patient received an exploratory laparotomy due to acute abdomen, in the other hospital. During the operation, 2800 ml intraabdominal blood clots and non-clotting blood were sucked out, and a 3*3*2 cm in diameter, cauliflower-like tumor was seen around the left tubal umbrella with active bleeding. Complete hysterectomy plus bilateral salpingo-oophorectomy and omentectomy was performed. Postoperative pathological report showed: diffuse granular follicular cell tumor of 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 with Cisplatin + Cyclophosphamide (PC). Then the patient never came back for following up.
Four years later (10 years ago), the patient received the second exploratory laparotomy in another hospital due to abdominal pain and pelvic mass. During the operation, a 3 cm in diameter, solid-cystic mass was observed between the sigmoid colon and the left bladder bottom. In addition, a 1 cm in diameter nodule at the left bladder bottom and a 0.5 cm in diameter nodule on the surface of sigmoid colon was seen. The tumor lesions were completely removed. Postoperative pathological result reported granulosa cell tumor. After surgery, she received 1 cycles of chemotherapy with Taxol + Carboplatin. But after that the patient lost follow-up.
This time she was admitted with abdominal pain and fever lasting for 1 day. Ultrasound showed a 10*8.6 cm in diameter, irregular, cystic mass with unclear boundary in the pelvic cavity; the wall is uneven in thickness and the size of cystic part is about 7.21*6.34 cm. MRI showed a double capsular structure in diameter of 8*9*7 cm was on the left side of the pelvic cavity. The mass was with thick wall and the edge was relatively clear, closely related to the right bowel. The cephalic size of the lesion was about 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 level of CA125 was 15.5 U/ml, Estradiol (E2) 25.8 pg/mL, Follicle Stimulating Hormone (FSH) 52.8mIU/mL。
We performed exploratory laparotomy and secondary cytoreductive surgery. During the operation, a 10 cm in diameter, solid-cystic, multilocular mass was observed between the sigmoid colon and the left lateral wall of the bladder. After sucking out 600 ml of yellow intracapsular fluid, it was completely removed. Then we performed adhesion decomposition, repair of sigmoid colon, and partial ileotomy anastomosis. The tumor was totally removed. Pathological results were ovarian fibrosarcoma. Immunohistochemical study showed: Melan-A (+), Vimentin (+), AE1/AE3 (+/-), CD99 (-), Calretinin (-), α-inhibin (-), Ki-67 (index10%).
After surgery, she suffered from incomplete intestinal obstruction and received conservative treatment. The patient’s condition gradually improved and fully recovered 2 weeks later. As the patient was elderly, she did not receive adjuvant chemotherapy. There were no signs of recurrence or increase of serum E2 level in more than 6 years after the operation.
Case 4
A 76-year-old woman was admitted with abdominal pain and abdominal mass. She had been post-menopausal for 28 years and had no abnormal vaginal bleeding. Ultrasound revealed a heterogeneous, irregular, hypoechoic mass without a clear boundary was at the upper right of the uterus, with a range of 11.4*13.5*8.6 cm. Punctiform blood flow signals could be seen in Color Doppler Flow Imaging (CDFI). MRI showed a large solid-cystic mass in the pelvic cavity, considering the possibility of malignant lesions from accessories, with the exception of mesenchymal tumors. Vaginal and abdominal examinations confirmed the presence of a hypertonic, cystic mass with a diameter of 12–15 cm and without pressing pain. Before surgery, the serum level of CA125 was 593.3 U/ml.
Her medical history included sleep apnea syndrome for more than 10 years and needed supplementary positive pressure ventilation at night
After multi-department consultation, surgical contraindications are eliminated and we performed an exploratory laparotomy. During the operation, bloody ascites about 100–200 ml were observed. A cystic-solid mass with a diameter of about 20 cm were seen below the incision. Cauliflower-like tumors could be seen on the surface of the mass with rich blood supply. The mass was widely adherent to the surrounding small intestine and colon, with cauliflower-like tumor lesions on the surface of bowels. The source of the mass and metastatic tumors was found to be the left accessory, so the left adnexa was removed. Pathological report revealed as malignant tumor with spindle cell, fibrosarcoma. The mitotic counts were evaluated as > 10 times/ 10 HPFs. Immunohistochemical study showed CA125 (-), CD10 (partial+), Desmin (-), Ki-67 (index40%), SMA (+), S-100 (-), Vimentin (+), p53 (-), α-inhibin (-).
