Oocyte accumulation for fertility preservation in women with benign ovarian tumours with a history of previous surgery, multiple or large cysts.

RESEARCH QUESTION: What are ovarian stimulation cycle outcomes and acceptance rates of an oocyte accumulation programme in young women with benign ovarian tumour (BOT)? DESIGN: Retrospective cohort study conducted at the Academic Assisted Reproductive Technology and Fertility Preservation Centre, Lille University Hospital, between January 2016 and December 2019. The number of metaphase II oocytes per cycle and per patient after accumulation were evaluated. Two groups were identified for the analysis: endometrioma ('endometrioma') and dermoid, mucinous or serous cyst ('other cysts'). RESULTS: A total of 113 fertility-preservation cycles were analysed in 70 women aged 27.9 ± 4.8 years. Almost all women had undergone previous ovarian surgery before fertility preservation (89%). Mean anti-Müllerian hormone levels before ovarian stimulation was 12.5 ± 8.7 pmol/l. A total of 6.4 ± 3.4 oocytes were retrieved, and 4.3 ± 3.4 metaphase II (MII) oocytes were vitrified per cycle. All agreed to the oocyte accumulation programme and all underwent at least one cycle. To date, 36 (51%) patients achieved two or three fertility- preservation cycles. After accumulation, 7.0 ± 5.23 MII oocytes were vitrified per patient. No difference was found in ovarian response and oocyte cohort between the 'endometrioma' and 'other cysts' groups. Questionnaires completed after oocyte retrieval revealed abdominal bloating and pelvic pain in most patients, with no difference according to the type of cyst. No serious adverse events occurred. CONCLUSIONS: Oocyte accumulation should be systematically offered to young women with BOT irrespective of histological type, as it seems to be well-tolerated. Long-term follow-up is needed to assess the efficiency of oocyte accumulation to optimize the chances of subsequent pregnancies.

ACG Clinical Guideline: Diagnosis and Management of Pancreatic Cysts.

Pancreatic cysts are very common with the majority incidentally identified. There are several types of pancreatic cysts; some types can contain cancer or have malignant potential, whereas others are benign. However, even the types of cysts with malignant potential rarely progress to cancer. At the present time, the only viable treatment for pancreatic cysts is surgical excision, which is associated with a high morbidity and occasional mortality. The small risk of malignant transformation, the high risks of surgical treatment, and the lack of high-quality prospective studies have led to contradictory recommendations for their immediate management and for their surveillance. This guideline will provide a practical approach to pancreatic cyst management and recommendations for cyst surveillance for the general gastroenterologist.

A case report and a literature review of primary retroperitoneal mucinous cystadenoma: the importance of imaging in diagnosis and management.

Primary retroperitoneal mucinous cystadenoma (PRMC) is an extremely rare tumor: its histogenesis and its biological behavior remain speculative. Since most retroperitoneal tumors are malignant, a preoperative diagnosis of benignity is essential and it can be reached through imaging examinations, allowing a conservative management approach. We describe the case of a 52-year-old woman with abdominal pain and a palpable mass. Computed tomography of the abdomen revealed a retroperitoneal cystic mass, which was resected successfully through laparoscopy and diagnosed as PRMC. Although there are no pathognomonic, clinical or radiological findings for PRMC, it should be included in the list of differential diagnoses and its imaging criteria of benignity should always be sought, with the aim to exclude malignant tumors.

Intraductal papillary mucinous neoplasms and mucinous cystadenomas: current status and recommendations.

The real prevalence of pancreatic cystic lesions remains unknown. The malignant potential of some of these lesions remains a cause for significant concern. Thus, it is mandatory to develop a strategy to clearly discriminate those cysts with a potential for malignant transformation from those that do not carry any significant risk. Intraductal papillary mucinous neoplasms and mucinous cystadenomas are mucinous cystic neoplasms with a known malignant potential that have gained greater recognition in recent years. However, despite the numerous studies that have been carried out, their differential diagnosis among other cysts subtypes and their therapeutic approach continue to be a challenge for clinicians. This review contains a critical approach of the current recommendations and management strategies regarding intraductal papillary mucinous neoplasms and mucinous cystadenomas, as well as highlighting the limitations exposed in current guidelines.

