Angiomixoma agresivo vulvar en mujer joven. Reporte de un caso / Aggressive vulvar angiomyxoma in a young woman. A case report

Resumen ANTECEDENTES: El angiomixoma agresivo profundo perineal es un tumor mesenquimatoso de muy limitada aparición que se origina debido a un crecimiento tumoral del tejido conjuntivo que se expande a pesar de su naturaleza benigna; se caracteriza por un comportamiento agresivo. CASO CLÍNICO: Paciente de 38 años, con un nódulo genital de 3 cm en el labio mayor izquierdo, con sospecha clínica de quiste de la glándula de Bartolino. Durante la intervención para su drenaje se objetivó una tumoración blanda, de aspecto mesenquimal, pediculado. El análisis histológico confirmó que se trataba de un angiomixoma agresivo profundo perineal. Posterior al estudio de extensión negativo, se completó la vulvectomía simple izquierda, con un posoperatorio favorable. CONCLUSIONES: La obtención de una biopsia inicial es decisiva, sobre todo en casos de tumores mesenquimales agresivos, como el angiomixoma agresivo perineal profundo. De esta manera puede establecerse un plan de tratamiento individual en función del diagnóstico histológico definitivo.

Abstract BACKGROUND: Aggressive deep perineal angiomyxoma is a mesenchymal tumor of very limited occurrence that originates due to a tumorous growth of connective tissue that expands despite its benign nature; it is characterized by an aggressive behavior. CLINICAL CASE: 38-year-old patient with a 3 cm genital nodule on the left labium majus, with clinical suspicion of Bartholin's gland cyst. During the intervention for its drainage, a soft, mesenchymal, pedunculated tumor was observed. Histological analysis confirmed that it was an aggressive deep perineal angiomyxoma. Following a negative extension study, a simple left vulvectomy was completed, with a favorable postoperative course. CONCLUSIONS: Obtaining an initial biopsy is critical, especially in cases of aggressive mesenchymal tumors, such as deep perineal aggressive angiomyxoma. In this way an individual treatment plan can be established based on the definitive histologic diagnosis.

Fibroepithelial stromal polyp of vagina in pregnancy - A mimicker

A rare mesenchymal neoplasm of the lower female genital tract, fibroepithelial stromal tumor, may be confused with other more aggressive lesions. The appearance in pregnancy may further complicate a normal vaginal delivery and lead to confusion with a more unfavorable diagnosis. Even on histological examination, it is pertinent to rule put other stromal tumors of the region. To avoid overdiagnosis, the pathologist must be aware of the specific histomorphology. We report a case of fibroepithelial stromal polyp in a 32-year-old pregnant woman as an incidental finding during a routine antenatal check-up.

Tumor fibroblástico inguinal superficial positivo para CD34: reporte de un caso y revisión de la literatura / Inguinal superficial cd34-positive fibroblastic tumor: case report and literature review

El tumor fibroblástico superficial de tejidos blandos positivo para antígeno CD34 (CD34) es un tumor raro, de baja frecuencia, que se caracteriza histológicamente por un marcado pleomorfismo, baja actividad mitótica, e inmunoreactividad difusa para CD34. Puede tener un comportamiento similar al de un tumor mesenquimal de malignidad intermedia. Existen sólo 52 casos publicados en la literatura. Se presenta el caso de una paciente de 31 años con una masa en tejidos blandos en región inguinal izquierda, de crecimiento progresivo, de varios meses de evolución dolorosa. La masa fue biopsiada y, con la sospecha de un tumor fibroblástico superficial positivo para CD34, fue posteriormente tratada con una resección ampliada de la lesión y con cobertura del defecto en la piel con un colgajo local de avance de V-Y, con una evolución satisfactoria en su seguimiento postquirúrgico. El reporte de patología confirmó la sospecha diagnóstica de un tumor con reactividad fuerte para CD34, con proteína P53 en 60% a 70%, antígeno Ki67 menor al 15%, sin pérdida de proteína nuclear INI-1, y negatividad para CD31, CD163, AE1AE3, CAM5.2, EMA, CD30, receptores de progestágenos, proteína S100, y desmina, con bordes negativos.

