Ovarian fibrosarcoma: A single-institution experience and a review of the literature.

Ovarian fibrosarcoma is an extremely rare and malignant sex cord-stromal tumor. Due to its low incidence and poor prognosis, until now, very few cases have been reported, and most of the reported cases have been sporadic. Therefore, the treatments and prognostic factors of ovarian fibrosarcoma are still debatable. Here, we report 5 cases of ovarian fibrosarcoma that presented at Peking Union Medical College Hospital over the past 20 years. The 5 patients were 41, 51, 54, 76, and 76 years of age when initial symptoms of pelvic mass or pain appeared. On ultrasound, this disease usually manifests as unilateral pelvic masses, within which uneven echo enhancement and some blood flow signals are observed. No significant increase was observed in the levels of preoperative tumor markers, such as serum CA125 and sex hormones. The final diagnosis depends on postoperative histopathological results since these tumors are easily misdiagnosed when intraoperative frozen sections are used for examination. Pathologic examinations showed that the tumor cells were spindle-shaped with moderate to severe atypia and high mitotic counts. The immunohistochemistry profile is not specific, but the positive rate of Ki-67 was consistent with the degree of malignancy and the prognosis of patients with this tumor. In addition, the tumor may also be positive for Vimentin, α-inhibin, SMA, estrogen receptor and progesterone receptor. Significant differences were observed in the surgical methods used, and no unified chemotherapy regimen has been established. The overall survival was > 15, >7, > 6, <1, and < 1 year for each patient. After reviewing the literature, evidence-based large-scale case studies were lacking. For treatments, complete cytoreductive surgery plus regimens typically used against malignant sex cord-stromal tumors, as described in the NCCN guidelines, are recommended. Due to its low incidence, both multicenter clinical studies and molecular studies are required to provide gynecologists with a better understanding and guidance for future management of patients with ovarian fibrosarcoma.

Surgical Treatment Strategies of Intravenous Leiomyomatosis withRight Cardiac Cavities Extension.

Objective To investigate the diagnosis and surgical treatment strategies of intravenous leiomyomatosis(IVL)extending through inferior vena cava into the right cardiac cavities. Methods Thirty patients of IVL extending through inferior vena cava into the right cardiac cavities were treated in Peking Union Medical College Hospital from November 2002 to January 2015.The following variables were studied: age,cardiopulmonary bypass time,deep hypothermic circulatory arrest time,origins of IVL,blood loss,duration of post-operative hospital stay,hospitalization expenses,edema of lower extremity,blood transfusion,postoperative complication,residual IVL,and re-grow or recurrence. Results Thirteen of 30 patients reported double lower limb edema. The cardiopulmonary bypass was applied in 27 cases,and the average duration of cardiopulmonary bypass was(106.9±53.7)min. Then,21 patients were treated with the deep hypothermic circulatory arrest,and the mean time was(28.2±11.6) min. The tumors originated from the genital veins in 9 cases,the iliac vein in 13 cases,and both veins in 8 cases. The average intra-operative blood loss volume was (2060.5±2012.3)ml,and 21 patients received blood transfusion. The average hospitalization time was(18.9±8.3)days and the average hospitalization expenses was (80 840.4±28 264.2)RMB yuan. While 14 patients had postoperative complications,there was no serious postoperative complication or death.All patients have shown a favorable outcome.Conclusions Tumor embolus extending through inferior vena cava into the right cardiac cavities should be suspected in patients with multiple hysteromyoma. Successful therapy for IVL with right cardiac cavities extension is dependent on reasonable surgical treatment strategies. Surgical removal of the ovaries is vital to avoid IVL re-grow or recurrence.

[Pregnant rate and pregnancy-relating factors of patients with early endometrial carcinoma and severe atypical hyperplasia of endometrium after fertility-preserving treatment by progestin].