Management of pancreatic cysts: a review of the current guidelines.

The number of patients diagnosed with cystic pancreatic neoplasms (CPN) has increased significantly during the last decade due to the widespread use of cross-sectional imaging. These CPN consist of a heterogeneous group of neoplastic and non-neoplastic lesions with variable histopathological features, clinical presentation, and outcome. Until now we are not able to reliably identify all CPNs that require additional analysis, surgical resection or surveillance. Hence, physicians and surgeons are confronted with a difficult dilemma as they do not want to miss a diagnosis of pancreatic carcinoma, but this often leads to the risk of over- or misuse of diagnostic examinations with a risk of complications and increased health care costs. Currently, four expert consensus guidelines on cystic lesions of the pancreas are available. Unfortunately, recommendations vary considerably between these guidelines. The purpose of this review therefore was to compare the different guidelines and elaborate upon the topics where these guidelines disagree.

Mucinous tumors of the ovary: current thoughts on diagnosis and management.

Mucinous tumors of the ovary represent a spectrum of neoplastic disorders, including benign mucinous cystadenoma, pseudomyxoma peritonei, mucinous tumors of low malignant potential (borderline), and invasive mucinous ovarian carcinoma. These tumors are related closely to each other and are distinct from other histologic subtypes of epithelial ovarian neoplasms from a clinical, histologic, and molecular standpoint. A continuum appears to be present from benign to borderline to malignant, which is different from other types of epithelial ovarian cancer. Mutational profiles are also distinct, as KRAS mutations are common, but p53 and BRCA mutations are infrequent. These characteristics lead to specific biologic behavior and guide both clinical management and research efforts in patients with mucinous ovarian tumors.

The N-terminally truncated p53 isoform Δ40p53 influences prognosis in mucinous ovarian cancer.

OBJECTIVE: The tumor suppressor p53 generates the N-terminally truncated isoforms Δ40p53 and Δ133p53 that possess the ability to modulate p53 function in vitro. The aim of the present study was to evaluate the clinical relevance of p53 isoforms in the main histological subtypes of ovarian cancer. METHODS: Δ40p53, Δ133p53, and full-length p53 (FLp53) expression was determined in 45 mucinous, 30 endometrioid, and 91 serous ovarian cancer specimens as well as 42 normal ovarian tissues using reverse transcriptase-quantitative polymerase chain reaction. In a subgroup of mucinous ovarian cancer cases, Δ40p53 expression was examined using Western blot analysis. A functional yeast-based assay and subsequent sequencing were performed to analyze the p53 mutational status. RESULTS: In endometrioid cancer specimens, Δ133p53 expression was significantly lower than in mucinous and serous cases (P = 0.016) or in normal tissues (P = 0.004). Mucinous cancer samples showed elevated Δ40p53 expression as compared with normal ovarian tissues (P = 0.003). In addition, high Δ40p53 expression constituted an independent prognostic marker for recurrence-free but not for overall survival in patients with mucinous ovarian cancer (hazard ratio, 0.267; 95% confidence interval, 0.094-0.756 [P = 0.013]; hazard ratio, 0.453, 95% confidence interval, 0.193-1.064 [P = 0.069]). Western blot analysis confirmed the presence of p53ß and Δ40p53α in a subset of patients with mucinous ovarian cancer. Expression of p53 isoforms was not associated with p53 mutational status or clinicopathologic parameters. CONCLUSIONS: We show that expression of p53 isoforms differs in histological subtypes, thus supporting the hypothesis that histological subtypes represent distinct disease entities. In addition, we provide first evidence for a favorable role of Δ40p53 in patients with mucinous ovarian cancer.

Awareness of guidelines and trends in the management of suspected pancreatic cystic neoplasms: survey results among general gastroenterologists and EUS specialists.

