Emergency laparotomy for abdominal catastrophes secondary to lymphoma: A systematic review.

Intestinal lymphomas can rarely present as abdominal catastrophes with perforation or small bowel obstruction. There is little data regarding their optimal surgical management and associated outcomes. We aimed to systematically review relevant published literature to assess the presentation, diagnosis, optimal surgical approach and associated post-operative outcomes. A systematic on-line literature search of Embase and Medline identified 1485 articles of which 34 relevant studies were selected, including 7 retrospective studies, 1 case series and 26 case reports. Selected articles were assessed by two reviewers to extract data. 95 patients with abdominal catastrophes secondary to lymphoma (predominately Burkitt (28 %) and Diffuse Large B-cell lymphoma (29 %)) were identified with a median age of 52 years, 40 % were female. Of the small bowel resections 25% (n = 18) suffered post-operative complications with a 13.8 % (n = 10) 30-day mortality. Ileocolonic resections had a 27 % complication rate with 18 % mortality and primary repair had a 25 % complications rate and 25 % mortality. Median follow-up was 8 days (range 1-96). Notable points of differences in the presentations between these different lymphomas included the majority of Burkitt's lymphoma were younger, had a known diagnosis, were on chemotherapy and presented with perforation in contrast to those with B cell lymphoma who were predominately older, had new diagnoses and presented with a balanced proportion of obstruction and perforation. Abdominal catastrophes secondary to intestinal lymphomas most commonly present with perforation. Aggressive surgical management, including small bowel resection, may offer similar remission rates for lymphoma patients presenting with abdominal catastrophes as those without such emergency complications.

Dual-modality emergency management of a rare cause of an aorto-oesophageal fistula: a case report.

Aorto-oesophageal fistula (AOF) is a life-threatening condition that usually presents with upper gastro-intestinal haemorrhage. This case report details the emergency presentation and management of a 51-year-old male who presented with hematemesis secondary to an impacted denture (ingested two years previously) in the oesophagus that had led to an AOF. This necessitated urgent thoracic endovascular aortic repair followed by thoracotomy, oesophagotomy, T-tube insertion and oesophagostomy. This is the first documentation in the literature of the dual-modality management for this rare cause of AOF and demonstrates the multidisciplinary approach to successful management of this complex yet rare presentation.

The surgical management of non-malignant aerodigestive fistula.

BACKGROUND: Acquired aerodigestive fistula (ADF) are rare, but associated with significant morbidity. Surgery affords the best prospect of cure. We present our experience of the surgical management of ADFs at a specialist unit, highlighting operative techniques, challenges and assess clinical outcomes following intervention. We also illustrate findings of a Hospital Episodes Statistics search for ADFs. METHODS: A prospectively-maintained database was searched to identify all patients diagnosed with an ADF who were managed at our institution. Of 48 patients with an ADF, eight underwent surgical intervention. RESULTS: Four patients underwent an exploration of the ADF with primary repair of the defect. Two of these patients had proximal ADFs, amenable to repair through a neck incision, and two required a thoracotomy. Two patients suffered fistulae secondary to endoscopic therapy and underwent oesophageal exclusion surgery, with subsequent staged reconstruction. Two patients with previous Tuberculosis had a lung segmentectomy and lobectomy respectively, and a further patient in remission after treatment for lymphoma underwent oesophageal resection with synchronous reconstruction. Three patients suffered a complication, with one post-operative mortality. The remaining seven patients all achieved normal oral alimentation, with no evidence of ADF recurrence at a median follow-up of 32 months. CONCLUSIONS: Surgery to manage ADFs is effective in restoring normal alimentation and alleviates soiling of the airway, with a very low risk of recurrence. Several operative techniques can be utilised dependent on the features of the ADF. Early referral to specialist units is advocated, where the expertise to facilitate the complete management of patients is present, within a multi-disciplinary setting.

Acquired Adult Aerodigestive Fistula: Classification and Management.