Superficial CD34 (CD34) antigen positive fibroblastic soft-tissue tumor is a rare, lowfrequency tumor, characterized histologically by marked pleomorphism, low mitotic activity, and diffuse immunoreactivity for CD34. It may behave like a mesenchymal tumor of intermediate malignancy. There are only 52 cases published in the literature. We present the case of a 31-year-old patient with a long progressive and painful growth of a soft-tissue lesion in the left inguinal region. The mass was biopsied and, with the suspicion of a superficial CD34-positive fibroblast tumor, it was subsequently treated with an enlarged resection of the lesion and covering the skin defect with a local V-Y advancement flap, with a satisfactory evolution in the postoperative follow-up. The pathology report confirmed the diagnostic suspicion of a tumor with strong reactivity for CD34, with P53 protein in 60% to 70%, Ki67 antigen in less than 15%, without loss of INI-1, and with negativity for CD31, CD163, AE1AE3, CAM5.2, EMA, CD30, progestin receptors, S100 protein and desmin, with negative borders.

Sarcoma gástrico primario: reporte de caso y revisión de la literatura / Primary gastric sarcoma: Case report and literature review

Resumen El cáncer gástrico, patología neoplásica de innegable importancia, corresponde en el 90 % de los casos a un adenocarcinoma. Dentro del 10 % restante, los linfomas y los tumores estromales gastrointestinales (Gastrointestinal Stromal Tumor, GIST) constituyen la mayoría. Sin embargo, los sarcomas no GIST siguen siendo un diagnóstico diferencial posible para tener en cuenta y configuran una patología neoplásica de tratamiento fundamentalmente quirúrgico. En particular, el leiomiosarcoma representa menos del 1 % de los tumores malignos del estómago y la literatura disponible al respecto consiste en reportes de caso o serie de casos. Por su rareza, presentamos este caso clínico y revisamos la literatura relacionada.

Abstract Gastric cancer, a neoplastic pathology of undeniable importance, accounts for 90% of cases to adenocarcinoma. GIST lymphomas and gastrointestinal stromal tumors are the majority of the other 10%. However, non-GIST sarcomas remain a possible differential diagnosis to keep in mind and constitute a neoplastic pathology whose treatment is fundamentally surgical. Leiomyosarcoma represents less than 1% of malignant stomach tumors, and the available literature consists of case reports or case series. Because of its rarity, we present this clinical case and review the literature.

Tumores ano-rectales del músculo liso / Smooth muscle ano-rectal tumors

Introducción: Los tumores ano-rectales del musculo liso son raros, la relación respecto de los de recto es de 0.1%, presentándose con un rango entre 40-70 años. El objetivo es analizar una serie de pacientes, el tratamiento empleado y actualización bibliográfica. Material y Método: Sobre una base de datos retrospectiva entre enero de 1983 y diciembre de 2018, sobre 421 pacientes operados por cáncer recto-anal, fueron extraídos 6 que correspondieron a tumores del musculo liso. Resultados: Correspondieron al sexo femenino 4, con edades entre 49 y 75 años (57.5 años); 4 de localización rectal, de ellos 3 fueron leiomiosarcoma, y 2 anales (leiomioma). En 2 se obtuvo diagnostico preoperatorio de certeza por punción mediante Tru-Cut. De 3 pacientes con leiomiosarcoma, a 2 se les realizo cirugía radical y al restante biopsia. Los 2 resecados recidivaron a los 6 meses y al año. Los 3 fallecieron entre los 2 y 16 meses por progresión de la enfermedad. La paciente con diagnóstico de leiomioma rectal, operada mediante cirugía radical, se encuentra sin recidiva a 18 meses. Los 2 pacientes resecados localmente por leiomioma de ano, presentaron en el postoperatorio absceso y fistula extraesfinteriana, uno de ellos con incontinencia severa. Ambos fueron re-operados y se encuentran asintomáticos, libres de recidiva a los 36 y 60 meses. Discusión: Los tumores del músculo liso ano-rectal son infrecuentes y presentan síntomas inespecíficos. La biopsia preoperatoria es imperiosa a fin de establecer una adecuada estrategia quirúrgica. Los malignos tienen alto índice de recidiva y mortalidad. (AU)

