A Narrative Review of Pheochromocytoma in VHL.

This systematic review aims to investigate the clinical presentation, diagnostic methods, and management strategies for pheochromocytoma in patients with von Hippel-Lindau (VHL) disease, an autosomal dominant disorder that predisposes individuals to the development of various tumors, including pheochromocytomas. Pheochromocytoma is a rare neuroendocrine tumor of the adrenal medulla that occurs sporadically or as part of an inherited syndrome. The incidence of pheochromocytoma in VHL patients is estimated to be between 10-20%, making it the second most common tumor associated with VHL. Early detection and management of pheochromocytoma in VHL patients are critical for patient outcomes, as these tumors can cause severe hypertension, cardiovascular complications, and death. This review highlights the importance of screening for pheochromocytoma in VHL patients and discusses the current diagnostic and management strategies to optimize patient care.

Robotic Radical Nephrectomy with Vena Cava Thrombus Extraction (RRN-VCTE) for Renal Cell Carcinoma: A Meta-Analysis of Surgical Technique and Outcomes.

Renal cell carcinoma (RCC) with vena cava tumor thrombus is a challenging condition, which requires complex surgical management. Robotic radical nephrectomy with vena cava thrombus extraction (RRN-VCTE) has emerged as a promising and minimally invasive technique. This meta-analysis aims to review the surgical technique and outcomes of RRN-VCTE in patients with RCC and vena cava tumor thrombus. A comprehensive literature search was conducted using databases, including PubMed, Embase, and Cochrane Library. Studies published in English till October 2021 were included. Keywords used for the search included "robotic radical nephrectomy," "vena cava tumor thrombus," "surgical technique," and "outcomes." Studies that reported on patient outcomes and surgical techniques of RRN-VCTE were included. Statistical analysis was performed to assess the pooled outcomes. The meta-analysis included 16 studies comprising 298 patients who underwent RRN-VCTE. The majority of patients were males (62.4%) with a median age of 58.9 years. The median tumor size was 7.2 cm, and 93.9% of patients had level 3 or 4 vena cava thrombus. The mean operating time was 328 min, with a range of 248-423 min. Blood loss ranged from 100 to 1500 mL. The overall complication rate was 26.5%, with no reported deaths. The average hospital stay was 9.5 days. The 2-year and 5-year survival rates were 77.5 and 53.1%, respectively. RRN-VCTE is a promising and minimally invasive surgical technique for RCC with vena cava tumor thrombus, whch is associated with low complication rates and acceptable oncological outcomes. Further research is needed to confirm the long-term survival rates and compare RRN-VCTE outcomes with conventional surgical techniques. Nonetheless, RRN-VCTE appears to be a valuable option for patients with RCC and vena cava tumor thrombus.

Polycystic Kidney Disease and Polycystic Liver Disease Associated to Advanced Gastric Cancer: an External Complication of Potter III Disease.

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent of the hereditary diseases affecting the kidney but it can also be associated with cysts in the pancreas, liver, arachnoid, seminal vesicles. In particular, ADPKD plus adult polycystic liver disease (APLD) is defined as Potter type III. In the literature, the association of malignant neoplasm with APLD and ADPKD is extremely rare. We have found only nine cases. Only one report described multiple gastric carcinomas associated with Potter type III cystic disease. We described a case of a man suffering from ADPKD and APLD plus a stage IV (TNM) gastric adenocarcinoma.

Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review.

Multiorgan tumors are a hallmark of the autosomal dominant genetic disorder known as Von Hippel-Lindau syndrome (VHL), which is typically the result of inherited aberrations of the VHL tumor suppressor gene. The most frequent cancer is retinoblastoma, which can also occur in the brain and spinal cord, renal clear cell carcinoma (RCCC), paraganglioma, and neuroendocrine tumors. There may also be lymphangiomas, epididymal cysts, and pancreatic cysts or pancreatic neuroendocrine tumors (pNETs). The most frequent causes of death are metastasis from RCCC and neurological complications from retinoblastoma or central nervous system (CNS). Pancreatic cysts are present in 35-70% of VHL patients. Simple cysts, serous cysts, or pNETs are possible presentations, and the likelihood of malignant degeneration or metastasis is no greater than 8%. Although VHL has been associated with pNETs, their pathological characteristics are unknown. Furthermore, it is unknown whether variations in the VHL gene cause the development of pNETs. Hence, this retrospective study was undertaken with the main aim to examine whether pNETs are connected to VHL from a surgical perspective.