BACKGROUND: Although pancreatic cystic neoplasms are widely recognized, practice habits among physicians and awareness of consensus guidelines are currently unknown. OBJECTIVES: To assess the awareness of guidelines and describe variability in practice habits among 2 groups: (1) "general group" of gastroenterologists and surgeons and (2) "EUS group" of specialists in EUS. DESIGN: An online survey was sent to randomly selected gastroenterologists and surgeons and e-mailed to members of the American Society for Gastrointestinal Endoscopy (ASGE) Special Interest Group in EUS (EUS-SIG). RESULTS: Response rate for the general group was 8.8% (220/2500) and 9.7% for the EUS group (42/431). EUS specialists were mostly in academic practice (66.7% vs 36.3%, P < .001) and reported seeing 21 to 50 cysts per year (54.8% vs 12.3%, P < .001). The majority of the general group (64.1%) was unaware of any published practice guidelines, compared with 33.3% of EUS specialists (P < .001). Awareness of ASGE guidelines was more frequently reported than other guidelines in both groups and yet was still <50% for each group. Both demonstrated moderate consistency with the International Association of Pancreatology guidelines, appropriately answering 66.7% of the questions. For 9-mm lesions, only 25% of the questions were correctly answered in each group. EUS specialists were less likely to refer main-duct intraductal papillary mucinous neoplasms (IPMN) for surgery and more likely to opt for EUS-guided FNA (compared with high-resolution CT, MRCP, or surgery) for 9-mm, 22-mm, and 34-mm branch-duct IPMNs (P

Mucinous cystic neoplasms of the mesentery: a case report and review of the literature.

BACKGROUND: Mucinous cystic neoplasms arise in the ovary and various extra-ovarian sites. While their pathogenesis remains conjectural, their similarities suggest a common pathway of development. There have been rare reports involving the mesentery as a primary tumour site. CASE PRESENTATION: A cystic mass of uncertain origin was demonstrated radiologically in a 22 year old female with chronic abdominal pain. At laparotomy, the mass was fixed within the colonic mesentery. Histology demonstrated a benign mucinous cystadenoma. METHODS AND RESULTS: We review the literature on mucinous cystic neoplasms of the mesentery and report on the pathogenesis, biologic behavior, diagnosis and treatment of similar extra-ovarian tumors. We propose an updated classification of mesenteric cysts and cystic tumors. CONCLUSION: Mucinous cystic neoplasms of the mesentery present almost exclusively in women and must be considered in the differential diagnosis of mesenteric tumors. Only full histological examination of a mucinous cystic neoplasm can exclude a borderline or malignant component. An updated classification of mesenteric cysts and cystic tumors is proposed.

Cystic lesions of the pancreas. A diagnostic and management dilemma.

BACKGROUND/AIMS: Due to enhanced imaging modalities, pancreatic cysts are being increasingly detected, often as an incidental finding. They comprise a wide range of differing underlying pathologies from completely benign through premalignant to frankly malignant. The exact diagnostic and management pathway of these cysts remains problematic and this review attempts to provide an overview of the pathology underlying pancreatic cystic lesions and suggests appropriate methods of management. METHODS: A search was undertaken with a Pubmed database to identify all English articles using the keywords 'pancreatic cysts', 'serous cystadenoma', 'intraductal papillary mucinous tumour', 'pseudocysts', 'mucinous cystic neoplasm' and 'solid pseudopapillary tumour'. RESULTS: The mainstay of assessment of pancreatic cysts is cross-sectional imaging incorporating CT and MRI. Fine-needle aspiration (FNA) (often with endoscopic ultrasound) may provide valuable additional information but can lack sensitivity. Symptomatic cysts, increasing age and multilocular cysts (with a solid component and thick walls) are predictors of malignancy. A raised cyst aspirate CEA, CA 19-9 and mucin content (including abnormal cytology), if present, can accurately distinguish premalignant and malignant cysts from benign ones. CONCLUSION: In summary, all patients with pancreatic cystic lesions, whether asymptomatic or symptomatic, must be thoroughly investigated to ascertain the underlying nature of the cyst. Small asymptomatic cysts (<3 cm) with no suspicious features on imaging or FNA may be safely followed up. Follow-up should continue for at least 4 years, with a repeat FNA if needed. An algorithm for the management of pancreatic cystic tumours is also suggested. and IAP.

