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2.
Clin Case Rep ; 12(6): e9075, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38883216

ABSTRACT

Endometriosis may contribute to Mullerian adenosarcoma development but makes diagnosis challenging given similar symptoms. Survival benefit has not been definitively shown for chemotherapy, hormonal therapy, or radiotherapy, consolidating surgery as the mainstay treatment. Local excision may be a treatment option for patients with confined tumors wishing to preserve their fertility.

3.
Int J Gynaecol Obstet ; 165(2): 535-541, 2024 May.
Article in English | MEDLINE | ID: mdl-37882505

ABSTRACT

Chronic, 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.


Subject(s)
Colorectal Neoplasms , Crohn Disease , Humans , Female , Adult , Crohn Disease/complications , Crohn Disease/surgery , Abscess/etiology , Abscess/surgery , Pelvis , Patient Care Team
4.
J Clin Med ; 12(19)2023 Oct 05.
Article in English | MEDLINE | ID: mdl-37835017

ABSTRACT

Mixed endometrial carcinoma (MEEC) refers to rare endometrial tumours that are composed of two or more distinct histotypes, at least one of which is serous or clear cell. The aim of this study was to evaluate the epidemiology, treatment outcomes and survival rates of patients with mixed endometrial carcinoma. The medical records of 34 patients diagnosed with MEEC between March 2010 and January 2020 were reviewed retrospectively. Clinicopathological variables and treatment strategies were assessed, and overall survival and disease-free survival rates were evaluated. The histology of endometrioid and serous component was found in 26 (76.5%) patients, followed by serous and clear-cell components (5/34, 14.5%) and mixed endometrioid serous and clear-cell components (3/34, 8.8%). The median age at diagnosis was 70 years (range 52-84), and the median follow-up time was 55 months. The 5-year disease-free survival and the 5-year overall survival were 50.4% and 52.4%, respectively. Advanced disease stage was identified as an independent predictor of inferior disease-free (<0.003) and overall survival (p < 0.001). Except for stage, none of the traditional prognostic factors was associated with disease recurrence or death from disease. MEECs represent rare high-risk endometrial carcinomas with significant diagnostic and treatment challenges. Undoubtedly, the implementation of a molecular analysis can offer further diagnostic and management insights.

5.
J Bone Oncol ; 41: 100489, 2023 Aug.
Article in English | MEDLINE | ID: mdl-37408735

ABSTRACT

Adamantinoma is a rare primary low-grade malignant tumour of the appendicular skeleton most commonly found in the tibia. It has an indolent course, with local recurrences and lung metastases occurring over a protracted duration. There have been several suggestions pertaining to a vascular origin in the literature, however, histogenesis remains unclear. Currently, guidelines are not available pertaining to clinical management. This paper presents an overview of the current literature regarding this unusual malignancy. It also explores disease etiology and acknowledges the benefits and challenges of investigations pertaining to diagnosis. It recognizes a paucity of recommendations regarding appropriate surveillance and follow up. This review aims to assist clinicians in the building of a consensus opinion for optimal adamantinoma case management under current circumstances where formal guidelines do not exist.

6.
Obstet Gynecol Sci ; 66(5): 459-461, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37381885

ABSTRACT

OBJECTIVE: We present an educational technique for the safe completion of complete cytoreduction of diaphragmatic disease for the management of advanced ovarian malignancy. METHODS: We demonstrated these steps with attention to anatomical landmarks and surgical approaches, considering intraoperative and postoperative morbidity and mortality. RESULTS: We present the case of a 49-year-old female patient diagnosed with suspected stage 3C ovarian malignancy following diagnostic laparoscopy. We demonstrate the surgical application of the Pringle manoeuvre, type 3 liver mobilisation, and full-thickness diaphragmatic resection. This was completed with a primary closure technique, with integrity ensured through the performance of an air test and Valsalva manoeuvre. Final histology confirmed a serous borderline tumour with invasive implants within a port site nodule (stage 4A). CONCLUSION: This technique affirms the essential skills in gynaecological oncology training and details a challenging case requiring advanced surgical skills and knowledge, with specific consideration for intraoperative multidisciplinary decision-making.

