Case Of Endometrial Stromal Sarcoma Research Articles

Metastatic endometrial stromal sarcoma of the lung: importance of immunohistochemical staining, clinical history and imaging studies

Proper evaluation of lung nodules is a difficult issue for clinical management of patients. Discriminating metastatic endometrial stromal sarcoma (ESS) from other primary spindle cell neoplasms of the lung using histological analysis can be challenging. This is particularly true when an adequate clinical history is lacking, because ESS metastasis can be delayed by a couple of decades. To emphasize the importance of the correlation of pathological findings with clinical history and imaging studies, we investigated 11 cases of ESS (seven low grade and four high grade) metastatic to the lung. All cases presented with one to multiple unilateral or bilateral lung nodules that were detected by chest computed tomography. Primary ESS was diagnosed from hysterectomy specimens except for one by endometrial biopsy, 0.5 to 23 years prior to metastasis. Immunohistochemical studies showed that all ESS cases were moderately to strongly positive for Bcl-2 and CD10 with > 50% of tumor cells stained, except for one high grade ESS that was negative for CD10. Eight (72.7%) and seven (63.6) of the 11 cases showed positive estrogen and progesterone receptors, respectively, with a majority of positive cases showing diffuse and moderate to strong staining. Strong but patchy staining for CD34 was detected in one (9.1%) case with smooth muscle differentiation. CK7 and TTF-1 were negative in all cases. Two (18.2%) cases exhibited patchy and strong positivity for caldesmon. Two (18.2%) low grade ESS cases showed moderate to strong AE1/AE3 positivity in > 50% of tumor cells, one of which also showed moderate CK19 and Cam 5.2 staining in >30% of tumor cells. One should be cautious when assessing spindle cell neoplasms of the lung in women with a history of hysterectomy. Correlation of clinical history and imaging studies with histological and immunohistochemical findings is essential to diagnosis of metastatic ESS to the lung.

Clinicopathologic features and prognostic factors of endometrial stromal sarcoma: a study of 55 cases

To investigate the clinicopathologic features and the prognostic factors of endometrial stromal sarcoma (ESS). 55 cases of endometrial stromal sarcoma were reviewed and categorized into 3 pathologic types based on the related literatures, i.e., low grade endometrial stromal sarcoma (LGESS), undifferentiated endometrial sarcoma with nuclear uniformity (UES-U) and undifferentiated endometrial sarcoma with nuclear pleomorphism (UES-P). Meanwhile, the pathologic features were reviewed, including fibroid, myoid, mucoid, and epithelioid differentiation and mitotic index. Clinical and follow-up data were collected. In endometrial stromal sarcoma, two or three pathologic types co-existed in one case, including 12.8% (5/39) of LGESS, 5/9 of UES-U, and 5/7 of UES-P. Mitotic index varied in different regions of one tumor from rare to high. Multi-differentiation was also commonly seen in ESS. The numbers of cases in LGESS, UES-U and UES-P were 39, 9 and 7, with recurrence rate of 51.6% (16/31), 5/6 and 2/3, respectively. There was no death case in LGESS, and 2 cases were died in UES-U and UES-P, respectively. In the 2 death cases of UES-U, both had focus of UES-P. There was a significant difference in the recurrence rate between cases with different mitotic index (≥ 10/10 HPF and < 10/10 HPF, P = 0.009), especially in LGESS group. All death cases had high mitotic index (> 30/10 HPF). It is a common phenomenon in ESS that two or three pathologic types may exist in one case, especially in UES-U and UES-P. And multi-differentiation is also commonly seen in ESS. So adequate pathologic sampling is important for pathologists to make a correct diagnosis of ESS in daily work. The recurrence rates are significantly higher in cases with high mitotic index, especially in LGESS. In addition, the presence of UES-P and high mitotic index may increase the risk of death in the patients.