Diagnosis, morphopathological profile and treatment of mucinous cystadenoma of the pancreas - a single center experience.

AIM: Pancreatic mucinous cystadenoma (MCA) occurs almost exclusively in perimenopausal women and represents between 10% and 45% of cystic neoplasm of the pancreas, being considered a premalignant lesion. MATERIALS AND METHODS: From 1983 to 2017, 31 patients underwent surgery for MCA of the pancreas in our Center. The median age was 47 years (range 17-81 years). All data were obtained retrospectively. RESULTS: The female∕male gender ratio was 14.5∕1. Most of the patients (90.3%) were symptomatic. The most common clinical manifestation was non-specific abdominal pain (58.06%), followed by fatigue and vomiting. The median cyst size was 7 cm, with a range between 2 cm and 15 cm. There were 35 procedures in 31 patients (in four patients the resection was preceded by a drainage procedure). From the 28 resections, most of them (89.28%) were performed by an open approach; a minimal invasive approach was used in three patients (robotic - two; laparoscopic - one). Most of the resections (82.14%) were distal pancreatectomies. In all cases, the final diagnosis was based on histological examination that revealed columnar epithelium and ovarian-type stroma. Postoperative complications occurred in 10 (34.48%) patients. Postoperative mortality was 3.44% (one patient) by septic shock secondary to acute postoperative pancreatitis. CONCLUSIONS: MCAs represent a rare pancreatic pathology with challenging diagnostic and therapeutic implications. Multi-detector computed tomography (MDCT) scan, endoscopic ultrasound (EUS) and magnetic resonance imaging (MRI)∕magnetic resonance cholangiopancreatography (MRCP) are useful in the differential diagnosis with other pancreatic fluid collections and treatment. Oncological surgical resections are recommended. Histopathological examination establishes the final diagnosis. The most common postoperative complication is pancreatic fistula.

Mucinous Cystadenoma in Children and Adolescents.

STUDY OBJECTIVE: Mucinous cystadenomas (MCAs) are benign epithelial ovarian tumors that occur rarely in children and adolescents. Because children and adolescents typically have their childbearing years ahead of them, conservative therapy is indicated. However, there is concern that ovarian cystectomy might be associated with significant recurrence risk in patients with MCA. Furthermore, guanine nucleotide binding protein, alpha stimulating (GNAS) gene mutations are associated with McCune-Albright syndrome, which is associated with cystic ovaries. We sought to evaluate the outcomes of children and adolescents with MCA treated conservatively. A subset of patients underwent GNAS gene testing. DESIGN, SETTING, PARTICIPANTS, AND INTERVENTIONS: After institutional board review approval, the pathology database of a large urban children's hospital was queried to identify adolescents with MCA between the years 2008 and 2014. Fourteen patients, aged 8-18 years (median, 14), were identified. A buccal swab for genetic testing was obtained from a subset of consenting patients. MAIN OUTCOME MEASURES: MCA recurrence; ovarian return to normal size; GNAS gene variants. RESULTS: Two patients underwent oophorectomies, and the remaining 12 underwent cystectomies. Follow-up ultrasound examination revealed slow return of ovary to normal size. Of the 10 patients with available follow-up data, there were no recurrences at a median of 225 days from surgery. Four patients consented to a buccal swab for genetic testing, and the GNAS gene was noted to have rare variants in 2 patients. CONCLUSION: This series supports the use of ovary-sparing surgery in the treatment of MCA. Further research exploring possible genetic variants such as the GNAS gene in children and adolescents diagnosed with MCA is warranted.

Less common neoplasms of the pancreas.

Recently, there has been an increased recognition of neoplasms of the pancreas other than ductal adenocarcinoma. Although not as well studied or characterized as pancreatic adenocarcinoma there are many distinct lesions which exhibit diverse biological behaviors and varying degrees of malignancy. These lesions include: endocrine neoplasms, cystic tumors, solid pseudopapillary tumors, acinar cell carcinoma, squamous cell carcinoma, primary lymphoma of the pancreas, and metastatic lesions to the pancreas. These less common neoplasms are being diagnosed more frequently as the number and sensitivity of diagnostic imaging studies increase. This review article discusses the clinical course, diagnosis, and treatment of these less common, but quite relevant, neoplasms of the pancreas.