7.
J Clin Med ; 12(9)2023 Apr 26.
Article in English | MEDLINE | ID: mdl-37176582

ABSTRACT

High grade endometrioid endometrial cancer (HGEEC) is a heterogeneous group of tumors with unclear prognostic features. The aim of the present study is to evaluate the independent risk factors for recurrence and mortality and to describe the recurrence patterns of HGEEC. Ninety-six consecutive cases of HGEEC treated with primary surgery in a single Tertiary Center were retrospectively reviewed. Clinicopathological and treatment details were recorded, and all patients were closely followed up. Disease-free, overall and cancer-specific survival rates were 83.8%, 77.8% and 83.6%, respectively. Cervical stromal involvement was independently related to recurrence (HR = 25.67; 95%CI 2.95-223.30; p = 0.003) and cancer-related death (HR = 15.39; 95%CI 1.29-183.43; p = 0.031) after adjusting for other pathological and treatment variables. Recurrence rate was 16%, with 60% of these cases having lung metastases and only one case with single vaginal vault recurrence. 81.81% of the recurrences presented with symptoms and not a single recurrence was diagnosed in routine follow-up clinical examination. In conclusion, the recurrence pattern may suggest that patient-initiated follow-up (PIFU) could be considered a potential alternative to clinical-based follow-up for HGEEC survivors, especially for patients without cervical involvement and after two years from treatment. Additional caution is needed in patients with cervical stromal involvement.

8.
Transl Cancer Res ; 12(1): 201-208, 2023 Jan 30.
Article in English | MEDLINE | ID: mdl-36760371

ABSTRACT

Background: Bartholin's gland carcinoma (BGC) accounts for approximately 5% of all vulval malignancies-making it an extremely rare malignancy of the female genital tract. It commonly manifests as a painless unilateral mass, near the introitus. BGC more commonly occurs in post-menopausal women. Unfortunately, over half of cases are associated with a missed or delayed diagnosis as it is often mistaken for a Bartholin's gland cyst or abscess. These tumours have a predilection for local and perineural invasion. Magnetic resonance imaging (MRI) is the imaging modality of choice for suspected Bartholin's tumour. Although no current guidance dedicated to the management of BGC exists, the majority of cases are treated by primary excision and bilateral groin node dissection (GND). Chemoradiotherapy has a role in both the adjuvant and palliative setting. BGC are typically associated with more advanced disease at presentation, higher rates of recurrence and poorer prognosis than other vulval cancer sub-types. Case Description: We share a case report of primary BGC-supported by high-quality radiological and surgical images; and further supplemented by a detailed review of current literature. Conclusions: We aim to generate improved clinician awareness of this rare pathology, highlighting the need for vigilance to avoid misdiagnosis and subsequent treatment delay; as well as contribute towards generating consensus on the approach to management of this gynaecological malignancy.

9.
J Clin Med ; 11(24)2022 Dec 10.
Article in English | MEDLINE | ID: mdl-36555968

ABSTRACT

INTRODUCTION: Cervical cancer is currently the fourth most common cancer in women and in the poorest countries this neoplasia still represents a widespread and potentially lethal disease. We present a rare case of cervical cancer in pregnancy, analyzing the historical changes behind the procedure of radical hysterectomy for cervical cancer and discussing variations in surgical techniques and anatomical definitions that have since been proposed. RESULTS: We present the case of a 33-year-old patient who attended with vaginal bleeding in the second trimester of pregnancy. Examination revealed an abnormal looking cervix, with investigations concluding stage IIb squamous cell carcinoma. Following extensive discussion regarding management options, the patient went on to have a peripartum foetocidal type III nerve sparing radical Wertheim hysterectomy at 18 weeks gestation with conservation and transposition of the ovaries above the level of the pelvic brim. The patient recovered well without significant morbidity and received further input from fertility and psychological medical teams in addition to adjuvant treatment within the department of clinical oncology. DISCUSSION: This case represents several elements of great interest and learning. Notably, we highlight this both due to the surgical challenges that a gravid uterus presents in the execution of a radical hysterectomy; and regarding the compassionate care demonstrated by the team - not only in supporting the patient and her partner in a period of profound turmoil in terms of the management of their cancer diagnosis and unborn child, but also regarding the uncertainty in consideration of the oncological and fertility related outcomes. CONCLUSION: This manuscript adds to the growing literature on the appropriate use of radical surgery for cervical cancer, more specifically during pregnancy and in consideration of such ethical dilemma, where management guidelines do not exist to aid clinicians further in their provision of treatment.

10.
BMJ Case Rep ; 15(11)2022 Nov 22.
Article in English | MEDLINE | ID: mdl-36414336

ABSTRACT

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.


Subject(s)
Osteolysis, Essential , Osteolysis , Ovarian Neoplasms , Pleural Effusion , Female , Humans , Osteolysis, Essential/diagnosis , Osteolysis, Essential/pathology , Diagnosis, Differential , Osteolysis/diagnosis , Ribs/pathology , Pleural Effusion/etiology , Pleural Effusion/diagnosis , Ovarian Neoplasms/complications , Ovarian Neoplasms/diagnosis
11.
BMJ Case Rep ; 15(11)2022 Nov 30.
Article in English | MEDLINE | ID: mdl-36450410

ABSTRACT

A patient in her 60s was referred to be investigated for an incidental large uterus with a history of renal cell carcinoma and melanoma. Uterine biopsy revealed features of perivascular epithelioid cell tumours (PEComas) and she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. Final histology confirmed PEComa with malignant features. Genomic studies did not reveal any deleterious germline variants; however, in view of her history, she is now under a 6-month follow-up with gynaecology-oncology. PEComas are rare tumours associated with tuberous sclerosis and melanoma, sharing genetic abnormalities. Gynaecological PEComas usually present with no or non-specific symptoms. Preoperative investigations are often misleading. Final histology and immunohistochemistry have overlapping features with smooth muscle tumours. Although rare, PEComas need to be treated aggressively to minimise the potential risk of spread. There is currently little evidence about further adjuvant treatment and no clear follow-up protocol. However, the literature suggests that the prognosis is generally good.