Endometrial stromal sarcoma: clinicopathological and immunophenotype study of 18 cases

Malignant tumors of the uterine corpus are uncommon. They originate from the endometrial stroma, smooth muscle, blood vessels, or from a mixture of them. The objective of this article was to know the frequency and the clinical, morphologic, and immunophenotype characteristics of the endometrial stromal sarcoma (ESS). We reviewed the cases of ESS observed from 2002 to 2008 at the Pathology Unit of the General Hospital of Mexico. The following data were analyzed: age, clinical stage, degree of differentiation, and immunophenotype. We found 18 cases, and the average age of patients was 48.6 years; 66% were in clinical stages 1 and 2. Fifteen cases (83.3%) were classified as low-grade sarcomas and 3 (16.6%) as high-grade or undifferentiated sarcomas. We determined immunohistochemical markers in 17 cases; receptors to estrogens were positive in 5 (29.4%) and to progesterone in 9 (52.9%). CD10 was expressed in 10 (58.8%) and p53 in 11 cases (64.7%). Two cases were associated to primary tumors of the ovary (papillary cystadenocarcinoma). In conclusion, ESS was present at 0.6% in our institution; and most were low grade. Expression of markers, such as p53, CD10, and hormonal receptors, was positive.

Incidence of lymph node and adnexal metastasis in endometrial stromal sarcoma

ObjectiveTo determine the incidence of adnexal and lymph node (LN) metastasis in newly diagnosed endometrial stromal sarcoma (ESS). MethodsWe identified all cases with a diagnosis of ESS evaluated at our institution from January 1, 1980 to October 31, 2009. All uterine pathology was reviewed at our center. High-grade or undifferentiated tumors and ESS arising in extrauterine sites were excluded. Pertinent clinical data were abstracted from electronic medical records. Appropriate statistical tests were performed using SPSS16.0. ResultsWe identified 94 cases of ESS. LN metastasis was identified in 7 (19%) of 36 patients who underwent LN evaluation. Six of the 7 cases with LN metastasis had lymphovascular invasion (LVI). LVI status was not reported in the other case. Five of the 7 patients with LN metastasis had grossly positive LNs with or without other gross extrauterine disease. Of 20 patients with disease grossly limited to the uterus and grossly normal LNs, 2 (10%) had LN metastasis. Both of these cases had LVI and extensive myoinvasion. Eighty-seven cases (93%) underwent salpingo-oophorectomy. Adnexal metastasis was identified in 11 (13%) of 87 cases, all manifested by gross adnexal tumor and occurring in patients with other gross pelvic extrauterine disease. ConclusionThe incidence of LN metastasis in ESS is commonly associated with gross extrauterine disease, extensive myoinvasion, and LVI. Since myoinvasion and LVI status often are not assessable at the time of hysterectomy, LN dissection remains a reasonable option at primary surgery. The rate of adnexal metastasis appears to be negligible in the absence of gross adnexal and extrauterine tumor.

Transition From Low-grade Endometrial Stromal Sarcoma to High-grade Endometrial Stromal Sarcoma

We report on a case of a primary low-grade endometrial stromal sarcoma (ESS) that progressed to a secondary high-grade ESS. In the secondary tumor, the immunohistochemical profile and focal tumor cell proliferation pattern suggested that this tumor was not truly undifferentiated, but possessed features of endometrial stroma. Low-grade ESS of our patient's primary tumor showed p53 protein overexpression, which is unusual in low-grade ESS, and her secondary high-grade ESS showed more prominent p53 immunoreactivity. This indicates that low-grade ESS that shows p53 immunoreactivity might progress to high-grade ESS, and it is considered that such cases of low-grade ESS should pay attention to the prognosis. Immunoreactivity for epidermal growth factor receptor was observed in both tumors, suggesting a relationship between the primary and secondary tumors in our case. Further study requires more immunohistochemical data for cases in which low-grade ESS transitions to high-grade ESS; in particular, data on epidermal growth factor receptor expression are necessary to define new therapeutic strategies for ESS.

