Hooman Soleymani majd

Clinicopathological Characteristics and Oncologic Outcomes of Endometrioid Ovarian Carcinoma: A Retrospective Study from a Tertiary Cancer Centre

Background/Objectives: To evaluate the clinicopathological features, treatment, and survival outcomes and to identify independent prognosticators for recurrence and mortality in patients with endometrioid ovarian cancer. Methods: The medical records of patients diagnosed with endometrioid ovarian carcinoma between January 2010 and December 2022 were reviewed retrospectively. Demographic and disease-related data were evaluated. Kaplan–Meier survival analysis using log rank test and Cox regression was performed. Results: Seventy-six patients were included in the study. The median age at diagnosis was 54 years (range 31–86). A total of 85.5% of the patients were diagnosed with early-stage disease and 88.1% of the tumours represented low-grade carcinomas. Synchronous endometrioid endometrial cancer was confirmed in 19.7% of the cases. All patients underwent surgical management and 65.8% received adjuvant chemotherapy. Median follow-up time was 67.5 months. The 5-year disease-free survival and overall survival were 92.1% and 93.4%, respectively. The risk of cancer-related death was higher in advanced stages (HR = 13.86; 95% CI 2.16–57.17; p < 0.001) and in the presence of residual disease (HR = 15.18; 95% CI 2.36–87.17; p < 0.002). Residual disease and advanced stages were also identified as independent risk factors for disease relapse with HR = 16.04 (95% CI 2.61–93.7; p < 0.002) and HR = 11.73 (95% CI 1.92–41.6; p < 0.001), respectively. Conclusions: Endometrioid ovarian carcinoma usually affects younger patients with the majority of the cases representing low-grade carcinomas diagnosed at early stages. Residual disease and advanced stages are independently associated with inferior survival outcomes. There was no significance of lymph node dissection and adjuvant chemotherapy in the overall and recurrence-free survival rates. Further research focusing on molecular profiling should aim to define the prevalence and the prognostic value of major molecular alterations and develop precise stratification models to plan personalised treatment for optimal care.

The role of the multidisciplinary team in surgical management of intractable tubo‐ovarian abscess as a late sequelae of challenging Crohn's disease in the modern era: A case report and review of current literature

AbstractChronic, severe Crohn's disease in a young female patient can result in surgical complexity. The rarity of the presentation of intractable pelvic abscesses within this etiology with additional considerations given to fertility concerns and hence requirement for input from a multi‐disciplinary team makes this a vital case in building a consensus for evidence‐based surgical management. A 29‐year‐old nulliparous woman was referred to our tertiary centre for surgical management of Crohn's disease with known tubo‐ovarian abscess and abdominoperineal and abdominal wall sinuses. Her previous surgical history included 4 midline laparotomies, subtotal colectomy and proctectomy with stoma formation. The patient underwent egg collection to preserve fertility. This was followed by midline laparotomy and abdominoperineal resection, which involved a retrograde radical modified hysterectomy using the Hudson technique, alongside excision of the perineal sinus, with reconstruction of the perineal defect using an internal pudendal artery perforator gluteal fold flap, and in addition to excision and drainage of the abdominal wall abscess. Involvement was sought from gynecological oncology, colorectal, urology, plastics, stoma, fertility, microbiology, and gastroenterology teams, which enabled successful preservation of end organ function and improvement in patient psychological well‐being. This case is a paradigm of surgical challenge, requiring expert gynecological oncology techniques including a retroperitoneal approach, nerve and vessel sparing considerations alongside colorectal and urological procedures. Moreover, we believe that our blueprint for effective multi‐disciplinary practice will inform the future management of gynecological surgery. Therefore this report aims to contribute towards the optimum management of the gynecological sequelae of Crohn's disease.

Radical abdomino-pelvic surgery in the management of uterine carcinosarcoma with concomitant para-aortic lymphadenopathy metastasising from anal carcinoma

Uterine carcinosarcomas are aggressive gynaecological cancers comprising less than 5% of uterine malignancies. We present the case of a woman in her 70s with a complicated history of advanced anal carcinoma treated with pelvic radiotherapy and multiple laparotomies, who was referred to gynae-oncology following MRI surveillance imaging showing evidence of endometrial carcinoma and para-aortic lymphadenopathy. Successful surgical excision required multidisciplinary teamwork between gynae-oncology, colorectal and urology surgeons. The patient underwent midline laparotomy, with adhesiolysis, ileum resection and side to side anastomosis, posterior exenteration, left kidney mobilisation and suspension, para-aortic lymph node debulking and left ureteric stent insertion. Significant challenge was posed by the extensive adhesions from previous laparotomies and the debulking of the para-aortic lymph nodes around the renal vessels. This case demonstrates the importance of a multidisciplinary approach in complex pelvic surgery and the vitality of good communication between colleagues in achieving effective patient care.

