Incidence Trends and Main Features of Gastro-Intestinal Stromal Tumours in a Mediterranean Region: A Population-Based Study.

Gastro-Intestinal Stromal Tumours (GISTs) are a kind of neoplasm whose diagnosis in common clinical practice just started in the current century, implying difficulties for proper registration. Staff from the Cancer Registry of Murcia, in southeastern Spain, were commissioned by the EU Joint Action on Rare Cancers into a pilot study addressing GIST registration that also yielded a population-based depiction of GISTs in the region, including survival figures. We examined reports from 2001 to 2015 from hospitals as well as cases already present in the registry. The variables collected were sex, date of diagnosis, age, vital status, primary location, presence of metastases, and risk level according to Joensuu's Classification. In total, 171 cases were found, 54.4% occurred in males, and the mean age value was 65.0 years. The most affected organ was the stomach, with 52.6% of cases. Risk level was determined as "High" for 45.0%, with an increment of lower levels in recent years. Incidence for the year 2015 doubled that of 2001. Overall, the 5-year net survival estimation was 77.0%. The rising incidence magnitude is consistent with trends in other European countries. Survival evolution lacked statistical significance. A more interventional approach in clinical management could explain the increase in the proportion of "Low Risk GISTs" and the first occurrence of "Very Low Risk" in recent years.

Updated Review and Clinical Recommendations for the Diagnosis and Treatment of Patients with Retroperitoneal Sarcoma by the Spanish Sarcoma Research Group (GEIS).

Soft tissue sarcomas (STS) are an uncommon and biologically heterogeneous group of tumors arising from mesenchymal cells. The incidence is estimated at five cases per 100,000 people per year. Retroperitoneal sarcomas (RPS) account for 10-15% of all STS, and their management depends on their anatomical characteristics and histotype. Due to their very low incidence, it is recommended that RPS be treated in reference centers and evaluated by an experienced multidisciplinary team (MDT). In Spain, the Spanish Group for Research in Sarcomas (GEIS) brings together experts from various specialties to promote research on sarcomas and improve treatment results. This paper summarizes the GEIS recommendations for the diagnosis, treatment, and follow-up of patients with RPS.

Postoperative negative-pressure incision therapy after liver transplant (PONILITRANS study): A randomized controlled trial.

BACKGROUND: Postoperative complications of surgical incisions are frequent in liver transplantation. However, evidence justifying the use of incisional negative pressure wound therapy to improve surgical wound outcomes remains limited. METHODS: Participating patients were randomly assigned to receive incisional negative pressure wound therapy or standard surgical dressing on the closed surgical incision of the liver transplantation. The primary endpoint was surgical site infection incidence 30 days postoperatively. The secondary endpoints included surgical site events (ie, surgical site infection, dehiscence, hematoma, and seroma) and wound quality of life. RESULTS: Between December 2018 and September 2021, 108 patients (54 in the incisional negative pressure wound therapy group and 54 in the control group) were enrolled in this study. The incidence of surgical site infection at 30 days postoperatively was 7.4% in the treatment group and 13% in the control group (P = .34). The rate of surgical site events was similar in the treatment in the and control group (27.8% vs 29.6%, P = .83). In relation to wound quality of life, the mean score was 75.20 ± 7.27 in the incisional negative pressure wound therapy group and 72.82 ± 10.57 in the control group (P = .23). CONCLUSION: The prophylactic use of negative pressure wound therapy on primarily closed incisions did not significantly reduce incisional surgical site infection and surgical site event rates after liver transplantation compared with standard surgical dressings.

National survey on the treatment of sarcomas in Spain.

Surgical units attending sarcomas in Spain are poor studied. The aim is to know the management of this pathology to identify areas of improvement through multicenter study based on a voluntary survey. The survey was completed by 74 surgeons of different hospitals, which 32,4% is exclusively dedicated to sarcomas. Only 24.3% declared to receive specific training in sarcomas. The most frequent type of hospital was the third level (56.8%), where 38,1% of the surgeons belong to societies or working-groups in sarcoma fields vs. 9,4% in first-second levels. The number of surgeons with specific theoretical training and papers published in this field are higher in third level hospitals. 55,4% belonged to a multidisciplinary unit. A multidisciplinary team was available in 57% of third level hospital vs 28% in others. Most services in charge of this patients are characterized by deficient specialization, low workload and the absence of a multidisciplinary team.