Endometrial stromal sarcoma located in the myometrium with a low‐intensity rim on T2‐weighted images: Report of three cases and literature review

Endometrial stromal sarcoma (ESS) most commonly grows from the uterine endometrium into the endometrial cavity; it is rarely located in the myometrium alone, where it may resemble degenerated leiomyoma on magnetic resonance imaging (MRI). We present three cases of intramyometrial ESS mimicking degenerated leiomyoma, all of which have a characteristic low-intensity rim on T2-weighted images. Histopathological examination revealed the rim to consist of fibrous tissue layers and/or a decrease in free water caused by distortion of myometrial tissue following tumor expansion. ESS should be included in the differential diagnosis of intramyometrial mass with low-intensity rim on T2-weighted image, especially if the mass shows degeneration with no or mild intratumoral hemorrhage.

Primary extrauterine endometrial stromal sarcoma: response to hormone therapy

Endometrial stromal sarcomas (ESS) of the uterus are hormone-sensitive tumors. There have been reports in the literature confirming the regression of ESS with progestins, gonadotropin analogues, and aromatase inhibitors. We report a case of primary extrauterine ESS of the rectovaginal septum (RVS), which was poorly responsive and, in fact, progressed on progestin therapy. The question arises: is the evident lack of response to oral progestin in our case an exception or a trend more commonly seen in primary extrauterine, extraovarian ESS? To the best of our knowledge, there has been no report in the literature to address this question. Therefore, we conducted a review of the literature to evaluate the response of these tumors to hormone therapy in relation to their estrogen and progesterone receptor status.

A case of multiple metastatic low-grade endometrial stromal sarcoma arising from an ovarian endometriotic lesion

The development of endometrial stromal sarcomas (ESSs) in foci of endometriosis is extremely rare, and few cases have been reported in the literature to date, particularly with regard to multiple extrauterine ESS. Here we report a case of endometrial stromal sarcoma with multiple metastasis that arose from an ovarian endometriotic lesion. The literature is also briefly reviewed.

Endometrial Stromal Sarcoma Presenting as Prevesical Mass Mimicking Urachal Tumor

Endometrial stromal sarcoma (ESS) is a mesenchymal neoplasm that usually occurs as a primary tumor of the uterine corpus, but rarely arises in other sites, such as the ovary, pelvic cavity, mesentery, omentum and intestine. Herein, we present a rare case of low-grade ESS presented as prevesical mass. A 60-yr-old woman who had undergone total hysterectomy for endometriosis eleven years ago was presented with incidentally detected prevesical pelvic mass. Since malignant transformation of urachal remnants was possible, the mass was suspected to be a urachal tumor. Extraction of the mass was performed, and the histopathologic diagnosis was low-grade ESS. In summary, prevesical tumor is rare but in patients with endometriosis, we suggest endometriosis and its possible malignant changes should be taken into account in the differential diagnosis of prevesical mass.

Endometrial Stromal Sarcoma of the Uterus with Arterial Tumor Embolus

Endometrial stromal sarcoma (ESS) of the uterus is extremely rare. We report a case of ESS of the uterus with tumor embolus in an artery in a 36 year-old woman. The patient is a Caucasian woman who presented with vaginal bleeding. Surgical biopsy revealed low grade ESS (LGESS). Subsequently, total abdominal hysterectomy and right salpingo-oophorectomy was performed. A 3.8 cm polypoid mass was identified in the endometrial cavity, invading into the outer half of the anterior uterine wall. About 5-10% of the tumor exhibited undifferentiated sarcoma. Based on the aggressive nature of the tumor, the patient underwent left salpingo-oophorectomy and was subsequently treated with chemotherapy. She was followed up with periodic CT scans and was found to have recurrent ESS in the retroperitoneum 20 months after total hysterectomy and bilateral salpingo-oophorectomy. Rebulking surgery was not performed as the tumor showed diffuse abdominal and retroperitoneal involvement. Although LGESS may behave as an indolent tumor, it can be associated with an aggressive process and a high recurrence rate. A poor prognosis may occur when LGESS is admixed with undifferentiated components especially with evidence of arterial tumor embolus, which is herein reported.

