Objective: To evaluate the incidence, treatment, and survival outcomes of Swyer syndrome with gonadal non-dysgerminoma malignant germ cell tumor (MGCT-NDG). Methods: A retrospective study was performed on Swyer syndrome patients with MGCT-NDG between January 2011 and December 2022 in Peking Union Medical College Hospital to investigate their characteristics and outcomes. Results: A total of 15 patients (4.9%, 15/307) with Swyer syndrome were identified in 307 MGCT-NDG patients. The average age at diagnosis of MGCT-NDG and Swyer syndrome were (16.8±6.7) and (16.7±6.6) years, respectively. Six cases were preoperatively diagnosed as Swyer syndrome, of which 4 cases received bilateral gonadectomy with or without hysterectomy, while the other 2 cases underwent removal of gonadal tumor and unilateral gonadectomy with hysterectomy, respectively. Of the 9 patients postoperatively diagnosed as Swyer syndrome, unilateral gonadectomy, removal of gonadal tumor, and unilateral gonadectomy with hysterectomy were performed in 6 patients, 2 patients, and 1 patient, respectively. Mixed malignant germ cell tumor (MGCT;10 cases), yolk sac tumor (4 cases), and immature teratoma (1 case) were the pathological subtypes, in the descending order. There were International Federation of Gynecology and Obstetrics (FIGO) stage Ⅰ in 6 cases, stage Ⅱ in 3 cases, stage Ⅲ in 5 cases, and stage Ⅳ in 1 case, respectively. Eleven patients received reoperation for residual gonadectomy after a average delay of (7.9±6.2) months, including 8 MGCT-NDG patients and 1 gonadoblastoma patient, no tumor involved was seen in the remaining gonads in the other 2 cases. Ten patients experienced at least one recurrence, with a median event free survival of 9 months (5, 30 months), of which 2 patients received surgery only at the time of initial treatment. All patients with recurrence received surgery and combined with postoperative chemotherapy. After a median follow-up of 25 months (15, 42 months), 10 patients were disease-free, 3 patients died of the tumor, 1 died of side effects of leukemia chemotherapy, and 1 survived with disease. Conclusion: The incidence rate of Swyer syndrome in patients with MGCT-NDG is about 4.9%; timely diagnosis and bilateral gonadectomy should be emphasized to reduce the risk of reoperation and second carcinogenesis in this population.
Female ; Humans ; Retrospective Studies ; Gonadal Dysgenesis, 46,XY/surgery* ; Gonadoblastoma/surgery* ; Neoplasms, Germ Cell and Embryonal/surgery* ; Ovarian Neoplasms/pathology*

Female ; Humans ; Teratoma/pathology* ; Ovarian Neoplasms/pathology* ; Carcinoma, Small Cell/surgery* ; Carcinoma, Squamous Cell/pathology* ; Carcinoma, Neuroendocrine/surgery*

Female ; Humans ; Melanoma/surgery* ; Neoplasms, Multiple Primary/pathology* ; Ovarian Neoplasms/pathology* ; Skin Neoplasms ; Teratoma/surgery*

Child ; Gonadoblastoma/surgery* ; Humans ; Male ; Ovarian Neoplasms/pathology* ; Testicular Neoplasms/pathology*

Carcinoma, Endometrioid/pathology* ; Carcinoma, Neuroendocrine/surgery* ; Carcinoma, Small Cell/surgery* ; Endometrial Neoplasms/pathology* ; Female ; Humans ; Ovarian Neoplasms/surgery* ; Uterus/pathology*