Subject(s)
Kidney Neoplasms , Melanoma , Perivascular Epithelioid Cell Neoplasms , Tuberous Sclerosis , Female , Humans , Genetic Predisposition to Disease , Perivascular Epithelioid Cell Neoplasms/genetics , Perivascular Epithelioid Cell Neoplasms/surgery
12.
BMJ Case Rep ; 15(11)2022 Nov 30.
Article in English | MEDLINE | ID: mdl-36450419

ABSTRACT

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.


Subject(s)
Anus Neoplasms , Carcinoma , Carcinosarcoma , Endometrial Neoplasms , Lymphadenopathy , Female , Humans , Pelvis , Anus Neoplasms/surgery , Carcinosarcoma/surgery
13.
BMJ Case Rep ; 15(2)2022 Feb 09.
Article in English | MEDLINE | ID: mdl-35140084

ABSTRACT

High-grade poorly differentiated sarcomas of unknown primary origin constitute a rare entity and are characterised by wide histopathological diversity and atypical presentations. We present such an unusual case attending with severe procidentia in a 68-year-old postmenopausal female. On review of the literature, there are no similar cases reported. Herein, we present this case as in view of its rare clinical appearance combined with the advanced and histologically uncertain nature of the tumour, which raised significant challenges regarding diagnosis and surgical management in considerations of oncological hygiene and risk of tumour spillage. This was further potentiated by delay in diagnosis and treatment due to the COVID-19 pandemic.


Subject(s)
COVID-19 , Sarcoma , Aged , Female , Humans , Pandemics , SARS-CoV-2 , Sarcoma/diagnosis
14.
J Invest Surg ; 35(5): 1067-1073, 2022 May.
Article in English | MEDLINE | ID: mdl-34865585

ABSTRACT

Objective: The ischiorectal fossa is a key anatomical location with boundaries and internal structures owing distinct contributions to function and quality of life. Methods: We highlight the importance of management of pathology in this deep anatomically complex location. Results: We present a ten-step multidisciplinary surgical approach; achieving en bloc radical excision with primary closure and reassuring outcomes. Schwannomas are benign slow-growing nerve sheath tumors. Conclusions: Pelvic schwannomas are rare with only 3 reported cases of the pudendal nerve. We also offer an overview of this rare pathology whilst acknowledging a paucity of recommendations regarding management of disease of the ischiorectal fossa.


Subject(s)
Neurilemmoma , Quality of Life , Humans , Neurilemmoma/diagnostic imaging , Neurilemmoma/pathology , Neurilemmoma/surgery , Rectum/pathology
15.
BMJ Case Rep ; 14(9)2021 Sep 16.
Article in English | MEDLINE | ID: mdl-34531237

ABSTRACT

Intravascular leiomyomatosis is a rare, benign tumour of myometrial smooth muscle. Despite being non-invasive, these tumours can proliferate aggressively within vascular structures including pelvic vessels, the vena cava and the heart. We discuss a 77-year-old woman presenting with a 9 cm uterine mass extending into the right adnexa and ovarian vein. Following hysteroscopic biopsy, palliative radical surgical resection was performed for suspected stage IV leiomyosarcoma. Tumour extension into the pelvic sidewall and obturator fossa indicated a modified laterally extended endopelvic resection combined with skeletonisation and preservation of the pelvic neurovasculature, ultimately providing a curative procedure with minimal functional neurological morbidity. We present this unusual case to assist in the development of a consensus for optimal case management where formal guidelines are not yet available. We summarise current understanding of intravascular leiomyomatosis and highlight the value of advanced surgical techniques using knowledge of complex ontogenetic and pelvic neuroanatomy in its management.


Subject(s)
Heart Neoplasms , Leiomyomatosis , Pelvic Neoplasms , Sarcoma , Uterine Neoplasms , Aged , Female , Humans , Leiomyomatosis/diagnosis , Leiomyomatosis/surgery , Pelvic Neoplasms/diagnosis , Pelvic Neoplasms/surgery , Sarcoma/diagnosis , Sarcoma/surgery , Uterine Neoplasms/diagnosis , Uterine Neoplasms/surgery , Vena Cava, Inferior
16.
BMJ Case Rep ; 14(7)2021 Jul 13.
Article in English | MEDLINE | ID: mdl-34257114

ABSTRACT

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.