Intravascular leiomyomatosis; mimicking low grade endometrial sarcoma

Minimally invasive surgery in the management of early stage cervical cancer after the publication of SHAPE trial

The Feasibility of Cardiophrenic Lymphnode Assessment and Removal in Patients Requiring Diaphragmatic Resection During Interval Debulking Surgery for Ovarian Cancer

Several studies have demonstrated the feasibility and role of bulky cardiophrenic lymph nodes (CPLNs) resection during primary debulking surgery (PDS) for stage IV ovarian cancer (OC). However, no studies, to date, investigated the accuracy and feasibility of CPLNs assessment and removal during interval debulking surgery (IDS) after neoadjuvant chemotherapy (NACT). A retrospective analysis of consecutive stage IV OC patients who underwent NACT followed by IDS with CPLNs assessment and/or resection from July 2017 to June 2018. Bulky CPLNs were considered for excision when a full-thickness diaphragmatic resection was required in order to achieve complete tumour resection. A total of 21 ovarian cancer stage IV patients treated with NACT followed by IDS were identified. Seven (33.3%) patients underwent CPLNs resection due to bulky appearance of the CPLNs at the intraoperative palpation. The final histological examination of the CPLNs reported metastatic disease in four (57%) of seven patients. Complete cytoreduction without residual disease was achieved in five cases (71.4%) while in two case (28.6%) optimal cytoreduction was performed. Intra-operative surgical complications occurred in one patient. One patient had a major postoperative complication (Clavien-Dindo 3). Two cases of postoperative cardiac arrhythmia were observed. CPLNs intraoperative assessment is less accurate during IDS compared to previous PDS studies. CPLNs removal during IDS after NACT for stage IV OC could be safely performed to achieve a complete resection.

Feasibility of laparoscopic diaphragmatic peritonectomy during Visceral-Peritoneal Debulking (VPD) in patients with stage IIIC-IV ovarian cancer

To describe the surgical technique and evaluate the safety, feasibility and efficacy of laparoscopic diaphragmatic peritonectomy during Visceral-Peritoneal Debulking (VPD) in patients with stage IIIC-IV ovarian cancer (OC). This report is part of a Service Evaluation Protocol (Trust number 3267) on laparoscopy in patients with OC following neo-adjuvant chemotherapy. Between April 2015 and November 2017, all patients underwent to exploratory laparoscopy and a selected court was offered laparoscopic VPD. Laparoscopic diaphragmatic surgery was considered if there was no full thickness involvement. Primary endpoints of this part of the study were the safety, feasibility and efficacy of laparoscopic diaphragmatic peritonectomy. We report the surgical technique and outcomes. Ninety-six patients underwent diaphragmatic surgery during the study period. Fifty patients (52.1%) had intra-operative exclusion criteria and/or full thickness diaphragmatic resection, 46 (47.9%) had peritonectomy and were included in the study. Laparoscopic diaphragmatic peritonectomy was performed in 21 patients (45.4%, group 1), while in 25 patients (54.6%, group 2) laparotomy was necessary. Extent of disease and complexity of surgery were similar. Reasons for conversions were disease coalescing the liver to the diaphragm preventing safe mobilization (22 patients) and accidental pleural opening (3 patients). Overall, intra- and post-operative morbidity was lower in group 1 and pulmonary specific morbidity was very low. Diaphragmatic peritonectomy can be safely accomplished by laparoscopy in almost half of the patients with OC whose disease is limited to the diaphragmatic peritoneum.

Interval Debulking Surgery for Advanced Ovarian Cancer in Elderly Patients (≥70 y): Does the Age Matter?