Introduction: Smooth muscle ano-rectal tumors are rare; the relation with respect to the rectum is 0.1%, in a patient's age range between 40-70 years. The objective is the analysis of a series of patients, the treatment used and bibliographic update. Material and method: On a retrospective, database between January 1983 and December 2018. About 421 patients operated for rectum-anal cancer, of which 6 corresponded to smooth muscle tumors. Results: Four were female, with ages between 49 and 75 years (57.5 years average); 4 were of rectal location, of which 3 were leiomyosarcoma, and 2 anal (leiomyoma). In two, a preoperative diagnosis of certainty was obtained by Tru-Cut. Two out of 3 patients with leiomyosarcoma, underwent radical surgery and the remaining one a biopsy. The two resected relapsed at 6 months and at one year. All 3 died between 2 and 16 months due to disease progression. The patient diagnosed with rectal leiomyoma, operated by radical surgery, is without recurrence at 18 months. The 2 patients resected locally for anus leiomyoma showed abscess and extrasphincteric fistula in the postoperative period, one of them with severe incontinence. Both were re-operated and are asymptomatic, free of recurrence at 36 and 60 months. Discussion: Ano-rectal smooth muscle tumors are uncommon and have nonspecific symptoms. Preoperative biopsy is imperative in order to establish an appropriate surgical strategy. Malignant tumors have a high rate of recurrence and mortality. (AU)

Angiomixoma agresivo postrasplante renal / Aggresive angiomyxoma after renal transplantation

La condición de inmunosuprimido aumenta el riesgo de cáncer en trasplantados renales, en comparación a la población general. La mejor supervivencia de esta población en los últimos años ha convertido a las neoplasias y a la enfermedad cardiovascular en las principales causas de morbi-mortalidad. Presentamos el caso de un paciente trasplantado renal que desarrolló cuatro años después del trasplante una forma inusual de tumor mesenquimatoso, el angiomixoma agresivo, que requirió resección quirúrgica amplia.

The condition of immunosuppressed increases the risk of cancer in kidney transplant patients, as compared to the general population. The best survival of inmunosupressed patients in recent years has turned both neoplasms and cardiovascular diseases into the main causes of morbidity and mortality. We present the case of a renal transplanted patient who developed an unusual form of mesenchymal tumor such as the aggressive angiomyxoma, four years after the implant and requiring wide surgical resection.

Angiomyofibroblastoma of the vulva: A diagnostic dilemma / Philippine Journal of Obstetrics and Gynecology

@#Angiomyofibroblastoma (AMFB) is a rare, benign, well-circumscribed mesenchymal soft tissue neoplasm redominantly occurring in the vulvovaginal region among women of reproductive age (35-45 years old). Histologically, it is characterized by presence of alternating hypo and hypercellular areas containing spindle and round stromal cells admixed with blood vessels. At present, there are 137 cases reported since it was first described in 1992. Currently, there are no published cases of Angiomyofibroblastoma in the Philippines. This is a case report of a 31 year old, nulligravid patient who presented with unilateral labial enlargement which was noted to be painless and slow growing. The initial impression was that of a benign tumor of the labia. The mass was excised and histopathologic results were consistent with the microscopic findings of Angiomyofibroblastoma. Simple excision of the mass is the only treatment, as in this case, and is noted to have extremely low rate of recurrence. The clinical importance to distinguish this unusual neoplasm from Aggressive Angiomyxoma and other mesenchymal malignant neoplasm with metastatic potential should be emphasized for appropriate management.