Robotic thymectomy: a review of techniques and results.

Thymectomy is a well-established therapeutic option in the multidisciplinary treatment of nonthymomatous myasthenia gravis (MG) and in thymoma treatment. Although many surgical procedures for thymectomy have been identified, the transsternal method is still regarded as the gold standard. Minimally invasive procedures, on the other hand, have achieved popularity in the last decades and are now extensively used in this field of surgery. Among them, robotic thymectomy has been the most cutting-edge surgical procedure. Several authors and meta-analyses have shown that a minimally invasive approach to thymectomy is associated with improved surgical results and fewer complications in surgery compared to transsternal open thymectomy, without any substantial changes in myasthenia gravis complete rates of remission. Hence, in the present review of the literature, we aimed to describe and delineate the techniques, advantages, outcomes, and future perspectives of robotic thymectomy. Existing evidence suggests that robotic thymectomy will likely become the gold standard for thymectomy in early stage thymomas and MG subjects. Many of the drawbacks related to other minimally invasive procedures appear to be resolved by robotic thymectomy, and long-term neurological outcomes are satisfactory. In addition, improved vision and high dexterity of instrument movements enable safe and complete thymic tissue dissection, superior to standard thoracoscopic procedures. The access with minimally invasive surgery VATS (video-assisted thoracoscopic surgery) or RATS (robot-assisted thoracic surgery) access in its various variants allows the extent of mediastinal fat resection due to the possibility of ectopic thymic foci in the mediastinum determining the long-term outcome in the group of patients operated on for myasthenia gravis. However, it was recommended to carry out better designed, multi-centre, randomized studies to arrive at definitive conclusions on robotic thymectomy for thymomas and myasthenia gravis treatment.

Image-guided techniques for localization of pulmonary nodules during video-assisted thoracoscopic surgery lobectomy.

In the modern era when screening and early surveillance of pulmonary nodules are increasing in importance, the management of the pulmonary nodule represents a different challenge to thoracic surgeons. The difficulty lies in the merging of sound surgical and oncological principles with more minimally invasive and appropriate lung-sparing surgery. The success rates of video-assisted thoracoscopic surgery (VATS) resection for smaller as well as subsolid nodules have increased as a result of radiologists' preoperative localization tools. Fast tracking in thoracic surgery is promoted by proper postoperative care for patients having lobectomies in combination with the VATS technique. Image-guided surgery refers to the application of a real-time correlation of the surgical field to preoperative imaging data collection that reflects the precise placement of a chosen surgical instrument in the adjacent anatomic structures. Among the cross-sectional digital imaging techniques the most widely utilized imaging modalities for image-guided therapy are computed tomography and magnetic resonance imaging. Additionally, surgical navigation devices, tracking tools, integration software, ultrasound, and angiography are used to support these procedures. For people who are thinking about implementing or optimizing a nodule localization program in view of workflow patterns, surgeon preferences, and institutional resources in a certain facility, this review provides in-depth, unbiased evaluation and offers useful information.

Outcomes and Complications of Duodenopancreatectomy in Octogenarian Patients: a Review.

Background: Duodenopancreatectomy is a surgical procedure that involves the removal of part of the pancreas, duodenum, and bile ducts. This procedure is commonly performed in patients with pancreatic cancer or other gastrointestinal disorders. However, the safety and efficacy of duodenopancreatectomy in older adults (octogenarians) remain unclear. Objectives:The goal of this review is to assess the outcomes and complications of duodenopancreatectomy in octogenarian patients. Methods:A systematic search of relevant literature was conducted using PubMed, Embase and the Cochrane Library databases. Studies reporting the outcomes and complications of duodenopancreatectomy in octogenarian patients were included. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed. Egger's test was used to evaluate publication bias. Results:A total of 14 studies were included in this review. The outcomes of duodenopancreatectomy in octogenarian patients were generally favorable, with a median 30-day mortality rate of 3.5% (range 0-16.7%). The most common complications were pancreatic fistula (12.2%), delayed gastric emptying (6.3%) and wound infection (5.5%). The overall long-term survival rate of octogenarian patients after duodenopancreatectomy was 21.2%. Conclusions:Duodenopancreatectomy can be safely performed in carefully selected octogenarian patients with good outcomes. However, this procedure is associated with a high risk of complications, particularly pancreatic fistula, in this age group.