Unresectable GIST liver metastases and liver transplantation: A review and theoretical basis for a new indication.

BACKGROUND: Liver metastases from gastrointestinal stromal tumors (GIST) can be found synchronously to a primary tumor and in the medium/long term after resection of a high-risk GIST. In these cases, treatment with tyrosine kinase inhibitors like imatinib has provided good results, but drug resistance is common after two years of treatment. Liver resection of potentially resectable metastases can improve the results of medical treatment. In cases of unresectability, the role of liver transplantation (LT) has not been well-studied. MATERIALS AND METHODS: A review of the literature was undertaken from January 1995 to December 2020 following the PRISMA and AMSTAR guidelines. RESULTS: Fifteen cases were identified. In eight cases, the LT was carried out before 2002. Mutational status was only studied in seven cases. LT was performed in 12 cases with a deceased donor and in three cases with a living donor. After a mean follow-up of 52.4 months, overall survival was 86.6% with disease-free survival of 53.3% CONCLUSION: LT in the management of unresectable GIST metastases has rarely been performed. Although its application has a solid theoretical basis, its use understood as a radical extension of a standard resection can only be recommended within prospective studies by groups with considerable experience in both GIST and transplantation care.

National survey on the treatment of sarcomas in Spain. / Encuesta nacional sobre el tratamiento de los sarcomas en España.

Surgical units attending sarcomas in Spain are poor studied. The aim is to know the management of this pathology to identify areas of improvement through multicenter study based on a voluntary survey. The survey was completed by 74 surgeons of different hospitals, which 32.4% is exclusively dedicated to sarcomas. Only 24.3% declared to receive specific training in sarcomas. The most frequent type of hospital was the third level (56.8%), where 38.1% of the surgeons belong to societies or working-groups in sarcoma fields vs. 9.4% in first-second levels. The number of surgeons with specific theoretical training and papers published in this field are higher in third level hospitals. 55.4% belonged to a multidisciplinary unit. A multidisciplinary team was available in 57% of third level hospital vs. 28% in others. Most services in charge of these patients are characterized by deficient specialization, low workload and the absence of a multidisciplinary team.

Clinical and pathological features of "small" GIST (≤2 cm). What is their prognostic value?

INTRODUCTION: Small GIST (<2 cm) are tumors whose biological behavior is benign and frequently involutes. Despite their increasing incidence, few studies have addressed the characteristics of these GIST. The aim of this work is to clarify the management of this entity. PATIENTS AND METHOD: The characteristics of ≤2 cm GIST were initially described, and then compared with those >2 cm. This series comprises 104 patients and they were divided according to tumor size in 4 groups: tumors which are ≤2 cm (group 1, G1), >2 and ≤ 5 cm (G2), >5 and ≤ 10 cm (G3) and >10 cm (G4). RESULTS AND DISCUSSION: Most of small GIST were asymptomatic and incidental, and were located in the stomach. There is an association between patients with associated tumors and asymptomatic GIST. A high overall mortality rate of up to 40% is observed being disease-specific mortality 4.5%. The disease-specific mortality increases proportionally with size. The overall survival (OS) at 5 years are lower for both <2 cm (61%) and >10 cm (53%) than the rest (85-91%). When analyzing the impact of tumor association on <2 cm GIST, we observed that the OS of patients with non-associated tumors was much higher than in the associated ones (90% vs 32% at 5 years, respectively), while no differences were observed in the disease specific survival. CONCLUSIONS: Small GIST are tumors that are very often incidentally discovered in the course of complementary examinations. Its prognosis is very good, but it depends on the associated tumor.

Right-colon mucinous adenocarcinoma mimicking a hydatid cyst.

Hydatid disease is a fecal-oral transmission zoonosis which is endemically distributed among Mediterranean countries such as Morocco. It usually presents asymptomatically and a diagnosis is made due to cyst complications. It is most frequently located in the liver and lungs and forms large masses. The incidence of colon cancer has increased amongst population under 50 years of age during the last few decades. It has been proposed that young adult cases may have histological characteristics with a more aggressive clinical behavior. These patients are frequently diagnosed in advanced stages and a mucinous histology is frequently detected. Diagnosis is usually delayed due to a low clinical suspicion and the attribution of symptoms to other diseases such as irritable bowel and parasitosis, among others.

