[Clinical and pathological features of gastrointestinal stromal tumors (GIST) in a single institution: A descriptive study and review of the literature]. / Perfil clínico y anatomopatológico de los tumores estromales gastrointestinales de un hospital de área: Estudio descriptivo y revisión de la literatura.

INTRODUCTION: This study was aimed to assess the main clinical, pathological and therapeutic characteristics of a cohort of gastrointestinal stromal tumors (GIST). METHODS: Observational study including 66 patients diagnosed with GIST admitted to our hospital between 2002 and 2015. Parameters related to medical history, clinical manifestations, medical and surgical treatment, histopathology, and morbi-mortality were studied. A review of the literature was included to correlate with the results. RESULTS: The most frequent location of GIST in our patients was the stomach (65.2%), in which the gastric fondo was the predominant region. The most common clinical manifestation was gastrointestinal hemorrhage (45.5%), followed by incidental finding after imaging or invasive procedures (33.3%). 58 patients underwent surgery (90.6%), 15.5% were urgent. A total of 69% of the GISTs had a size between 2 and 10cm. The one-year mortality was 7.9%, all cases related to local or remote extension, or surgical complications. CONCLUSION: There is a large clinical variability among GIST cases. The first choice of treatment is surgery, which is feasible in most cases and should be as conservative as possible. The prognosis varies depending on the size and proliferation index, thus close follow-up should be performed. No tumor marker is clearly associated with a poor prognosis. New molecular biology studies are needed in order to find therapeutic targets.

Diffuse pulmonary meningotheliomatosis diagnosed by transbronchial lung biopsy.

Minute pulmonary meningothelial-like nodules (MPMNs) are usually unique lesions in the lung parenchyma. Diffuse pulmonary meningotheliomatosis, which is presented as multiple MPMNs, has been less frequently described. MPMNs are mainly asymptomatic and are diagnosed after lung surgery or during autopsy. We report on a patient with multiple and bilateral pulmonary nodules, some of which were cavitated, diagnosed with diffuse pulmonary meningotheliomatosis by transbronchial lung biopsy. Diffuse pulmonary meningotheliomatosis should be included in the differential diagnosis of bilateral lung nodules.

Pulmonary Oncocytoma Located at the Intermediate Bronchus Diagnosed by Bronchial Biopsy / Oncocitoma pulmonar localizado en bronquio intermediario diagnosticado mediante biopsia bronquial

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Perfil clínico y anatomopatológico de los tumores estromales gastrointestinales de un hospital de área: Estudio descriptivo y revisión de la literatura / Clinical and pathological features of gastrointestinal stromal tumors (GIST) in a single institution: A descriptive study and review of the literature

Introducción: Describir las principales características clínicas, anatomopatológicas, terapéuticas y evolutivas de una serie amplia de tumores estromales gastrointestinales (GIST). Métodos: Estudio observacional de una serie de 66 casos de GIST tratados en nuestro hospital de 2002 a 2015. Seleccionamos variables relacionadas con los antecedentes personales, las manifestaciones clínicas, el tratamiento médico y quirúrgico, la anatomía patológica y la morbimortalidad. Añadimos una revisión de la literatura para correlacionarla con nuestros resultados. Resultados: La localización más frecuente fue el estómago (65,2%), en el que destacó como región predominante el fondo. La manifestación clínica más habitual fue la hemorragia digestiva (45,5%), seguida del hallazgo casual tras la realización de alguna prueba de imagen o procedimiento invasivo (33,3%). Recibieron cirugía 58 pacientes (90,6%), el 15,5% de carácter urgente. El 69% de los GIST tenían un tamaño entre 2 y 10 cm. La mortalidad al año debida al tumor fue de un 7,9% (5 casos), todos ellos relacionados con extensión local o a distancia, o complicación quirúrgica. Conclusiones: La variabilidad clínica de los GIST es muy amplia. El tratamiento de primera elección es la cirugía, que es factible en la mayoría de los casos y debe ser lo más conservadora posible. El pronóstico es variable, dependiendo del tamaño y del índice de proliferación, por lo que debe realizarse un seguimiento estrecho. No existe un marcador tumoral claramente asociado a un peor pronóstico, por lo que se necesitan nuevos estudios de biología molecular con el objetivo de encontrar dianas terapéuticas (AU)