OBJECTIVE: Distal pancreatectomy with splenectomy may be required for optimal cytoreductive surgery in patients with epithelial ovarian cancer (EOC) metastasized to splenic hilum. This study evaluates the morbidity and treatment outcomes of the uncommon procedure in the management of advanced or recurrent EOC. METHODS: This study recruited 18 patients who underwent distal pancreatectomy with splenectomy during cytoreductive surgery of EOC. Their clinicopathological characteristics and follow-up data were retrospectively analyzed. RESULTS: All tumors were confirmed as high-grade serous carcinomas. The median diameter of metastatic tumors located in splenic hilum was 3.5 cm (range, 1 to 10 cm). Optimal cytoreduction was achieved in all patients. Eight patients (44.4%) suffered from postoperative complications. The morbidity associated with distal pancreatectomy and splenectomy included pancreatic leakage (22.2%), encapsulated effusion in the left upper quadrant (11.1%), intra-abdominal infection (11.1%), pleural effusion with or without pulmonary atelectasis (11.1%), intestinal obstruction (5.6%), pneumonia (5.6%), postoperative hemorrhage (5.6%), and pancreatic pseudocyst (5.6%). There was no perioperative mortality. The majority of complications were treated successfully with conservative management. During the median follow-up duration of 25 months, nine patients experienced recurrence, and three patients died of the disease. The 2-year progression-free survival and overall survival were 40.2% and 84.8%, respectively. CONCLUSION: The inclusion of distal pancreatectomy with splenectomy as part of cytoreduction for the management of ovarian cancer was associated with high morbidity; however, the majority of complications could be managed with conservative therapy.
Adult ; Aged ; *Cytoreduction Surgical Procedures ; Disease-Free Survival ; Female ; Humans ; Middle Aged ; Neoplasms, Glandular and Epithelial/mortality/pathology/*surgery ; Ovarian Neoplasms/mortality/pathology/*surgery ; *Pancreatectomy/adverse effects ; Postoperative Complications/epidemiology/therapy ; *Splenectomy/adverse effects ; Splenic Neoplasms/pathology/*secondary/*surgery

No abstract available.
Asia ; China ; Female ; *Genital Neoplasms, Female ; *Gynecologic Surgical Procedures ; Humans ; Laparoscopy ; Laparotomy ; *Medical Oncology ; Ovarian Neoplasms/pathology/*surgery ; Republic of Korea

OBJECTIVE: Fertility-sparing surgery (FSS) is becoming an important technique in the surgical management of young women with early-stage epithelial ovarian cancer (EOC). We retrospectively evaluated the outcome of laparoscopic FSS in presumed clinically early-stage EOC. METHODS: We retrospectively searched databases of patients who received laparoscopic FSS for EOC between January 1999 and December 2012 at Samsung Medical Center. Women aged < or =40 years were included. The perioperative, oncological, and obstetric outcomes of these patients were evaluated. RESULTS: A total of 18 patients was evaluated. The median age of the patients was 33.5 years (range, 14 to 40 years). The number of patients with clinically stage IA and IC was 6 (33.3%) and 12 (66.7%), respectively. There were 7 (38.9%), 5 (27.8%), 3 (16.7%), and 3 patients (16.7%) with mucinous, endometrioid, clear cell, and serous tumor types, respectively. Complete surgical staging to preserve the uterus and one ovary with adnexa was performed in 4 patients (22.2%). Two out of them were upstaged to The International Federation of Gynecology and Obstetrics stage IIIA1. During the median follow-up of 47.3 months (range, 11.5 to 195.3 months), there were no perioperative or long term surgical complications. Four women (22.2%) conceived after their respective ovarian cancer treatments. Three (16.7%) of them completed full-term delivery and one is expecting a baby. One patient had disease recurrence. No patient died of the disease. CONCLUSION: FSS in young patients with presumed clinically early-stage EOC is a challenging and cautious procedure. Further studies are urgent to determine the safety and feasibility of laparoscopic FSS in young patients with presumed clinically early-stage EOC.
Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Female ; *Fertility Preservation ; Humans ; Laparoscopy ; Live Birth ; Neoplasm Recurrence, Local/blood/diagnosis/*therapy ; Neoplasm Staging ; Neoplasms, Glandular and Epithelial/drug therapy/*pathology/*surgery ; *Organ Sparing Treatments ; Ovarian Neoplasms/drug therapy/*pathology/*surgery ; Pregnancy ; Pregnancy Rate ; Retrospective Studies ; Term Birth ; Treatment Outcome ; Young Adult