Utility of neoadjuvant therapy in rectal GIST.

Neoadjuvant therapy with Imatinib is the preferred treatment for rectal GISTs in order to reduce the aggressiveness of a surgical resection. At present, surgical resection does not require wide surgical margins. There is also a lot of controversy with regard to the need for a broad anatomical resection or if a local resection is sufficient and maintains maximum oncological safety and minimum morbidity and mortality. We present a case report of a rectal GIST with a good response to neoadjuvant treatment which allowed a transanal resection.

[Controversies in the surgical treatment of primary gastrointestinal stromal tumours (GIST)]. / Controversias en el tratamiento quirúrgico de los tumores del estroma gastrointestinal (GIST) primarios.

It is estimated that 50% of GIST are located at the time of their diagnosis. A complete surgical resection can be performed in up to 95% of these cases, making this the most important prognostic factor. This surgery must fulfil a series of technical requirements so as to be really effective, as it has to be R0, with no tumour rupture, with preservation of the pseudo-capsule, etc. Although the majority of GIST are gastric, their location in other anatomical areas, such as the oesophagus, duodenum or rectum, require the surgeon to use more complex techniques. Laparoscopy is increasingly used; however, we must avoid its use, due to there being few experienced groups or if there are large tumours. The use of neoadjuvant therapy has revived great interest by allowing to resection tumours that were once non-resectable or in very compromised anatomical locations, with less aggressive surgery. The use of pre-surgical biopsy is not exempt from serious risks, thus it should only be used for establishing a neoadjuvant treatment or if there are diagnostic doubts with other diseases in high risk patients.

[Surgical treatment of an advanced GIST the age of imatinib]. / Tratamiento quirúrgico del GIST avanzado en la era del imatinib.

The use of imatinib in the management of gastrointestinal stromal tumours has radically changed their prognosis, particularly in their advanced forms, whether they are metastasic (the majority) or locally advanced. The high rates of response obtained means that, in many cases, surgery can be performed in situations where it was impossible to do so, even to the extent of considering surgery as a first line therapeutic weapon in combination with imatinib. Even so, it must not be used indiscriminately. It will be the different responses of these tumours to imatinib that will determine its usefulness and the way it is used. The combined use of surgery and imatinib is a clear and successful example of multimodal treatment in the context of the so-called targeted molecular therapy.

[Intra-abdominal hypertension and abdominal compartment syndrome. What should surgeons know and how should they manage these entities?]. / La hipertensión intraabdominal y el síndrome compartimental abdominal: qué debe saber y cómo debe tratarlos el cirujano?

Correct monitoring of medicosurgical critically-ill patients aids the early diagnosis and appropriate treatment of intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS). The abdominal cavity and the retroperitoneum act sealed compartments and any change in the volume of their contents can increase intraabdominal pressure (IAP). IAH is only one measure of elevated IAP, and ACS represents the end result of sustained IAH with the appearance of organ dysfunction. To diagnose IAH and ACS, measurement of IAP, abdominal perfusion pressure and intramucous gastric pH must be performed and the results correlated with signs of clinical deterioration in the patient. Medical therapeutic measures in ACS are limited and abdominal decompression is the established symptomatic treatment of this entity.

[What are the main errors made by surgeons in the management of pancreatic cancer?]. / Cuáles son los principales errores que cometemos los cirujanos en el tratamiento del cáncer de páncreas?

