2023 GEIS Guidelines for gastrointestinal stromal tumors.

Gastrointestinal stromal tumor (GIST) is the most common malignant neoplasm of mesenchymal origin. GIST spans a wide clinical spectrum that ranges from tumors with essentially no metastatic potential to malignant and life-threatening spread diseases. Gain-of-function mutations in KIT or PDGFRA receptor tyrosine kinases are the crucial drivers of most GISTs, responsible for tumor initiation and evolution throughout the entire course of the disease. The introduction of tyrosine kinase inhibitors targeting these receptors has substantially improved the outcomes in this formerly chemoresistant cancer. As of today, five agents hold regulatory approval for the treatment of GIST: imatinib, sunitinib, regorafenib, ripretinib, and avapritinib. This, in turn, represents a success for a rare neoplasm. During the past two decades, GIST has become a paradigmatic model in cancer for multidisciplinary work, given the disease-specific particularities regarding tumor biology and tumor evolution. Herein, we review currently available evidence for the management of GIST. This clinical practice guideline has been developed by a multidisciplinary expert panel (oncologist, pathologist, surgeon, molecular biologist, radiologist, and representative of patients' advocacy groups) from the Spanish Group for Sarcoma Research, and it is conceived to provide, from a critical perspective, the standard approach for diagnosis, treatment, and follow-up.

Biomarkers of Early Liver Graft Damage in Circulatory Death and Brain Death Donors: A Propensity Score Matching Analysis.

BACKGROUND: Donation after circulatory death (DCD) is related to a warm ischemia time and more complications compared with traditional donors (donation after brain death [DBD]). METHODS: This study included biopsy samples retrospectively collected from November 2014 to December 2018 to compare histologic and biological markers of DCD and DBD liver grafts. The analysis includes marker of early apoptosis (p21), senescence (telomerase reverse transcriptase [TERT]), cell damage (caspase-3 active), endothelial damage (vascular endothelial growth factor), stem cell (CD90), hypoxia (HIF1A), inflammatory activation (COX-2), and cross-organ allograft rejection (CD44). A propensity score matching (PSM) was used to match patients receiving DCD livers to those receiving DBD livers. We analyzed the immunohistochemical initial liver damage-related warm ischemia time. RESULTS: Positive staining expression of liver damage biomarkers (COX-2, CD44, TERT, HIF1A, and CD90) was found, but no significant differences were found between DCD and DBD and with ischemic cholangiopathy. After PSM, there was a significant relationship between CD90 and male donors (odds ratio [OR], 0.26; 95% confidence interval [CI], 0.07-0.91), TERT with donor sodium (OR, 1.11; 95% CI, 1.02-1.2), HIF1A with steatosis (OR, 0.33; 95% CI, 0.13-0.83), and CD44 with donor vasoactive drugs (OR, 0.36; 95% CI, 0.13-1) and glutamic oxaloacetic transaminase 1 week increase (OR, 1.01; 95% CI, 1-1.03). CONCLUSIONS: DCD immunohistochemical initial liver damage was found to behave similarly to DBD. The increase in complications and cholangiopathy associated with warm ischemia could be related to a different later phenomenon.

[Giant lipomas or retroperitoneal liposarcomas? Controversies in their diagnosis and treatment]. / ¿Lipoma gigante o liposarcoma retroperitoneal? Controversias en su diagnóstico y tratamiento.

BACKGROUND: Retroperitoneal lipomas are extremely rare tumors that must be differentiated from well-differentiated liposarcomas (WD-LPS). OBJECTIVES: To summarize the evidence about giant retroperitoneal lipomas or liposarcomas; and to elaborate recommendations for their management. DATA SOURCES: A systematic literature search from January 1985 to December 2019 and a review of our own cases was performed. RESULTS: Our series comprises four patients, two females and two males. The diagnosis was incidental in two cases. The medium size was 26 cm, being two cases located exclusively in the retroperitoneum, one in the inguinal region and one in the buttock via pelvic space. All cases were surgically removed being confirmed the initial diagnosis of retroperitoneal lipomas in two cases, as the rest two cases were classified as WD_LPS after MDM2/CDK4 genetic analysis. The review of the available literature plus our own cases revealed 30 cases, of which 58% were woman. Only two cases were asymptomatic. The main symptom was abdominal mass (53%) followed by abdominal pain (40,6%). The median size of the lesions was 24,9 cm with a median weight of 4.576,3 g. All cases were surgically removed, being necessary to remove contiguous organs in only four cases (12,5%). CONCLUSIONS: Retroperitoneal lipoma is a rare tumor which must be differentiated from WD-LPS. This is a very difficult task, being necessary to determinate MDM2 status (by FISH or MLPA), present in liposarcoma but not in lipomas, for its correct diagnosis. The treatment must be based on a complete surgical resection with negative margins.

