Extrauterine low-grade endometrial stromal sarcoma with florid endometrioid glandular differentiation.

Endometrial stromal sarcoma of the uterus (ESS) is a rare lesion that can cause diagnostic difficulty especially when it presents with unusual histologic features such as diffuse endometrioid glandular differentiation. Only three such cases have been reported, all primary in the uterus. We report the first case of an extrauterine low-grade ESS with extensive glandular differentiation that appeared to arise in endometriosis.     

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.     

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.     

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.     

A case of endometrial stromal sarcoma with curvilinear calcification

BACKGROUND: Although many histopathological, clinical, and imaging findings of ESS have been described, intense calcifications have not been reported previously. CASE: A 54-year-old female was admitted with vaginal bleeding and abdominal pain. Gynecologic examination revealed a huge, firm pelvic mass extending up to the epigastrium. Transabdominal ultrasonography and computed tomography demonstrated a well-demarcated uterine mass containing heterogeneous solid and cystic areas and a centric curvilinear calcification. Histopathological diagnosis was established as undifferentiated high-grade endometrial stromal sarcoma. CONCLUSION: Endometrial stromal sarcomas should be considered in the differential diagnosis of uterine masses containing curvilinear calcifications.     

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.     

High-grade pelvic sarcoma after radiation therapy for low-grade endometrial stromal sarcoma

A high-grade heterologous pelvic sarcoma arose in a 60-year-old woman 15 years after she received whole-pelvic radiation for a low-grade endometrial stromal sarcoma. This complication must be considered in determining therapy for low-grade endometrial sarcomas, which are usually inherently of indolent biological behavior.     

Expression of progesterone receptor A and B isoforms in low-grade endometrial stromal sarcoma.

The progesterone receptor (PR) exists as two isoforms, PRA and PRB. In vitro studies have shown that these proteins are functionally distinct, suggesting that their relative expression can influence progesterone response. Low-grade endometrial stromal sarcoma (LGESS) is an uncommon tumor that usually expresses PR. In normal endometrial stroma, both PR isoforms are present with PRA predominant throughout the menstrual cycle. The relative expression of PRA and PRB in LGESS has not been previously reported. All nine cases of primary LGESS (seven uterine, two extrauterine) expressed PRB. Eight tumors also contained PRA and it was the predominant isoform in seven cases. These tumors had similar histopathologic appearances, whereas a case with approximately equal PR isoform expression showed features of sex cord or smooth muscle differentiation. An extrauterine tumor expressing only PRB had myxoid stroma. Recurrent tumor in two cases, which expressed predominantly PRA in the primary, contained reduced levels of PR consisting predominantly or entirely of PRB after prolonged interval progestin therapy. Most primary LGESSs showed PR isoform expression similar to normal endometrial stroma, consistent with the highly differentiated phenotype of this tumor. Variant differentiation or disease recurrence was accompanied by an altered PR isoform profile that could impact on hormone response.     

Atypical endometrial stromal cells related with a polypoid leiomyoma with bizarre nuclei: a case report.

We presented a polypoid leiomyoma with bizarre nuclei having atypical endometrial stromal cells adjacent to the leiomyoma. Although atypical stromal cells of the female genital tract have been described, to our knowledge, no lesion identical to ours has been reported thus far. These atypical cells may be mistaken for malignant cells on histologic examination, especially in small endometrial samples. To differentiate these kinds of atypical-featured lesions from sarcoma is important, especially in curettage materials. The etiology and precise nature of multinucleated giant cells and atypical stromal cells in the endometrium are unclear. To prevent radical surgery, pathologists must be familiar with these atypical cells in the female genital tract.     

Endolymphatic stromal myosis (endometrial low-grade stromal sarcoma) presenting with hematuria: a diagnostic challenge

In this case of a 41-year-old white woman who presented with hematuria, the diagnosis of metastatic endolymphatic stromal myosis (ESM) in the urinary bladder was reached on the basis of the histologic pattern and with the aid of intermediate filament typing. In the hysterectomy specimen the tumor foci of ESM were of minute size. To the best of our knowledge, this is the first report of such an unusual clinical presentation of this lesion.     

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.     

Multilocular intrauterine cystic mass,atypical presentation of endometrial stromal sarcoma: a case report

Endometrial stromal sarcoma (ESS) is a rare neoplasm comprising only 0.2% of all uterine malignancies and 15–26% of primary uterine sarcomas. Pathology of the tumor was described as soft, fleshy, smooth polypoidal mass, which may protrude in the uterine cavity. This case report, represent atypical presentation of ESS as multilocular cystic intrauterine mass, which can be misdiagnosed as multilocular ovarian cyst.     

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.     

Ovary and uterus-sparing procedures for low-grade endometrial stromal sarcoma: A retrospective study of 153 cases

Objective

To discuss the optimal treatment options for low grade endometrial stromal sarcoma (LG-ESS).

Methods

Medical records of consecutive patients with LG-ESS in our institute were collected. The pertinent data, including clinicopathological characteristics, treatment and prognostic information were evaluated.

Results

A total of 153 cases of LG-ESS were included. The 5-year relapse free survival (RFS), overall survival (OS) and survival after relapse (SAR) rates were 66.1%, 95.8% and 82.9%, respectively. Ovary-sparing procedures, positive resection-margins, and myomectomy were the independent adverse factors for relapse (P < 0.0001, = 0.0041, and = 0.0075, respectively). Post-menopause, cervical involvement, and positive lymphovascular space involvement were significantly associated with survival (P < 0.0001, = 0.0020, and = 0.0163, respectively). Distance recurrence and macroscopically residual tumors negatively affected SAR (P = 0.0137 and = 0.0004, respectively). No benefit was found for lymphadenectomy in terms of both RFS and OS (P = 0.1187 and = 0.5138, respectively). Initial ovary-sparing procedures and myomectomy had no impact on OS (P = 0.0810 and = 0.8845, respectively). Adjuvant treatment had a slightly beneficial effect both on OS and SAR.

Conclusion

Hysterectomy with bilateral salpingo-oophorectomy and complete resection of the macroscopic lesion should be treated as the initial and salvage mainstay treatments for LG-ESS patients. Ovary-sparing procedures could be considered for young women without cervical involvement; however, long-term follow-up should be mandatory. Myomectomy should only be conserved for young patients with a strong desire for future fertility, with fully informed consent; hysterectomy was recommended after the completion of pregnancy and delivery. However, the roles of lymphadenectomy and adjuvant treatment deserve further investigation.