Endometrial stromal sarcoma mimicking a myoma

Uterine malignancies are not uncommonly misdiagnosed for the more ubiquitous leiomyoma. A case of endometrial stromal sarcoma with ultrasound and color Doppler imaging is described.

Immunohistochemical profiling of cytokeratin expression by endometrial stroma sarcoma

Endometrial stromal sarcomas can be confused with several neoplasms because of their inconsistent and widely varied morphologic appearance and frequent immunohistochemical expression of a variety of antigens including cytokeratin. The resulting diagnostic challenge becomes problematic particularly in the diagnosis of metastases resulting from such tumors. Because of the sometime epithelioid appearance of the tumor cells and their expression of cytokeratin, the metastases may be misdiagnosed as poorly differentiated carcinoma. We therefore studied the profile of cytokeratin proteins expression in 17 cases of endometrial stromal sarcomas using a panel of antibodies including cytokeratin cocktail antibody (AE1/AE 3), CK5/6, CK7, CK14, CK16, Cam5.2 (CK8), CK19, CK20, and 34Ebeta12 (CK1, 5, 10, and 14). Of the 17 cases, 8 (47%) stained positive with the cytokeratin cocktail antibody (AE1/AE 3). Of the 8 cases with cytokeratin expression, 5 (63%) stained positive with CK19, and 3 of them stained positive with Cam5.2. The 3 cases that stained positive with Cam5.2 also expressed CK19. Of the 5 cases with CK19, 1 was focally positive for CK5/6, CK7, and 34Ebeta12. None of the cases expressed CK14, CK16, or CK20. These results show that CK19 is most commonly expressed cytokeratin in endometrial stromal tumors. Hence, the inclusion of CK19 in the panel of immunostains may help resolve the diagnostic confusion created by keratin expression in endometrial stromal sarcoma and may also help in the correct diagnosis of endometrial stromal sarcoma at extrauterine sites.

Endometrial stromal sarcoma of the small bowel: a case report and review of literature

Endometrial stromal sarcoma (ESS) is a rare mesenchymal neoplasm of the uterus, which is predominantly composed of endometrial stromal cells. When this feature is encountered in the extragenital area, the diagnosis is sometimes difficult especially if endometriosis is not present. We report a case of ESS arising in the small bowel without associated endometriosis in a 75-year-old woman and review the literatures for 16 cases of extrauterine extraovarian ESS. The most common site of the extrauterine extraovarian ESS is the gastrointestinal tract (8/16 cases). It is intimately associated with endometriosis (12/16 cases) as the case of ovarian ESS. Most ESSs were immunoreactive for CD10 (5/5 cases), progesterone receptor (10/10 cases), and estrogen receptor (9/11 cases), and negative for CD34 (0/7 cases). It may have a higher tendency for dissemination beyond its site of origin (12/16 cases) than its uterine counterpart. In conclusion, a careful morphological examination combined with immunohistochemical studies and consideration of ESS in the differential diagnosis would help in obtaining an accurate diagnosis in these rare circumstances.

A case of primary retroperitoneal undifferentiated endometrial stromal sarcoma after concurrent chemoradiation therapy for cervical cancer

Endometrial stromal sarcoma (ESS) is a relatively rare uterine sarcoma, especially extrauterine ESS. Furthermore, retroperitoneal ESS are extremely rare. Up to now, there are only four cases of primary retroperitoneal ESS reported in the literature. We report one case of primary retroperitoneal undifferentiated endometrial stromal sarcoma after concurrent chemoradiation therapy for cervical cancer with a brief review of the literature.