Actualización en el manejo de sarcomas retroperitoneales y pélvicos; el papel de la cirugía compartimental / Update in pelvic and retroperitoneal sarcoma management: The role of compartment surgery

La cirugía de los sarcomas retroperitoneales debe ser compartimental «en bloque», lo que implica la resección de órganos adyacentes al tumor. Su empleo «de entrada» permite un elevado porcentaje de resecciones con márgenes negativos, lo que supone un mejor control local y mayor supervivencia en muchos pacientes. La preservación de órganos debe hacerse de forma personalizada, especialmente en la pelvis, y adaptarla a la agresividad histológica del tumor. La biopsia preoperatoria permite establecer el subtipo de sarcoma y una adecuada estrategia perioperatoria. Estos pacientes deben ser manejados por cirujanos expertos en centros de referencia, con unidades multidisciplinarias y comités oncológicos. El uso de quimioterapia y radioterapia aún no está bien definido, por lo que solo se recomienda en centros de referencia con ensayos clínicos. En la actualidad esta es la única opción para ofrecer las mejores tasas de morbimortalidad, y las posibles mejoras en la supervivencia de estos pacientes

Surgery for retroperitoneal sarcomas should be "en bloc" compartmental, which involves resection of unaffected organs. Its upfront use is key, providing a high percentage of resections with negative margins, resulting in a better local control and increased survival in many patients. Preservation of organs should be done in an individualized manner, especially in the pelvic location, and adapted to the histological aggressiveness of the tumor. Preoperative biopsy is able to establish the diagnosis of sarcoma subtype and consequently an adequate perioperative strategy. These patients should be managed by expert surgeons at referral centers with multidisciplinary units and oncology committees. The use of chemotherapy and radiotherapy is not yet well defined, so it is only recommended at referral centers with clinical trials. Currently, this is the only option to offer the best morbidity and mortality rates, as well as possible improvements in the survival of these patients

Update in pelvic and retroperitoneal sarcoma management: The role of compartment surgery. / Actualización en el manejo de sarcomas retroperitoneales y pélvicos; el papel de la cirugía compartimental.

Surgery for retroperitoneal sarcomas should be "en bloc" compartmental, which involves resection of unaffected organs. Its upfront use is key, providing a high percentage of resections with negative margins, resulting in a better local control and increased survival in many patients. Preservation of organs should be done in an individualized manner, especially in the pelvic location, and adapted to the histological aggressiveness of the tumor. Preoperative biopsy is able to establish the diagnosis of sarcoma subtype and consequently an adequate perioperative strategy. These patients should be managed by expert surgeons at referral centers with multidisciplinary units and oncology committees. The use of chemotherapy and radiotherapy is not yet well defined, so it is only recommended at referral centers with clinical trials. Currently, this is the only option to offer the best morbidity and mortality rates, as well as possible improvements in the survival of these patients.