Tumores del estroma gastrointestinal / Gastrointestinal stromal tumors

Antecedentes: Los tumores del estroma gastrointestinal (TEG) son los tumores mesenquimales más comunes del tracto gastrointestinal (TGI), se considera que surgen de las células de Cajal, ocurren principalmente en adultos mayores (60-65 años) y se localizan en estómago (50%-70%), intestino delgado (25%-35%), colon-recto (5%-10%) y esófago (<5%). La mayoría se presenta de manera esporádica y hasta el 70% son clínicamente sintomáticos. El diagnóstico definitivo se realiza en el estudio anatomopatológico. El pronóstico de estos tumores se determina por el tamaño, recuento mitótico y localización del tumor, clasificandose: riesgo muy bajo, riesgo bajo, riesgo intermedio y riesgo alto. La cirugía es la opción terapéutica principal...

Background: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastrointestinal tract (GI), areconsidered to arise from the Cajal cells. Theyoccur mainly in older adults, 60-65 years. Theypresent in the stomach (50%-70%), small intestine (25%-35%), colon and rectum (5%-10%)and esophagus (<5%). Most GISTs are sporadicand are clinically symptomatic. The definitivediagnosis is made through anatomic pathology study. To determine the prognosis of this type of tumors we use the size, mitotic count and location of the tumor, classified them in: very low risk, low risk, intermediate risk and high risk. Surgery is the main treatment...

Leiomiosarcoma de duodeno: reporte de caso / A Case of Duodenal Leiomyosarcoma

El leiomiosarcoma de duodeno es una patología poco frecuente con mal pronóstico. Los pacientes presentan un cuadro clínico poco específico y el diagnóstico se realiza a través del estudio histopatológico en donde se reconoce una lesión mesenquimal conformada por células tumorales malignas; en el estudio de inmunohistoquímica se observa positividad para actina de músculo liso, desmina, actina de músculo específico, caldesmon y calponina. Se presenta el caso de una mujer de 56 años con leiomiosarcoma primario de duodeno con metástasis al páncreas y al tronco celíaco.

Duodenal leiomyosarcoma is a rare disease with poor prognoses for patients. The disease does not have a clear set of signs and symptoms that allows easy diagnosis. Diagnosis is made on the basis of histopathological identification of a mesenchymal lesion composed of malignant tumor cells and immunohistochemistry positive for smooth muscle actin, desmin, muscle specific actin, calponin and caldesmon. We report a case of a 56 year old woman with primary duodenal leiomyosarcoma which had metastasized to the pancreas and the celiac artery.

Synchronous Gastrointestinal Stromal Tumors: A Rare Case Report.

Gastrointestinal stromal tumors (GISTs) are a subset of GI mesenchymal tumors showing neural, smooth muscle, epitheloid types of varying differentiation accounting for approximately 80% of GI mesenchymal tumors. Over 90% of GISTs occur in adults over 40 years, the most common location being stomach (50-60%) and small intestine (30-40%). We report a case of a 70-year-old man who presented with diffuse dull pain in the abdomen, a gradual loss of appetite and weight since last eight months. He was investigated and a diagnosis suggestive of GI stromal tumor-NOS type with synchronous multicentric origin having an indeterminate malignant potential was made.