High-grade endometrial stromal sarcoma arising from colon endometriosis

Dear Editor, Malignant transformation is a rare but well-documented complication of endometriosis, occurring only 0.7 to 1% of the cases. Among these cases, nearly 76% arose in the ovary, and colorectal involvement was only 5%. Most of these malignant neoplasms are endometrial adenocarcinoma. Yantiss et al. reported only one endometrial stromal sarcoma (ESS) identified among 17 malignant neoplasms arising in gastrointestinal endometriosis. Experience of treatment with this tumor is limited due to their rare occurrence. We present a case of high-grade ESS of the sigmoid colon arising in extrauterine endometriosis managed by complete surgical excision and adjuvant radiotherapy. A 42-year-old woman presented with a 1-month history of difficult defecation and rectal bleeding. The bleeding was bright red in color, occurred at irregular intervals, and was associated with bowel movements. She had a 5-year history of non-small-cell lung cancer, undergoing chemotherapy at an outpatient department. She was multipara and had no history of endometriosis or taking hormonal drugs. The physical examination did not show an abdominal tenderness or a palpable mass. The digital rectal examination did not reveal any mass or blood. The results of the laboratory tests were unremarkable. Barium enema showed an apple-core-filling defect with mucosal destruction at the sigmoid colon. The computed tomography (CT) of the abdomen revealed a segmental wall thickening of the rectosigmoid junction with adjacent mesocolon soft tissue nodules, which was considered as an adenocarcinoma of the sigmoid colon with regional lymph nodes metastasis. The subsequent colonoscopy found a polypoid mass in the sigmoid colon with a narrowing of the bowel lumen. The biopsy results showed endometriosis characterized by pictures of the stromal cell’s hyperplasia with uniform, round to spindle nuclei, and focal lymphocytic infiltration, and positive immunohistochemical stains for CD10, estrogen receptor, and progesterone receptor. The surgical intervention arranged was due to the symptoms of stenosis of the rectosigmoid colon. At laparotomy, multiple red and brown cystic-like masses over the omentum, sigmoid colon, and left ovary were found. The frozen sections showed ESS arising from endometriosis. The surgical procedures included rectosigmoidectomy, total hysterectomy, bilateral salpingo-oophorectomy, and partial omentectomy. Grossly, the resected sigmoid colon had multiple 1to 3-cm nodular masses involving areas from the mucosa to the pericolic fatty tissue. Histologically, the sections of the rectosigmoid colon showed high-grade ESS characterized by multiple foci of tumor cell nests with crowded hyperchromatic nuclei extending from the colon mucosa to the pericolic fatty tissue. The tumor showed frequent mitosis, accounting more than ten mitotic figures per 10 high power fields (MF/10 HPF), and increased the proliferating index to approximately 12%. There were multiple foci of tumor necrosis and lymphovascular identification. The tumor cells were immunohistochemically positive for vimentin, MIB-1 (Ki-67), estrogen receptor (ER), progesterone receptor (PR), and CD10, but negative for cytokeratin, smooth muscle actin, and Int J Colorectal Dis (2007) 22:1551–1553 DOI 10.1007/s00384-006-0267-6

Cytomorphologic Features of Low-Grade Endometrial Stromal Sarcoma

Endometrial stromal sarcomas are uncommon tumors of the uterus, and the cytologic features have only been reported in a number of case reports that mostly discuss the features of higher grade undifferentiated sarcomas. This article discusses the cytologic features of a large series of low-grade endometrial stromal sarcomas (ESSs) sampled by a variety of methods. We reviewed our histologic files for all confirmed cases of low-grade ESS, and followed this by a computerized search for any corresponding cytologic specimens. We identified 12 Papanicolaou tests and 9 nongynecologic specimens. Most cases showed moderate to marked cellularity with a combination of single cells and stromal fragments. Blood vessels, interspersed between the clusters of stromal cells, were seen in 15 cases; 4 of these had supporting hyaline matrix. The cells were predominantly spindle shaped with scant to moderate cytoplasm, round to ovoid nuclei, and fine chromatin. Mitotic figures and nucleoli were rare. Most cases had a clean background.

