Endometrial stromal sarcoma: A review of rare mesenchymal uterine neoplasm

2020 ◽  
Vol 46 (11) ◽  
pp. 2221-2236
Author(s):  
V.A. Capozzi ◽  
L. Monfardini ◽  
V. Ceni ◽  
A. Cianciolo ◽  
D. Butera ◽  
...  
Keyword(s):  
Endometrial Stromal Sarcoma ◽  
Stromal Sarcoma ◽  
Uterine Neoplasm

scholarly journals Pleural Metastasis of High-Grade Endometrial Stromal Sarcoma With YWHAE Gene (17p13.3) Rearrangement, A Rare Case Report

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S41-S41
Author(s):  
A C Re ◽  
M Enrique ◽  
S Ren
Keyword(s):  
Case Report ◽  
Spindle Cell ◽  
Malignant Neoplasm ◽  
Endometrial Stromal Sarcoma ◽  
Low Grade ◽  
High Grade ◽  
Stromal Sarcoma ◽  
Uterine Tumors ◽  
Uterine Neoplasm ◽  
Left Pleural Effusion

Abstract Introduction/Objective Endometrial stromal sarcoma (ESS), a rare malignant neoplasm of endometrial stroma, accounts for less than 1% of all uterine tumors. High grade ESS (HGESS) is aggressive and commonly relapses even after surgical and neoadjuvant therapy. Abdominal and pelvic regions are common sites of metastasis, however, distant metastases to the liver, lung, vertebrae, and brain have been reported. Methods/Case Report We encountered a 49-year-old female who presented with shortness of breath, found to have a left pleural effusion and multiple pleural masses. She initially presented three years ago with heavy irregular menses and left pelvic pain for one year. D&C revealed prominent small spindle cells for which a stromal nodule and low-grade or malignant process was probable. CT scan showed an enlarged uterus. Hysterectomy with bilateral salpingo- oophorectomy, bilateral pelvic and para-aortic lymph node dissection, and partial omentectomy were performed. The uterus revealed an intramural 7 cm mass with a serpiginous growth pattern and lymphovascular invasion. Tumor cells were plump to spindled with areas of high cellularity, rounded nuclei, increased atypia and mitosis. Atypical areas were positive for cyclin D1, focally positive for CD10, and negative for ER, PR, SMA, desmin, AE1/3 and CAM5.2. FISH studies showed rearrangement of YWHAE gene (17p13.3) and no rearrangement of JAZF1 or PHF1 gene regions. Findings supported the diagnosis of HGESS. The patient received post-operative chemotherapy. Biopsy of the current pleural lesion revealed a nonspecific malignant spindle cell neoplasm positive for BCL1, CD56, CD117, CD99, TLE1 and INI1, while negative for AE1/3, CAM5.2, EMA, ER, PR, CK5/6, calretinin, SMA, desmin and S100. The CD10 stain was inconclusive. FISH studies showed rearrangement of YWHAE gene (17p13.3) and no rearrangement involving JAZF1 or PHF1 gene regions. No rearrangement of the SS18 gene region was observed and synovial sarcoma was excluded. Overall findings support the diagnosis of metastatic HGESS. Results (if a Case Study enter NA) NA Conclusion HGESS, a rare tumor with a nonspecific immunostain profile, has the ability to metastasize to rare body sites, such as the pleura in our case. Display of spindle cell morphology is a nonspecific finding that raises broad differential diagnoses. In women, with or without a history of uterine neoplasm, HGESS is a clinically worthwhile diagnosis to be mindful of.

A ruptured ovarian cystadenofibroma presenting with life-threatening sepsis and an incidental synchronous endometrial stromal sarcoma

2021 ◽  
Vol 14 (11) ◽  
pp. e245473
Author(s):  
Alistair Boyd ◽  
Hemant Sheth ◽  
Faris Kubba ◽  
Mohammad Aziz
Keyword(s):  
Rare Complication ◽  
Iliac Fossa ◽  
Endometrial Stromal Sarcoma ◽  
Uterine Sarcoma ◽  
Stromal Sarcoma ◽  
Gene Testing ◽  
Uterine Neoplasm ◽  
Life Threatening ◽  
History Of ◽  
The Right

A woman in her 60s presented with a rare complication of an ovarian cyst which many clinicians may not consider at first presentation. She was admitted with life-threatening staphylococcus aureus sepsis. She presented shocked with a collapse following a 2-day history of diarrhoea, vomiting and pain in the right iliac fossa. She was taken to theatre where a ruptured, widely infarcted left ovarian serous cystadenofibroma was discovered with over 2 litres of purulent fluid exuding from the cyst into the abdomen. She had a left cyst removal, hysterectomy and bilateral salpingo-oophorectomy performed. Histological analysis and molecular gene testing of an incidentally discovered uterine neoplasm revealed an undifferentiated uterine sarcoma. She successfully recovered as an inpatient and was discharged under the care of an oncology team for ongoing management.