BACKGROUND: Acquired aerodigestive fistulae (ADF) are rare, but associated with a high mortality rate. We present our experience of the diagnosis, management and outcomes of patients with ADFs treated at a tertiary centre. Utilising our findings, we propose an anatomical classification system, demonstrating how specific features of an ADF may determine management. METHODS: A clinical database was searched and 48 patients with an ADF were identified. A classification system was developed based on anatomical location of the ADF and differences in clinico-pathological features based on this categorisation were performed, with the chi-squared test used for inferential analyses and Kaplan-Meier curves with log-rank test to assess survival. RESULTS: Twenty (41.6%) patients developed an ADF secondary to malignancy, with previous radiotherapy (18.7%), post-operative anastomotic dehiscence and endotherapy (14.6% each) representing other causes. Thirty-one patients were managed with tracheal and/or oesophageal stents and eight underwent surgical repair. The classification system demonstrated benign causes of ADF were either proximally or distally sited, whilst a malignant cause resulted in mid-tracheal fistulae (p = 0.001), with the latter associated with poorer survival. ADFs over 20 mm in size were associated with poor survival (p = 0.011), as was the use of previous radiotherapy. Proximal and distal ADFs were associated with improved survival (p = 0.006), as were those patients managed surgically (p = 0.001). CONCLUSION: By classifying ADFs, we have demonstrated that anatomical location correlates with the size, history of malignancy, previous radiotherapy and aetiology of ADF, which can affect management. The proposed classification system will aid in formulating multi-modality individualised treatment plans.

Intraoperative Pyloric Interventions during Oesophagectomy: a Multicentre Study.

BACKGROUND: Denervation of the pylorus after oesophagectomy is considered the principal factor responsible for delayed gastric emptying. Several studies have attempted to delineate whether surgical or chemical management of the pylorus during oesophagectomy is of benefit, but with conflicting results. The aim of this multicentre study was to assess whether there was any difference in outcomes between different approaches to management of the pylorus. METHODS: A prospectively maintained database was used to identify patients who underwent oesophagectomy for malignancy. They were divided into separate cohorts based on the specific pyloric intervention: intra-pyloric botulinum toxin injection, pyloroplasty and no pyloric treatment. Main outcome parameters were naso-gastric tube duration and re-siting, endoscopic pyloric intervention after surgery both as in- and outpatient, length of hospital stay, in-hospital mortality and delayed gastric emptying symptoms at first clinic appointment. RESULTS: Ninety patients were included in this study, 30 in each group. The duration of post-operative naso-gastric tube placement demonstrated significance between the groups (p = 0.001), being longer for patients receiving botulinum treatment. The requirement for endoscopic pyloric treatment after surgery was again poorer for those receiving botulinum (p = 0.032 and 0.003 for inpatient and outpatient endoscopy, respectively). CONCLUSION: We did not find evidence of superiority of surgical treatment or botulinum toxin of the pylorus, as prophylactic treatment for potential delayed gastric emptying after oesophagectomy, compared to no treatment at all. Based on our findings, no treatment of the pylorus yielded the most favourable outcomes.

Minimally invasive and robotic esophagectomy: Evolution and evidence.

Esophageal cancer has a poor prognosis, with little improvement in outcomes in recent years. Surgery maintains its pivotal role in cure, but this involves two or three compartment dissection with associated high risks. Chiefly, pulmonary complications following surgery are most common, and can be life-threatening. As a consequence, minimally invasive and robotic esophagectomy have been performed with improving efficacy and equivalent oncological outcomes. This is a review of the pertinent literature regarding these techniques. J. Surg. Oncol. 2016;114:731-735. © 2016 Wiley Periodicals, Inc.

Parathyroid cancer.

Parathyroid carcinoma is an exceedingly rare endocrine malignancy first described in 1933. It accounts for between 0.5% and 5% of all cases of primary hyperparathyroidism. Parathyroid carcinoma is unusual among endocrine malignancies, being more hormonally active than its benign counterpart. Parathyroid carcinoma poses a diagnostic challenge both clinically and histologically due to the lack of features which can definitively distinguish malignant from benign disease early in its clinical course. Here, we describe the clinical features of the disease, and present the current opinion on optimal management. Further, we analyse the most recent histological advances made to aid in the diagnosis and management of this rare, but potentially devastating, disease.