OBJECTIVE: To summarize the pregnant rate of patients with early endometrial carcinoma and severe atypical hyperplasia after fertility-preserving treatment and analyze their pregnancy-relating factors. METHODS: Endometrial curettage was used to evaluate the therapy response of endometrium after every 3 months of administration of high-dose progestin as fertility-sparing treatment for 51 patients with stage I endometrial carcinoma or severe endometrial atypical hyperplasia from Jun. 1996 to Jan. 2010. Individualized maintained treatment was given to patients after achieving complete remission of the endometrium. Pregnant results and pregnancy-relating factors were analyzed retrospectively. RESULTS: The median age of all the 51 patients was 29 years old. Forty-five (88%, 45/51) achieved complete response. Of the 34 cases who desired to conceive after complete response, 16 of them had 22 pregnancies, the pregnant rate was 47% (16/34); and 12 women obtained healthy live birth baby, the fertility rate was 35% (12/34). The pregnant rate of patients at age >35 or ≤ 35 was 0/2 and 50% (16/32) respectively (P > 0.05). The pregnant rate of patients with or without infertility was 40% (8/20) and 8/14, with endometrial cancer or severe atypical hyperplasia was 40% (10/25) and 6/9, respectively (all P > 0.05). The pregnant rate of patients who received in vitro fertilization-embryo transfer, ovulation promotion, or no treatment was 7/7, 6/16 and 3/11 respectively (P < 0.01). CONCLUSIONS: Fertility-preserving treatment for early endometrial cancer and severe atypical hyperplasia with high-dose progestin could achieve higher response rate. Assisted reproductive technologies could significantly increase the chance of conception.

[Management of invasive cervical cancer in pregnancy: clinical analysis of 13 cases].

OBJECTIVE: To analyze the clinical characteristics and assess the outcome of treatment for cervical cancer during pregnancy. METHODS: A cohort of 13 patients with cervical cancer diagnosed during pregnancy from January 2001 to September 2011 in Peking Union Medical College Hospital (PUMCH) was retrospectively studied. Clinical information, gestational age at diagnosis, treatment options and maternal and child outcomes were collected and analyzed. RESULTS: Thirteen patients out of 2030 cases of invasive cervical cancer were diagnosed during pregnancy with an incidence of 0.64% (13/2030). The Mean gestational age at diagnosis of 13 patients is 21(+6) weeks. Two cases were diagnosed during the first trimester, 8 cases at second trimester and 3 cases at third trimester respectively. Vaginal bleeding during the pregnancy was main clinical manifestation presented in 8 patients and all thirteen cases were diagnosed by biopsy with pathological types of squamous cell carcinoma in 10 cases. The International Federation of Gynecology and Obstetrics (FIGO) stage was I in eleven cases and stage II in two cases. Six patients of them received treatment promptly after diagnosis. The other 7 patients had delayed treatment with mean diagnosis-treatment interval time of 65 days due to fertility reasons, who ended pregnancy by cesarean section at mean gestational age of 34(+6) weeks, two of them received chemotherapy with cisplatin + fluorouracil (PF) or cisplatin respectively before the end of the pregnancy, while the one with PF chemotherapy experienced neonatal death. The rest 6 neonatal outcomes were good. As follow-up of 13 cases: 11 cases in stage I received surgical treatment, and two of which had recurrence respectively, 15 months and 7 months post surgery, and one case had died. One case of Stage II patients died and one had recurrence after 53 months after radiotherapy. The recurrence rate in 13 cases was 3/13 and the mortality rate was 2/13. CONCLUSIONS: Most cases of cervical cancer diagnosed during pregnancy were in early FIGO stage. For those patients diagnosed in late pregnancy with strong fertility demand, considering delayed treatment according to FIGO stage of the disease and fetus maturity is appropriate. Chemotherapy during pregnancy may cause neonatal complications.

[Clinicopathologic features and prognostic factors of endometrial stromal sarcoma: a study of 55 cases].

