Imaging in gynecological disease (19): clinical and ultrasound features of extragastrointestinal stromal tumors (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. Copyright © 2020 ISUOG. Published by John Wiley & Sons Ltd.

Utility of diffusion-weighted and contrast-enhanced magnetic resonance imaging in diagnosing and differentiating between high- and low-grade uterine endometrial stromal sarcoma.

BACKGROUND: Endometrial stromal sarcoma (ESS) is a rare uterine malignancy that features different prognoses for its high- and low-grade subtypes. We investigated the diagnostic accuracy of magnetic resonance (MR) imaging in diagnosing and differentiating between high- and low-grade ESS. METHODS: We retrospectively reviewed the preoperative pelvic MR images of consecutive patients who received histologically confirmed diagnoses of high-grade ESS (n = 11) and low-grade ESS (n = 9) and T2-hyperintense leiomyoma (n = 16). Two radiologists independently evaluated imaging features in T1-, T2-, and diffusion-weighted and contrast-enhanced MR images. Statistical analysis included Mann-Whitney tests and Fisher's exact test, with sensitivity, specificity and diagnostic accuracy of imaging features. RESULTS: High-grade ESS was associated with significantly more extensive necrosis and hemorrhage and distinct feather-like enhancement compared with low-grade ESS (P < .05 for all). The feather-like enhancement pattern yielded a diagnostic accuracy of 95%, sensitivity of 91%, and specificity of 100% in differentiating high-grade from low-grade ESS. This imaging characteristic was significantly superior to the necrosis (80%, P = .033) or hemorrhage (75%, P = .007). Both high- and low-grade ESS demonstrated T2 hypointense bands, marginal nodules, intratumoral nodules, and worm-like intra-myometrial nodules, and their tumor apparent diffusion coefficient (ADC) values were significantly lower than those of T2-hyperintense leiomyomas (P < .001). CONCLUSIONS: Diffusion-weighted MR imaging is useful in diagnosing ESS against T2-hyperintense leiomyomas, and contrast enhancement aids in further differentiating between high- and low-grade ESS.

Endometrial Stromal Sarcoma Arising from Endometrial Polyp: A Case Report.

Endometrial stromal sarcoma (ESS) is a rare malignant tumor of the uterus. We report an uncommon case of ESS composed of both low-grade ESS and high-grade ESS arising from an endometrial polyp. On the findings of magnetic resonance imaging and contrast computed tomography, a patient was suspected of having uterine malignant tumor. She underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. Macroscopically, the tumor was a polypoid lesion in the uterine cavity. The tumor was an endometrial polyp with ESS components. ESS was composed of low-grade ESS and high-grade ESS. By immunohistochemistry, both an endometrial polyp and low-grade ESS showed a positivity for CD10, estrogen receptor (ER), and progesterone receptor (PR). However, high-grade ESS showed only a focal and weak CD10 positivity with no immunostaining for ER and PR. A focal or diffuse positivity for α-smooth muscle actin and desmin was noted in both low-grade and high-grade ESS. The positive rates of Ki-67 and p53 in high-grade ESS were elevated up to over 95%. She was diagnosed as having ESS in a stage IA. After surgery, she received no further treatment. She has been without recurrence for 4 years since an initial surgery. In conclusion, immunohistochemical analyses are useful for make an accurate diagnosis of ESS showing a transition from low-grade ESS to high-grade ESS in addition to the conventional method.

Treatment of low-grade endometrial stromal sarcoma in a nulligravid woman.

A 32 year-old nulligravid woman with a uterine mass underwent exploratory laparotomy with myomectomy. Final pathology revealed a low-grade endometrial stromal sarcoma (ESS) with positive margins. She subsequently underwent definitive robotic hysterectomy and bilateral salpingectomy with ovarian preservation. She was diagnosed with a stage IB low-grade ESS. She is currently undergoing observation. Discussion of classification, surgical options, and adjuvant therapy is presented.