Osteomalacia por tumor secretor de FGF-23 / Ostemalacia due to a tumor secreting FGF-23

Se presenta un caso de osteomalacia oncogénica en un varón de 50 años, con fuertes dolores óseos y gran debilidad muscular durante 4 años. Tenía varias deformidades vertebrales dorsales en cuña, fracturas en ambas ramas iliopubianas y en una rama isquiopubiana, y una zona de Looser en la meseta tibial derecha. Se localizó un tumor de 2 cm de diámetro en el hueco poplíteo derecho mediante centellograma con octreótido marcado con tecnecio. El tumor fue extirpado quirúrgicamente. La microscopía mostró un tumor mesenquimático fosfatúrico, de tejido conectivo mixto. La inmunotinción demostró FGF-23. Hubo rápida mejoría, con consolidación de las fracturas pelvianas y de la pseudofractura tibial y normalización de las alteraciones bioquímicas.

A case of oncogenic osteomalacia in a 50-year-old male is here presented. He suffered severe bone pain and marked muscular weakness of 4 years' duration. There were several vertebral deformities in the thoracic spine, bilateral fractures of the iliopubic branches, another fracture in the left ischiopubic branch, and a Looser's zone in the right proximal tibia. An octreotide-Tc scan allowed to identify a small tumor in the posterior aspect of the right knee. It was surgically removed. Microscopically, it was a phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT). Expression of FGF-23 was documented by immune-peroxidase staining. There was rapid improvement, with consolidation of the pelvic fractures and the tibial pseudo-fracture. The laboratory values returned to normal.

Tumores estromales gastrointestinales: evaluación clinicopatológica y sobrevida en el Hospital Rebagliati / Gastrointestinal stromal tumors: clinicopathologic and survival evaluation in Rebagliati Hospital

OBJETIVO: Determinar las manifestaciones clínicas, radiológicas, histopatológicas y sobrevida de los pacientes con Tumor Estromal Gastrointestinal (GIST) en el Hospital Nacional Edgardo Rebagliati Martins (HNERM), de Lima, Perú. MATERIAL Y MÉTODOS: El presente es un estudio descriptivo, retrospectivo. El cual se realizó a partir de las historias clínicas de 103 pacientes con GIST confirmado por inmunohistoquímica que fueron evaluados y tratados en el HNERM, desde Enero del 2002 hasta Diciembre de 2010. RESULTADOS: En los 103 pacientes el promedio de edad fue 64 años (entre 30 y 88 años). Predominó en mujeres (52%). El tiempo de enfermedad promedio fue 7 meses. La forma de presentación más frecuente fue sangrado digestivo (48.3%). El diagnóstico se hizo más por endoscopía (50.5%). La prevalencia por órganos fue más frecuente en estómago 56.3%. El promedio de tamaño fue 98mm, (49% entre 50mm y 100mm), el tumor de mayor tamaño alcanzaba 260 mm. El estadio tumoral más frecuente fue localizado (70.9%). Presentaron otro cáncer asociado el 9.7% de pacientes El patrón histológico predominante fue fusiforme 73.8%. El 84.5% tuvo bajo índice mitótico. La inmunohistoquímica mostró la expresión de KIT (CD 117) 94.17%, CD 34 77.5%, Vimentina 96.6%, NSE 84.9%, Alfa actina 52.7%, CD56 44.4%, S-100 32.3% y Actina 20.0%. La característica tomográfica más frecuente fue tumor heterogéneo (43.6%). La distribución según clasificación de riesgo fue: muy bajo riesgo 3.9%, bajo riesgo 28.2%, riesgo intermedio 37.7% y alto riesgo 30.1%. La resección quirúrgica completa se realizó en 87.4% de pacientes, 4.9% de pacientes recibió Imatinib. La sobrevida global acumulada a 5 años fue 31.07%. En el análisis bivariado se encontró asociación estadística entre el haber sobrevivido con: ausencia de cáncer asociado p= 0.004, CD 34 p=0.01, índice mitótico bajo p=0.00 y tratamiento quirúrgico recibido p= 0.000. En el análisis multivariado se encontró asociación estadística de mayor sobrevida con: los de menor tamaño del tumor p=0.015 (IC -3.67, -0.41), estadio tumoral localizado p=0.036 (IC -5.83, -0.19), menor índice mitótico p=0.038 (IC -0.86, 0.02), paciente asintomático p=0.009 (IC 1.25, 8.62), no recidiva del tumor p=0.01 (IC -8.49, -1.17) y el no presentar metástasis p=0.001 (IC 2.66, 10.62). CONCLUSIONES: Los resultados de nuestro estudio fueron similares a lo que reporta la literatura internacional. Los factores que se asociaron a mayor sobrevida fueron: haber recibido tratamiento quirúrgico, pacientes con menor tamaño tumoral, estadio tumoral localizado, índice mitótico bajo, paciente asintomático, no recidiva del tumor, no metástasis y no cáncer asociado.