Pseudotumor gástrico ulcerado con afectación pancreática por enfermedad relacionada con inmunoglobulina G4: descripción de un caso y revisión de la literatura / An ulcerated gastric ulcer and pseudotumour with pancreatic affectation associated with immunoglobulin G4-related disease: a case report and literature review

Presentamos el caso de un varón de 67 años con una úlcera gástrica de 9 años de evolución, con clínica de hematemesis y melenas, que a nivel histológico mostró lesiones de fibrosis y acúmulos de células plasmáticas con positividad para inmunoglobulina G4, sin evidencia de malignidad. Esta lesión alcanzaba al páncreas, donde se observaron lesiones histológicas superponibles a las gástricas. El diagnóstico final fue el de pseudotumor gástrico ulcerado con afectación pancreática por enfermedad relacionada con inmunoglobulina G4 (AU)

We report the case of a 67 year old male who presented with a nine year history of a gastric ulcer with symptoms of hematemesis and melena. Histological analysis identified fibrotic lesions and the accumulation of immunoglobulin G4-positive plasma cells with no evidence of malignancy. The lesion extended into the pancreas, where histological lesions and gastric lesions were also observed. This is a case of an ulcerated gastric ulcer and pseudo-tumor with pancreatic affection that is associated with immunoglobulin G4-related disease (AU)

An ulcerated gastric ulcer and pseudotumour with pancreatic affectation associated with immunoglobulin G4-related disease: a case report and literature review.

We report the case of a 67 year old male who presented with a nine year history of a gastric ulcer with symptoms of hematemesis and melena. Histological analysis identified fibrotic lesions and the accumulation of immunoglobulin G4-positive plasma cells with no evidence of malignancy. The lesion extended into the pancreas, where histological lesions and gastric lesions were also observed. This is a case of an ulcerated gastric ulcer and pseudo-tumor with pancreatic affection that is associated with immunoglobulin G4-related disease.

Asociación de tumores del estroma gastrointestinal con otros tumores primarios. Propuesta de una nueva clasificación / Additional primary malignancies in patients with gastrointestinal stromal tumors. Proposal for a new classification

La asociación de gastrointestinal stromal tumor (GIST, «tumor del estroma gastrointestinal») con otras neoplasias primarias en un mismo paciente es un hecho no solo frecuente, sino con un interés creciente en la literatura científica. Esta asociación tiene una enorme importancia tanto por el desafío clínico, diagnóstico y terapéutico como por el impacto pronóstico que implica. En las series publicadas existe una tendencia a agrupar a estos pacientes para determinar que los GIST asociados a otras neoplasias tienen características concretas y diferenciables. Por el contrario, no existe un consenso general ni una clasificación unificada. Esta clasificación sería de gran interés, pues permitiría unificar criterios, consensuar los grupos para comparar las distintas series y demostrar si realmente la etiopatogenia subyacente a ambos tumores y las características del propio GIST varían según el tipo de que se trate. Realizamos una revisión de la literatura médica actual y una propuesta de nueva clasificación para pacientes con GIST asociados a otros tumores (AU)

Additional primary malignancies in patients with gastrointestinal stromal tumor (GIST) is not only common but of growing interest in the scientific literature. This association is of great importance in terms of clinical challenge, diagnosis and therapy as well as for the prognosis impact it implies. In the published series there is a tendency to group these patients to determine the specific and distinguishable characteristics of GIST associated with other malignancies. On the other hand, there is no general consensus or unified classification. This classification would be of great interest, as it would unify criteria, agree groups to compare different series and demonstrate whether the aetiology underlying both tumours and the GIST’s own characteristics really vary according to the type in question. We undertook a medical literature review and proposed a new classification for patients with GIST associated with other tumours (AU)

The assessment of the Newborn Hearing Screening Program in the Region of Murcia from 2004 to 2012.