Because of her medical complications and elderly age, the patient refused to receive adjuvant chemotherapy. One week later she discharged and went back home. The tumor relapsed 2 months later and she died within 1 year after the first operation.
Case 5
A 54-year-old woman was admitted with fever and abdominal dull pain. She had been post-menopausal for 18 months and had no abnormal vaginal bleeding. Physical examination confirmed the presence of a 10 cm in diameter, cystic mass on the right rear of the uterus, with clear boundaries, poor activity and no tenderness. The serum level of CA125 was 88.8 U/ml and CA199 was 40.4 U/ml. Vaginal ultrasonography showed: a cystic mass full of fine spots was in the right ovarian area, with clear boundaries, irregular low echo protrusions on the wall and no blood flow signals in CDFI; a solid mass with clear boundaries and a diameter of 7.3*5.9*6.5 cm was below the former cystic mass, and abundant arteriovenous blood flow signals can 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. During the operation, small amount of bloody ascites was observed. We saw the size of uterine was about 6 weeks of pregnancy and no obvious abnormity was seen in the left ovarian area. A cystic-solid mass with a diameter of about 10*15 cm were seen in the right ovarian area. After sucking out 600 ml of thin, chocolate-like intracapsular fluid, we observed the mass was densely adherent to the surrounding pelvic peritoneum, with rectal pouch totally closed. The source of the mass was found to be the left accessory, so the left adnexa was completely removed. After cutting open the mass, the contents seemed as pale white, crisp and vortex-free, with chocolate-like liquid inside. Intraoperative rapid pathological report showed: right ovarian spindle cell tumor with large necrosis, considered as a sexostromal tumor, not excluding malignant tumor. After communicating with the patient's family, they asked to wait for the final pathological results before proceeding further surgery. Therefore, total hysterectomy and bilateral adnexectomy were performed.
However, 10 days later the final pathologic diagnosis turned to be fibrosarcoma of the right ovary, with extensive necrosis. The mitotic counts were evaluated > 40 times/ 10 HPFs. Immunohistochemical study showed: CD31 (+), Ki-67 (+ 70%), SMA (+), AE1/AE3 (-), CD34 (-), CD117 (-), ER (-), PR (-), Desmin (-). No tumor cell was found in the peritoneal washes.
After surgery, the serum level of CA125 was 109.0 U/ml and CA19-9 5.6 U/ml. The patient was given 1 cycles of systemic chemotherapy with Cisplatin + Epirubicin + Ifosfamide (PEI). After the 1 course of chemotherapy, the serum level of CA125 was 24.9 U/ml and CA19-9 4.3 U/ml.
Then we performed a second laparoscopic exploration, adhesion decomposition and partial removal of pelvic mass, at 5 weeks after the first surgery and 3 weeks after the chemotherapy. During operation, a hard solid mass of about 5 cm was palpable at the top of the stump, located below the adhesion of bladder and rectum. The surface of the mass was not visible. No obvious tumor lesions were seen at the pelvic peritoneal surfaces and visceral surfaces. Because of the tight adhesion, the separation was extremely difficult, and the mass was without capsule or boundaries. In the end, only 2/3 of the tumor was removed. Cutting open the mass, solid brittle gray materials were present.
Pathological report still revealed as fibrosarcoma. One week after the second surgery, the serum level of CA125 was 35.1 U/ml and she received 1 cycles of chemotherapy with Taxol + Carboplatin (TC). After the 1 course of TC, the serum level of CA125 was 32.9 U/ml. Then the patient stopped chemotherapy since and died within 1 year after surgery.