OBJECTIVE: To determine the clinical, radiological, histopathological manifestations and survival of patients with gastrointestinal stromal tumor (GIST)in the National Hospital Edgardo Rebagliati Martins (HNERM) from Lima, Perú. MATERIAL AND METHODS: This is a descriptive and retrospective study, which was based on the medical records of 103 patients with confirmed GIST with immunohistochemical. All the patients were evaluated and treated at the HNERM, from January 2002 until December 2010. RESULTS: In 103 patients between 30 and 88 years the average age was 64 years. The tumor was more frequent in females (52%). The mean disease duration was 7 months. The most frequent form of presentation was gastrointestinal bleeding (48.3%). The diagnosis was made more by endoscopy (50.5%). The prevalence of GISTs by organs was more frequent in stomach (56.3%). The average size of the tumors was 98mm, 49% had a size between 50mm and 100mm, the largest tumor was 260 mm. Tumor stage more frequent was localized (70:9%). GIST associated with another cancer was 9.7% of patients. The predominant histologic pattern was fusiform (73.8%). The 84.5% had low mitotic index. Immunohistochemistry showed expression KIT (CD 117) was 94.17%, CD34 77.5%, Vimentin 96.6%, NSE 84.9%, alpha actin 52.7%, CD56 44.4%, S-100 32.3% and Actin 20%. The tomographic characteristic more frequent was heterogeneous tumor (43.6%).The distribution according to risk classification was: very low risk 3.9%, low risk 28.2, intermediate risk 37.7% and high risk 30.1%. Complete surgical resection was performed in 87.4% of patients and 4.9% of patients received imatinib. The cumulative overall survival at 5 years was 31.07%. In bivariate analysis statistical association was found between surviving with: no presence of cancer associated p = 0.004, CD 34 p = 0.01, low mitotic index p = 0.00 and received surgical treatment p = 0.000. In multivariate analysis one found statistical association of longer survival with smaller tumor size p = 0.015 (CI -3.67, -0.41), localized tumor stage p = 0.036 (CI -5.83, -0.19), lower mitotic index p = 0.038 (CI -0.86, 0.02), asymptomatic patient p=0.009 (CI 1.25, 8.62), no tumor recurrence p = 0.01 (CI -8.49, -1.17), and no metastasis p = 0.001 (CI 2.66, 10.62). CONCLUSIONS: The results of our study were similar to what was reported in international literature. Factors that were associated with longer survival were receiving surgical treatment, patients with smaller tumor size, tumor stage localized, low mitotic index, asymptomatic patient, not tumor recurrence, not metastasis and no cancer associated.

Clinicopathological spectrum of 19 adenosarcomas of female genital tract, including uncommon clinical associations and immunohistochemical profile, reviewed at a single institution.