Preoperative prediction of lymph node metastasis and deep stromal invasion in women with invasive cervical cancer: prospective multicenter study using 2D and 3D ultrasound.

OBJECTIVES: To determine how various objective two-dimensional (2D) and three-dimensional (3D) ultrasound parameters allow prediction of deep stromal tumor invasion and lymph node involvement, in comparison to subjective ultrasound assessment, in women scheduled for surgery for cervical cancer. METHODS: This was a prospective multicenter trial including 104 women with cervical cancer at FIGO Stages IA2-IIB, verified histologically. Patients scheduled for surgery underwent a preoperative ultrasound examination. The value of various 2D (size, color score) and 3D (volume, vascular indices) ultrasound parameters was compared to that of subjective assessment in the prediction of deep stromal tumor invasion and lymph node involvement. Histology obtained from radical hysterectomy or trachelectomy and pelvic lymphadenectomy was considered as the gold standard for assessment. RESULTS: All women underwent pelvic lymphadenectomy, with 99 (95%) undergoing subsequent radical surgery; five underwent only pelvic lymphadenectomy because of the presence of a positive sentinel lymph node. Women with deep stromal invasion or lymph node involvement had significantly larger tumors (diameter and volume) but there was no correlation with vascular indices measured on 3D ultrasound. Subjective evaluation was superior (AUC, 0.93; sensitivity, 90.5%; specificity, 97.2%) in the prediction of deep stromal invasion when compared to any objective measurement technique, with maximal tumor diameter at 20.5-mm cut-off (AUC, 0.83; sensitivity, 90.5%; specificity, 61.1%) and 3D tumor volume at 9.1-mm(3) cut-off (AUC, 0.85; sensitivity, 79.4%; specificity, 83.3%) providing the best performance among the objective parameters. Both subjective assessment and objective measurements were poorly predictive of lymph node involvement. CONCLUSIONS: In women with cervical cancer, subjective ultrasound evaluation allowed better prediction of deep stromal invasion than did objective measurements; however, neither subjective evaluation nor objective parameters were adequate to predict lymph node involvement. 3D vascular indices were ineffective in the prediction of advanced stages of the disease.

Metastatic endometrial sarcoma with inferior vena caval and cardiac involvement: a combined surgical approach.

Low-grade endometrial stromal sarcoma with intracaval and intracardiac extension represents a complex and often lethal condition. A case of a 40-year-old woman, who underwent hysterectomy for endometrial stromal sarcoma 4 years previously and developed recurrence with intracaval and intracardiac metastases, is presented. Operative management of resection of the intracaval and intracardiac tumor is described. The literature is reviewed in order to highlight the data on diagnosis and management of recurrent endometrial stromal sarcoma with vascular involvement, including the multimodality approach required in treating this disease.

Study of the sentinel node in endometrial cancer at early stages: preliminary results.

AIM: To investigate the applicability of the sentinel lymph node biopsy technique in early stages of endometrial cancer. MATERIAL AND METHODS: A prospective study that included consecutive patients with a histological diagnosis of clinical state I endometrial carcinoma was performed. Two doses of 2 mCi (74 MBq) of (99m)Tc-albumin nanocolloid were injected in the uterine cervix, and planar and SPECT-CT images were obtained at one hour, and at 24 hours if no migration of the tracer was observed. Methylene blue dye was also injected into the cervix immediately prior to the surgery. A gamma probe was used during the surgical procedure for sentinel lymph node identification. In all cases, a hysterectomy, double adnexectomy and pelvic lymphadenectomy were performed, carrying out a histological analysis (hematoxylin-eosin) of the sentinel lymph nodes and the lymphadenectomy specimen. RESULTS: We included 19 patients, with a final diagnoses of endometrioid carcinoma (18 cases) and endometrial stromal sarcoma (1 case). At least one sentinel lymph node was identified in 17 of them (89.5% detection rate). Twenty-nine sentinel lymph nodes were identified during surgery, all of them negative for neoplastic infiltration. No metastatic invasion was found in the pelvic lymphadenectomy specimens as well. CONCLUSIONS: The sentinel lymph node biopsy technique seems to be a reliable tool in nodal staging of endometrial cancer at early stages, with an acceptable detection rate and high histological correlation. The low prevalence of lymphatic spread in this group of patients and the encouraging results obtained could make the sentinel lymph node an alternative to routine complete lymphadenectomy.