The present review identifies two major conceptual errors. Therapeutic nihilism, which should be discounted in view of the results currently achieved by surgery, and noncentralization, since better results have been demonstrated, both in terms of morbidity and mortality and in survival, in high-volume centers than in low volume centers. The present review also identifies errors in management, the most important of which are: undervaluing the medical record, which is of great utility and continues to be the pillar on which the entire diagnostic process is based; the systematic use of preoperative biliary drainage, which used to be considered mandatory but should be used highly selectively in patients with severe jaundice or biliary tract infections, and viewing preoperative imaging tests as unreliable, when current radiological techniques, particularly helical computed tomography (CT), are highly reliable in establishing tumor resectability and consequently they should be used in all treatment planning. Moreover, because radiological tests are highly reliable, laparoscopic staging has lost diagnostic value; obtaining a preoperative histological diagnosis, which is not mandatory except when neoadjuvant therapy is planned or when tumors requiring nonsurgical treatment are suspected; undervaluing the use of surgical palliation, since this technique provides better long-term results than nonsurgical palliation, and consequently still plays a role in patients with good general health status and prolonged life expectancy; systematically performing gastrojejunostomy with bilio-enteric bypass, as this procedure should only be performed in tumors of the uncus or when there is imminent biliary or gastroduodenal obstruction; the use of supraradical surgical techniques such as regional, total or extensive pancreatectomy, since these techniques do not prolong survival after resection. Furthermore, the use of vascular resections would only be justified if resection with disease-free margins could be performed; undervaluing close postoperative monitoring within specialized units since this is the key to reducing morbidity and mortality rates in this type of surgery; and lastly when an intraoperative pancreatic incidentaloma is present, performing diagnostic maneuvers such as biopsy or pancreatic mobilization, since these procedures hamper subsequent radiological interpretation and possible surgical intervention.

Translumbar retroperitoneal endoscopy: an alternative in the follow-up and management of drained infected pancreatic necrosis.

BACKGROUND: The follow-up of drained infected pancreatic necrosis (IPN) is usually done with data on the patient's clinical evolution and information obtained from serial helical computed tomographic scans. Management often requires necrosectomies and periodic debridements. HYPOTHESIS: Translumbar retroperitoneal endoscopy is effective in the management of drained IPN. DESIGN: A prospective observational study. SETTING: University tertiary care hospital. PATIENTS: A series of 11 consecutive patients with drained IPN undergoing postoperative follow-up with translumbar retroperitoneal endoscopy. INTERVENTIONS: Initially, the IPN was drained via the posterior extraperitoneal translumbar approach; then, a superficial necrosectomy was performed during the same surgical intervention by flushing and endoscopic aspiration; and, finally, a lavage and drainage system was fitted. In the immediate postoperative period, for management of the IPN, we removed the drainage tube and inserted a flexible endoscope as far as the pancreatic area to eliminate the infected necrotic material by flushing and aspiration. MAIN OUTCOME MEASURES: In these patients, we studied control of the infection of the pancreatic area, quantification variables of the necrosectomy, technique-related morbidity and mortality, and the need for subsequent operations. RESULTS: The 11 patients studied showed good results regarding the control and complete elimination of the infected necrosis. There was no technique-related morbidity or mortality or need for subsequent operations. CONCLUSION: Translumbar retroperitoneal endoscopy allows exploration of the retroperitoneal space under direct visual guidance, facilitates lavage and aspiration, avoids subsequent surgical operations for debridement, decreases the need for repeated computed tomographic scans to evaluate the evolution of the IPN, and has no added morbidity or mortality.

[Toward zero mortality in liver resection. Presentation of 200 consecutive cases]. / Hacia la mortalidad cero en las resecciones hepáticas. Presentación de 200 casos consecutivos.

INTRODUCTION: Liver resection (LR) morbidity and mortality rates have dropped in recent decades. Mortality is now below 5% and morbidity is less than 30%. Our objective was to present a series of 200 LRs without mortality and to analyze the factors that may be related to complications. PATIENTS AND METHOD: Between January 1996 and October 2003, 200 LRs were performed in 177 patients. The most common indication was liver metastases in 123 patients (61.5%), primary malignant liver tumors in 27 patients (13.5%), bile duct tumors in 27 patients (13.5%) and benign disease in 23 patients (11.5%). Fifty-one percent of the resections were performed under hemihepatic vascular control and 49% were resections of central segments, segmentary and atypical resections. We studied the association between morbidity and age, sex, previous comorbidity, liver status, indication for surgery, number of resections, major and minor resections, resection extended to other organs, type of vascular occlusion, transfusion requirements, operating time, length of hospital stay and experience of the surgical team. RESULTS: There was no postoperative mortality. The morbidity rate was 17.5% (35 patients) and the most common complications were biliary (8%). Morbidity was related to transfusion (transfused patients presented more complications) (P < .001). Transfusion was greater in major resections, the first 100 resections and prolonged operations. Among the segmentary resections the Pringle maneuver reduced transfusion requirements but this difference was not statistically significant. Morbidity decreased in the second 100 resections, without significant differences. CONCLUSION: LRs can be performed with low mortality and morbidity. Biliary complications and blood transfusion should be avoided whenever possible.

