Low-grade endometrial stromal sarcoma of the ectocervix after therapy for breast cancer

OBJECTIVE: Low-grade endometrial stromal sarcoma is an uncommon, indolent uterine sarcoma that can arise in extrauterine locations. The objective of this study was to report on a previously unpublished site of origin for a low-grade endometrial stromal sarcoma. METHODS: A case of a low-grade endometrial stromal sarcoma arising in the ectocervix after goserelin hormonal therapy for breast cancer was studied. RESUTLS: Low-grade endometrial stromal sarcoma can arise in the ectocervix even in the absence of endometriosis. CONCLUSION: Low-grade endometrial stromal sarcoma should be included in the differential diagnosis of sarcomas of the ectocervix.     

Endometrial stromal sarcoma of uterus

Endometrial stromal sarcomas are the second most common uterine sarcomas. Currently, they are classified into low-grade endometrial stromal sarcomas and undifferentiated endometrial sarcoma. Low-grade endometrial stromal sarcomas are biologically low-grade uterine sarcomas, and typically composed of uniform cells intimately associated with prominent arterioles, resembling the endometrial stroma in proliferative phase. There is usually little cytological atypia or pleomorphism, and mitoses are scanty. In contrast, undifferentiated endometrial sarcomas are frankly malignant, lack specific differentiation and any features of normal endometrial stroma. It is a highly aggressive neoplasm, often exhibiting myometrial invasion, haemorrhage and necrosis, as well as marked nuclear pleomorphism and high mitotic activity. The diagnosis of undifferentiated endometrial sarcoma is reached after excluding other uterine tumours with a sarcomatous component, such as adenosarcoma and malignant mixed Müllerian tumour. Histological variants of endometrial stromal sarcomas, including the so called 'high-grade endometrial stroma sarcomas' are addressed. The problems with histologic diagnosis and application of immunohistochemical studies and molecular pathology are highlighted.     

Lymph node metastases in low-grade endometrial stromal sarcoma

OBJECTIVES: The objective of this study was to review our experience on lymphatic dissemination in patients with low-grade endometrial stromal sarcoma. METHODS: All cases diagnosed as low-grade endometrial stromal sarcoma or endolymphatic stromal myosis before October 2003 and who had lymph node sampling at some point in their evolution were retrieved from the files of the pathology and gynecologic oncology departments of l'Hotel-Dieu de Quebec University Hospital (HDQ). RESULTS: Fifteen patients with either limited lymph node biopsies or a complete lymph node dissection at some point in the course of their disease were found. Five of these patients (33%) presented lymph node metastases either at the initial hysterectomy, during a subsequent staging procedure, or at the time of a recurrence. CONCLUSION: These findings suggest that the incidence of lymph node involvement in low-grade endometrial stromal sarcoma is higher than expected. More extensive sampling of lymph nodes in a larger number of patients may allow a better understanding of the frequency and prognostic significance of these metastases.     

Endometrial stromal sarcoma: objective response to letrozole

BACKGROUND: Low-grade endometrial stromal sarcoma is generally an indolent tumor rich in estrogen and progesterone receptors. Objective responses to hormonal therapy, most commonly with megestrol acetate, have been reported. CASE: The patient is a 51-year-old woman who presented with low-grade endometrial stromal sarcoma confined to the uterus in 1991 and was treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy. Approximately 5 years later, the patient had recurrent pelvic disease treated with radiation therapy, followed by an attempt at resection. She was treated with megestrol acetate during the period she received radiation therapy with poor tolerance. Tamoxifen was then given with no tumor response. Megestrol acetate was restarted with progression of disease in the pelvis and abdomen. Letrozole was then given at a daily dose of 2.5 mg with partial response for a duration of 9 months. CONCLUSION: Letrozole at a daily dose of 2.5 mg may be effective in low-grade endometrial stromal sarcoma with positive estrogen receptors.     

Estrogen receptor expression in an endometrial stromal sarcoma after tamoxifen therapy

INTRODUCTION: Several cases of low-grade endometrial stromal sarcomas in women with breast cancer have been reported to be associated with tamoxifen therapy. Estrogen receptor expression has been used to characterize the partial estrogenic action of tamoxifen on the endometrium and has been found in tamoxifen-associated endometrial pathologies. CASE: A low-grade endometrial stromal sarcoma in a woman with a history of breast cancer treated with adjuvant tamoxifen is presented. Steroid receptor studies performed on the tumor were negative for estrogen and positive for progesterone. CONCLUSION: The absence of estrogen receptor expression suggests that endometrial stromal sarcomas are not necessarily caused by the estrogenic properties of tamoxifen.     