Introduction: This study was aimed to assess the main clinical, pathological and therapeutic characteristics of a cohort of gastrointestinal stromal tumors (GIST). Methods: Observational study including 66 patients diagnosed with GIST admitted to our hospital between 2002 and 2015. Parameters related to medical history, clinical manifestations, medical and surgical treatment, histopathology, and morbi-mortality were studied. A review of the literature was included to correlate with the results. Results: The most frequent location of GIST in our patients was the stomach (65.2%), in which the gastric fondo was the predominant region. The most common clinical manifestation was gastrointestinal hemorrhage (45.5%), followed by incidental finding after imaging or invasive procedures (33.3%). 58 patients underwent surgery (90.6%), 15.5% were urgent. A total of 69% of the GISTs had a size between 2 and 10 cm. The one-year mortality was 7.9%, all cases related to local or remote extension, or surgical complications. Conclusion: There is a large clinical variability among GIST cases. The first choice of treatment is surgery, which is feasible in most cases and should be as conservative as possible. The prognosis varies depending on the size and proliferation index, thus close follow-up should be performed. No tumor marker is clearly associated with a poor prognosis. New molecular biology studies are needed in order to find therapeutic targets (AU)

Quiste de colédoco tipo II intrapancreático del adulto malignizado: duodenopancreatectomía / Type II intrapancreatic choledochal malignant cyst in adults: Duodenopancreatectomy

Presentamos el caso de una paciente de 62 años que ingresó de urgencias por pancreatitis aguda leve. En las pruebas de imagen se observó una lesión sólido-quística en la cabeza de páncreas comunicante con la vía biliar distal, y se diagnosticó quiste de colédoco tipo II de Todani con degeneración neoplásica en su interior confirmada tras una biopsia por punción en una ecoendoscopia. La paciente fue tratada con una duodenopancreatectomía cefálica con intención curativa

A 62-year-old female patient was admitted for abdominal pain and vomiting. Imaging tests revealed a solid-cystic lesion at the head of the pancreas communicating with the distal bile duct. A Todani type II choledochal cyst was diagnosed with neoplastic degeneration after cytological diagnosis with endoscopic ultrasound-guided puncture. The patient was treated with a cephalic duodenopancreatectomy with curative intention (AU)

[Utility of frozen section in the surgical treatment of thyroid nodules]. / Utilidad de la biopsia intraoperatoria en el tratamiento quirúrgico del nódulo tiroideo.

OBJECTIVE: To present our experience of the use of frozen section (FS) in the operative management of thyroid nodules and determine the utility of this procedure when deciding the extent of thyroidectomy. PATIENTS AND METHOD: We performed a prospective and comparative study. The FS of patients who underwent surgery for thyroid nodules between 1995 and 2002 were evaluated. The results were compared with those of fine-needle aspiration cytology (FNA) and definitive biopsy. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and diagnostic accuracy of both FNA and FS were calculated. RESULTS: There were 469 thyroidectomies and 179 FS were performed. The results obtained for FNA and FS were, respectively: PPV: 100%/100%; NPV: 89%/90%; diagnostic accuracy: 89.5%/91%. When only "follicular hyperplasia" was included as the cytologic diagnosis, the PPV and NPV for FS were 100% and 86.7%, respectively. CONCLUSION: The diagnostic accuracy of FNAC and FS is similar. In cases of follicular hyperplasia, the sensitivity of FS is too low to rule out malignancy.

[Gastrointestinal stromal tumors. Diagnosis, prognosis and current surgical treatment. Follow-up of 18 treated patients]. / Tumores gástricos estromales. Diagnóstico, pronóstico y tratamiento quirúrgico actual. Seguimiento de 18 pacientes tratados.

INTRODUCTION: Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumors of the digestive tract and their growth is directed through the signaling of the KIT or PDFGRA genes. The objective of the present study was to describe a series of cases of GIST tumors located in the stomach. PATIENTS AND METHODS: We performed a retrospective, descriptive study of 18 patients diagnosed with gastric GIST and treated by means of gastric resection between July 1996 and June 2004. There were 12 women and six men, with a mean age of 63 years (range 33-84). In all patients, diagnosis was performed with immunohistochemical markers, such as CD34 and CD117. Investigations included endoscopy, abdominal ultrasonography, abdominal computerized axial tomography and fine-needle aspiration biopsy. RESULTS: The main symptoms were digestive hemorrhage with severe anemia in 10 patients and abdominal pain in seven. In two patients, the tumors were incidental findings during laparotomy. Four patients underwent emergency surgery and the remainder underwent elective surgery. In all patients, gastric resection of variable extension was performed, according to tumoral location. Multicentric tumors were found in two patients. All patients were CD117- and CD34-positive. There were few postoperative complications. One patient died from acute myocardial infarction (5.6%). The mean follow-up was 47.5 months (range, 12-106). One patent died due to spread of a pancreatic neoplasm and the remaining patients are alive and without tumoral recurrence (94.1%). CONCLUSIONS: Initial symptoms consist of upper gastrointestinal hemorrhage and abdominal pain. Gastroscopy and imaging techniques lead to a suspected diagnosis, which can be confirmed by immunohistochemical studies, in which the "gold standard" is positivity for CD117; CD34 (+), vimentin (+), actin (-) and protein S-100 (-) are also used. Treatment consists of tumoral resection with negative margins.

