ZC3H7B–BCOR high-grade endometrial stromal sarcoma may present as myoma nascens with cytoplasmic signet ring cell change

Ondič, Ondřej; Bednarova, Barbora; Ptáková, Nikola; Hájková, Veronika; Šteiner, Petr; Šidlová, Henrieta; Presl, J; Bouda, J; Alaghehbandan, Reza; Michal, Michal

doi:10.1007/s00428-020-02744-5

Cited by 13 publications

(14 citation statements)

References 11 publications

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“…Most of the clinical data published on patients diagnosed with HG‐ESS showed stage 3 disease or the patient had a recurrence. However, ESS harboring a ZC3H7B/BCOR fusion may be clinically as well as morphologically heterogeneous 54‐56 . A unique case of ZC3H7B/BCOR‐ positive HG‐ESS was identified at an early stage when an endocervical polypoid mass from the lower uterine segment was examined 56 .…”

Section: Chromosomal Aberrations and Their Molecular Productsmentioning

confidence: 99%

“…The gene's orientation in the different chimeric fusions is intriguing, whether it is 3′ or 5′. Furthermore, some but not all ZC3H7B/BCOR positive HG‐ESS are characterized by the presence of also the reciprocal transcript 51,52,56 whose role in tumorigenesis and/or progression is still unknown. Additionally, a new BCOR ‐rearranged HG‐ESS was recently reported by Kommoss et al 69 in which RNAseq identified a fusion between BCOR and LPP .…”

Section: Chromosomal Aberrations and Their Molecular Productsmentioning

confidence: 99%

“…However, ESS harboring a ZC3H7B/BCOR fusion may be clinically as well as morphologically heterogeneous 54‐56 . A unique case of ZC3H7B/BCOR‐ positive HG‐ESS was identified at an early stage when an endocervical polypoid mass from the lower uterine segment was examined 56 . Though macroscopically the tumor presented as a polypoid mass descending into the cervical canal in a myoma nascens‐like fashion, the histomorphologic and immunohistochemical profiles were suggestive of HG‐ESS.…”

Section: Chromosomal Aberrations and Their Molecular Productsmentioning

confidence: 99%

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Molecular pathogenesis and prognostication of "low‐grade'' and "high‐grade" endometrial stromal sarcoma

Micci

Heim

Panagopoulos

2020

Genes Chromosomes & Cancer

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Endometrial stromal sarcomas (ESS) are a heterogeneous group of rare mesenchymal cancers. Considerable knowledge has been gained in recent years about the molecular characteristics of these cancers, which helps to classify them in a more meaningful manner leading to improved diagnosis, prognostication, and treatment. According to this classification, ESS is now grouped as low‐ or high‐grade. ESS may have overlapping clinical presentation, morphology, and immunohistochemical profile. Their genetic characteristics allow subdivision of many of them depending on which pathogenetically important fusion genes they carry, but clearly much more needs to be unraveled in this regard. We here provide an overview of the molecular pathogenetic knowledge gained so far on low‐ and high‐grade ESS.

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Section: Chromosomal Aberrations and Their Molecular Productsmentioning

confidence: 99%

Section: Chromosomal Aberrations and Their Molecular Productsmentioning

confidence: 99%

Section: Chromosomal Aberrations and Their Molecular Productsmentioning

confidence: 99%

See 1 more Smart Citation

Molecular pathogenesis and prognostication of "low‐grade'' and "high‐grade" endometrial stromal sarcoma

Micci

Heim

Panagopoulos

2020

Genes Chromosomes & Cancer

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“…HGESS has a poor prognosis, as more than 50% of patients will present with an advanced stage of the disease. Lung metastases are most common [ 41 , 44 , 58 , 59 , 60 , 61 , 62 ].…”

Section: Uterine Sarcomasmentioning

confidence: 99%

“…Grossly, HGESS is a pink-to-yellow-tan polypoid friable tumor with extensive invasion through the myometrium [ 60 ]. Cases of HGESS appearing as myoma nascens or multiple intrauterine tumors have been described [ 47 , 60 , 61 ].…”

Section: Uterine Sarcomasmentioning

confidence: 99%

New Aspects of Sarcomas of Uterine Corpus—A Brief Narrative Review

Kostov

Kornovski

Ivanova

et al. 2021

Clinics and Practice

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Sarcomas of the uterine corpus are rare malignant neoplasms, which are further classified into mesenchymal tumors, and mixed (epithelial plus mesenchymal) tumors. The main issues concerning these neoplasms are the small number of clinical trials, insufficient data from evidence-based medicine, insignificant interest from the pharmaceutical industry, all of which close a vicious circle. The low frequency of these malignancies implies insufficient experience in the diagnosis, hence incomplete surgical and complex treatment. Additionally, the rarity of these sarcomas makes it very difficult to develop clinical practice guidelines. Preoperative diagnosis, neoadjuvant and adjuvant chemoradiation, target and hormone therapies still raise many controversies. Disagreements about the role and type of surgical treatment are also often observed in medical literature. There are still insufficient data about the role of pelvic lymph node dissection and fertility-sparing surgery. Pathologists’ experience is of paramount importance for an accurate diagnosis. Additionally, genetics examinations become part of diagnosis in some sarcomas of the uterine corpus. Some gene mutations observed in uterine sarcomas are associated with different outcomes. Therefore, a development of molecular classification of uterine sarcomas should be considered in the future. In this review, we focus on the epidemiology, pathogenesis, pathology, diagnosis and treatment of the following sarcomas of the uterine corpus: leiomyosarcoma, low- and high-grade endometrial stromal sarcomas, undifferentiated sarcoma and adenosarcoma. Uterine carcinosarcomas are excluded as they represent an epithelial tumor rather than a true sarcoma.

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The clinical, morphological, and genetic heterogeneity of endometrial stromal sarcoma

2020

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ZC3H7B–BCOR high-grade endometrial stromal sarcoma may present as myoma nascens with cytoplasmic signet ring cell change

Cited by 13 publications

References 11 publications

Molecular pathogenesis and prognostication of "low‐grade'' and "high‐grade" endometrial stromal sarcoma

Molecular pathogenesis and prognostication of "low‐grade'' and "high‐grade" endometrial stromal sarcoma

New Aspects of Sarcomas of Uterine Corpus—A Brief Narrative Review

The clinical, morphological, and genetic heterogeneity of endometrial stromal sarcoma

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