Effects of leuprolide acetate on low-grade endometrial stromal sarcoma

We describe a low-grade endometrial stromal sarcoma coexistent with leiomyoma and adenomyosis treated with leuprolide acetate. We describe its histologic characteristics and clinical significance.     

Endometrial stromal sarcoma diagnosed after uterine morcellation in laparoscopic supracervical hysterectomy

Endometrial stromal sarcoma is a rare uterine cancer with no reliable method for preoperative diagnosis. A 30-year-old parous woman underwent laparoscopic supracervical hysterectomy because of a leiomyoma. The uterus was removed from the abdominal cavity with an electric morcellator with a spinning blade. The pathology report revealed low-grade endometrial stromal sarcoma. Two months after the initial surgery, a second laparoscopic procedure was performed. The final pathology report confirmed low-grade endometrial stromal sarcoma involving the ovary, fallopian tube, and ovarian artery. It was concluded that morcellation of leiomyomas at laparoscopic supracervical hysterectomy may potentially increase metastasis if the tumor is a sarcoma.     

A rare case of low grade endometrial stromal sarcoma of the uterus

Endometrial stromal sarcoma (ESS) is a rare disease. Low-grade endometrial stromal sarcoma (LGESS) is characterized by proliferations composed of cells with endometrial stromal cell differentiation and typically show extensive worm-like vessel invasion. We present a case of a 38-year-old woman with complaints of abdominal pains and ultrasound findings of a tumour formation in the pelvis. The biopsy examination showed a low-grade endometrial stromal sarcoma (LGESS), with a primary location in the uterine corpus and a tumour infiltration in the myometrium and cervix of the uterus, with tumour emboli in the lymph vessels and tumour methastases in the peritoneum--FIGO III Stage. The patient underwent postoperative polychemotherapy and radiotherapy. The gynecological and ultrasound examination found no pathological changes in the pelvic region five years after the operation.     

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.     

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.     

Endolymphatic Stromal Myosis Associated with Tamoxifen Use

A case of low-grade endometrial stromal sarcoma (endolymphatic stromal myosis) occurring in a patient who had received tamoxifen citrate for 3 years following surgical treatment for breast cancer is presented. Endometrial adenocarcinomas have been the most frequently reported tumor associated with tamoxifen use. More recently, uterine sarcomas have also been described in association with the use of tamoxifen. This report adds only the second case of a low-grade endometrial stromal sarcoma associated with tamoxifen use. As in the first report, the tumor demonstrated a sex-cord-like pattern of differentiation, an uncommon feature of endometrial stromal sarcomas. This suggests a possible association between tamoxifen use and the subsequent development of low-grade endometrial stromal sarcoma.     

The high-grade endometrial sarcoma: a rare entity

Introduction: With an estimated incidence of one to two per one million women, the endometrial stromal sarcoma (ESS) is a rare disease. It is subclassified into a high-grade and a prognostically better low-grade type. Evidence-based data for a standardized therapy is lacking. Case report: A 32-year-old obese nulligravida presented with persistent vaginal bleeding after the operation of an acute adnextorsion at another hospital. The repeat gynecological ultrasound examination showed a 5.3×5.3×3.6 cm vascularized, partially inhomogeneous mass in the uterus. A fractioned curettage yielded a differential diagnosis of malignant muellerian mixed tumor or a non-differentiated endometrial sarcoma. For completion of the operative treatment, laparotomy with hysterectomy, adnexectomy, and pelvine lymphonodectomy were performed. The final histological report described a 7 cm non-differentiated endometrial sarcoma with infiltration of the left ovary and 25 tumor-free lymph nodes. Discussion: Standard therapy for resectable sarcoma is abdominal hysterectomy and bilateral adnexectomy. So far, there is little data from studies reporting radio- or chemotherapy treatment of small patient numbers in an adjuvant setting. Conclusion: The ESS is a very rare disease of the uterus. Due to missing clinical data, it remains a multidisciplinary therapeutic challenge requiring individual decisions. To receive more information on this rare disease, treatment should be performed according to international protocols.     

Low-grade endometrial stromal sarcoma with florid intravenous component

A case of low-grade endometrial stromal sarcoma (LGESS) with striking involvement of pelvic veins is reported. The pattern of vascular involvement resembled that seen in intravenous leiomyomatosis. The report illustrated that the possibility of venous involvement should always be considered in the management of LGESS. Search for tumor involvement of the venous system at the time of diagnosis of metastasis or recurrence is essential. Surgical management of extensive venous recurrence of LGESS is feasible.     