An unusual ectopic location of a parathyroid carcinoma arising within the thyroid gland.

INTRODUCTION: Parathyroid carcinomas are very rare tumors and may arise in the normally located or ectopic gland. The latter present certain diagnostic difficulties, and there are no specific guidelines on their management. We present a case of parathyroid carcinoma arising within an ectopically located intrathyroid gland and discuss the diagnosis, management, and difficulties encountered. Furthermore, we review all six previously reported cases of this rare event and demonstrate the patterns in presentation, as well as the differences in management. CASE PRESENTATION: A 39-yr-old male presented with a right neck mass with biochemical derangement suggestive of a parathyroid lesion. However, radiological investigations were inconclusive as to the true nature of this lesion because they demonstrated a mass within the right thyroid lobe. A sestamibi (99m)technetium subtraction study was performed, which suggested an intrathyroid parathyroid carcinoma. The patient underwent successful surgical intervention. CONCLUSION: Although rare, ectopically located parathyroid glands can harbor malignant disease. Those located within the thyroid gland can be difficult to diagnose, and thus a combination of radiological modalities, including sestamibi (99m)technetium studies, need to be utilized. Although surgical resection is the most effective treatment, there are no specific guidelines as to the radicality of such treatment.

Trefoil factor family peptides in normal and diseased human pancreas.

OBJECTIVES: Trefoil factor family (TFF) peptides promote wound healing in the gut. Recent evidence has suggested that TFF3 may be a pancreatic mitogen, an unusual role for TFF peptides. We sought to clarify human pancreatic TFF and mucin expression and performed in vitro experiments to see how pancreatic cell lines respond to TFF3 in particular. METHODS: Samples of normal and diseased pancreas (chronic pancreatitis, pancreatic intraepithelial neoplasia, neuroendocrine tumors, and pancreatic ductal adenocarcinoma [PDAC]) were studied by immunohistochemistry and in situ hybridization. Pancreatic cell lines were challenged with TFF2 and TFF3 in wound and migration assays. RESULTS: In normal islets, colocalization of insulin or glucagon with TFF3 was common. All TFF messenger RNAs were seen in ductal epithelium. Adenocarcinomas expressed all TFF messenger RNAs. Normal ducts were mucin free; MUC5AC was strongest in pancreatic intraepithelial neoplasia and chronic pancreatitis but was reduced in PDAC. TFF2 induced Panc-1 migration and accelerated wound closure in Capan-2 and COLO-357. Double immunohistochemistry for insulin or TFF3 and Ki67 colabeled only very rare islet cells. TFF3-positive PDAC ducts showed some Ki67 colocalization. CONCLUSIONS: No correlation between TFF3 or insulin and Ki67 was seen without ductal hyperplasia. TFF2 may assist pancreatic tumor cell movement, but TFF3 may not be a pancreatic mitogen.

How to do it: the difficult thyroid.

There is a paucity of publications detailing how to deal with the difficult thyroid cancer. When compared to other cancers, it is relatively rare with several histopathological subtypes which run differing clinical courses and respond to different therapies. It is a condition predominately treated by specifically trained General and now ENT surgeons who already have a thorough knowledge of vocal fold assessment and rehabilitation as well as emergency airways management both to avoid and treat common complications should they occur.Good surgery involves a team effort to produce good results consistently. All members of the team are essential to quality service delivery. Communication with the team and the patient is paramount. We describe our approach to the difficult thyroid.

Management of benign retroperitoneal schwannomas: a single-center experience.