Mucinous tumors of the ovary: a clinicopathologic analysis of 75 borderline tumors (of intestinal type) and carcinomas.

With the exception of benign cystadenomas, mucinous ovarian tumors are rare and heterogeneous neoplasms. They have been classified as either borderline tumors or carcinomas for almost 30 years. Subsequently, the borderline tumors have been subclassified into endocervical-like and intestinal types. The diagnostic criteria for distinguishing borderline tumors of the intestinal type from mucinous carcinomas have varied, making difficult the interpretation of prognostic information. More recently, a further subdivision of the former tumors into forms with only epithelial atypia and variants with focal intraepithelial carcinoma has been proposed. Consequently, in this study of 41 mucinous borderline tumors of intestinal type and 34 mucinous carcinomas, the former were also subdivided into 30 cases with mild to moderate atypia only and 11 with areas of intraepithelial carcinoma. All 30 purely borderline tumors were stage I tumors, and all 15 with follow-up information (including one case with microinvasion) were clinically benign. All 11 mucinous borderline tumors that had foci of intraepithelial carcinoma were also stage I neoplasms, and none of the eight patients with follow-up data (including one with microinvasive carcinoma) recurred. Thirty-four invasive carcinomas were subclassified into 15 expansile and 19 infiltrative subtypes. All 15 carcinomas with only expansile invasion were stage I; none of the 11 with follow-up data recurred. Three of nine patients with stage I infiltrative carcinomas with follow-up information had a fatal recurrence. Eight of the remaining 10 infiltrative carcinomas had extended beyond the ovary at the time of diagnosis (stages II and III); of the six patients with follow-up data, four died of tumor and two were alive with disease. In stage I carcinomas nuclear grade and tumor rupture correlated with unfavorable prognosis, but less than infiltrative invasion. However, all three fatal tumors were infiltrative carcinomas that had ruptured, and two contained grade 3 malignant nuclei. Combination of infiltrative invasion, high nuclear grade, and tumor rupture is a strong predictor of recurrence for stage I mucinous ovarian tumors. Among the 19 infiltrative tumors, 13 contained foci of anaplastic carcinoma. Of the seven patients with stage I tumors and follow-up data, only one patient whose tumor had ruptured intraoperatively had a fatal recurrence. The presence of anaplastic components in stage Ia (intact) carcinomas did not have an adverse effect in their outcome, even when the undifferentiated carcinomatous elements appeared in the form of mural nodules.

Lymph node disorders and prognostic value of nodal involvement in patients treated for a borderline ovarian tumor: an analysis of a series of 42 lymphadenectomies.

BACKGROUND: The aim of this study is to evaluate the rate and the clinical outcomes of lymph node involvement in patients treated for borderline ovarian tumor (BOT). STUDY DESIGN: Forty-two patients were treated for BOT with a procedure that included lymphadenectomy. Twenty-four patients underwent a pelvic lymphadenectomy, 6 a paraaortic lymphadenectomy, and 12 both procedures. Thirty-two patients underwent systematic lymphadenectomy, five because of associated cancer (uterine cervix or corpus) and five because of bulky nodes discovered during the surgical procedure. RESULTS: An endosalpingiosis was present in 11 (26%) patients who underwent lymphadenectomy. Eight patients had nodal involvement related to the BOT. All patients with nodal involvement had serous BOT with peritoneal implants. None of the patients with a mucinous tumor had nodal involvement. None of the patients with early-stage disease (without peritoneal disease) had nodal involvement discovered after routine lymphadenectomy. None of the patients with nodal involvement died of borderline tumor. One patient died of a complication of adjuvant therapy (leukemia after chemotherapy). CONCLUSIONS: The prognosis of patients with borderline tumors of the ovary and nodal involvement is excellent. Routine lymphadenectomy should not be performed in patients with early-stage disease. This procedure should be carried out in patients with serous tumor and enlarged lymph nodes.