Endometrial stromal sarcoma invading the abdominal aorta treated with aortic replacement.

Endometrial stromal sarcomas are extremely rare tumors with few reports describing invasion of major blood vessels. Tumors that primarily involve the abdominal aorta or invade the vessel secondarily are uncommon. Outcomes from radical tumor resection and aortic reconstruction are thus poorly understood. We present a rare case of a symptomatic recurrent endometrial stromal sarcoma with encasement of the infrarenal abdominal aorta requiring en bloc tumor resection and in situ aortic replacement.

Characterization of papillary projections in benign versus borderline and malignant ovarian masses on conventional and color Doppler ultrasound.

OBJECTIVE: The purpose of our study was to evaluate on endovaginal ultrasound the morphologic and color Doppler characteristics of papillary projections in benign compared with borderline and malignant epithelial stromal ovarian tumors. MATERIALS AND METHODS: A total of 283 women (mean age, 52 years; age range, 20-85 years) with 343 operated adnexal masses comprising 167 epithelial stromal tumors of the ovary with 76 tumors containing papillary projections at pathology were retrospectively studied on ultrasound. We systematically evaluated the topography of the papillary projections, the morphologic features of the largest papillary projection, and the presence or absence of color Doppler findings. All these findings were correlated with macroscopic and microscopic features. RESULTS: Ultrasound detected papillary projections in 78% of tumors. Papillary projections were disseminated in 33% of malignant, 20% of borderline, and 0% of benign tumors (p = 0.0049). The mean size of the papillary projections was 9.6, 15.7, and 35.3 mm in benign, borderline, and malignant tumors, respectively (p = 0.0007). An acute angle was present in 68% of benign tumors and an obtuse angle in 40% of borderline and 89% of malignant tumors (p = 0.0001). The surface was regular in 77% of benign tumors and irregular in 50% of borderline and 88% of malignant tumors (p = 0.0000). Calcifications were present only in benign tumors (18%). For papillary projections ≥ 10 mm, color flow was present in all malignant, in 86% of borderline, and absent in all benign tumors. CONCLUSION: Association of morphologic and vascular ultrasound findings can highly suggest the diagnosis of benign or malignant papillary projection.

Gastrointestinal stromal tumor arising in the rectovaginal septum.

We report herein a rare case of malignant gastrointestinal stromal tumor (GIST) originated from the rectal wall, which presented as a tumor on the rectovaginal septum. A 54-year-old Japanese woman, gravida 4, para 3, was admitted complaining of anuresis and severe constipation. She had a history of hysterectomy and right salpingo-oophorectomy for uterine leiomyoma 11 years previously. Pelvic examination revealed an 8.5 x 7.5 x 7.5 cm hard mass in the rectovaginal space. The inferior border of the tumor was 2 cm from the vaginal introitus and 2 cm from the anus. Computed tomography and magnetic resonance imaging showed a well-circumscribed soft-tissue mass filling the rectovaginal space. Urinary bladder and rectum were markedly compressed and displaced. Colon fiberscopy revealed invasion of the tumor into the rectal mucosa. An abdominoperineal resection of the rectum with posterior vaginal wall resection and pelvic lymphadenectomy was performed. The resected specimen showed a rectal submucosal tumor that was 8 x 8 x 7 cm in size. The tumor was diagnosed as a malignant GIST. Immunohistochemical analysis confirmed this diagnosis. The patient is now healthy without evidence of recurrence at 13 months after surgery. Gynecologists should be aware of rectal GIST arising in the rectovaginal space as a differential diagnosis of vaginal submucosal tumor.