Mesenchymal Tumors of the Uterus

Oliva, Esther; Zaloudek, Charles; Soslow, Robert A.

doi:10.1007/978-1-4614-3165-7_10-2

Cited by 4 publications

(12 citation statements)

References 577 publications

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“…Die Tumortypisierung erfolgt nach der WHO-, die Stadieneinteilung nach der FIGO-bzw. TNM-Klassifikation [2,3].…”

Section: Tumortypisierung Bzw Klassifikationunclassified

“…In der WHO-Klassifikation sind folgende Entitäten als maligne mesenchymale Tumoren bzw. maligne gemischte epithelial-mesenchymale Tumoren aufgeführt [3,4]: ▪ Leiomyosarkome (LMS) ▪ low-grade endometriale Stromasarkome (LG-ESS) ▪ high-grade endometriale Stromasarkome (HG-ESS) ▪ undifferenzierte uterine Sarkome (UUS) ▪ Adenosarkome ▪ Rhabdomyosarkome ▪ PECome (perivascular epitheloid cell tumor), maligne Variante Die wesentlichen morphologischen Kriterien werden nachfolgend entitätsbezogen kurz dargestellt.…”

Section: Tumortypisierung Bzw Klassifikationunclassified

“…Low-Grade-ESS (LG-ESS) sind definiert als maligne Tumoren bestehend aus mesenchymalen Zellen, die dem endometrialen Stroma des Endometriums in der Proliferationsphase ähneln [3]. Der Nachweis intratumoraler Hämorrhagien und/oder Nekrosen ist variabel.…”

Section: Histopathologische Diagnoseunclassified

“…Wie bereits erwähnt, ist der Terminus "undifferenziertes endometriales Sarkom (UES)", der noch in der WHO-Klassifikation von 2003 geführt wurde [88] in der aktuellen WHO-Klassifikation nicht mehr enthalten [3] und sollte somit nicht mehr verwendet werden.…”

Section: Merkeunclassified

“…Die WHO-Klassifikation fordert für die Diagnose eines LMS den Nachweis von Mitosen und atypischen Tumorzellen[3]. Aufgrund der zum Teil schwierigen Unterscheidung von infarktoiden Nekrosen und Tumorzellnekrosen wird dieser Parameter für die Diagnose nicht zwingend gefordert.Für das Grading von LMS und anderen seltenen Sarkomen gibt es in der WHO-Klassifikation kein System[3]; für das Grading endometrialer Stromasarkome (ESS) erfolgt in der WHO-Klassifikation eine Unterteilung in Low-und High-Grade-Tumoren[3].Bei extrauterinen Weichteilsarkomen favorisiert die WHO-Klassifikation zur Prognoseabschätzung und Festlegung des therapeutischen Vorgehens das Gradingsystem der Fédération Nationale des Centres de Lutte Contre le Cancer, das sog. FNCLCC-Gradingsystem[28 -31], welches auch von der European Organisation for Research and Treatment of Cancer (EORTC) empfohlen wird.…”

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Uterine Sarkome

Denschlag¹

2018

Frauenheilkunde up2date

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“…Die Tumortypisierung erfolgt nach der WHO-, die Stadieneinteilung nach der FIGO-bzw. TNM-Klassifikation [2,3].…”

Section: Tumortypisierung Bzw Klassifikationunclassified

Section: Histopathologische Diagnoseunclassified

Section: Merkeunclassified

unclassified

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Uterine Sarkome

Denschlag¹

2018

Frauenheilkunde up2date

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Sarcoma of the Uterus. Guideline of the DGGG and OEGGG (S2k Level, AWMF Register Number 015/074, February 2019)

Denschlag

Ackermann

Battista

et al. 2019

Geburtshilfe Frauenheilkd

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Aims This is an official guideline published and coordinated by the German Society of Gynecology and Obstetrics (DGGG) and the Austrian Society of Gynecology and Obstetrics (OEGGG). Because of their rarity and heterogeneous histopathology, uterine sarcomas are challenging in terms of how they should be managed clinically, and treatment requires a multidisciplinary approach. To our knowledge, there are currently no binding evidence-based recommendations for the appropriate management of this heterogeneous group of tumors. Methods This S2k guideline was first published in 2015. The update published here is the result of the consensus of a representative interdisciplinary group of experts who carried out a systematic search of the literature on uterine sarcomas in the context of the guidelines program of the DGGG, OEGGG and SGGG. Members of the participating professional societies achieved a formal consensus after a moderated structured consensus process. Recommendations The consensus-based recommendations and statements include the epidemiology, classification, staging, symptoms, general diagnostic work-up and general pathology of uterine sarcomas as well as the genetic predisposition to develop uterine sarcomas. Also included are statements on the management of leiomyosarcomas, (low and high-grade) endometrial stromal sarcomas and undifferentiated uterine sarcomas and adenosarcomas. Finally, the guideline considers the follow-up and morcellation of uterine sarcomas and the information provided to patients.

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Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Case Report

Chai

Ding

Zhou

et al. 2020

Preprint

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Background: Low-grade endometrial stromal sarcoma (ESS) is rare mesenchymal neoplasm, which has an indolent history with late recurrences. ESS usually spread through the lymph nodes and venous system but very seldom involve large vessels or the heart.Case presentation: A 38-year-old Chinese woman was admitted to our department due to pelvic mass found on physical examination. The superior and inferior vena cava CT angiography (CTA) showed an enlarged uterine as well as low density image in the left internal iliac vein, the left common iliac vein, the inferior vena cava, the left renal vein adjacent to the heart and the right atrium, with a range of 110*16mm. The filling defect of right atrium was about 30*14mm. The three-dimensional computed tomography reconstruction showed that the mass originated from the uterine and invaded into the reproductive vein, subsequently extended along the inferior vena cava to the right atrium. Needle biopsy of the pelvic mass was performed and the tissue indicates smooth muscle. The preoperative diagnosis was intravascular leiomyomatosis and the patient underwent radical resection: thrombectomy and total hysterectomy with bilateral salpingo-oophorectomy. Postoperative histopathology revealed low grade endometrial stromal sarcoma. Microscopically, the tumors in both original uterine lesions and intravascular and intracardiac metastases shared morphologic features characterized by neoplastic cells similar to proliferative-phase endometrial stromal cells, in which small spiral artery differentiation was recognized and tumor tissue showed invasive growth pattern by inserting into the surrounding smooth muscle. Immunohistochemistry showed tumor cells were reactive to Estrogen Receptor, Progesterone Receptor,CD10. Primary uterine foci showed cyclin D1(5%+) and Ki-67(20%+),whereas metastatic lesions of the intracardiac and the intravascular component identified cyclin D1(negative) and Ki-67(2%+). The patient is alive without evidence of recurrence 3 months after surgery.Conclusions: Distant metastasis of low-grade endometrial stromal sarcoma is rare, especially involving large vessels or the heart. This case demonstrates that malignant tumor metastasis should be considered as a differential diagnosis of intracardiac and intravascular masses. The treatment rely on multidisciplinary cooperation.

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Mesenchymal Tumors of the Uterus

Cited by 4 publications

References 577 publications

Uterine Sarkome

Uterine Sarkome

Sarcoma of the Uterus. Guideline of the DGGG and OEGGG (S2k Level, AWMF Register Number 015/074, February 2019)

Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Case Report

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