OBJECTIVES: Newborn (NB) auditory deficit has a prevalence of 1-2% in the world. Since the 1990s different screening programs have been put into practice. The Newborn Hearing Screening Program has been in operation since 2002 in our hospital (HCUVA) in Murcia (Spain) and two years later it was introduced into the whole of the Autonomous Community of the Region of Murcia as part of universal healthcare. The objective of this study was to analyze and assess its results. METHOD: The newborn (NB) population is divided into two groups: not-at-risk NBs and at-risk NBs. In the first case we carry out acoustic otoemissions (AOEs) 48 h after birth and depending on the result the child is either discharged or, in negative cases, the infant undergoes a series of tests in a period of 30-45 days to confirm or rule out the existence of hearing anomalies. In the at-risk group we combine AOEs with brain trunk potentials (BERA) following the stages in a decision tree diagram similar to the ones for not-at-risk children in order to provide a clinical diagnosis in the first three months of life. RESULTS: The screening performance was assessed for the 156,122 children studied, of which 151,258 belonged to the group of not-at-risk children; and 4864 to the group at risk of hypoacusia. As a result of the screening only 410 (0.26%) were sent to consultation, 213 in the not-at-risk group (0.14%) and 197 (24.7%) in the at-risk group. A total of 7452 false positives were identified (4.7%), 6951 (4.5%) in the not-at-risk group; and 501 (10.3%) in the group with risk factors; and there were 53 false negatives (0.03%). Sensitivity in the screening program was 88.5%, with a specificity of 95%. CONCLUSIONS: The Region of Murcia has a Newborn Hearing Screening Program with tests that provide a high level of sensitivity and specificity in accordance with the findings of the literature. Our results endorse the program and the patients were treated in a way that met the objective of providing a correct diagnosis and the appropriate therapeutic action.

[Additional primary malignancies in patients with gastrointestinal stromal tumors. Proposal for a new classification]. / Asociación de tumores del estroma gastrointestinal con otros tumores primarios. Propuesta de una nueva clasificación.

Additional primary malignancies in patients with gastrointestinal stromal tumor (GIST) is not only common but of growing interest in the scientific literature. This association is of great importance in terms of clinical challenge, diagnosis and therapy as well as for the prognosis impact it implies. In the published series there is a tendency to group these patients to determine the specific and distinguishable characteristics of GIST associated with other malignancies. On the other hand, there is no general consensus or unified classification. This classification would be of great interest, as it would unify criteria, agree groups to compare different series and demonstrate whether the aetiology underlying both tumours and the GIST's own characteristics really vary according to the type in question. We undertook a medical literature review and proposed a new classification for patients with GIST associated with other tumours.

Colangitis por obstrucción de asa aferente tras duodenopancreatectomía cefálica / Cholangitis due to afferent loop obstruction after cephalic duodenopancreatectomy

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Cirugía de los tumores del estroma gastrointestinal primarios no metastásicos. Resultados del estudio GRISK / Primary, non-metastatic gastrointestinal stromal tumour surgery: Results of the GRISK study

La cirugía radical es el tratamiento estándar en el GIST primario localizado. Se valora una serie de GIST primarios, su tratamiento y el riesgo preestablecido de recaída tras seguimiento de los mismos. Material y métodos Estudio retrospectivo multicéntrico y descriptivo de GIST primarios no metastásicos intervenidos entre junio de 2007 y diciembre de 2008. Se analizan las variables de mayor relevancia: localización, tamaño, índice mitótico y criterios pronóstico de recidiva NHI y AFIP y su correlación con la supervivencia libre de enfermedad (SLE) de los pacientes. Resultados Serie de 141 pacientes, edad media 65 años, con GIST de localización más frecuente en estómago (70,8%) e intestino delgado (22,9%) y con tamaño medio tumoral de 6,7cm (0,5-35). La cirugía fue R0 en el 97,2% de los casos (abordaje laparoscópico 21,5%). Distribución según criterios de NHI/Flescher: alto (31,95) e intermedio (26,4%), y según criterios AFIP/Miettinen: alto (22,9%) e intermedio (12,5%). Tras un seguimiento medio de 20,3 meses se detectó recaída en 7,1% (10 casos) perteneciendo tan solo 2 casos al grupo de «bajo riesgo» por criterios pronóstico NHI y AFIP. La SLE a un año fue del 95,5% y a los 2 años del 91,5%.ConclusionesLa serie mostró una alta SLE y una buena correlación con los criterios pronóstico tanto de Flescher como de Mietinen. No obstante, el riesgo de recaída varió siguiendo los criterios de NIH (intermedio/alto 58,3%) o los criterios de AFIP (intermedio/alto 35,4%) que incluyen la localización del tumor. Por ello consideramos estos últimos criterios como los más adecuados para la valoración prónostica de riesgo de recidiva del GIST (AU)

