Endometrial Stromal Sarcoma Presenting Research Articles

Advanced-stage Endometrial Stromal Sarcoma Presenting as Primary Infertility in a Young Nulligravida: A Case Report

Advanced-stage Endometrial Stromal Sarcoma Presenting as Primary Infertility in a Young Nulligravida: A Case Report

Advanced-stage Endometrial Stromal Sarcoma Presenting as Primary Infertility in a Young Nulligravida: A Case Report.

Endometrial stromal sarcoma (ESS) rarely causes infertility in young women. We report a nulligravida in her 30s who presented with primary infertility of 15 years. Hysteroscopy revealed a submucosal necrotic fibroid polyp. Ultrasonography detected multiple intramural fibroids. Open myomectomy with polypectomy was performed. Histopathology revealed low-grade ESS (LGESS) within the fibroid polyp. Subsequently, the patient underwent completion surgery. Her final diagnosis was estrogen-receptor positive LGESS stage IIIB, and she was suggested anastrozole adjuvant therapy and long-term surveillance. ESS with abnormal perimenopausal bleeding, though the most common presentation, may not always observed. Hence, a high index of suspicion of ESS should always be kept as a differential diagnosis in uterine fibroid polyp, though rare. Considering the scarcity of more extensive studies on ESS, reporting of cases will aid in formulating management protocols.

Recurrent Low-grade Endometrial Stromal Sarcoma Presenting as Isolated Pedunculated Vaginal Cuff Polyp.

Recurrent Low-grade Endometrial Stromal Sarcoma Presenting as Isolated Pedunculated Vaginal Cuff Polyp.

YWHAE-FAM22 high grade endometrial stromal sarcoma presenting as a cervical mass

YWHAE-FAM22 high grade endometrial stromal sarcoma presenting as a cervical mass

Endometrial stromal sarcoma presenting as large bleeding left upper quadrant mass.

Endometrial stromal sarcoma (ESS) represents only 0.2% of all uterine malignancies. Even fewer cases have been reported on patients presenting with symptomatic disease. We report the case of young lady presenting with a large bleeding abdominal mass. At the time of surgery, an en bloc excision of the mass with a partial pancreatectomy, splenectomy, transverse colectomy, left nephrectomy, left adrenalectomy and resection of the diaphragm was performed. The final pathology revealed a malignant spindle and epithelial cell neoplasm with features favoring a variant of ESS. ESS can present in an atypical fashion as an intra-abdominal mass; a multi-disciplinary treatment approach for patients with this disease is crucial.

Frequent expression of KIT in endometrial stromal sarcoma with YWHAE genetic rearrangement

Endometrial stromal sarcomas with the YWHAE-NUTM2A/B genetic fusion characteristically contain high-grade round to epithelioid cell component that is strongly and diffusely cyclin D1-positive and it may or may not show an associated low-grade fibroblastic/myxoid cell component. They are clinically more aggressive than endometrial stromal sarcomas with the JAZF1-SUZ12 genetic fusion and frequently demonstrate extrauterine extension at initial clinical presentation. In this setting, the tumor may be misdiagnosed as gastrointestinal stromal tumor. This study examines the expression of KIT and ANO1 in 14 YWHAE-NUTM2A/B tumors by immunohistochemistry. Staining localization was determined as membranous and/or cytoplasmic, and the staining intensity was assessed (negative, weak, moderate and strong). Of the 14 tumors, 6 contained only a high-grade round cell component, 2 only a low-grade fibroblastic component and 6 had both components in the slides evaluated. The high-grade round cell component displayed moderate to strong membranous/cytoplasmic KIT staining in all tumors (12 of 12). The low-grade fibroblastic cell component showed only weak cytoplasmic KIT staining in 3 of 8 tumors. In contrast, ANO1 was negative in all 14 neoplasms, irrespective of the component evaluated. Sanger sequencing analysis (exons 9, 11, 13 and 17) and Ampliseq Cancer Panel mutation screen (Ion Torrent) demonstrated no KIT mutations in three KIT-positive YWHAE-NUTM2A/B tumors. This study shows that the high-grade round cell component of YWHAE-NUTM2A/B endometrial stromal sarcoma consistently expresses KIT but lacks KIT hotspot mutations. KIT expression may represent a potential diagnostic pitfall in the evaluation of YWHAE-NUTM2A/B endometrial stromal sarcoma presenting with pelvic/abdominal mass, particularly in situations where its uterine origin is not definitive, and thus a panel of antibodies that includes ANO1 and cyclin D1 is necessary.

Low grade endometrial stromal sarcoma presenting as a cervical polyp in a young female: A rare case report

Endometrial stromal sarcomas ESS are rare malignant mesenchymal tumors that usually develop in the uterus in perimenopausal women. The report describes a rare extra uterine low grade ESS presenting as a cervical polyp in a young female for which local tumor resection was performed. Histological examination revealed a spindle cell tumor. The diagnostic challenge was to differentiate ESS from cellular leiomyoma and other spindle cell tumors for which immunohistochemistry was carried out. The case report highlights that ESS is to be included in the differential diagnosis of spindle cell tumor in cervix even though rare. We also discuss the differential diagnosis of cervical polypoidal lesions.

Endometrial stromal sarcoma presenting as multiple primary tumor

Endometrial Stromal Sarcoma (ESS) is a rare uterine malignancy. The annual incidence of ESS has been reported as 1 - 2 per million women. In the general population approximately 10 percent of cancer patients may develop a second primary cancer. Synchronous multiple primary tumors constitute only 20% of all multiple primary tumors, and by definition, occur within a period of two month. This case report is presented to document the occurrence of ESS as a “Synchronous Multiple Primary Tumor”, which is an extremely rare event. Previously only one case report has documented such an occurrence. The literature is also briefly reviewed. This case underlines the requirement of a thorough initial evaluation for a patient with a gynecological malignancy and a regular follow-up. Follow-up includes patient education regarding symptoms of any recurrence, physical examination repeated at 6 - 12 monthly interval, along with imaging studies when indicated.

