Uterus Sarcomas

There are a large number of gynaecological cancers with rare histologies, for which the available data are limited and usually retrospective. Because of their rarity and poor prognosis, the management of these cancers must be centralized in expert centres, for both histological diagnosis and treatment. With the exception of sarcomas, most endometrial or cervical cancers with rare histologies respond to the same radiation treatment modalities than cancers with more common histologies, although there are some specificities regarding treatments such as neuroendocrine carcinomas (chemotherapy with platinum and etoposide, major role of surgery)...

Diagnosis of soft tissue tumors is often challenging, given the large number of entities, often with non-specific or overlapping morphology. Although morphology still plays an important part in diagnostic process, additional studies including immunohistochemistry and molecular genetics are often needed to arrive at correct diagnosis. We report a case of 61-year-old male with subcutaneous tumor in right hip area, that was surgically removed. The tumor was composed of uniform bland spindle cells in mild to moderately cellular myxoid nodules, with limited areas of collagenization and the diagnosis of low grade fibromyxoid sarcoma was made...

During a year, myomas may undergo radical changes in their dimensions - from decreasing by 90% to growing by 200%. On average, myomas of the uterus increase in volume by 20-30% annually in the premenopausal period. On the other hand, myomas regress spontaneously in about 20% of women. After menopause uterine fibroids stabilize or regress. Every new or growing lesion of the uterus after menopause has to be diagnosed. There is no general definition of fast growing uterine myoma. The presence of fast growing uterine myoma, regardless of its definition, is associated with some clinical issues: it may become symptomatic (pain, bleeding, bulk symptoms), may be responsible for infertility, and a malignant process (leiomyosarcoma) may be present...

Uterine sarcomas are a group of rare malignant tumors of mesenchymal tissue of the uterus, and their diagnosis is often difficult because they have variable morphologies and no typical immunophenotype. This report describes a 48-year-old woman who underwent laparoscopic myomectomy and relapsed within 5 years with a large mass in the pelvic cavity. Morphologically, the tumor was composed of oval cells and small arteries, and the cells showed moderate to severe atypia. Immunohistochemical results showed that the tumor cells expressed desmin, smooth muscle actin, and h-caldesmon, which supported myogenic differentiation...

A 49-year-old female patient was referred to a tertiary care hospital with a history of postmenopausal bleeding and abdominal pain that had persisted for the last two months. An abdominal examination revealed a huge mass that had been present for 12 weeks. A fibroid uterus was suggested by USG. A biopsy was done and sent to histopathology which revealed myxoid leiomyoma. Subsequently, the patient underwent total abdominal hysterectomy without bilateral salpingo-oophorectomy. Histopathological examination confirmed the diagnosis of myxoid leiomyosarcoma (MLMS) of the uterus...

BACKGROUND: Differential diagnosis between uterine leiomyomas and sarcomas is challenging. Ultrasound shows an uncertain role in the clinical practice as pooled estimates about its diagnostic accuracy are lacking. OBJECTIVES: To assess the accuracy of ultrasound in the differential diagnosis between uterine leiomyomas and sarcomas. DATA SOURCES: A systematic review was performed searching 5 electronic databases (MEDLINE, Web of Sciences, Google Scholar, Scopus, and ClinicalTrial...

The marine environment is an excellent source of molecules that have a wide structural diversity and a variety of biological activities. Many marine natural products (MNPs) have been established as leads for anticancer drug discovery. Most of these compounds are alkaloids, including several chemical subclasses. In this review, we focus on the bis-indolyl alkaloid Nortopsentins and their derivatives with antiproliferative properties. Nortopsentins A-C were found to exhibit in vitro cytotoxicity against the P388 murine leukaemia cell line...

INTRODUCTION AND IMPORTANCE: A case of Low-grade endometrial stromal sarcoma (LG-ESS) invading the great vessels is rare. CASE PRESENTATION: A 34-year-old female who had no past history presented to a previous hospital with abdominal distension. Magnetic resonance imaging revealed a 15 cm pelvic mass beside the uterus, and only the pelvic mass was removed at the surgery. The tumor was judged to be a LG-ESS. The patient chose to be observed to preserve her fertility, and no adjuvant treatment was undertaken...

BACKGROUND: Differential diagnosis between uterine leiomyomas and sarcomas is challenging. Magnetic resonance imaging (MRI) represents the second-line diagnostic method after ultrasound for the assessment of uterine masses. OBJECTIVES: To assess the accuracy of MRI in the differential diagnosis between uterine leiomyomas and sarcomas. SEARCH STRATEGY: A systematic review and meta-analysis was performed searching five electronic databases from their inception to June 2023...

Uterine sarcomas are rare neoplasms of the uterus, some of which are associated with distinctive gene fusions. COL1A1::PDGFB fusion uterine sarcoma is a recently described entity that shares the same genetic alteration as dermatofibrosarcoma protuberans. These uterine sarcomas have a nonspecific spindle cell sarcoma appearance and are CD34 positive by immunohistochemistry. Accurate diagnosis relies on identification of the characteristic fusion by molecular genetic methods. The importance of diagnosing this entity lies in its potential response to targeted therapy with imatinib, a tyrosine kinase inhibitor successfully used in dermatofibrosarcoma protuberans, but only one prior case of COL1A1::PDGFB fusion uterine sarcoma treated with imatinib has been reported...