Introduction and objectives: Radical surgery is the standard treatment for localised gastrointestinal stromal tumours (GIST). A series of primary GIST, their treatment and pre-established risk of recurrence after their follow-up is evaluated. Material and methods: A retrospective, descriptive and multicentre study was conducted on primary, non-metastatic GIST operated on between June 2007 and December 2008. The variables of greater relevance were analysed, including, location, size, mitotic index, and NHI and AFIP recurrence prognostic criteria, and their correlation with the disease-free survival (DFS) of the patients. Results: The series included 141 patients with a mean age of 65 years. The most frequent GIST location was in the stomach (70.8%) and small intestine (22.9%), and with a mean tumour size of 6.7 cm (0.5-35 cm). The surgery was R0 in 97.2% of cases ( laparoscopic approach, 21.5%). The distribution according to NHI/Flescher criteria was, high (31.95%),and intermediate (26.4%), and according to AFIP/Miettinen criteria it was, high (22.9%) and intermediate (12.5%). After a mean follow-up of 20.3 months, there was a 7.1% (10 cases)recurrence, with only 2 cases belonging to the group with a «low risk» using the NHI and AFIP prognostic criteria. The DFS at one year was 95.5% and 91.5% at 2 years. Conclusions: The series showed a high DFS and a good correlation with both the Flescher and the Miettinen criteria. However, the risk of recurrence varied according to the AFIP criteria(intermediate/high, 58.3%), or the AFIP criteria (intermediate/high, 35.4%) which included the tumour location. For this reason, we consider these latter criteria as the most adequate for assessing the prognostic risk of GIST recurrence (AU)

[Primary, non-metastatic gastrointestinal stromal tumour surgery: results of the GRISK study]. / Cirugía de los tumores del estroma gastrointestinal primarios no metastásicos. Resultados del estudio GRISK.

INTRODUCTION AND OBJECTIVES: Radical surgery is the standard treatment for localised gastrointestinal stromal tumours (GIST). A series of primary GIST, their treatment and pre-established risk of recurrence after their follow-up is evaluated. MATERIAL AND METHODS: A retrospective, descriptive and multicentre study was conducted on primary, non-metastatic GIST operated on between June 2007 and December 2008. The variables of greater relevance were analysed, including, location, size, mitotic index, and NHI and AFIP recurrence prognostic criteria, and their correlation with the disease-free survival (DFS) of the patients. RESULTS: The series included 141 patients with a mean age of 65 years. The most frequent GIST location was in the stomach (70.8%) and small intestine (22.9%), and with a mean tumour size of 6.7 cm (0.5-35 cm). The surgery was R0 in 97.2% of cases (laparoscopic approach, 21.5%). The distribution according to NHI/Flescher criteria was, high (31.95%), and intermediate (26.4%), and according to AFIP/Miettinen criteria it was, high (22.9%) and intermediate (12.5%). After a mean follow-up of 20.3 months, there was a 7.1% (10 cases) recurrence, with only 2 cases belonging to the group with a «low risk¼ using the NHI and AFIP prognostic criteria. The DFS at one year was 95.5% and 91.5% at 2 years. CONCLUSIONS: The series showed a high DFS and a good correlation with both the Flescher and the Miettinen criteria. However, the risk of recurrence varied according to the AFIP criteria (intermediate/high, 58.3%), or the AFIP criteria (intermediate/high, 35.4%) which included the tumour location. For this reason, we consider these latter criteria as the most adequate for assessing the prognostic risk of GIST recurrence.

Hemorragia diferida de la arteria hepática derecha tras iatrogenia biliar por colecistectomía laparoscópica que precisó trasplante hepático por insuficiencia hepática aguda: caso clínico y revisión de la literatura / Delayed Right Hepatic Artery Haemorrhage After Iatrogenic Gallbladder by Laparoscopic Cholecystectomy that Required a Liver Transplant Due to Acute Liver Failure: Clinical Case and Review of the Literature