High-grade endometrial stromal sarcoma presenting in a 28-year-old woman during pregnancy: a case report

IntroductionTo the best of our knowledge, soft tissue sarcomas have not prevously been reported as a complication during pregnancy.Case presentationA 28-year-old Caucasian woman was diagnosed with a transperitoneal sarcoma during pregnancy. Morphological, immunohistochemical, chromosomal and mutational analyses pointed towards a high-grade endometrial stromal sarcoma. Although surgery and chemotherapy are possible during pregnancy, we were unable to perform these in this case.ConclusionThe potential to treat gynecological cancer during pregnancy should always be assessed individually.

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.

Uterine epithelioid endometrial stromal sarcoma presenting as a “cervical polyp”

Although appearance of conventional uterine endometrial stromal sarcoma is easily recognized on histology, it may uncommonly assume unusual appearances such as uterine tumor resembling ovarian sex-cord tumor, thereby hindering its diagnosis. Recently, its manifestation as an epithelioid neoplasm was described. In this report, we detail yet another instance where this tumor adopted an epithelioid morphology, presenting itself as a polyp extruding from the cervical os in a 41-year-old Chinese woman. Both the polypectomy and subsequent hysterectomy specimens revealed a predominant proliferation of CD10-negative, caldesmon-negative, and CD117-positive epithelioid cells set within a stroma containing vascular proliferation resembling endometrial stromal tumor. Areas of typical low-grade endometrial stromal sarcoma containing spindle cells that were focally positive for CD10 and negative for CD117 were present in close association with the epithelioid areas. The differential diagnoses and possible implication of CD117 positivity are discussed.

Low-grade endometrial stromal sarcoma presenting as vaginal nodule

Endometrial stromal sarcoma is a rare neoplasm of the uterus. Extrauterine locations of this neoplasm, excluding metastases or local extension, are even more unusual and are usually associated with the presence of endometriosis. The authors report a case of endometrial stromal sarcoma presenting as a vaginal wall nodule, without any sign of primary uterine tumor after extensive evaluation or presence of endometriosis. The morphology, immunohistochemical profile, differential diagnoses, and pathogenesis are discussed, as well as a review of the literature on this issue.

Endometrial stromal sarcoma presenting as postpartum haemorrhage: report of a case with a sole t(10;17)(q22;p13) translocation

Background Although the clinical picture of endometrial stromal sarcoma (ESS) is variable, it was never reported to present as a postpartum hemorrhage. In addition, ESS is a tumor type of which, due to its rarity, little is known regarding chemosensitivity and genetic changes. Case A 28-year-old woman complaining of persistent postpartum bleeding was referred to our hospital, where she was diagnosed with ESS. At laparotomy, the invasion of nervous and vascular pelvic structures rendered her inoperable, and chemotherapy (doxorubicin 50 mg/m 2 for 15 min; ifosfamide 5 g/m 2/24 h; mesna 5 g/m 2, every 3 weeks) was initiated. The ESS appeared to be chemosensitive because after three treatment cycles the tumor iliac metastase significantly decreased in volume and became surgically removable. Chemosensitivity was confirmed microscopically. Three additional courses of chemotherapy and pelvic irradiation were administered. Cytogenetic evaluation of both the primary as well as the metastatic lesions revealed a t(10;17)(q22;p13) as the sole cytogenetic abnormality. Conclusion Three interesting features of this particular case put ESS in a new perspective. First, the fundal ESS permitted normal conception and pregnancy but caused a postpartum haemorrhage. Second, the ESS was clearly chemosensitive. Third, we report a novel cytogenetic aberration in ESS, the molecular characterization of which might lead to the identification of the deregulated pathway(s) triggering tumor development in ESS.

Endometrial Stromal Sarcoma Presenting as Extrauterine Metastases

A case of endometrial stromal sarcoma that initially presented as an umbilical mass 5 years before and as a lung lesion 8 months before the primary tumor was detected in the uterus is reported. The morphologic features and immunohistochemical profile of the two initial extrauterine lesions suggested metastases from a primary endomerial stromal sarcoma of the uterus, which was confirmed by subsequent endometrial curettage. The tumors in all three locations were characterized histologically by small to medium-sized uniform cells with fewer than 10 mitoses per 10 high-power fields and distinct arterial vasculature that resembled the spiral arteries of the normal enometrium. Both the endometrial and umbilical tumors were immunoreactive for estrogen and progesterone receptors but showed no immunoreactivity for cytokertin. Additionally, the tumor cells of the umbilical mass showed no immunoreactivty for S-100 protein, HMB-45, smooth-muscle actin, or desmin. Endometrial stromal sarcomas are usually confined to the uterus at the time of diagnosis. When they preent initially as an extrauterine metastasis, diagnostic difficulties may arise. A recogition of the morphologic features, together with the immunoreactivity pattern, is needed in evaluating endometrial stromal sarcomas that present as extrauterine metastases.

Recurrent endometrial stromal sarcoma resembling intravenous leiomyomatosis

A case of recurrent low-grade endometrial stromal sarcoma (endolymphatic stromal myosis) presenting as an intracardiac neoplasm is reported. Pronounced leiomyomatous differentiation was noted in the recurrence. This together with the gross pathology closely resembled intravenous leiomyomatosis.