In 2014, 9 topics were selected as major advances in clinical research for gynecologic oncology: 2 each in cervical and corpus cancer, 4 in ovarian cancer, and 1 in breast cancer. For cervical cancer, several therapeutic agents showed viable antitumor clinical response in recurrent and metastatic disease: bevacizumab, cediranib, and immunotherapies including human papillomavirus (HPV)-tumor infiltrating lymphocytes and Z-100. The HPV test received FDA approval as the primary screening tool of cervical cancer in women aged 25 and older, based on the results of the ATHENA trial, which suggested that the HPV test was a more sensitive and efficient strategy for cervical cancer screening than methods based solely on cytology. For corpus cancers, results of a phase III Gynecologic Oncology Group (GOG) 249 study of early-stage endometrial cancer with high-intermediate risk factors are followed by the controversial topic of uterine power morcellation in minimally invasive gynecologic surgery. Promising results of phase II studies regarding the effectiveness of olaparib in various ovarian cancer settings are summarized. After a brief review of results from a phase III study on pazopanib maintenance therapy in advanced ovarian cancer, 2 outstanding 2014 ASCO presentations cover the topic of using molecular subtypes in predicting response to bevacizumab. A review of the use of opportunistic bilateral salpingectomy as an ovarian cancer preventive strategy in the general population is presented. Two remarkable studies that discussed the effectiveness of adjuvant ovarian suppression in premenopausal early breast cancer have been selected as the last topics covered in this review.
Biomedical Research/*trends ; Endometrial Neoplasms/drug therapy/pathology/surgery ; Female ; Genital Neoplasms, Female/diagnosis/*therapy ; Humans ; Ovarian Neoplasms/drug therapy/pathology/surgery ; Uterine Cervical Neoplasms/drug therapy/pathology/surgery

OBJECTIVE: The aim of this paper was to describe the operative details for en bloc removal of the adnexal tumor, uterus, pelvic peritoneum, and rectosigmoid colon with colorectal anastomosis in advanced epithelial ovarian cancer patients with widespread pelvic involvement. METHODS: The patient presented with good performance status and huge pelvic tumor extensively infiltrating into adjacent pelvic organs and obliterating the cul-de-sac. The patient underwent en bloc pelvic resection as primary cytoreductive surgery. En bloc pelvic resection procedure is initiated by carrying a circumscribing peritoneal incision to include all pan-pelvic disease within this incision. After retroperitoneal pelvic dissection, the round ligaments and infundibulopelvic ligaments are divided. The ureters are dissected and mobilized from the peritoneum. After dissecting off the anterior pelvic peritoneum overlying the bladder with its tumor nodules, the bladder is mobilized caudally and the vesicovaginal space is developed. The uterine vessels are divided at the level of the ureters, and the paracervical tissues (or parametria) are divided. The proximal sigmoid colon is divided above the most proximal extent of gross tumor using a ligating and dividing stapling device. The sigmoid mesentery is ligated and divided including the superior rectal vessels. The pararectal and retrorectal spaces are further developed and dissected down to the level of the pelvic floor. The posterior dissection is progressed and moves to the right and then to the left of the rectum. The rectal pillars including the middle rectal vessels are ligated and divided. Hysterectomy is completed in a retrograde fashion. The distal rectum is divided using a linear stapler. The specimen is removed en bloc with the uterus, adnexa, pelvic peritoneum, rectosigmoid colon, and tumor masses leaving a macroscopically tumor-free pelvis. Colorectal anastomosis was completed using stapling device. RESULTS: En bloc pelvic resection was performed by total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic peritonectomy, and rectosigmoid colectomy with colorectal anastomosis using a stapling device. Complete clearance of pelvic disease leaving no gross residual disease was possible using en bloc pelvic resection. CONCLUSION: En bloc pelvic resection is effective for achieving maximal cytoreduction with the elimination of the pelvic disease in advanced primary ovarian cancer patients with extensive pelvic organ involvement.
Anastomosis, Surgical ; Colon, Sigmoid/pathology/surgery ; Disease Progression ; Female ; Humans ; Hysterectomy/*methods ; Neoplasm Invasiveness ; Neoplasm, Residual ; Neoplasms, Glandular and Epithelial/*pathology/*surgery ; Ovarian Neoplasms/*pathology/*surgery ; Ovary/pathology/surgery ; Pelvic Exenteration/*methods ; Pelvis/pathology/surgery ; Rectum/pathology/surgery ; Salpingectomy ; Surgical Stapling