Schwannoma epitelioide con cambios tipo ancient. A propósito de un caso / Epithelioid schwannoma with ‘ancient’ changes. A case report

Los tumores benignos de la vaina del nervio periférico son relativamente frecuentes, siendo los principales representantes el neuroma solitario circunscrito, el neurofibroma y el schwannoma. Sin embargo, la variante epitelioide benigna del schwannoma de partes blandas es extremadamente rara y debe plantearse un cuidadoso diagnóstico diferencial con otras entidades benignas y malignas. Se presenta el caso de un schwannoma epitelioide en un varón de 48 años y se revisa la literatura así como su diagnóstico diferencial histológico e inmunohistoquímico (AU)

Benign peripheral nerve sheath tumours are relatively common and the three main types are neuroma, neurofibroma, and schwannoma. However, the benign epithelioid variant of soft tissue schwannoma is extremely rare and a careful differential diagnosis must be made with other benign and malignant entities. A case of an epithelioid schwannoma in a 48 year-old-man is presented and the histological and immunohistochemical differential diagnosis is discussed. The relevant literature is reviewed (AU)

Hiperplasia nodular oncocítica multifocal de glándula parótida. A propósito de un caso con revisión del espectro de lesiones oncocíticas / Multifocal nodular oncocytic hyperplasia in the parotid gland. A case report and review of oncocytic lesions of the parotid gland

La hiperplasia nodular oncocítica multifocal es una lesión infrecuente de la glándula parótida. Se presenta un caso de hiperplasia nodular oncocítica multifocal de la glándula parótida en espacio parafaríngeo de una mujer de 74 años y se revisan las lesiones oncocíticas de la glándula parótida(AU)

Multifocal nodular oncocytic hyperplasia is a rare lesion of the parotid gland. A case of multifocal nodular oncocytic hyperplasia of the parotid gland in the parapharyngeal space in a 74-year-old woman is reported and the oncocytic lesions of the parotid gland are reviewed(AU)

Utilidad de la biopsia intraoperatoria en el tratamiento quirúrgico del nódulo tiroideo / Utility of frozen section in the surgical treatment of thyroid nodules

Objetivo. Presentar nuestra experiencia en la utilización de la biopsia intraoperatoria (BIO) en el manejo diagnóstico del nódulo tiroideo, a la hora de decidir la extensión de la tiroidectomía. Pacientes y método. Estudio prospectivo y comparativo. Se estudia la biopsia intraoperatoria de pacientes intervenidos por nódulo tiroideo entre enero de 1999 y diciembre de 2002. El resultado se compara con el estudio citológico preoperatorio y con el resultado de la biopsia diferida. Hemos calculado la sensibilidad, la especificidad y los valores predictivos positivos y negativos (VPP y VPN), así como la exactitud diagnóstica, tanto para la BIO como para la punción-aspiración con aguja fina (PAAF), en el diagnóstico de cáncer tiroideo. Resultados. Se realizaron 179 BIO sobre un total de 469 tiroidectomías. Los valores obtenidos para PAAF y BIO son, respectivamente: VPP, el 100 y el 100%; VPN, el 89 y el 90%; exactitud diagnóstica, el 89,5 y el 91%. Si incluimos sólo los diagnósticos de "proliferación folicular" en la PAAF, los VPP y VPN para la BIO son del 100 y el 86,7%, respectivamente. Conclusiones. La exactitud diagnóstica es similar para la BIO y la PAAF. En los casos de proliferación folicular la BIO, debido a su baja sensibilidad, tiene poco valor para descartar cáncer (AU)