Elderly ovarian cancer (OC) patients are more likely to be managed suboptimally, with worse clinical outcomes as a result. Strategies to decrease morbidity are lacking. A total of 153 patients were referred during the study period. 114 patients underwent IDS after NACT (74.5%), 46 in Group 1 and 68 in Group 2. Elderly patients were more likely to receive more than three cycles of NACT prior to IDS compared to younger patients (39% vs. 19%, Older age should not preclude clinicians from offering ultra-radical resection to patients with advanced OC after NACT. In our series, elderly patients received the same treatment with similar outcomes to the younger group. Clinicians should be encouraged to use CPET for patients' selection following NACT.

Tozzi classification of diaphragmatic surgery in patients with stage IIIC–IV ovarian cancer based on surgical findings and complexity

To introduce a systematic classification of diaphragmatic surgery in patients with ovarian cancer based on disease spread and surgical complexity. For all consecutive patients who underwent diaphragmatic surgery during Visceral-Peritoneal debulking (VPD) in the period 2009-2017, we extracted: initial surgical finding, extent of liver mobilization and type of procedure. Combining these features, we aimed to classify the surgical procedures necessary to tackle different presentation of diaphragmatic disease. We also report histology, intra- and post-operative specific complication rate based on the classification. A total of 170 patients were included in this study, 110 (64.7%) had a peritonectomy, while 60 (35.3%) had a full thickness resection with pleurectomy. We identified 3 types of surgical procedures. Type I treated 28 out of 170 patients (16.5%) who only had anterior diaphragm disease, needed no liver mobilization, included peritonectomy and had no morbidity recorded. Type II pertained to 105 out of 170 patients (61.7%) who had anterior and posterior disease, needed partial and sometimes full liver mobilization, had a mix of peritonectomy and full thickness resection, and experienced 10% specific morbidity. Type III included 37 out of 170 patients (21.7%) who needed full mobilization of the liver, always had full thickness resection, and suffered 30% specific morbidity. Diaphragmatic surgery can be classified in 3 types. The adoption of this classification can facilitate standardization of the surgery, comparison of data and define the expertise required. Finally, this classification can be a benchmark to establish the training required to treat diaphragmatic disease.

Thoracic Gorham-Stout disease masquerading as an ovarian malignancy: a differential diagnosis in non-malignant causes of raised Ca-125 and overview of this rare pathology

Gorham-Stout disease (GSD) is thought to be due to uncontrolled proliferation of vascular and lymphatic structures within bone tissue causing destruction and osteolysis of bone. We present a patient in her mid-40s who reported chronic shoulder pain, a pleural effusion and irregular periods. Investigations showed osteolysis of her ribs, pleural effusions, an ovarian mass and a raised carbohydrate antigen 125 (Ca-125). She was subsequently diagnosed with GSD, and referred to gynaecology-oncology in consideration of potential ovarian malignancy. GSD is a diagnosis of exclusion that requires a high degree of clinical suspicion, as well as multiple investigations to achieve diagnosis. Clinicians rely on a small number of case reports to provide guidance for this. Therefore, this report provides an overview of a rare pathology, considers the differentials of a raised Ca-125 and describes how a pleural effusion, which links them both, alarmed us regarding an incidental finding of an ovarian cyst.

Primary peritoneal mucinous cystadenocarcinoma mimicking possible recurrent ovarian mucinous cystadenoma: coincidental pathology or a spectrum of disease?

We present the case of a 32-year-old woman with a previous surgical history of benign mucinous cystadenoma resected in 2012 who underwent magnetic resonance cholangiopancreatography following her second pregnancy in 2020. This demonstrated a large cystic mass in the left subdiaphragmatic space. Histopathology confirmed a well-differentiated primary peritoneal mucinous cystadenocarcinoma displaying cells of a Mullerian origin. We subsequently discuss the aetiology of these conditions separately and explore the possibility of a connection between the two regarding origin or potential malignant transformation that may otherwise have occurred coincidentally in this young patient. We also acknowledge a paucity of evidence regarding subsequent management strategies.

Adamantinoma: metastatic disease masquerading as a gynaecological malignancy

Adamantinoma is a rare low-grade malignancy of the appendicular skeleton with unclear histogenesis. We present the case of a 65-year-old woman with known recurrent and metastatic right tibial disease despite clear resection margins. On further investigation, a positron emission tomography–CT scan identified a primary breast lesion and an 11 cm mass in the right iliac fossa of suspected ovarian malignancy amenable to surgical resection. The patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy and resection of a retroperitoneal mass arising from the pelvic sidewall encompassing the iliac vasculature. The patient made an uneventful recovery with histology confirming disease metastasising to the pelvis. Currently, clinical management guidelines are not available. We present an overview of adamantinoma and highlight a previously undocumented gynaecological oncology surgical approach to this novel disease location. Regarding metastases, we acknowledge the challenges of investigation pertaining to disease site and origin as well as a paucity of recommendations for surveillance and follow-up.