A Review on Treatment of Perforated Peptic Ulcer by Minimally Invasive Techniques.

Peptic ulcers are caused by acid peptic damage to the mucosal layer in the gastro-duodenal area of the gut, which results in mucosal erosion that exposes the underlying tissues to the digestive action of gastro-duodenal secretions. This pathology was traditionally related to a hypersecretory acid environment, dietary factors and stress. There are other causes of ulcers such as Helicobacter pylori infection, excessive use of NSAIDs, and smoke and alcohol abuse. Perforation and bleeding are two major complications of the disease. A typical symptom of perforated peptic ulcers (PPU) is a sudden onset of abdominal pain or acute deterioration of the ongoing abdominal pain. Perforated peptic ulcer can be diagnosed by a simple X-ray and CT scan of the abdomen. Laboratory tests are also run to rule out differential diagnosis. Although there are several choices for surgical intervention, minimally invasive techniques have been taking over as a frequent option in feasible cases. Techniques like laparoscopy have been surfacing because of their advantages. But the efficiency of minimally invasive techniques compared to conventional approach is yet to be defined. Hence, the present review of the literature aims to describe and delineate the current perspective on PPU management by minimally invasive and low risk techniques.

Current perspective on uniportal and multiportal video-assisted thoracic surgery during lobectomy for lung cancer.

Non-small cell lung cancer (NSCLC) is the most typical type of lung cancer, and it is the leading cancer-related mortality globally. Lobectomy for early-stage NSCLC has been characterized in the previous decade using a wide range of methodologies. The development of video-assisted thoracoscopic surgery (VATS) allowed surgeons first to reduce the thoracotomy size, which is generally anterior, limiting it to trocar incisions or a single portal approach. This review aimed to describe current perspectives on operative outcomes, lymph node removal, oncologic outcomes, and advantages for surgeons performing uniportal VATS (uVATS) and multiportal (mVATS) lobectomy. The advantages of uVATS include comfortable operating for surgeons with a direct view and safety, and for patients more favourable operative outcomes. Also, the uVATS approach has previously been demonstrated to be effective and safe, with positive outcomes not just with respect to cosmetics but also in terms of a speedy recovery. Oncological uVATS clearance is comparable to multiportal VATS with respect to early mid-term survivability as well as nodal staging, as per retrospective comparison studies. However, the interpretation of outcomes must be made cautiously due to selection bias as well as lack of long-term follow-up; the choice of which VATS approach to utilize for patients' treatment following pulmonary resection is largely based on the preferences of the surgeon. As a result, it is difficult to say if one VATS method is better than another.

Small bowel obstruction: a prognostic score index for surgery - a review.

Bowel obstruction is an important cause of morbidity and mortality, accounting for nearly 30,000 deaths and more than $3 billion per year in direct medical costs. Small bowel obstruction (SBO) is caused mainly by postoperative adhesions. Adhesive small bowel obstruction (ASBO) is one of the leading causes of surgical emergencies and in particular of surgical emergencies that require an emergent operation. Hence, in this review article we attempted to narrate the role of the prognostic score index in the management of small bowel obstruction. Various prediction score index models developed by various authors were discussed in this review article, with the aim to delineate the role of the prognostic score index for making a decision about the surgery of patients suffering from SBO to prevent further complications like strangulation, malignancy etc. The use of prediction score index models was deliberated in this review article; widespread implementation in the treatment of patients with SBO has the potential to improve patient outcomes and reduce resource consumption.

Use of the Desarda Technique in Emergency Settings: a Comprehensive Review.

One of the most frequent surgeries performed across the world is an inguinal hernia repair, with at least twenty million people undergoing this procedure each year. The prevalence of groin hernia, defined as projections of adipose tissue or viscera through the femoral or inguinal canal, is 3-6% for women and 27-43% for men. Obstruction of the small bowel is one of the most frequent reasons for a strangulated hernia. The risk of strangulation per annum for a groin hernia is approximately 1-3%. The surgical method used to treat an emergency inguinal hernia surgery remains controversial. The elevated risk of complications and infections using prosthetic as opposed to pure tissue repair accounts for an increased likelihood of contamination of the surgical site and necrosis. To ascertain the utility of the Desarda technique in emergency hernia surgery, a systematic review in conjunction with a meta-analysis was performed, with a particular focus on duration of hospital stay, estimated operating time, relapse rate, and postoperative complications.