OBJECTIVE: To investigate the clinicopathologic features and the prognostic factors of endometrial stromal sarcoma (ESS). METHODS: 55 cases of endometrial stromal sarcoma were reviewed and categorized into 3 pathologic types based on the related literatures, i.e., low grade endometrial stromal sarcoma (LGESS), undifferentiated endometrial sarcoma with nuclear uniformity (UES-U) and undifferentiated endometrial sarcoma with nuclear pleomorphism (UES-P). Meanwhile, the pathologic features were reviewed, including fibroid, myoid, mucoid, and epithelioid differentiation and mitotic index. Clinical and follow-up data were collected. RESULTS: In endometrial stromal sarcoma, two or three pathologic types co-existed in one case, including 12.8% (5/39) of LGESS, 5/9 of UES-U, and 5/7 of UES-P. Mitotic index varied in different regions of one tumor from rare to high. Multi-differentiation was also commonly seen in ESS. The numbers of cases in LGESS, UES-U and UES-P were 39, 9 and 7, with recurrence rate of 51.6% (16/31), 5/6 and 2/3, respectively. There was no death case in LGESS, and 2 cases were died in UES-U and UES-P, respectively. In the 2 death cases of UES-U, both had focus of UES-P. There was a significant difference in the recurrence rate between cases with different mitotic index (≥ 10/10 HPF and < 10/10 HPF, P = 0.009), especially in LGESS group. All death cases had high mitotic index (> 30/10 HPF). CONCLUSIONS: It is a common phenomenon in ESS that two or three pathologic types may exist in one case, especially in UES-U and UES-P. And multi-differentiation is also commonly seen in ESS. So adequate pathologic sampling is important for pathologists to make a correct diagnosis of ESS in daily work. The recurrence rates are significantly higher in cases with high mitotic index, especially in LGESS. In addition, the presence of UES-P and high mitotic index may increase the risk of death in the patients.

[Comparative study on the effects of LEEP and laser CO(2) vaporization in cervical intraepithelial neoplasia II].

OBJECTIVE: to compare the effect and complications of loop electro-surgical excision procedure (LEEP) and laser CO(2) vaporization in the treatment of cervical intraepithelial neoplasia II. METHODS: a total of 338 CINII women were recruited into this multi-center comparative study. The diagnosis was confirmed by histopathological examination for cervical epithelial cell abnormalities. And colposcopic examination was submitted to LEEP (n = 195) or laser CO(2) vaporization (n = 143) respectively. A post-treatment follow-up of 3, 6 and 12 months was carried out to compare the effect of two methods. RESULTS: among 195 women undergoing LEEP, the frequency of cure, persistent and recurrent CIN was 89.2% (n = 174), 4.1% (n = 8) and 3.6% (n = 7) respectively. And among 143 women receiving laser CO(2) vaporization, the frequency of cure, persistent and recurrent CIN was 86.7% (n = 124), 4.9% (n = 7) and 0.70% (n = 1) respectively. There was no statistical difference in cure rates, persistence or recurrence of CIN (P > 0.05). The recovery time, the operative frequency and intra-operative blood loss were significantly different in two groups. CONCLUSION: both LEEP and CO(2) vaporization are both effective and reliable for the treatment of cervical intraepithelial neoplasia II. However, pathological specimens may be harvested during LEEP. It is of vital importance to conduct preoperative colposcopic assessment and standard postoperative follow-ups.

[Ovarian growing teratoma syndrome clinical study of 22 cases].

OBJECTIVE: To describe the essential points for the correct diagnosis and best treatment for ovarian growing teratoma syndrome (GTS) developed after surgery and chemotherapy for ovarian immature teratoma. METHODS: Retrospective review of the clinical characteristics and long term follow up results of 22 cases of ovarian GTS to illustrate the unique biological behavior of the tumor and good prognosis of the disease. RESULTS: Pathological examination of the tumors revealed completely benign mature teratoma with G0 grading in 20 cases. The other 2 cases were found to be G0 mature teratoma with concurrent association of malignant somatic cell tumor: carcinoid and primitive neuroectodermal tumor (PNET) respectively. Among the 22 cases of ovarian GTS there are 6 cases with recurrent tumors developed repeatedly, so totally surgical treatments had been performed for 31 times. Time interval in between the development of the ovarian GTS and the initial surgery for their primary immature teratoma is equal to or exceeding one year in 94% (29/31) of the cases. Such a time factor is of high significance for the diagnosis of ovarian GTS. As the benign behavior of the ovarian GTS together with its poor response to chemotherapy have just been recognized in recent years, they were treated as malignant tumors as their original primary immature teratoma before the year of 1987. Postoperative chemotherapy of various kinds was applied. By the year of 1988 postoperative chemotherapy began to be abandoned and since then most of the patients (9/10) had not received postoperative chemotherapy. After long periods of follow up (3.6 -23.0 years) 20 of the 22 patients are found to be living and well. The rest 2 patients died of the concurrent association of malignant somatic cell tumors with carcinoid and PNET in 0.1 and 0.3 years respectively. CONCLUSIONS: Ovarian GTS is a tumor developed after surgical and chemotherapeutic treatment of malignant ovarian immature teratoma. Pathologic grading of the tumors showed retroconversion of the malignancy of the tumor from G3, G2 or G1 to G0 with good prognosis. The tumor usually remained to be quiescent for long periods of time. But there are also some potential of progressive growth, the tumor may grow to huge size and the recurrent tumor may develop repeatedly for several times more than 10 or 20 years later. Surgical removal should be the main treatment either for the primary or the recurrent tumors. Chemotherapy and radiotherapy are not effective and can do nothing but harm to patients. Only correct knowledge about the benign biological behavior of the ovarian GTS and reasonable therapeutic regimen can have the disease ends with good prognosis.