Malignant struma ovarii: surgical, histopathological and survival outcomes for thyroid-type carcinoma of struma ovarii with recommendations for standardising multi-modal management. A retrospective case series sharing the experience of a single institution over 10 years

Struma ovarii is rare, accounting for 0.3-1% of ovarian tumours. Malignant transformation may occur, most often into papillary thyroid carcinoma. There is a paucity of data pertaining to malignant struma ovarii. This paper shares a decade of experience of a single institution in the management of this rare ovarian cancer, exploring the characteristics of this tumour and suggesting a standardised approach to treatment and follow-up. All patients treated for malignant struma ovarii within a large cancer centre over one decade were identified and data collected retrospectively on presentation, diagnosis, management, follow-up and survival outcomes. A literature review was also undertaken. Eleven cases of malignant struma ovarii were managed in the Oxford Cancer Centre between 2010 and 2019, 6 of which were of papillary thyroid carcinoma sub-type. No cases were correctly diagnosed pre-operatively. All patients had stage I disease and were managed surgically-but with variation in radicality. Patients identified as high-risk based on final histopathology underwent additional thyroidectomy and radio-active iodine ablation therapy. One case of synchronous malignancy of the thyroid gland proper was identified. No disease recurrence occurred. Malignant struma ovarii present a diagnostic challenge. Multi-disciplinary team (MDT) input is essential. Unilateral salpingo-oophrectomy may be adequate if stage I; reserving more radical surgery for advanced disease. Histopathological risk-stratification should be used to identify those most likely to benefit from adjuvant thyroid-targeting therapies. Patients require follow-up, anticipating an overall good prognosis.

Cervical lymph nodes and ovarian teratomas as germinal centres in NMDA receptor-antibody encephalitis

Abstract Autoantibodies against the extracellular domain of the N-methyl-d-aspartate receptor (NMDAR) NR1 subunit cause a severe and common form of encephalitis. To better understand their generation, we aimed to characterize and identify human germinal centres actively participating in NMDAR-specific autoimmunization by sampling patient blood, CSF, ovarian teratoma tissue and, directly from the putative site of human CNS lymphatic drainage, cervical lymph nodes. From serum, both NR1-IgA and NR1-IgM were detected more frequently in NMDAR-antibody encephalitis patients versus controls (both P < 0.0001). Within patients, ovarian teratoma status was associated with a higher frequency of NR1-IgA positivity in serum (OR = 3.1; P < 0.0001) and CSF (OR = 3.8, P = 0.047), particularly early in disease and before ovarian teratoma resection. Consistent with this immunoglobulin class bias, ovarian teratoma samples showed intratumoral production of both NR1-IgG and NR1-IgA and, by single cell RNA sequencing, contained expanded highly-mutated IgA clones with an ovarian teratoma-restricted B cell population. Multiplex histology suggested tertiary lymphoid architectures in ovarian teratomas with dense B cell foci expressing the germinal centre marker BCL6, CD21+ follicular dendritic cells, and the NR1 subunit, alongside lymphatic vessels and high endothelial vasculature. Cultured teratoma explants and dissociated intratumoral B cells secreted NR1-IgGs in culture. Hence, ovarian teratomas showed structural and functional evidence of NR1-specific germinal centres. On exploring classical secondary lymphoid organs, B cells cultured from cervical lymph nodes of patients with NMDAR-antibody encephalitis produced NR1-IgG in 3/7 cultures, from patients with the highest serum NR1-IgG levels (P < 0.05). By contrast, NR1-IgG secretion was observed neither from cervical lymph nodes in disease controls nor in patients with adequately resected ovarian teratomas. Our multimodal evaluations provide convergent anatomical and functional evidence of NMDAR-autoantibody production from active germinal centres within both intratumoral tertiary lymphoid structures and traditional secondary lymphoid organs, the cervical lymph nodes. Furthermore, we develop a cervical lymph node sampling protocol that can be used to directly explore immune activity in health and disease at this emerging neuroimmune interface.