Objective. To present our experience of the use of frozen section (FS) in the operative management of thyroid nodules and determine the utility of this procedure when deciding the extent of thyroidectomy. Patients and method. We performed a prospective and comparative study. The FS of patients who underwent surgery for thyroid nodules between 1995 and 2002 were evaluated. The results were compared with those of fine-needle aspiration cytology (FNA) and definitive biopsy. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and diagnostic accuracy of both FNA and FS were calculated. Results. There were 469 thyroidectomies and 179 FS were performed. The results obtained for FNA and FS were, respectively: PPV: 100%/100%; NPV: 89%/ 90%; diagnostic accuracy: 89.5%/91%. When only "follicular hyperplasia" was included as the cytologic diagnosis, the PPV and NPV for FS were 100% and 86.7%, respectively. Conclusion. The diagnostic accuracy of FNAC and FS is similar. In cases of follicular hyperplasia, the sensitivity of FS is too low to rule out malignancy (AU)

Tumores gástricos estromales. Diagnóstico, pronóstico y tratamiento quirúrgico actual. Seguimiento de 18 pacientes tratados / Gastrointestinal stromal tumors. Diagnosis, prognosis and current surgical treatment.Follow-up of 18 treated patients

Introducción. Los tumores gastrointestinales estromales (GIST) son los tumores de origen mesenquimal más frecuentes del tracto digestivo y su crecimiento está dirigido a través de la señalización de los genes KIT o PDFGRA. El objetivo del presente estudio es describir una serie de casos de tumores GIST localizados en el estómago. Pacientes y método. Estudio retrospectivo y descriptivo de 18 pacientes con GIST gástricos diagnosticados y tratados mediante resección gástrica entre julio de 1996 y junio de 2004, de los que 12 eran mujeres y 6 varones con una edad media de 63 años (rango, 33-84 años). En todos los casos, el diagnóstico se ha realizado con marcadores inmunohistoquímicos, como CD34 y CD117. Los estudios incluyeron endoscopia, ecografía abdominal, tomografía computarizada y punción-aspiración con aguja fina. Resultados. La clínica predominante fue hemorragia digestiva alta con anemia grave en 10 casos y dolor abdominal en 7. En 2 pacientes resultaron ser hallazgo casual en una laparotomía. Se intervino de urgencia 4 pacientes y electivamente el resto, realizándose siempre resección gástrica extensión de variable según la localización. Se apreció multicentricidad en 2 casos. Todos los casos fueron CD117 y CD34 positivos. Las complicaciones postoperatorias fueron bajas y 1 paciente falleció por infarto agudo de miocardio (5,6%). El seguimiento medio ha sido 47,5 meses (rango, 12-106) y se ha producido el fallecimiento de 1 paciente por una neoplasia avanzada de páncreas, mientras que el resto permanece vivo y sin recidiva (94,1%). Conclusiones. Los datos clínicos iniciales son la hemorragia digestiva alta y el dolor abdominal. La endoscopia y las técnicas de imagen hacen presumir el diagnóstico, que se confirma con estudios de inmunohistoquímica cuyo patrón de referencia es la positividad para CD117, utilizándose además CD34 (+), vimentina (+), actina (­) y proteína S-100 (­). El tratamiento implica la resección del tumor con bordes sanos (AU)

Introduction. Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumors of the digestive tract and their growth is directed through the signaling of the KIT or PDFGRA genes. The objective of the present study was to describe a series of cases of GIST tumors located in the stomach. Patients and methods. We performed a retrospective, descriptive study of 18 patients diagnosed with gastric GIST and treated by means of gastric resection between July 1996 and June 2004. There were 12 women and six men, with a mean age of 63 years (range 33-84). In all patients, diagnosis was performed with immunohistochemical markers, such as CD34 and CD117. Investigations included endoscopy, abdominal ultrasonography, abdominal computerized axial tomography and fine-needle aspiration biopsy. Results. The main symptoms were digestive hemorrhage with severe anemia in 10 patients and abdominal pain in seven. In two patients, the tumors were incidental findings during laparotomy. Four patients underwent emergency surgery and the remainder underwent elective surgery. In all patients, gastric resection of variable extension was performed, according to tumoral location. Multicentric tumors were found in two patients. All patients were CD117- and CD34-positive. There were few postoperative complications. One patient died from acute myocardial infarction (5.6%). The mean follow-up was 47.5 months (range, 12-106). One patent died due to spread of a pancreatic neoplasm and the remaining patients are alive and without tumoral recurrence (94.1%). Conclusions. Initial symptoms consist of upper gastrointestinal hemorrhage and abdominal pain. Gastroscopy and imaging techniques lead to a suspected diagnosis, which can be confirmed by immunohistochemical studies, in which the "gold standard" is positivity for CD117; CD34 (+), vimentin (+), actin (­) and protein S-100 (­) are also used. Treatment consists of tumoral resection with negative margins (AU)