Extragenital endometrial stromal sarcoma of transverse mesocolon: A diagnostic conundrum

2021 ◽  
pp. 106689692110704
Author(s):  
Aishwarya Sharma ◽  
Munita Bal ◽  
Santosh Menon
Keyword(s):  
Estrogen Receptor ◽  
Progesterone Receptor ◽  
Gastrointestinal Stromal Tumor ◽  
Clinical Diagnosis ◽  
Rare Case ◽  
Abdominal Mass ◽  
Endometrial Stromal Sarcoma ◽  
Transverse Mesocolon ◽  
Stromal Sarcoma ◽  
Uterine Neoplasm

Endometrial stromal sarcoma (ESS) is a rare uterine neoplasm infrequently arising in extra-genital sites. Herein, we report an extremely rare case of primary extra-genital ESS of transverse mesocolon occurring in a 51-year-old female presenting with gradually increasing abdominal mass. The clinical diagnosis considered was a gastrointestinal stromal tumor. Intra-operatively, the mass was confined exclusively to the transverse mesocolon. Microscopy revealed a cellular tumor composed of oval to elongate neoplastic cells with hyperchromatic nuclei, inconspicuous nucleoli and were immunoreactive for CD10, progesterone receptor (PR), estrogen receptor (ER), and PAX8; negative for KIT, CD34, SMA, S100, synaptophysin, chromogranin, WT-1, and calretinin. A distinct arborizing network of arterioles along with foci of endometriosis was also seen. We present this case for its extreme rarity and the challenges entailed in its diagnosis.

scholarly journals CT, MRI, and FDG-PET imaging findings of low-grade extrauterine endometrial stromal sarcoma arising from the mesentery: A case report

2021 ◽  
Vol 16 (9) ◽  
pp. 2774-2779
Author(s):  
Satoshi Suzuki ◽  
Ryo Kurokawa ◽  
Tetsushi Tsuruga ◽  
Mayuyo Mori‑Uchino ◽  
Haruka Nishida ◽  
...  
Keyword(s):  
Case Report ◽  
Pet Imaging ◽  
Fdg Pet ◽  
Endometrial Stromal Sarcoma ◽  
Low Grade ◽  
Imaging Findings ◽  
Stromal Sarcoma

scholarly journals Fusion genes in gynecologic tumors: the occurrence, molecular mechanism and prospect for therapy

2021 ◽  
Vol 12 (8) ◽  
Author(s):  
Bingfeng Lu ◽  
Ruqi Jiang ◽  
Bumin Xie ◽  
Wu Wu ◽  
Yang Zhao
Keyword(s):  
Tumor Progression ◽  
Massively Parallel Sequencing ◽  
Gynecologic Cancer ◽  
Endometrial Stromal Sarcoma ◽  
Driver Mutations ◽  
Fusion Genes ◽  
Stromal Sarcoma ◽  
Uterine Tumors ◽  
Rearrangement Mechanism ◽  
Gynecologic Tumors

AbstractGene fusions are thought to be driver mutations in multiple cancers and are an important factor for poor patient prognosis. Most of them appear in specific cancers, thus satisfactory strategies can be developed for the precise treatment of these types of cancer. Currently, there are few targeted drugs to treat gynecologic tumors, and patients with gynecologic cancer often have a poor prognosis because of tumor progression or recurrence. With the application of massively parallel sequencing, a large number of fusion genes have been discovered in gynecologic tumors, and some fusions have been confirmed to be involved in the biological process of tumor progression. To this end, the present article reviews the current research status of all confirmed fusion genes in gynecologic tumors, including their rearrangement mechanism and frequency in ovarian cancer, endometrial cancer, endometrial stromal sarcoma, and other types of uterine tumors. We also describe the mechanisms by which fusion genes are generated and their oncogenic mechanism. Finally, we discuss the prospect of fusion genes as therapeutic targets in gynecologic tumors.

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