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PubMed2004

Inverse correlation of secreted frizzled-related protein 4 and beta-catenin expression in endometrial stromal sarcomas.

Endometrial stromal sarcomas are rare uterine tumours. Whereas the histology and immunohistochemistry of these tumours are well documented, almost nothing is known about the molecular mechanisms involved in their pathogenesis. To characterize the genes altered in these malignancies, a genome-wide cDNA library was generated by suppression subtractive hybridization and a set of differentially expressed clones was isolated. These were then used to produce custom-spotted cDNA arrays. Genes deregulated in endometrial stromal sarcomas were identified by cDNA array hybridization and were confirmed by quantitative real-time PCR analyses and in situ hybridization. Following cDNA array analysis, more than 300 genes deregulated in endometrial stromal sarcoma were selected and sequenced. Among the most significantly deregulated genes were those of secreted frizzled-related proteins (SFRPs), in particular secreted frizzled-related protein 4 (SFRP4). SFRPs are putative modulators of the Wnt-signalling pathway and play a role in different cellular events including cell proliferation. Compared with normal endometrium, the expression of SFRP4 was decreased in both low-grade endometrial stromal sarcoma (ESS; n = 10) and undifferentiated endometrial sarcoma (UES; n = 4), being lower in the latter more aggressive form. These results were verified on paraffin wax-embedded tissue by quantitative real-time PCR analysis and in situ hybridization. Furthermore, the expression of beta-catenin, an important component of the Wnt-signalling pathway, was regulated in an opposite manner to SFRP4, being particularly increased in undifferentiated sarcomas. The activation of the Wnt-signalling pathway was additionally supported by the immunohistochemical demonstration that beta-catenin was translocated to the nucleus in UES. SFRP4 may therefore be a putative tumour suppressor involved in deregulation of the Wnt pathway and in the pathogenesis of ESS and UES.

Cytoskeletal Proteins↗

PubMed2004

Multicystic endometrial stromal sarcoma.

Endometrial stromal sarcoma usually has the gross appearance of a single nodule, multiple masses, or a poorly demarcated lesion with occasional cystic degeneration; however, a multilocular form has not been described in the literature. We report the case of a 25-year-old woman with a cystic multilocular lesion with thin septae measuring 8 cm, discovered by a pelvic ultrasonography. Grossly, it was a multicystic mass located in uterine fundus that was attached to myometrium and showed infiltrating borders. We propose that cystic endometrial stromal sarcoma should be included in the differential diagnosis of cystic uterine tumors.

Adult↗

PubMed2006

Consistent rearrangement of chromosomal band 6p21 with generation of fusion genes JAZF1/PHF1 and EPC1/PHF1 in endometrial stromal sarcoma.

Endometrial stromal sarcomas (ESS) represent <10% of all uterine sarcomas. Cytogenetic data on this tumor type are limited to 32 cases, and the karyotypes are often complex, but the pattern of rearrangement is nevertheless clearly nonrandom with particularly frequent involvement of chromosome arms 6p and 7p. Recently, a specific translocation t(7;17)(p15;q21) leading to the fusion of two zinc finger genes, juxtaposed with another zinc finger (JAZF1) and joined to JAZF1 (JJAZ1), was described in a subset of ESS. We present three ESS whose karyotypes were without the disease-specific t(7;17) but instead showed rearrangement of chromosomal band 6p21, twice as an unbalanced t(6p;7p) and once as a three-way 6;10;10 translocation. All three tumors showed specific rearrangement of the PHD finger protein 1 (PHF1) gene, located in chromosomal band 6p21. In the two tumors with t(6;7), PHF1 was recombined with the JAZF1 gene from 7p15, leading to the formation of a JAZF1/PHF1 fusion gene. The third tumor showed a t(6p;10q;10p) as the sole karyotypic abnormality, leading to the fusion of PHF1 with another partner, the enhancer of polycomb (EPC1) gene from 10p11; EPC1 has hitherto not been associated with neoplasia. The PHF1 gene encodes a protein with two zinc finger motifs whose involvement in tumorigenesis and/or tumor progression has not been reported before, but its rearrangement clearly defines a new pathogenetic subgroup of ESS.