BACKGROUND: We describe the presentation, diagnostic pathway, and management of 28 patients with benign retroperitoneal schwannomas. METHODS: A prospectively kept soft-tissue tumor database was reviewed to identify patients who presented to the Royal Marsden Hospital with retroperitoneal schwannomas. RESULTS: From 2001 to 2009, 28 patients presented with retroperitoneal schwannomas. In 13 patients, tumors were identified incidentally, 8 patients presented with pelvic or abdominal symptoms and 7 patients presented with a palpable mass. Seventeen occurred in the pelvis and 11 occurred in the abdominal retroperitoneal space. The median age was 47 years and 21 patients were women; the mean follow-up period was 39 months. Twenty patients underwent resection and 8 patients were managed conservatively with radiologic surveillance. An initial histologic diagnosis was accomplished in 19 of 28 patients. Complete resection was achieved in 17 of 20 patients and 3 patients with pelvic schwannomas underwent a subtotal resection. Resected tumor size ranged from 5 to 23 cm (median, 9 cm), and weighed between 64 and 2,300 g (median, 500 g). There was no surgical mortality. In the 3 subtotal resected tumors, no progression of residual disease or malignant transformation has been noted on follow-up imaging. All 8 patients in the surveillance group had a histologic diagnosis and typical radiologic findings. CONCLUSIONS: An accurate preoperative diagnosis is important because the risks of complicated surgery in the retroperitoneum and pelvis for what is a benign lesion should be considered carefully. Cross-sectional imaging combined with a needle biopsy should in all cases provide the correct diagnosis. Management options include radiologic surveillance in asymptomatic patients or surgical resection in symptomatic patients.

Angiosarcoma developing in a non-functioning arteriovenous fistula post-renal transplant.

BACKGROUND: Angiosarcomas comprise less than 1% of all sarcomas, arising from endothelial cells of blood or lymph vessels. Chronic immunosuppression increases the risk of many malignancies and an association between the development of angiosarcoma with an immunosuppressed state is established. A few cases have been reported of angiosarcomas arising in the post-renal transplant patient. Specifically, there have been six cases of an angiosarcoma arising in arteriovenous (AV) fistulae in this patient population. We describe a further case and review the relevant literature with specific emphasis on a possible mechanism for the development of angiosarcoma in the post-transplant patient. CASE PRESENTATION: We report the case of a 48-year-old male who developed an angiosarcoma in a ligated native AV fistula. The lesion arose on the background of immunosuppression following a successful ABO-incompatible renal transplant for chronic renal failure. CONCLUSION: Angiosarcomas are extremely rare tumours but should be considered as a differential diagnosis for an evolving mass near the site of an AV fistula. Diagnosis relies on an index of suspicion and obtaining a definitive histological diagnosis. Both clinicians and patients should be aware that an evolving mass within or around an AV fistula should prompt urgent biopsy.

The implications of fraud in medical and scientific research.

Fraud is an important issue that affects research of both scientific and clinical fields because of its potential to adversely affect patient care. Fraud can take many forms, each with varying prevalence and implications. A number of ethical institutions have been created to investigate and regulate this malpractice. Using case examples, we describe the various forms of fraud and their potential implications. The increasing importance of editorial responsibility is highlighted and discussed. Finally, we suggest solutions for how to effectively regulate and eradicate this practise.

Late widespread skeletal metastases from myxoid liposarcoma detected by MRI only.

BACKGROUND: Myxoid liposarcoma is the second most commonly occurring sub-type of liposarcomas. In contrast to other soft tissue sarcomas, it is known to have a tendency to spread toward extrapulmonary sites, such as soft tissues, retroperitoneum, and the peritoneal surface. Bony spread, however, is not as common. CASE PRESENTATION: We report an unusual case of diffuse skeletal metastases from myxoid liposarcoma occurring 13 years after treatment of the primary tumour in the left lower limb. The skeletal spread of the disease was demonstrated on MRI only after other imaging modalities (plain radiography, CT and TC99 bone scans) had failed to detect these metastases. CONCLUSION: MRI is an extremely sensitive and specific screening tool in the detection of skeletal involvement in these types of sarcomas, and therefore, should be a part of the staging process.