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.

Tumor mucinoso de colon simulando un quiste hidatídico / Right-colon mucinous adenocarcinoma mimicking a hydatid cyst

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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.

Utilidad de la neoadyuvancia en el manejo del GIST rectal / Utility of neoadjuvant therapy in rectal GIST

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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.

Rotura espontánea de aneurisma de arteria esplénica en el síndrome de Ehlers-Danlos tipo clásico / Spontaneous rupture of a splenic aneurysm in classic Ehlers-Danlos syndrome

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

Se calcula que un 50% de los tumores del estroma gastrointestinal están localizados en el momento de su diagnóstico. Hasta en el 95% de estos casos es posible efectuar una resección quirúrgica completa que se convierte en el factor pronóstico más importante. Esta cirugía debe cumplir una serie de requisitos técnicos para que sea realmente efectiva como que debe ser R0, sin rotura tumoral, con preservación de la pseudocápsula, etc. Si bien la mayoría de los tumores del estroma gastrointestinal son gástricos, su localización en otras zonas anatómicas como el esófago, duodeno o recto, obligan al cirujano al empleo de técnicas más complejas. La laparoscopia es cada vez más usada; sin embargo, debemos evitar su uso por grupos poco experimentados o en caso de tumores de gran tamaño. El empleo de la neoadyuvancia ha suscitado un gran interés al permitir resecar con cirugías poco agresivas tumores antes irresecables o en localizaciones anatómicas muy comprometidas. El empleo de la biopsia preoperatoria no esta exento de grandes riesgos de ahí que solo deba emplearse en caso de plantearse un tratamiento neoadyuvante o en caso de existir dudas diagnosticas con otras patologías en pacientes de alto riesgo (AU)

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 (AU)

[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.

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

La aplicación clínica del imatinib en el manejo de los tumores de la estroma gastrointestinal ha cambiado radicalmente su pronóstico, especialmente en sus formas avanzadas, ya sean metastásicas, la mayoría, o localmente avanzadas. Las elevadas tasas de respuesta obtenidas permiten, en muchos casos, el empleo de la cirugía en situaciones en que antes era imposible, e incluso se llega a considerar la cirugía como un arma terapéutica de primera línea en combinación con imatinib. Aun así, su empleo no debe ser indiscriminado. Serán las diferentes respuestas que estos tumores poseen al imatinib lo que determine su utilidad y su forma de empleo. El uso combinado de cirugía e imatinib supone un claro y exitoso ejemplo de tratamiento multimodal en el contexto de lo que se ha denominado terapia molecular dirigida (AU)

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 (AU)

[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.

Vine pollen allergy in areas with a high density of vineyards.

BACKGROUND: In Castilla-La Mancha, Spain, the area with the highest density of vineyards in the world, 2 cases of Vitis vinifera pollen allergy have been previously reported. OBJECTIVE: To determine the clinical relevance and biochemical characteristics of vine pollen in the Spanish province of Ciudad Real. METHODS: We designed a prospective study of patients treated in the allergy units from Puertollano and Ciudad Real for respiratory symptoms of 4 months' duration in the year 2000. Skin prick tests with a standard aeroallergen battery and V vinifera pollen extract were performed on all patients. We also performed conjunctival and bronchial provocation tests and serum specific IgE and sodium dodecyl sulfate-polyacrylamide gel electrophoresis immunoblotting on the patients who tested positive for V vinifera pollen. RESULTS: We included 200 patients, 98 sensitized to any pollen and 9 to V vinifera pollen. We found 8 of 9 positive conjunctival provocation test results and a positive bronchial provocation result to vine pollen in a vine grower. Serum specific IgE against V vinifera pollen was detected in 9 of 9 patients. Immunoblotting with V vinifera pollen extract showed IgE-binding bands at 45 and 67 kDa. CONCLUSIONS: Vine pollen could be the cause of pollinosis in exposed patients living in areas with a high density of vineyards.

[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.