Adult↗

PubMed2003

Long-term survival of patients given hormonal therapy for metastatic endometrial stromal sarcoma.

Endometrial stromal sarcoma (ESS) is a rare neoplasm, mainly observed in premenopausal women. We describe two women 44 and 34 years old at the time ESS diagnosis, who developed lung metastases 3 and 6 years, respectively, after initial treatment: hysterectomy without (case 1) or with oophorectomy (case 2), followed by hormone replacement therapy (HRT) for the latter. Their estrogen (ER) and progesterone receptors (PR) were analyzed biochemically in metastatic lung tissue, yielding respective concentrations of ER 242 and 184, and PR 910 and 100 fmol/mg of cytosol protein. Both patients started treatment with the aromatase inhibitor aminoglutethimide (500 mg qid) after surgery for the first patient and after stopping HRT for the second. Under aromatase-inhibitor therapy, both patients achieved a complete response, patient 1 remains disease- free with 14+ years of follow-up, and patient 2 with 7+ years. Our data suggest that an aromatase inhibitor may be an effective treatment for ESS. Furthermore, routine ER and PR analyses could be useful to predict the response to hormonal therapy in ESS.

Adult↗

PubMedDate not supplied

Utility of MIB-1 and estrogen and progesterone receptor in distinguishing between endometrial stromal sarcomas and endometrial stromal nodules, highly cellular leiomyomas.

It is difficult to differentiate between an endometrial stromal nodule (ESN) and endometrial stromal sarcoma (ESS) in curettage specimen, and the recommended therapy of endometrial stromal neoplasm is hysterectomy. If we could discriminate ESS from ESN in curettage specimens, there would be an opportunity to treat ESN by local excision rather than by hysterectomy. We analyzed MIB-1 and estrogen and progesterone receptor (ER/PR) expression in a retrospective series of 8 ESSs, 7 ESNs, and 17 highly cellular leiomyomas obtained from hysterectomy specimens. ESSs expressed MIB-1 more frequently than ESNs (P < 0.05), and ESSs had a tendency to express ER less frequently than ESNs (P= 0.08). We observed that in spite of showing MIB-1 expression to some extent, highly cellular leiomyomas usually could not reach ESSs' level and frequency of MIB-1 expression in the current study. Although MIB-1 and ER appear to be promising markers in the differential diagnosis of ESSs, a larger study would be necessary to confirm their validity.

Antibodies, Antinuclear↗

PubMed2001

MR imaging findings of uterine endometrial stromal sarcoma: differentiation from endometrial carcinoma.

Endometrial stromal sarcoma (ESS) is a rare malignant neoplasm of the uterus. We retrospectively analyzed pelvic MR imaging in 8 cases of ESS to determine the characteristic MR findings of ESS. Magnetic resonance images of 8 cases of ESS were evaluated for findings including the size, margin of the tumor, nodular lesions at the tumor margin, intramyometrial worm-like nodular extension, multiple nodular mass formation, hemorrhage, and necrosis in the tumor. The degree of contrast enhancement was also analyzed. The MR imaging findings of 21 consecutive cases of endometrial carcinoma (EC) were also evaluated and compared with those of ESS. The mean and the standard deviation of the maximum diameter of ESS and EC were 8.80 +/- 4.99 and 3.93 +/- 2.47 cm, respectively. Increased enhancement in at least a part of the tumor was observed in five of the six analyzed ESS cases and in 2 of 12 analyzed EC cases. The irregular margin, nodular lesions at the margin, intramyometrial nodular extension, and multiple nodular mass formation were more frequently seen in cases of ESS than in cases of EC. Magnetic resonance imaging can play a role in both diagnosing ESS as well as in differentiating ESS from EC.

Aged↗

PubMed1975

Ultrastructure of endometrial stromal sarcoma.

An endometrial stromal sarcoma from a 48-year-old woman was studied by light and electron microscopy. Ultrastructurally most of the tumor cells resembled the stromal cells of midproliferative endometrium. There was no evidence of a pericytic differentiation of the tumor cells. These findings lend further support to the concept of an endometrial histogenesis of stromal sarcoma.

Cell Nucleus↗

PubMed1989

Ultrasound findings in uterine mixed müllerian sarcomas and endometrial stromal sarcomas.