Giant Retroperitoneal Mass: Case Report in a Primary Hospital and Review of the Literature.

Retroperitoneal liposarcoma (RPLS) is a rare form of cancer that mostly affects adults. Symptoms may be undetected unless the tumor begins to grow. Diagnosis is made when the tumor is large and puts vital organs such as the pancreas, liver, kidneys at risk. Symptoms of a liposarcoma depend on tumor location. Liposarcoma in the arms, legs, particularly thighs may be painful, swollen, with patients citing weakness in the affected area; 15% of soft tissue sarcomas are retroperitoneal, with two of the most common types being the well-differentiated liposarcoma (WDLPS) and high-grade dedifferentiated liposarcoma (DDLPS). The tumor can weigh between 15 kg and 20 kg, with 20 to 25 cm in diameter (4). Surgery is the main form of treatment provided along with adjuvant therapies. We present the case of a 60-year-old woman who was referred to the Emergency Department with acute onset of abdominal distension, nausea, vomiting, right back pain, lipothymia and a giant abdominal mass.

Laparoscopic distal pancreatectomy for benign and malignant disease: a review of techniques and results.

Diagnosis of pancreatic cancer is challenging in the initial phases because its progression is rapid. The pancreatic tail and body roughly accounts for 20-30% of all cancerous cases. The standard treatment for symptomatic benign, malignant, and premalignant diseases of the pancreatic tail and body is distal pancreatectomy. This technique has been modified over the years to fit certain indications, with the goal of enhancing post-operative results as well as reducing patient trauma. In cases of a premalignant and symptomatic benign condition, the spleen must be preserved either using Kimura's splenic vessel preservation technique or Warshaw's splenic vessel resection technique. A better long-term prognosis is ensured by regional lymph node dissection and radical R0 resection. Radical antegrade modular pancreatosplenectomy was proposed to tackle the shortcomings of traditional surgery for pancreatic tail and body cancer. In this review, study techniques and results of laparoscopic distal pancreatectomy for malignant and benign pancreatic ailments were described with the intention of providing knowledge on various suitable techniques reported for pancreatic cancer treatment. Furthermore, this study will serve as a ready reckoner for surgeons and could serve to boost their confidence levels during surgery by avoiding confusion on the selection of suitable for the pancreatic diseases diagnosed.

Von Hippel-Lindau Syndrome: Medical Syndrome or Surgical Syndrome? A Surgical Perspective.

Von Hippel-Lindau syndrome (VHL) is an autosomal dominant disease caused by a genetic aberration of the tumor suppressor gene VHL and characterized by multi-organ tumors. The most common neoplasm is retinal or cerebral hemangioblastoma, although spinal hemangioblastomas, Renal Clear Cell Carcinoma (RCCC), pheochromocytomas (Pheo), paragangliomas, Pancreatic Neuroendocrine Tumors (PNETs), cystadenomas of the epididymis, and tumors of the lymphatic sac can also be found. Neurological complications from retinal or CNS hemangioblastoma and metastases of RCCC are the most common causes of death. There is a strong association between pheochromocytoma and VHL syndrome, and pheochromocytoma is often a classic manifestation of the syndrome. RCCCs are often incidental and identified during other tests. Between 35 and 70% of patients with VHL have pancreatic cysts. These can manifest as simple cysts, serous cysto-adenomas, or PNETs with a risk of malignant degeneration or metastasis of no more than 8%. The objective of this retrospective study is to analyze abdominal manifestations of VHL from a surgical point of view.

Clear Cell Hidradenocarcinoma: a Rare Identity Difficult to Recognize - Case Report and Review of the Literature.

Hidradenocarcinoma (HC) is a malignant and aggressive sweat gland tumour. It is a rare occurrence, accounting for about 6% of malignant eccrine cancers and less than 1% of all cutaneous neoplasms. It is a malignant tumor which can manifest itself in any area of the body, including the anterior surface of the trunk, the torso, scalp, face, and extremities. It is apparent that HC has a high propensity for lymphatic metastasis. The suggested treatment approach for HC implies making a wide surgical excision with negative margins. Nevertheless, such tumours are aggressive and recur locally in 50% of cases; they also produce metastases in 60% of cases. The overall five-year survival rate is 30%. Here, we present the case of a 68-year-old Caucasian male with unknown diagnosis of malignant neoplasm of inguinal cutaneous skin of sweat gland called hydrocarcinoma.