Introducción La lesión de la arteria hepática derecha (AHD) tras colecistectomía laparoscópica (CL) puede pasar desapercibida clínicamente, aunque a veces ocasiona una necrosis del lóbulo derecho. En situaciones excepcionales, cuando la necrosis se extiende al segmento IV, podría ocurrir un fallo hepático fulminante (FHF) y requerir un trasplante hepático urgente (TH).Pacientes y método Presentamos una revisión de la literatura médica de los pacientes en los que se indicó TH debido a una lesión vascular secundaria a lesión biliar por CL. También presentamos el cuarto paciente descrito en la literatura especializada que precisó TH secundario a lesión de la AHD tras CL, el segundo por FHF. Resultados El TH debido a la lesión de la AHD se realizó en 3 de 13 pacientes recogidos en la literatura médica: uno se realizó a los 3 meses de la lesión por FHF, tras realizar una hepatectomía derecha ampliada y los otros 2 debido a cirrosis biliar secundaria. Nuestro paciente se trasplantó a los 15 días de la lesión por FHF. Conclusiones La lesión de la AHD tras CL puede requerir TH por FHF, aunque es excepcional, es necesario pensar en esta posibilidad ante complicaciones de la AHD que requieran su oclusión (AU)

Introduction Right hepatic artery (RHA) injury after laparoscopic cholecystectomy (LC) may go unnoticed clinically, but can sometimes cause necrosis of the right lobe. Exceptionally, when the necrosis spreads to segment IV, fulminant liver failure (FLF) may occur, and an urgent liver transplantation (LT) may be required. Patients and method We provide a review of the literature on patients with indication for an LT due to vascular damage caused by bile duct injury following LC. The case reported herein is the fourth described in the specialized literature of LT due to RHA injury after LC and the second of FLF after RHA injury. Results LT due to RHA injury was performed in 3 of 13 patients reported in the literature: one LT was performed at 3 months due to FLF, after an extended right hepatectomy was performed, and the remaining two were performed due to secondary biliary cirrhosis. Our patient was transplanted due to FLF 15 days after the injury. Conclusions RHA injury after LC may require LT due to FLF. Although exceptional, this possibility should be considered when there are RHA complications that may require occlusion (AU)

[Delayed right hepatic artery haemorrhage after iatrogenic gallbladder by laparoscopic cholecystectomy that required a liver transplant due to acute liver failure: clinical case and review of the literature]. / Hemorragia diferida de la arteria hepática derecha tras iatrogenia biliar por colecistectomía laparoscópica que precisó trasplante hepático por insuficiencia hepática aguda: caso clínico y revisión de la literatura.

INTRODUCTION: Right hepatic artery (RHA) injury after laparoscopic cholecystectomy (LC) may go unnoticed clinically, but can sometimes cause necrosis of the right lobe. Exceptionally, when the necrosis spreads to segment IV, fulminant liver failure (FLF) may occur, and an urgent liver transplantation (LT) may be required. PATIENTS AND METHOD: We provide a review of the literature on patients with indication for an LT due to vascular damage caused by bile duct injury following LC. The case reported herein is the fourth described in the specialized literature of LT due to RHA injury after LC and the second of FLF after RHA injury. RESULTS: LT due to RHA injury was performed in 3 of 13 patients reported in the literature: one LT was performed at 3 months due to FLF, after an extended right hepatectomy was performed, and the remaining two were performed due to secondary biliary cirrhosis. Our patient was transplanted due to FLF 15 days after the injury. CONCLUSIONS: RHA injury after LC may require LT due to FLF. Although exceptional, this possibility should be considered when there are RHA complications that may require occlusion.

Anatomía patológica de los tumores del estroma gastrointestinal primarios no metastásicos en España. Resultados del estudio GRISK / The pathology of primary, non mestastatic gastrointestinal stromal tumours (GIST) in Spain. The GRISK study results