Sonographic findings in five cases of mixed mullerian tumors and two cases of endometrial stromal sarcoma which were evaluated before surgery for staging are reviewed. Assessment of myometrial invasion was in agreement with surgical findings in five cases; in the two other cases invasion was overestimated. The echo pattern of these tumors presented some peculiarities. In five patients, four with mixed mullerian tumors and one with endometrial stromal sarcoma, a heterogenous pattern with high-intensity and hypoechoic areas scattered in the myometrium was seen. In the other two cases the sonographic picture was indistinguishable from that of leiomyomas. Sonography is an accurate imaging technique for preoperative staging of uterine sarcomas. If an heterogenous echo pattern of the uterus as described is found at sonography, sarcoma should be suspected and laparotomy performed urgently.

Adult↗

PubMed1984

[Detection of endometrial stromal sarcoma using endometrial cytology. Apropos of a case].

The authors report a case of endometrial stromal sarcoma which was detected by endometrial cytology. The tumour originates either in the stroma of the endometrium or in foci of myometrial adenomyosis. It occurs most frequently in patients who are more than 60 years of age. It appears as an intra-uterine polypoid mass and causes bleeding and pelvic pain. Its incidence is difficult to establish. Endometrial cytology is highly suggestive of stromal sarcoma when smears harbour numerous atypical sarcomatous cells mixed with regular endometrial cells. Biopsies of the endometrium show decidua-like arrangements of malignant cells. Stromal sarcoma constitutes the homologous form of mesenchymal tumours of the uterus. The prognosis depends on whether local recurrences occur and on the sites of metastases in which the sarcomatous component seems to be latent with lesions that resemble adenocarcinomata . The five years salvage rate is approximately 26 to 28%. Treatment is surgical with or without irradiation.

Aged↗

PubMed2005

Chromosomal alterations in low-grade endometrial stromal sarcoma and undifferentiated endometrial sarcoma as detected by comparative genomic hybridization.

OBJECTIVE: Endometrial stromal sarcomas (ESS) are very rare neoplasms constituting less than 0.5% of all malignant uterine tumors. The aim of the present study was to characterize the karyotypic abnormalities in these malignant mesenchymal tumors and to find specific chromosomal aberrations with eventual correlation with histologic grades. METHODS: Twelve cases of endometrial stromal sarcomas consisting of nine low-grade ESS and three undifferentiated endometrial sarcomas (UES) were investigated by comparative genomic hybridization (CGH). RESULTS: Ten of the twelve cases (83.3%) displayed chromosomal gains or losses. Deletions occurred more frequently than gains (63.4% versus 36.6%). In low-grade ESS, gains on 1, 6q, 9q, 16p, 19, 20q, 22q and losses on 2, 4q, 6, 7, 11q, 13q, 15q, 16q, 20p, X were detected. CGH with UES exhibited gains on 2q, 4q, 6q, 7p, 9q, 20q and losses on 3q, 10p, 14q. One low-grade ESS and one UES did not reveal any chromosomal aberration. CONCLUSIONS: Chromosomal aberrations in endometrial sarcomas are heterogeneous and do not clearly correlate with the histologic grades. There is no increased accumulation of aberrations from low-grade ESS to UES. Despite the karyotypic variations, chromosomal deletion on 7p was the most common finding (55.6%) in low-grade ESS and may play a role in tumor development and progression.

Chromosome Aberrations↗

PubMed2006

Transition of endometrial stromal sarcoma into high-grade sarcoma.

BACKGROUND: Endometrial stromal sarcoma typically is of low grade and hormone-sensitive. Although these characteristics result in an indolent behavior, little data are available on the evolution over time. CASE: We report on two cases where microscopic and immunohistochemic assessment of the tumor on several occasions during 8 and 25 years of follow up enabled us to document a transition of a low-grade into a high-grade malignancy. These were mainly characterized by increased cellular atypia, absence of spiral arterioles and an increased mitotic index and proliferation index (MIB1). Since this transition was related to clinical loss of hormone sensitivity, the therapeutic approach consisting of hormonal treatment in combination with repetitive surgery was switched to chemotherapy only. CONCLUSION: These long-term follow up data provide insight in endometrial stromal sarcoma tumor biology and highlight the importance of sampling recurrent tumors to estimate biologic behavior in order to tailor subsequent treatment.