Subject(s)
Cystadenocarcinoma, Mucinous , Cystadenocarcinoma , Cystadenoma, Mucinous , Cystadenoma , Ovarian Neoplasms , Adult , Cell Transformation, Neoplastic , Cystadenoma, Mucinous/diagnostic imaging , Cystadenoma, Mucinous/surgery , Female , Humans , Ovarian Neoplasms/diagnostic imaging , Ovarian Neoplasms/surgery
17.
BMJ Case Rep ; 14(6)2021 Jun 03.
Article in English | MEDLINE | ID: mdl-34083182

ABSTRACT

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.


Subject(s)
Adamantinoma , Ovarian Neoplasms , Adamantinoma/diagnostic imaging , Adamantinoma/surgery , Aged , Female , Humans , Hysterectomy , Ovarian Neoplasms/surgery , Pelvis , Salpingo-oophorectomy
18.
Med Sci Sports Exerc ; 46(9): 1778-86, 2014 Sep.
Article in English | MEDLINE | ID: mdl-25134001

ABSTRACT

PURPOSE: The effects of glucose-and-fructose (GF) coingestion on cycling time trial (TT) performance and physiological responses to exercise were examined under postprandial conditions. METHODS: Eight trained male cyclists (age, 25 ± 6 yr; height, 180 ± 4 cm; weight, 77 ± 9 kg; V˙O2max, 62 ± 6 mL·kg·min) completed the study. Subjects ingested either an artificially sweetened placebo (PL), a moderate-glucose beverage (MG, 1.03 g·min), a high-glucose beverage (HG, 1.55 g·min), or a GF beverage (1.55 g·min, 2:1 ratio) during approximately 3 h of exercise, including 2 h of constant-load cycling (55% Wmax, 195 ± 17 W), immediately followed by a computer-simulated 30-km TT. Physiological responses (V˙E, V˙O2, RER, HR, blood glucose level, blood lactate level, and RPE) and incidences of gastrointestinal distress were assessed during early (15-20 min), middle (55-60 min), and late exercise (115-120 min) and during the TT. Magnitude-based qualitative inferences were used to evaluate differences between treatments. RESULTS: In comparison with that in PL (52.9 ± 3.7 min), TT performances were faster with GF (50.4 ± 2.2 min, "very likely" benefit), MG (51.1 ± 2.4 min, "likely" benefit), and HG (52.0 ± 3.7 min, "possible" benefit). GF resulted in a "likely" improvement versus HG (3.0%) and an "unclear" effect relative to MG (1.2%). MG was "possibly" beneficial versus HG (1.8%). Few incidences of GI distress were reported in any trials. CONCLUSIONS: GF ingestion seems to enhance performance, relative to PL and HG. However, it is unclear whether GF improves performance versus moderate doses of glucose.


Subject(s)
Athletic Performance/physiology , Bicycling/physiology , Fructose/administration & dosage , Glucose/administration & dosage , Performance-Enhancing Substances/administration & dosage , Administration, Oral , Adult , Blood Glucose/metabolism , Colic/chemically induced , Dizziness/chemically induced , Double-Blind Method , Exercise Test , Fructose/adverse effects , Glucose/adverse effects , Headache/chemically induced , Heart Rate , Humans , Lactic Acid/blood , Male , Nausea/chemically induced , Oxygen Consumption , Performance-Enhancing Substances/adverse effects , Physical Exertion , Postprandial Period , Pulmonary Gas Exchange , Pulmonary Ventilation , Vomiting/chemically induced , Young Adult
19.
Cases J ; 2: 6691, 2009 May 18.
Article in English | MEDLINE | ID: mdl-19829843

ABSTRACT

INTRODUCTION: Oesophageal carcinomas represent 3% of all cancers in the UK accounting for 7650 new cases per annum. Oesophageal cancer may be associated with swallowing abnormalities, localised mass pressure effects, lymphatic or distant metastatic spread. CASE PRESENTATION: We report a 50-year-old man who presented with a painful, enlarging, solid, fixed lesion adjacent to the left buttock with associated dysphagia. Initial endoscopic assessment suggested severe oesophageal inflammation while the lesion in the buttock area was presumed to be a primary soft-tissue neoplasm. However, subsequent histological assessment confirmed a primary oesophageal squamous carcinoma with metastatic spread to the buttock. CONCLUSION: We discuss the clinical presentation, investigative modalities, and current therapeutic guidelines associated with this rare metastasis and present other atypical oesophageal musculoskeletal metastases. We emphasise the need to consider all aspects of patient symptomatology during the investigation of any atypical lesion.

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