perfil clínico, endoscópico e histológico de los tumores estromales gastrointestinales en el Hospital Rebagliati

ABSTRACT RESUMEN INTRODUCCIÓN: Los Tumores Estromales Gastrointestinales (GIST) son lesiones que se originan a partir de las células intersticiales de Cajal, y pueden tomar cursos asintomáticos o producir complicaciones, como hemorragia digestiva y degeneración maligna. OBJETIVO: Determinar el perfil clínico, endoscópico e histológico de los pacientes con GIST diagnosticados en el Hospital Nacional Edgardo Rebagliati Martins (Lima-Perú), en enero del 2002 y diciembre del 2004. MÉTODO: Estudio descriptivo, transversal, retrospectivo en el cual se revisaron las historias clínicas e informes anatomopatológicos de 34 pacientes con diagnóstico de GIST, vertiéndose los datos en una ficha de recolección. RESULTADOS: . Predominó el sexo masculino (58.82%) sobre el femenino. El grupo etáreo más afectado fue el de 71-80 años, con 26.47%, la edad promedio fue 64.7 años, con un rango de 30 a 86 años. La forma de presentación más frecuente fue dolor abdominal (47.06%) seguido de melena (44.12%). El diagnóstico se hizo por método endoscópico en 58.82%. El tipo histológico predominante fue el fusiforme (47.06%). Hubo riesgo de malignidad alto en 50% de casos, y la localización más frecuente fue estómago (61.77%). Endoscópicamente se manifiesta por la presencia de tumoración submucosa con ulceración y tamaño promedio de 6.44 cm. Se halló relación estadísticamente significativa entre método endoscópico y melena como forma de presentación (p<0.05). CONCLUSIÓN: Los pacientes con GIST en el HNERM son en su mayoría varones, mayores de 60 años, que se presentan con dolor abdominal y melena, y es la endoscopía el mejor método para diagnosticarlos si acuden por melena (p < 0.05). Predomina la histología fusiforme. ABSTRACT INTRODUCTION: Gastrointestinal Stromal Tumors (GIST) are lesions which origin from Intersticial Cells of Cajal that may be asymptomatic or cause complications like digestive bleeding or malignant transformation. AIM: To determine the clinical, endoscopic and histological profile, as well as the main demographic features of patients with GIST in Hospital Edgardo Rebagliati (Lima-Perú) from January 2002 to December 2004. METHODS: Descriptive, transversal and retrospective study in which we reviewed clinical and anatomopathological archives from 34 patients with GIST during the above mentioned period. The collected data were placed on a sheet of recollection. RESULTS: There was male predominance (58.82%).The most affected age group was 71-80 years old (26.47%). The average age was 64.7 years old. The most frequent clinical presentation was abdominal pain (47.06%) followed by melena (44.12%). Diagnosis was made by endoscopic method in 58.82%. The main histological type was spindle (47.06%). There was high risk of malignancy in 50% and the most often involved site was stomach (61.77%). Endoscopicall the characteristic image was a submucosal tumor with ulceration, the average size was 6.44 cm. There was significant statistical relation (p< 0.05) between endoscopic method and melena.

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    ABSTRACT: The interstitial cells of Cajal (ICC) form a complex cell network within the gastrointestinal tract wall where they function as a pacemaker system. Expression of the kit proto-oncogene is essential for the development of this system. The aim of our study was to examine the hypothesis that gastrointestinal stromal tumors differentiate toward cells with an ICC phenotype. Ultrastructurally, 58 stromal tumors were characterized and found to share many features with ICC. Seventy-eight stromal tumors were immunophenotyped, particularly with regard to the kit receptor. All 78 tumors revealed strong, homogeneous immunoreactivity for the kit receptor as did ICC of adjacent and control gastrointestinal walls. Focal hyperplasia and hypertrophy of kit receptor positive cells were also observed in the gastrointestinal wall adjacent to the tumors. CD34 immunoreactivity observed in interstitial cells surrounding Auerbach's ganglia suggests that a subpopulation of ICC is CD34 positive and may explain why 56 of 78 stromal tumors were CD34 positive. Thirty control tumors, including gastrointestinal leiomyomas and leiomyosarcomas, were all negative for the kit receptor. We conclude that gastrointestinal stromal tumors show striking morphological and immunophenotypic similarities with ICC and that they may originate from stem cells that differentiate toward a pacemaker cell phenotype. We propose that the noncommittal name "gastrointestinal stromal tumor" be replaced by gastrointestinal pacemaker cell tumor.