Adult↗

PubMed1982

Endometrial stromal sarcoma and poorly differentiated endometrial sarcoma.

U.T.M.D. Anderson Hospital cases filed as endometrial sarcoma were reviewed. Malignant mixed Müllerian tumors, Müllerian adenosarcomas, and poorly differentiated sarcomas of uncertain origin were excluded, and a minimum of 36 months follow-up was required. On pathologic examination, the remaining cases fell into two distinct groups: endometrial stromal sarcoma (11 cases) and poorly differentiated endometrial sarcoma (seven cases). The former were characterized histologically by small to medium-sized uniform cells and distinctive arterial vasculature resembling the spiral arteries to the normal endometrium; they were thus considered to exhibit endometrial stromal differentiation. The latter showed topographic configurations indicative of endometrial origin but did not demonstrate endometrial stromal differentiation; they were composed of generally larger cells with nuclear hyperchromatism and pleomorphism and had no distinctive vascular pattern. The mitotic rate was usually low in endometrial stromal sarcoma and high in poorly differentiated endometrial sarcoma, although two examples of the former had more than 20 mitoses per 10 high-power fields. All but one of the patients with endometrial stromal sarcoma were less than 50 years of age, whereas all of those with poorly differentiated endometrial sarcoma were older than 50 years. One patient with endometrial stromal sarcoma died of tumor, two were living with tumor at latest follow-up, and eight were clinically tumor-free. By contrast, six of those with poorly differentiated endometrial sarcoma died of tumor, all within 34 months. The extent of tumor was the major prognostic factor in both groups; the three patients with endometrial stromal sarcoma who were dead or living with tumor all had extrauterine extension at the time of hysterectomy (only one of the other eight did so) and all six who died with poorly differentiated endometrial sarcoma had myometrial invasion (the one survivor had a tumor limited to the endometrium). Other pathologic factors, including mitotic rate, had no prognostic significance in either category; of particular interest was that both patients who had endometrial stromal sarcomas with high mitotic rates were tumor-free. It is concluded that endometrial stromal sarcoma is a distinct tumor entity whose diagnosis should not depend on mitotic rate, and it is suggested that poorly differentiated endometrial sarcoma is closely related to malignant mixed Müllerian tumor, in view of clinical and pathologic similarities.

Adult↗

PubMed2006

A case of c-kit positive high-grade stromal endometrial sarcoma responding to Imatinib Mesylate.

BACKGROUND: High-grade endometrial stromal sarcoma (HGES) is an aggressive disease with dismal prognosis. Surgery is the standard treatment, and radiotherapy seems to reduce local relapse. No other treatment has proved to be useful in the case of non-resectable diseases. CASE: A 41-year-old woman suffering HGES, with positive staining for CD117 (c-kit) and large abdominal and retroperitoneal mass progressing after chemotherapy, was treated with Imatinib Mesylate. After 3 months, objective response was documented and radical surgery performed. Two years after surgical intervention, she is currently free of the disease. CONCLUSION: The response seen with Imatinib should be further investigated in a larger number of cases.

Adult↗

PubMed2000

Endometrial stromal sarcoma diagnosed after hysteroscopic endometrial resection.

Endometrial ablation and resection is now common therapy for dysfunctional uterine bleeding that is unresponsive to conservative management. Opponents argue that it may predispose patients to potentially hazardous malignancies of the uterus. In our patient, endometrial resection was performed to treat menorrhagia after a negative workup. Pathologic interpretation of the resected tissue showed a low-grade stromal sarcoma of the endometrium. The woman underwent definitive treatment, which included total abdominal hysterectomy, and has remained recurrence free over the past 3 years. Although rare, uterine malignancies have been documented after hysteroscopic management of menorrhagia. It is suggested that intraoperative endometrial resection or tissue sampling be done to prevent or diagnose endometrial hyperplasia and uterine malignancies.

Endometrial Neoplasms↗

PubMed1996

Endometrial stromal sarcoma of twenty cases.