[Liver transplantation in the management of unresectable bilateral neuroendocrine metastases]. / Trasplante hepático en metástasis hepáticas irresecables de origen neuroendocrino.

INTRODUCTION: Neuroendocrine tumors are rare neoplasms that present bilateral liver metastases at diagnosis. Because of the good initial results obtained, liver transplantation is considered as a potentially curative treatment. OBJECTIVE: Present our experience of the use of liver transplantation in the management of unresectable bilateral neuroendocrine metastases. PATIENTS AND METHODS: We retrospectively reviewed the medical records of eight patients (four men and four women), with a mean age of 45 years, who underwent liver transplantation due to bilateral neuroendocrine liver metastases between January 1996 and January 2005. The most frequent location of the primary tumor was the pancreas in five patients (one carcinoid, one gastrinoma and three non-functioning tumors). The remaining three tumors were located in the small bowel (two) and in the lung. RESULTS: Only one patient died due to technical complications related to the transplant, representing a mortality rate for the entire group of 14%. After a median follow-up of 3 years (range: 1 month-6 years), two patients died due to tumoral recurrence at 15 and 17 months, representing a tumoral recurrence rate of 33%. The survival rate at 1 and 3 years was 86% and 57%, respectively. CONCLUSIONS: Despite the initial promising results obtained with liver transplantation in the management of unresectable neuroendocrine liver metastases, our results indicate that careful patient selection is required. The key to obtaining good results is individualization of the indication for this procedure.

[Laparoscopic resection of solid liver tumors. Presentation of our experience]. / Resección de tumores hepáticos sólidos por laparoscopia. Presentación de nuestra experiencia.

INTRODUCTION: Laparoscopic liver resection (LLR) of solid liver tumors (SLT) has not become widespread due to technical problems, the risk of air embolism, and possible tumoral spread in malignant lesions. We present our experience of LLR in SLT. PATIENTS AND METHOD: Between January 2003 and May 2005, we performed the laparoscopic approach in 16 patients with SLT. Preoperative diagnosis was liver metastases from colorectal carcinoma in 11 patients and benign tumor in the remaining five patients. Five of the lesions were located in the left lobe, another 10 in the right lobe (two in S. V, four in S. VI and four in S. VII) and the remaining lesion was bilobar (S. III and VI). LLRs were performed by complete laparoscopic hepatectomy (CLH) (n=8) and assisted laparoscopic hepatectomy (ALH) (n=8). LLR was completed in 13 patients (81%). Surgical technique (n=13) consisted of three left lobectomies, one with partial resection of S. IV, three bisegmentectomies (two of S. VI and VII and one of S. III and IV, the latter associated with metastasectomy in S. VIII), five segmentectomies (one of S. II, two of S. V and two of S. VI, one of the latter associated with metastasectomy in S. VII) and two local resections of benign tumors. RESULTS: There was no intra- or postoperative mortality. With CLH the LLR was completed in five patients (62%), whereas with ALH there were no conversions. Only one of the 13 resected patients required transfusion. Seventeen nodules were excised in the 13 LLR, and 12 of 17 required the Pringle maneuver. The mean length of hospital stay was 4.9 days (3-14 days). Only one female patient (7.7%) developed an infected hematoma, requiring radiological drainage. CONCLUSION: LLR of benign SLTs shows all the advantages of laparoscopy. In the case of malignant lesions, greater experience is needed to confirm the safety and effectiveness of the open approach.

Laparoscopic iatrogeny of the hepatic hilum as an indication for liver transplantation.

The introduction of biliary laparoscopic surgery led to an increase in the incidence of liver hilum injuries. These types of lesions are very serious, because they can lead to secondary biliary cirrhosis or fulminant hepatic failure and the need for liver transplantation (LT). We present three cases of liver hilum injuries, which were treated with LT; one case was due to severe and persistent cholangitis, and two cases were due to fulminant hepatic failure. The world literature is also reviewed, and published cases of iatrogenic lesions of the liver hilum caused by laparoscopic surgery and requiring LT are presented. These iatrogenic lesions of the hepatic hilum are complex and technically demanding, due to their high morbidity and mortality and even the need for LT. In conclusion, these lesions must be always managed in centers with experience in hepatobiliary surgery.