    American Journal Of Pathology 06/1998; 152(5):1259-69. · 4.59 Impact Factor
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    ABSTRACT: From 1984 to 1989 the clinicopathological aspects of 21 patients with smooth muscle tumours of the stomach were reviewed. Ten patients had leiomyomas: 7 males, 3 females, average age 53 +/- 8 years. Four out of seven leiomyomas presenting with haematemesis were diagnosed correctly by an endoscopist but biopsy was positive in only two patients. Three leiomyomas were found incidently at laparotomy, and a total of nine leiomyomas were resected with a cuff of normal stomach. Their size ranged from 2 to 20 cm. All patients who underwent local resection are alive. Eight patients had leiomyosarcomata: 3 males, 5 females, average age 62 +/- 9 years. Six of these patients had an epigastric mass and four had haematemesis or melaena. The remaining patient in this group presented with dysphagia. Seven of these patients, who had no evidence of metastatic disease, underwent exploratory laparotomy. In four, palliative resections of the stomach and distal oesophagus were performed. In two, exogastric lesions involving colon and liver respectively were resected en bloc with a cuff of stomach. In one, biopsy alone was carried out. Two patients in this group are alive 1 and 2 years following surgery. Adjuvant treatment was not given to any of our patients. The remaining three tumours comprised a smooth muscle tumour of undetermined malignant potential whose clinical behaviour was unpredictable, and two leiomyoblastomas which mimicked malignant tumours in their presentation.
    Journal of the Royal College of Surgeons of Edinburgh 03/1993; 38(1):23-7.
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    ABSTRACT: The authors carry out a retrospective review of 30 patients with gastrointestinal stromal tumours (GISTs) who underwent surgical treatment over the period from 1974 to 2001. Sixteen were male and 14 female, with an average age of 60.9 years. Histologically, 19 tumours showed evidence of differentiation towards smooth muscle elements (10 benign and 9 malignant), 9 towards neural elements (3 benign and 6 malignant) and 2 iacked differentiation towards either cell type. Twenty-one tumours were located in the stomach, 1 in the duodenum, 3 in the jejunum and 5 in the ileum. The main symptoms were abdominal pain and abdominal masses, and the most sensitive diagnostic techniques were abdominal CT scan and endoscopy in gastroduodenal locations. In 21 gastric GISTs, the surgical procedures were local resection (15 cases), partial gastric resection (3 cases), subtotal gastrectomy (2 cases) and total gastrectomy (1 case). In 8 small bowel GISTs, we performed a typical intestinal resection while duodenal undifferentiated GIST was managed by pancreatico-duodenectomy. There was no operative mortality or morbidity. Among the 13 patients with benign GISTs, 1 died of causes unrelated to the disease, while 12 patients are still alive and in good health after a mean follow-up of 148.5 months (range: 6-262). Among patients with malignant muscular GISTs (6 gastric and 3 ileal), 3 with gastric tumours were lost to follow-up, 3 with gastric and 1 with ileal neoplasms are alive and free from disease after a median follow-up of 58 months (range 3-108), while 2 patients with ileal neoplasms died of metastatic disease 39 and 29 months after the surgical procedure. Among 6 patients with malignant neural GISTs (2 gastric, 2 jejunal and 2 ileal) 1 with a gastric tumour and 1 with a jejunal tumour were lost to follow-up, while 3 are still alive and in good health after a median follow-up of 67.6 months (range 19 to 94); another with jejunal disease developed liver metastases 14 months after small bowel resection and died 12 months later. The two patients with undifferentiated GIST both died of liver metastasis 38 months after pancreatico-duodenectomy and 43 months after total gastrectomy. The most frequent symptoms were abdominal pain and a palpable mass, but no specific signs were detected. In gastroduodenal lesions endoscopy plays a very important diagnostic role and CT scan is the most sensitive diagnostic technique in the evaluation of location, size, invasion of adjacent organs and metastases. Prognostic prediction on the basis of histological findings is difficult and in our experience undifferentiated tumours are always malignant.
    Chirurgia italiana 01/2001; 53(6):809-20.
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