Background: Adenosarcomas of the female genital tract have been rarely documented as case series from our continent. Materials and Methods: Over a seven-year period, 19 adenosarcomas were critically reviewed. Results: Nineteen tumors occurred in the age range of 21-65 years (mean: 43), in the endometrium (8), endometrium and cervix (4), cervix (4), and ovary (3). Four cases displayed coexisting leiomyomas; two, adenomyosis; two on background endometriosis; and one in post-treated cervix carcinoma. Histopathologically, the tumors were low grade (10; 52.6%) and high grade (9; 47.3%), the latter with sarcomatous overgrowth (SO) (7/9 cases). Dedifferentiation (8, 42.1%) and conspicuous decidualization (2) were noted. Immunohistochemically, the tumors focally expressed CD10 (4/6), smooth muscle actin (SMA) (3/8), desmin (8/11); diffuse vimentin (7/7), and estrogen receptor/progesterone receptor (ER/PR) (2/4). Ki-67 (6 cases) varied 5-20%. Seventeen patients underwent surgery and four received adjuvant treatment (3/4 high-grade tumors). Five tumors recurred (4 high-grade tumors with SO) and one metastasized. Among 11 patients, five were alive with disease (AWD) (mean: 29.4 months) and six, free of disease (FOD) (mean: 15 months), the latter mostly with low-grade type tumors (83.3% cases). Conclusions: Diverse clinicopathological spectrum was noted within adenosarcomas. Low-grade tumors were less aggressive than high-grade ones, with SO. Immunohistochemically, lower CD10 and ER/PR positivity was noted in high-grade tumors. Surgery formed the mainstay of treatment. Adjuvant treatment was offered in high-grade subtypes, including in tumors with SO.

Tumores mesenquimales diagnosticados en la unidad de gastroenterología del HMPC entre los años 2007 aL 2009 / Mesenchymal tumors diagnosed in the gastroenterology unit of HPMC from 2007 to 2009

Se evaluaron las historias médicas con diagnóstico de tumores mesenquimales confirmados por análisis inmunohistoquímico entre los años 2007 y 2009. El objetivo fue describir a la población según características clínicas, epidemiológicas, diagnósticos por imágenes y análisis anatomopatológico. Se obtuvieron 11 pacientes con diagnósticos de GIST(7), Leiomiomas(2), GANT(1) y Leiomiosarcoma(1). Del género femenino (82%) y masculino (18%). Con un promedio de 55 años de edad. Clínicamente presentaron dolor abdominal (45%), mareos (27%), pirosis (9%) y disfagia (1%). El 18% permaneció asintomático. Entre los signos encontrados figuran: melena (36%), pérdida de peso (27%), palidez cutánea (9%) y vómitos (9%). El 36% de los pacientes no presentaron hallazgos al examen físico. Se realizó ultrasonido abdominal en el 100% de los pacientes, con hallazgos patológicos relacionados con el tumor en el 27 % de los casos. Al 90% de los pacientes se les realizó una Endoscopia Digestiva Superior, el 82% de los hallazgos se describieron como Tumores Submucosos y 9% se reportó como normal. Las lesiones se ubicaron en el estómago (60%), 27% en el intestino delgado ( duodeno y yeyuno); 9% en esófago y 9% en retroperitoneo. El ultrasonido endoscópico fue practicado al 82% de los pacientes, con un porcentaje de aciertos diagnósticos en el 90% de los tumores localizados en esófago, estómago e intestino delgado, y de 87,5% si se incluye el tumor retroperitoneal. La Tomografía Computada (TC) de abdomen fue practicada en 45% de los pacientes, con hallazgos patológicos en el 100% de los casos, descritos como Lesiones Ocupantes de Espacio (LOE), en estómago, intestino, esófago y retroperitoneo; y en el 20% de los pacientes se encontró enfermedad metastásica hepática Recibieron tratamiento quirúrgico el 82%, el 18% restante no se realizó por contraindicación médica o se encuentran en espera del procedimiento. Y un 18% de los pacientes recibieron tratamiento médico con Imatinib...