Low-grade endometrial stromal sarcoma recurring with multiple bone and lung metastases: report of a case

BACKGROUND: Approximately 50% of patients with low-grade endometrial stromal sarcoma (ESS) develop recurrent disease, mainly in lung or pelvis. Bone metastasis of low-grade ESS is an extremely rare phenomenon. CASE: A 63-year-old Japanese female developed multiple bone and lung metastases 18 years after initial treatment for low-grade ESS. Bone scintigram showed a high uptake area at thoracic spine (Th6, Th8-9 and Th12), right 9th rib, iliac bone, and sacrum. Radiation therapy with Liniac of 4500cGy to the Th6 vertebra and Liniac of 4200cGy to sacrum was performed for the palliation of the pain. Radiotherapy was effective for the pain relief, although the size of recurrent tumor was unchanged. CONCLUSION: This is the first detailed reported case of multiple bone recurrence in a patient with low-grade ESS. The long-term follow-up after treatment is recommended.     

Excellent results of postoperative radiotherapy for endometrial stromal sarcoma of low-grade malignancy

Endometrial stromal sarcoma of low-grade malignancy (ESSL) is a rare neoplasm, and neither preoperative diagnostic procedures nor standard therapy have yet been established. We treated 3 cases of ESSL in the past 27 years, and we report here one of these cases that was classified as stage III (according to the FIGO classification of endometrial carcinoma). Postoperative radiotherapy was used to treat a residual tumor, and the patient showed a complete response.     

Asymptomatic intracardiac metastasis from a low-grade endometrial stromal sarcoma with successful surgical resection

BACKGROUND: The endometrial stromal sarcoma (ESS) is a rare neoplasm of the uterine origin. Intracardiac metastasis from the low-grade ESS is an extremely rare event. A case of a patient who successfully underwent surgical extraction of metastatic tumors of the low-grade ESS in the right ventricle is described in the present report. CASE: A 48-year-old woman was considered recurrence of the low-grade ESS 4 years after the initial operation for this disease. The CT scanning and magnetic resonance imaging demonstrated solid masses in the pelvis, the lungs, the inferior vena cava, and the right ventricle. The chemotherapy was effective against the recurrent tumors except for intracardiac site. Although she had no symptoms of right-sided heart failure, surgical resection of the intracardiac masses was successfully performed. Pathological examination confirmed intracardiac recurrent low-grade ESS. CONCLUSION: Surgical approach to intracardiac metastasis of the low-grade ESS is considered viable because of an excellent long-term prognosis in this disease and the likelihood of fatal heart failure or sudden death in untreated cases.     

Endometrial stromal sarcoma: a clinicopathologic study.

BACKGROUND: This study is a clinicopathologic evaluation of five patients with endometrial stromal sarcoma. PATIENTS AND METHODS: Over a period of 9 years 5 cases of ESS were observed in our Unit. The patients were retrospectively staged according to the FIGO staging system for endometrial cancer. The neoplasm was divided into two groups based on mitotic activity. Patients underwent endouterine curettage, surgery therapy and, except one of them, chemotherapy. RESULTS: Two patients had low-grade ESS stage Ib and Ic. The other three had high-grade ESS, and were in stage IIIa. Treatment was surgery for all patients, and adjuvant chemotherapy for 4 out of 5. Both patients in stage I are alive, clinically free from the disease, 25 and 36 months after diagnosis. In stage III all patients died 14, 25 and 36 months after diagnosis. CONCLUSION: ESS is a rare uterine neoplasm. Histologic grade is the most important prognostic factor.     

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.     

Hemangiopericytoma of the uterus vs. hemangiopericytoma-like endometrial stromal sarcoma: a histopathological approach to differential diagnosis

A case of uterine hemangiopericytoma is reported and compared to a case of endometrial stromal sarcoma of low-grade malignancy (also termed endolymphatic stromal myosis). Their clinical features are quite similar, whereas their morphological appearances are fairly unalike. The actual existence of the uterine hemangiopericytoma is emphasized, although the very nature of this tumour is still disputed by some authors. Light-microscopy findings supplemented by special stains allow a definite diagnosis even without ultrastructural study. Histological criteria for differentiating hemangiopericytoma of the uterus from uterine mesenchymal neoplasms with a pericytomatous-like pattern are discussed.