AntecedentesLos tumores del estroma gastrointestinal (GIST) son los tumores mesenquimales más frecuentes del tracto gastrointestinal, suelen expresar CD117 y la mutación en KIT o PDGFRA es el evento clave de su oncogénesis.MétodosEstudio retrospectivo, multicéntrico, en pacientes con GIST primario no metastásico sometidos a resección completa entre junio de 2007 y diciembre de 2008.ResultadosLocalizaciones: en el estómago (70,8%) y el intestino delgado (22,9%). Tipo de cirugía: R0 (97,2%) y R1 (2,8%). Tamaño: media de 6,7cm (0,5–35). El estado de los bordes figuraba en el 67,4%. El subtipo histológico constaba en el 84%: células fusiformes (64,6%), epitelioides (6,9%) y mixto (12,5%). Presencia de necrosis (33,6%). Mitosis (índice mitótico por 50 campos de gran aumento): superior a 5 (34,1%). Riesgo alto del National Institutes of Health (31,9%), riesgo nivel iv de National Institutes of Health modificado (13,2%), riesgo alto del AFIP (22,9%), muy alto riesgo del AFIP modificado (11,1%). La inmunohistoquímica se hizo en todos los GIST con positividad para CD117 (97,2%), CD34 (89,2%) y actina (31,9%).ConclusionesExiste buena adherencia de los patólogos a las guías del Grupo Español de Investigación en Sarcomas de GIST. El informe patológico debe incluir no sólo el tamaño, el índice mitótico por 50 campos de gran aumento y la localización, sino también el estado de los bordes, el tipo celular y la presencia de necrosis. Se debe hacer inmunohistoquímica con CD117, CD34, actina, desmina, proteína S-100 y Ki-67. El riesgo de progresión difiere entre las clasificaciones que serán validadas con el seguimiento(AU)

IntroductionGastrointestinal stromal tumours (GIST) are the most frequent mesenchymal tumours of the gastrointestinal tract. They usually express CD117 and KIT or PDGFRA mutations are a key factor in their oncogenesis.MethodWe carried out a multicentre, retrospective study of patients with primary, non metastatic GIST who had undergone complete surgical resection between June 2007 and December 2008.ResultsLocalization: stomach (70.8%) and small intestine (22.9%). Type of surgery: R0 (97.2%) and R1 (2.8%). Average tumour size: 6.7cm (0.535cm). Description of tumoural borders was reported in 67.4%. Histological subtype was reported in 84%: spindle cells (64.6%), epithelioid cells (6.9%) and mixed (12.5%). Necrosis was present in 33.6%. Mitoses (number per 50 hmf) >5 in 34.1%. High risk NIH (31.9%), level IV risk NIH mod (13.2%), high risk AFIP (22.9%) and very high risk AFIP mod (11.1%). Immunohistochemistry was carried out in all the GIST with positivity for CD-117 (97.2%), CD34 (89.2%) and actin (31.9%)(AU)

[Unilocular macrocystic serous cystoadenoma of the pancreas]. / Cistoadenoma seroso unilocular macroquístico de páncreas.

The differential diagnosis between macrocystic serous cystoadenoma and mucinous cystoadenoma is very difficult but is highly important due to the latter's potential for malignant transformation. We present a case of unilocular macrocystic serous cystoadenoma. Due to the impossibility of obtaining an accurate preoperative diagnosis, all macrocystic unilocular lesions, in an appropriate clinical context, must be resected.

Cistoadenoma seroso unilocular macroquístico de páncreas / Unilocular macrocystic serous cystoadenoma of the pancreas

El diagnóstico diferencial entre el cistoadenoma seroso macroquístico y el cistoadenoma mucinoso es muy dificultoso, pero de gran importancia, debido al potencial maligno de esta última entidad. Se presenta un caso clínico de cistoadenoma seroso unilocular macroquístico. Dada la imposibilidad de un diagnóstico preciso preoperatorio, las lesiones macroquísticas uniloculares, en el adecuado contexto clínico, deben ser resecadas (AU)

The differential diagnosis between macrocystic serous cystoadenoma and mucinous cystoadenoma is very difficult but is highly important due to the latter's potential for malignant transformation. We present a case of unilocular macrocystic serous cystoadenoma. Due to the impossibility of obtaining an accurate preoperative diagnosis, all macrocystic unilocular lesions, in an appropriate clinical context, must be resected (AU)

Confección de la ventanapleuropericárdica porvideotoracoscopia / Construction of the pleuropericardial window for videothoracoscopy

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