BACKGROUND: Endometrial stromal sarcoma is a rare neoplasm. We reviewed twenty cases to study the characteristics of this disease. METHODS: Twenty cases of endometrial stromal sarcoma were treated at our hospital. The clinical stage, treatment and outcome were retrospectively analyzed. RESULTS: Endometrial stromal sarcoma comprised 4.3% of corpus cancers and 46.4% of uterine sarcomas at our hospital. Seven cases were stage I, one was stage II, ten were stage III, and two were stage IV at the time of diagnosis. Histopathologically, seventeen cases were classified as low-grade sarcoma and three were high-grade sarcoma. Seven patients had recurrence and five of them had already died of disease. Among these recurrent patients, one was stage II and six were stage III. All three patients with high-grade sarcoma and four with low-grade sarcoma had recurrence. CONCLUSIONS: We think mitotic count is an important prognostic factor in low-grade endometrial stromal sarcoma and high-grade endometrial stromal sarcoma has a poor prognosis even with post-operative adjuvant treatment.

Adjuvants, Pharmaceutic↗

PubMed1990

Post-hysterectomy extra-uterine endometrial stromal sarcoma: a case report.

Endometrial stromal sarcoma is a relatively rare form of uterine sarcoma. The present paper reports on a patient who was found to have contracted endometrial stromal sarcoma eight years after a hysterectomy. A postoperative specimen revealed this sarcoma to have originated in extra-uterine endometrium of the rectovaginal septum.

Adult↗

PubMed2000

Endometrial stromal sarcoma of the vagina.

Endometrial stromal sarcoma is a rare tumor and has unique histopathologic features. Most tumors of this kind occur in the uterus; thus, the vagina is an extremely rare site. A 34-year-old woman presented with endometrial stromal sarcoma arising in the vagina. No correlative endometriosis was found. Because of the uncommon location, this tumor was differentiated from other more common neoplasms of the vagina, particularly embryonal rhabdomyosarcoma and other smooth muscle tumors. Although the pathogenesis of endometrial stromal tumors remains controversial, the most common theory of its origin is heterotopic Müllerian tissue such as endometriosis tissue. Primitive cells of the pelvis and retroperitoneum are an alternative possible origin for the tumor if endometriosis is not present. According to the literature, the tumor has a fairly good prognosis compared with other vaginal sarcomas. Surgery combined with adjuvant radiotherapy appears to be an adequate treatment.

Adult↗

PubMed1999

Expression of metalloproteinases endometrial stromal sarcoma: immunohistochemical study using image analysis.

AIM: To investigate the expression of matrix metalloproteinases (MMP), a group of proteolytic enzymes with a central role in extracellular matrix invasion and degradation, in stromal sarcomas. METHODS: 11 endometrial stromal sarcomas (four low grade tumours, seven high grade) were stained for MMP-2, MMP-3, and MMP-9 using immunohistochemical stains. The surgical material consisted of nine hysterectomy specimens and two pelvic recurrences. Three hysterectomy specimens, removed for leiomyomas, were studied as controls. Staining area was evaluated using image analysis. RESULTS: Age at the time of diagnosis ranged from 21 to 67 years. Four of the 11 patients (three with high grade tumours and one with a low grade tumour) died of the disease, six remained free of disease, and one was lost to follow up. Staining for MMP-2, MMP-3, and MMP-9 was more diffuse in high grade tumours than in low grade tumours and controls. Staining for MMP-3 and MMP-9 was more pronounced in high grade than in low grade tumours (p = 0.04; p = 0.05). Staining for MMP-9 was significantly greater in all stromal sarcomas than in controls (p < 0.001 for high grade tumours v controls; p < 0.01 for low grade tumours v controls). Diffuse staining for MMP-2, exceeding 90% of the tumour area, was observed in three of seven high grade tumours but in no low grade tumours. There was no apparent correlation between staining for any of the three enzymes and survival. CONCLUSIONS: Both low and high grade endometrial stromal tumours express matrix metalloproteinases. MMP-3 and MMP-9 are expressed more diffusely in high grade than in low grade tumours. In the individual case, diffuse staining for MMP-2 appears to best characterise the high grade tumours. Thus staining for MMP-2 may aid in differentiating high grade from low grade tumours, and MMP-9 in differentiating normal endometrial stroma from low and high grade endometrial stromal sarcomas. MMP expression does not appear to predict disease outcome in endometrial stromal sarcoma.

Adult↗