[Repair of abdominal wall defect after resection of abdominal wall endometriosis].

OBJECTIVE: To study the techniques to repair the fascia layer of abdominal wall after the resection of abdominal wall endometriosis (AWE). METHODS: Fifty-five AWE patients aged 28 approximately 38 underwent resection of the lesion. After the resection a defect fascia in abdominal wall larger than 2 cm(2) was seen in 29 patients (large fascia defect group), and in the other 26 patients the fascia defect was less than 2 cm(2) (small fascia defect group). In the large fascia defect group, 11 cases underwent routine closure of the abdominal wall, 2 underwent abdominal wall reconstruction by applying tension suture, 1 case underwent fascia layer/skin tension-relieving suture, 4 cases abdominal wall reconstruction by PDS-II suture, 4 cases underwent fascia patch grafting, and 7 cases underwent abdominal wall plastic repair plus fascia patch grafting, the different techniques being selected according to the size of the defect. Routine abdominal wall closure was performed on all the 26 patients in the small fascia detect group. The features of the lesion and operation, and the outcomes were compared. RESULTS: Primary healing was achieved in all the patients. In comparison with the small fascia defect group, the mean size of the masses measured by pre-operational ultrasonography of the large fascia defect group was significantly bigger [(3.8 +/- 1.4) cm vs. (2.5 +/- 1.1 cm)], the mean size of the masses resected in operation was significantly larger [(5, 5 +/- 2.0) cm vs. (3.7 +/- 1.9) cm, P = 0.004], the operation time was significantly longer [(66 +/- 42) min vs. (35 +/- 24) min, P = 0.002], and the intra-operational blood loss was significantly more [(52 +/- 50) ml vs. (23 +/- 19) ml, P = 0.006]. Relapse occurred in 1 case in the large fascia defect group. CONCLUSION: Ultrasonography helps estimate the extension of AWE before operation. Fascia layer/skin tension-relieving suture can be used in the fascia defect of abdominal wall larger than 2 cm(2). Abdominal wall plastic repair plus fascia patch grafting is capable of repairing larger fascia layer and skin defects of abdominal wall.

[Diagnosis and management of uterine arteriovenous fistula with massive vaginal bleeding].

OBJECTIVE: To study the clinical manifestation, diagnosis, management and prognosis of uterine arteriovenous fistula with massive vaginal bleeding. METHOD: The clinical records of 15 patients who satisfied the diagnostic criteria were retrospectively analyzed. RESULTS: All patients had massive vaginal bleeding, with a history of caesarean section, curettage or gynecologic carcinoma. The disease could be diagnosed by angiography or color Doppler ultrasound. Vaginal bleeding was aggravated by dilation and curettage. Fourteen patients were treated with uterine artery embolization, and no complications occurred postembolization. Of the eleven successful embolizations, all patients returned normal menstrual cycling. Five of these patients became pregnant later. CONCLUSIONS: Uterine arteriovenous fistula is a rare and potentially life threatening disease. Uterine artery embolization is a safe and effective treatment for this disease, and it can preserve normal function of uterus and ovaries.