[The treatment of borderline ovarian tumors and its prognosis].

Twenty patients with ovarian borderline epithelial tumors were analysed. The tumor in 9 patients were serous and in 11 patients mucinous in nature type. 15 cases were classified as of stage I, 3 cases of stage II and 2 cases of stage III, according to the FIGO staging. All pathologic slides were re-reviewed to confirm the diagnosis and no patients were lost to follow-up. Total hysterectomy and unilateral or bilateral salpingo-oophorectomy were done in 12 patients, among whom, 2 died of tumor recurrences. Unilateral adnexectomy was done in 8 patients, and none died of tumor recurrence. Fourteen patients received post operative chemotherapy 1-8 courses with PAC regimen, 12 patients were alive up to the present. Six patients did not receive chemotherapy and among these 3 with stage Ia were alive and 1 with stage Ib died of tumor recurrences. The survival rate of stage I was 92.3%. Our results indicated that the prognosis was closely related to clinical staging. We suggested that patients of stage I who wish to preserve childbearing function may be treated with unilateral salpingo-oophorectomy, and chemotherapy should be given to the patients with high risk factors. Because of the indolent nature of these tumors, long-term follow-up at least 10 years is necessary.

[The clinical features and management of borderline ovarian tumors].

OBJECTIVE: To study the clinical features and management of borderline ovarian tumor (BOT). METHODS: Retrospectively study 70 cases of BOT. RESULTS: Age 16-76 years old, average 45.6 year old; histological type: mucinous 46(65.7%), serous 21(30%); stage: I 46 (65.7%), II 1(1.4%), III 11(15.7%), IV 0%, relapse 12(17.1%). Tumor marker: elevated CA125 11/21, carcinoembryonic antigen (CEA) 6/10. TREATMENT: all patients were operated, 45 were given adjuvant therapy. OUTCOME: No evidence of disease (NED) 45, with evidence of disease (WED) 4, died of disease (DOD) 11, died of intercurrent disease (DID) 10. 11 patients who were given conservative surgery were NED, there were 2 long term CR among 16 patients with residual disease who were given adjuvant treatment. The 5, 10, 20 and 30-year survival is 90.0%, 87.1%, 87.1% and 85.6% respectively. COX hazard regression model analysis showed that histological type (r = 24.825), stage (r = 2.236) and residual disease (r = 2.083) were the main factors which impact on the prognosis of BOT. CONCLUSIONS: (1) The proportion of mucinous tumor is higher than that of serous tumor in this group; (2) Surgery is the proved only effective treatment of BOT till now, without any residual disease is expected; (3) Adjuvant therapy can't improve the prognosis of BOT; (4) CA125 and CEA can be two effective tumor markers in monitoring of BOT.

[Current strategy to cure pancreatic cancer].

For more than a decade extensive retroperitoneal dissection, chemotherapy, or radiotherapy has not prolonged the survival of patients with pancreatic cancer. Two prospective randomized studies addressing the clinical significance of extensive dissection or pancreatic resection for advanced cancer are now in progress. Nonetheless, at present, resection offers the patient the only possibility of cure. Although the diagnosis of curable pancreatic cancer is difficult, recent evidences have given a few hints. The first is pancreatic duct dilatation caused by cancerous stricture. The second is diabetes as a sign of pancreatic cancer. Our prospective pancreatographic screening of diabetic patients selected by our criteria(Table 1) revealed 7 cancers in 98 patients(7.1%). Within 3 years from diagnosis, the prevalence was 15%. Although the 7 cancers were advanced, this suggests that earlier examinations in diabetic patients may possibly lead to earlier diagnosis. The third is a small cystic lesion as a sentinel of pancreatic cancer. Endoscopic retrograde cholangiopancreatography with cytology of the pancreatic juice may show the presence of in situ cancer in patients with a pancreatic cyst. At the moment, careful checks for the presence of these hints seem to be the only strategy to offer a chance for cure to patients with pancreatic cancer.