M Ambrosio1, A C Testa2,3, F Moro2, D Franchi4, M C Scifo1, N Rams5, E Epstein6, J L Alcazar7, J J Hidalgo8, C Van Holsbeke9, A Burgetova10, P Dundr11, D Cibula12, D Fischerova12. 1. Department of Obstetrics and Gynecology, S. Orsola Malpighi Hospital, University of Bologna, Bologna, Italy. 2. Dipartimento Scienze della Salute della Donna e del Bambino, Fondazione Policlinico Universitario A. Gemelli, IRCCS, Rome, Italy. 3. Dipartimento Scienze della Vita e Sanità pubblica, Università Cattolica del Sacro Cuore, Rome, Italy. 4. Division of Gynecology, European Institute of Oncology, Milan, Italy. 5. Hospital Santa Cruz y San Pablo, Barcelona, Spain. 6. Department of Clinical Science and Education, Karolinska Institutet & Department of Obstetrics and Gynecology, Södersjukhuset, Stockholm, Sweden. 7. Department of Obstetrics and Gynecology, Clinica Universidad de Navarra, University of Navarra, Pamplona, Spain. 8. Department of Obstetrics and Gynecology, Clinic Hospital Valencia, Valencia, Spain. 9. Ziekenhuis Oost-Limburg, ZOL, Genk, Belgium. 10. Department of Radiology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic. 11. Department of Pathology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic. 12. Department of Obstetrics and Gynecology, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic.
Abstract
OBJECTIVE: To describe the clinical and sonographic characteristics of extragastrointestinal stromal tumors (eGISTs). METHODS: This was a retrospective multicenter study. The data of patients with a histological diagnosis of eGIST who had undergone preoperative ultrasound examination were retrieved from the databases of nine large European gynecologic oncology centers. One investigator from each center reviewed stored images and ultrasound reports, and described the lesions using the terminology of the International Ovarian Tumor Analysis and Morphological Uterus Sonographic Assessment groups, following a predefined ultrasound evaluation form. Clinical, surgical and pathological information was also recorded. RESULTS: Thirty-five women with an eGIST were identified; in 17 cases, the findings were incidental, and 18 cases were symptomatic. Median age was 57 years (range, 21-85 years). Tumor marker CA 125 was available in 23 (65.7%) patients, with a median level of 23 U/mL (range, 7-403 U/mL). The vast majority of eGISTs were intraperitoneal lesions (n = 32 (91.4%)); the remaining lesions were retroperitoneal (n = 2 (5.7%)) or preperitoneal (n = 1 (2.9%)). The most common site of the tumor was the abdomen (n = 23 (65.7%)), and less frequently the pelvis (n = 12 (34.3%)). eGISTs were typically large (median largest diameter, 79 mm) solid (n = 31 (88.6%)) tumors, and were less frequently multilocular-solid tumors (n = 4 (11.4%)). The echogenicity of solid tumors was uniform in 8/31 (25.8%) cases, which were all hypoechogenic. Twenty-three solid eGISTs were non-uniform, either with mixed echogenicity (9/23 (39.1%)) or with cystic areas (14/23 (60.9%)). The tumor shape was mainly lobular (n = 19 (54.3%)) or irregular (n = 10 (28.6%)). Tumors were typically richly vascularized (color score of 3 or 4, n = 31 (88.6%)) with no shadowing (n = 31 (88.6%)). Based on pattern recognition, eGISTs were usually correctly classified as a malignant lesion in the ultrasound reports (n = 32 (91.4%)), and the specific diagnosis of eGIST was the most frequent differential diagnosis (n = 16 (45.7%)), followed by primary ovarian cancer (n = 5 (14.3%)), lymphoma (n = 2 (5.7%)) and pedunculated uterine fibroid (n = 2 (5.7%)). CONCLUSIONS: On ultrasound, eGISTs were usually solid, non-uniform pelvic or abdominal lobular tumors of mixed echogenicity, with or without cystic areas, with rich vascularization and no shadowing. The presence of a tumor with these features, without connection to the bowel wall, and not originating from the uterus or adnexa, is highly suspicious for eGIST.
OBJECTIVE: To describe the clinical and sonographic characteristics of extragastrointestinal stromal tumors (eGISTs). METHODS: This was a retrospective multicenter study. The data of patients with a histological diagnosis of eGIST who had undergone preoperative ultrasound examination were retrieved from the databases of nine large European gynecologic oncology centers. One investigator from each center reviewed stored images and ultrasound reports, and described the lesions using the terminology of the International Ovarian Tumor Analysis and Morphological Uterus Sonographic Assessment groups, following a predefined ultrasound evaluation form. Clinical, surgical and pathological information was also recorded. RESULTS: Thirty-five women with an eGIST were identified; in 17 cases, the findings were incidental, and 18 cases were symptomatic. Median age was 57 years (range, 21-85 years). Tumor marker CA 125 was available in 23 (65.7%) patients, with a median level of 23 U/mL (range, 7-403 U/mL). The vast majority of eGISTs were intraperitoneal lesions (n = 32 (91.4%)); the remaining lesions were retroperitoneal (n = 2 (5.7%)) or preperitoneal (n = 1 (2.9%)). The most common site of the tumor was the abdomen (n = 23 (65.7%)), and less frequently the pelvis (n = 12 (34.3%)). eGISTs were typically large (median largest diameter, 79 mm) solid (n = 31 (88.6%)) tumors, and were less frequently multilocular-solid tumors (n = 4 (11.4%)). The echogenicity of solid tumors was uniform in 8/31 (25.8%) cases, which were all hypoechogenic. Twenty-three solid eGISTs were non-uniform, either with mixed echogenicity (9/23 (39.1%)) or with cystic areas (14/23 (60.9%)). The tumor shape was mainly lobular (n = 19 (54.3%)) or irregular (n = 10 (28.6%)). Tumors were typically richly vascularized (color score of 3 or 4, n = 31 (88.6%)) with no shadowing (n = 31 (88.6%)). Based on pattern recognition, eGISTs were usually correctly classified as a malignant lesion in the ultrasound reports (n = 32 (91.4%)), and the specific diagnosis of eGIST was the most frequent differential diagnosis (n = 16 (45.7%)), followed by primary ovarian cancer (n = 5 (14.3%)), lymphoma (n = 2 (5.7%)) and pedunculated uterine fibroid (n = 2 (5.7%)). CONCLUSIONS: On ultrasound, eGISTs were usually solid, non-uniform pelvic or abdominal lobular tumors of mixed echogenicity, with or without cystic areas, with rich vascularization and no shadowing. The presence of a tumor with these features, without connection to the bowel wall, and not originating from the uterus or adnexa, is highly suspicious for eGIST.