Metastatic low-grade endometrial stromal sarcoma of clitoris: report of a case

Low-grade endometrial stromal sarcoma (ESS) is an uncommon neoplasm, which has a highly recurrent nature. A review of the literature revealed that only one case of low-grade ESS, arising within the vulva from a focus of endometriosis, has been previously published. We describe an additional case of low-grade ESS arising within the vulva and to the best of our knowledge the first report of low-grade ESS metastasized to clitoris. A 46-year-old woman was admitted to our hospital due to a heavy uterine bleeding. A physical examination revealed a lesion in clitoris, which exhibited a densely cellular mesenchymal neoplasm on microscopy. On the basis of the pathologic features alone, a differential diagnosis of a low-grade ESS and cellular leiomyoma was considered. Seven months later, the patient presented again with excessive uterine bleeding and a total hysterectomy was performed. A tumor of white-tan, whorled appearance was found. Its features were suggestive of low-grade ESS. Taking into account the possible extrauterine location of an ESS and reviewing the first case, a diagnosis of rare low-grade ESS metastasized to clitoris was made.

Clinicoprognostical features of endometrial cancer patients with somatic mtDNA mutations

Somatic mitochondrial DNA (mtDNA) mutations have been found in a subset of endometrial cancers (EC) from different populations. We have investigated the relationship between mtDNA changes and clinical and pathological variables of women affected by EC. mtDNA mutations were detected both in early (3/32; 9%) and in advanced (1/8; 12%) stages of uterine tumors. However, patients carrying the mtDNA mutations or the normal mtDNA sequence had indistinguishable clinicopathological data, including age, clinical stage, histological grade and type or depth of myometrial invasion. It is noteworthy that mtDNA mutations were not detected in hyperplastic endometrial tissues or in ECs coexisting with hyperplasia, nor in a single case of endometrial stromal sarcoma. LOH at the tumor suppressor genes RB1 and TP53 as well as p16INK4A alterations (LOH, gene deletion) were found in tumors carrying mtDNA mutations. These results suggest that somatic mtDNA mutations are detected in a subset of ECs, although they are unrelated to clinicopathological variables of cancer.

Uterine tumor resembling ovarian sex cord tumors in a patient using tamoxifen: report of a case and review of literature

Increasing number of uterine malignancies have been reported in breast cancer patients using tamoxifen. Most of these are endometrial adenocarcinomas. However, only a few cases of endometrial stromal sarcomas have been reported to be linked with tamoxifen usage. A 58-year-old postmenopausal women who had been using tamoxifen for 4 years after a surgery for breast cancer is presented with chronic pelvic pain. Preoperative investigations were indicative of a uterine myoma so that a standard total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Postoperative histologic diagnosis was a uterine tumor resembling ovarian sex cord tumors, which is an exceedingly rare entity itself. The present case is the first designated diagnosis of this rare tumor, with a possible association of tamoxifen usage.

Uterine tumor resembling ovarian sex cord tumors in a patient using tamoxifen: report of a case and review of literature

Increasing number of uterine malignancies have been reported in breast cancer patients using tamoxifen. Most of these are endometrial adenocarcinomas. However, only a few cases of endometrial stromal sarcomas have been reported to be linked with tamoxifen usage. A 58-year-old postmenopausal women who had been using tamoxifen for 4 years after a surgery for breast cancer is presented with chronic pelvic pain. Preoperative investigations were indicative of a uterine myoma so that a standard total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Postoperative histologic diagnosis was a uterine tumor resembling ovarian sex cord tumors, which is an exceedingly rare entity itself. The present case is the first designated diagnosis of this rare tumor, with a possible association of tamoxifen usage.