We evaluated the clinical records with a diagnosis of mesenchymal tumors confirmed by immunohistochemical analysis, from 2007 to 2009. The objective was to describe the population according to clinical and epidemiological features, diagnostic imaging and histopathological analysis. We found 11 patients with diagnoses of GIST(7), leiomyoma(2), leiomyosarcoma(1) and GANT(1); (82%) female and (18%) male, with a mean age of 55 years. They clinically presented abdominal pain (45%), dizziness (27%), heartburn (9%) and dysphagia (1%). 18% remained asymptomatic. Among the symptoms were: melena (36%), weight loss (27%), paleness (9%) and vomiting (9%). 36% of the patients had no findings at physical examination. Abdominal ultrasound was performed in 100% of the patients, with pathological findings related to the tumor in 27% of cases. In 90% of patients an upper digestive endoscopy was performed. 82% of findings were described as submucosal tumors and 9% was reported as normal. Lesions were located in stomach (60%); 27% in small bowel (duodenum and jejunum), 9% in esophagus and 9% in retroperitoneum. Endoscopic ultrasound was performed to 82% of patients, with a diagnostic accuracy of 90% for the tumors located in esophagus, stomach and small bowel; and 87,5% if the retroperitoneal tumor is included. The abdomen Computed Tomography (CT) was performed in 45% of patients with pathological findings in 100% of the cases, described as Space occupying lesion (SOL) in stomach, intestine, esophagus and retroperitoneum; and, in 20% of patients metastatic liver cancer was found. 82% received surgical treatment, the remaining 18% was not performed due to contraindication or are waiting for the procedure. And 18% of patients received medical therapy with Imatinib...

Diagnosis and treatment of gastrointestinal stromal tumors: an analysis of 26 cases / 中国基层医药

Objective To explore the diagnosis and treatment of gastrointestinal stromal tumors(GIST).Methods 26 patients with gastrointestinal stromal tumors in Ji'an Central People's Hospital from 1998 to 2006 Were retrospectively analyzed.Results All patients received operation.Of them,low malignant GIST wag in 17 eases,high malignant GIST in 9.All patients were follwod-up from 6 to 84 months.4 ofthem had local recurrence and metastasis and 5 eases died.Condusion The diagnosis of GIST Was dimcult because of the lack of clinical features.Final diagnosis should be based on IHC pathology.The main choice of treatment Was operation.Glivee could prolong the life and improve the prognosis of the patients in recurrent and metastatic cases.

Tumor estromal gastrointestinal del intestino delgado con expresión negativa del marcador oncogénico KIT (CD117): A propósito de un caso

Los tumores estromales del tracto gastrointestinal (GIST), aunque infrecuentes, son tumores mesenquimatosos importantes de origen mesodérmico. Macroscópicamente se presentan como una masa intramural submucosal, siendo los mas comunes dentro de los tumores mesenquimatosos. La forma maligna, típicamente agresiva y resistente al tratamiento, es afortunadamente rara. Un importante avance ha sido la caracterización del marcador oncogénico KIT y su relación a la patogénesis del Tumor Estromal Gastrointestinal. Sin embargo, recientes investigaciones han demostrado la génesis de los GIST que son KIT negativos. Por lo tanto, la positividad del marcador oncogénico KIT, si bien es altamente sensible, no es requerimiento indispensable para el diagnóstico de GIST. Presentamos nuestra paciente con un GIST del intestino delgado y negatividad al c-KIT(CD 117).

Gastrointestinal stromal tumors (GIST) are uncommon but important mesenchymal tumors of the Gastrointestinal Tract, defined as being of mesodermal origin. Macroscopically they present as intramural submucosal masses, being the most common mesenchymal tumors to arise from the gastrointestinal tract. The malignant form, typically aggressive and resistant to treatment, in fortunately rare. An important development has been the characterization of the oncogene marker KIT, and its relation to the pathogenesis of GIST. However recent research has shed light on the genesis of GIST that is KIT negative. KIT is highly sensitive for GIST (though not 100%) but is not a marker of malignancy or essential requirement to the diagnosis of GIST. We show a patient with a GIST of the small intestine, KIT- negative.