Tumor cells need to activate a telomere maintenance mechanism, enabling limitless replication. The bulk of evidence supports that sarcomas predominantly use alternative lengthening of telomeres (ALT) mechanism, commonly associated with alterations in ATRX and DAXX. In our dataset, only 12.3% of sarcomas harbored alterations in these genes. Thus, we checked for the presence of other genomic determinants of high telomeric content in sarcomas. Our dataset consisted of 13555 sarcoma samples, sequenced as a part of routine clinical care on the FoundationOne®Heme platform...

Vulvar leiomyomas are extremely rare smooth muscle tumors that are easily mistaken for other lesions, as the differential diagnosis must consider a wide spectrum of benign and malignant lesions. We present the case of a 52-year-old woman with a three-year history of progressive abdominal distension and pain and an enlarging vulvar mass distorting the labia majora and causing gait disturbance. Imaging confirmed an enormous pelvic mass originating in the uterus, compatible with a leiomyoma/sarcoma, and large perineal and vulvar masses with similar characteristics...

INTRODUCTION: Leiomyosarcoma is a malignant neoplasm that is derived from smooth muscle cells in walls of small blood vessels or branch of the inferior vena cava, the uterus and the gastrointestinal tract. Different treatment options are present for the treatment of LMS. However, due to the rarity of LMS, the optimal treatment option is still to be discussed and determined. PRESENTATION OF CASE: A 51-year-old male patient, previously healthy, presented for perianal pain...

Data on genetic and immunophenotypical characteristics of uterine mesonephric-like adenocarcinoma (MLA) remain limited. Therefore, we aimed to investigate the clinicopathological, immunohistochemical, and molecular features of uterine MLA. We performed targeted sequencing, array comparative genomic hybridization, and immunostaining in 17, 13, and 17 uterine MLA cases, respectively. Nine patients developed lung metastases. Eleven patients experienced disease recurrences. The most frequently mutated gene was Kirsten rat sarcoma viral oncogene homolog ( KRAS ; 13/17)...

The benign tumor uterine leiomyoma (UL) develops from the smooth muscle tissue that constitutes the uterus, whereas malignant tumor uterine sarcoma develops from either the smooth muscle tissue or stroma and is different from UL and endometrial cancer. Uterine sarcoma is broadly classified into three types: uterine leiomyosarcoma, endometrial stromal sarcoma (ESS), and carcinosarcoma. Although uterine leiomyosarcoma and ESS are both classified as uterine sarcoma, they significantly differ in terms of their sites of occurrence, symptoms, and treatment methods...

OBJECTIVE: Investigate the incidence of homologous recombination DNA damage response (HR-DDR) genomic alterations among patients with uterine sarcoma. METHODS: The American Association for Cancer Research GENIE v13.0 database was accessed and patients with uterine leiomyosarcoma, adenosarcoma, undifferentiated uterine sarcoma, high-grade endometrial stromal sarcoma, low-grade endometrial stromal sarcoma, and endometrial stromal sarcoma not otherwise specified were identified...

Leiomyosarcomas of the uterine cervix are rare, mostly occurring in perimenopausal women. Diagnosis is based on pathology and immunohistochemistry. Surgery with a total abdominal hysterectomy and bilateral salpingo-oophorectomy remains the standard. A female patient in her 60s presented with heavy postmenopausal bleeding. Vaginal ultrasound scan and magnetic resonance imaging showed a large strongly vascularized cervical mass with features suspicious of sarcomatous degeneration. Positron Emission Tomography-Computed Tomography (PET-CT) did not reveal any evidence of metastases nor lymphadenopathy, but presence of right hydronephrosis...

OBJECTIVE: Brain metastases (BMs) secondary to sarcoma are rare, and their incidence ranges from 1% to 8% of all bone and soft tissue sarcomas. Although stereotactic radiosurgery (SRS) is widely used for BMs, only a few papers have reported on SRS for sarcoma metastasizing to the brain. The purpose of this study was to evaluate the safety and effectiveness of SRS for sarcoma BM. METHODS: The authors retrospectively reviewed the clinical and radiological outcomes of patients with BM secondary to histopathologically confirmed sarcoma treated with SRS, either as primary treatment or as adjuvant therapy after surgery, at their institution between January 2005 and September 2022...

BACKGROUND: Uterine sarcomas are uncommon mesenchymal tumors of the uterus. The clinical problem is that the features of uterine sarcomas can sometimes mimic uterine fibroids. This study aims to investigate the clinical characteristics of patients with uterine sarcomas who were preoperative presenting mainly with uterine masses. METHODS: A retrospective analysis of patients who underwent gynecological surgery for uterine sarcomas at the Obstetrics & Gynecology Hospital of Fudan University, between January 2016 and December 2021...

OBJECTIVES: To analyze the impact of perioperative characteristics on the risk of recurrence in patients with uterine leiomyosarcomas. METHODS: A sub-analysis of the SARComa of the UTerus (SARCUT) study, which is a multicentric cross-sectional pan-European study that included 390 patients diagnosed with leiomyosarcoma, between 2001 and 2007. Perioperative factors related to risk of recurrence and survival were analyzed. RESULTS: The 5-year and 10-year disease-free survivals (DFS) were 46% and 55%, respectively...