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

2021 ◽  
Vol 11 (4) ◽  
pp. 878-900
Author(s):  
Stoyan Kostov ◽  
Yavor Kornovski ◽  
Vesela Ivanova ◽  
Deyan Dzhenkov ◽  
Dimitar Metodiev ◽  
...  
Keyword(s):  
Low Frequency ◽  
Molecular Classification ◽  
Malignant Neoplasms ◽  
Insufficient Data ◽  
Mesenchymal Tumors ◽  
Epithelial Tumor ◽  
Undifferentiated Sarcoma ◽  
Uterine Sarcomas ◽  
Uterine Corpus ◽  
Fertility Sparing

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.

scholarly journals New advances in the molecular classification of pediatric mesenchymal tumors

2018 ◽  
Vol 58 (2) ◽  
pp. 100-110 ◽  
Author(s):  
Albert J. H. Suurmeijer ◽  
Yu-Chien Kao ◽  
Cristina R. Antonescu
Keyword(s):  
Molecular Classification ◽  
Mesenchymal Tumors

Regression of chemotherapy-refractory metastatic tumor from CD117-negative prostate sarcoma with imatinib mesylate plus thalidomide

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 9557-9557 ◽  
Author(s):  
V. C. Kok ◽  
K. Yeh ◽  
W. Chang
Keyword(s):  
Pleural Effusion ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Imatinib Mesylate ◽  
Tumor Regression ◽  
Performance Status ◽  
Clinical Activity ◽  
Malignant Neoplasms ◽  
Dramatic Improvement ◽  
Undifferentiated Sarcoma

9557 Background: Chemoresistant relapsing prostate sarcoma has no standard treatment and the prognosis is extremely poor. Imatinib mesylate may target on tyrosine kinases of platelet-derived growth factor receptor (PDGFR) which has been shown to be important in tumor growth of certain types of soft tissue sarcoma. Thalidomide, itself a potent immunomodulatory agent has exhibited clinical activity in several malignant neoplasms which have been linked to abnormal angiogenesis and tumor vasculature. Case Report: A 38-year-old man who underwent radical cystoprostatectomy in June 2004 for a stage III high-grade soft tissue sarcoma of spindle and round cell type of prostate staged pT2b(8cm) pN0(0/26) M0, received adjuvant radiotherapy up to 6,660 cGy/37Fx postoperatively. Immunohistochemistry study showed vimentin+, CD34+, CD117−, desmin+, SMActind- and S-100−. Distant relapse in lungs was noted in April 2005. Shortly later massive pleural effusion developed. Three cycles of salvage chemotherapy with MAID were given. Tumor assessment disclosed Progressive Disease status and metastatic tumors were chemoresistant. Imatinib mesylate 400 mg once a day plus thalidomide with increment dose to 200 mg/day was chosen as biologic treatment for his devastating condition after an informed consent was obtained. PDGFR immunohistochemistry staining of the paraffin block and gene sequencing are planned. Results: Marked tumor regression reaching very good partial remission and resolution of pleural effusion without recurrence. Dramatic improvement in terms of performance status and cancer-associated symptoms are obvious. Except for skin changes and mild periorbital edema from imatinib, no other significant adverse reactions were noted. Postulated mechanism of action may suggest PDGFR tyrosine kinase inhibition as well as antiangiogenesis. Conclusions: We documented a case of undifferentiated sarcoma of prostate with pulmonary metastases which were refractory to MAID chemotherapy but subsequently regressed on a combination of double biologic agents, imatinib mesylate plus thalidomide. No significant financial relationships to disclose.

Malignant mesenchymal tumors of the uterine corpus

1974 ◽  
Vol 120 (4) ◽  
pp. 452-460 ◽  
Author(s):  
Eero Saksela ◽  
Väinö Lampinen ◽  
Berndt-Johan Procopé
Keyword(s):  
Mesenchymal Tumors ◽  
Uterine Corpus

scholarly journals Sonodynamic and Photodynamics Used as a Combined Therapy in the Treatment of Malignant Neoplasms: Facts and Open Questions

2020 ◽  
Author(s):  
Heber Lopes de Mello ◽  
Luiz Anastacio Alves ◽  
Evellyn Araujo Dias ◽  
Sabrina de Sá Pereira Magalhães ◽  
Vinicius Cotta-de-Almeida ◽  
...  
Keyword(s):  
Cell Death ◽  
Combined Therapy ◽  
Low Frequency ◽  
Malignant Neoplasms ◽  
Tumor Treatment ◽  
New Approach ◽  
Common Path ◽  
Open Questions ◽  
Low Frequency Ultrasound ◽  
Anti Tumor Activity

Photodynamic therapy (PDT) used in combination with sonodynamic therapy (SDT) is a new approach that aims to increase the effectiveness of tumor treatment when compared to the effect of each independent therapy. PDT is based on stimulating sensitizers with photons, while the most accepted theory for SDT is that sensitizers are stimulated by the sonoluminescence phenomenon. However, after the excitation of the sensitizer, both therapies follow a common path, leading to the generation of free radicals and inducing cell death. One of the positive aspects of this combination is the augmentation of anti-tumor activity with fewer side effects, since cell death may be induced using lower sensitizer concentrations or less exposure to ultrasound or light. Another benefit of combining PDT and SDT, especially with the use of low-frequency ultrasound is the induction of sonophoresis. For instance, on the skin, it may facilitate the absorption of the sensitizer. However, research involving both PDT and SDT exhibit many variants, including differences in irradiation sources and their intensities, among others. These aspects contribute to a lack of standardization, leading to result variations, hindering assessment on the real contribution that these combined therapies can offer in tumor treatment. Thus, further research in the pre-clinical and clinical areas are crucial.

scholarly journals Uterine Preservation Treatments in Sarcomas: Oncological Problems and Reproductive Results: A Systematic Review

Cancers ◽  
2021 ◽  
Vol 13 (22) ◽  
pp. 5808
Author(s):  
Giulia Dondi ◽  
Eleonora Porcu ◽  
Alessandra De Palma ◽  
Giuseppe Damiano ◽  
Eugenia De Crescenzo ◽  
...  
Keyword(s):  
Systematic Review ◽  
Early Stage ◽  
Uterine Sarcoma ◽  
Low Grade ◽  
Cochrane Central Register ◽  
Childbearing Age ◽  
Uterine Sarcomas ◽  
Oncological Outcomes ◽  
Uterine Tumors ◽  
Fertility Sparing

Uterine sarcomas are rare cancers, sometimes diagnosed in women of childbearing age. Hysterectomy is the standard treatment in early stages. The option of lesion removal to save fertility is described in the literature, but it is still considered experimental. The objective of this systematic review is to report on the available evidence on the reproductive and oncological outcomes of fertility-sparing treatment in women with uterine sarcomas. PubMed, Scopus and Cochrane Central Register of Controlled Trials were searched between 1 January 2011 and 21 June 2021 for publications in English about women with uterine sarcoma treated with a fertility-sparing intervention. Thirty-seven studies were included for a total of 210 patients: 63 low-grade endometrial stromal sarcomas, 35 embryonal rhabdomyosarcomas of the cervix, 19 adenosarcomas, 7 leiomyosarcomas and 2 uterine tumors resembling an ovarian sex cord. Conservative treatment ensured pregnancy in 32% of cases. In terms of oncological outcomes, relapse was related to histology and the worst prognosis was reported for leiomyosarcoma, followed by low-grade endometrial stromal sarcoma, which relapsed in 71% and 54% of cases, respectively. The highest death rate was associated with leiomyosarcoma (57.1%). This study demonstrated that fertility-sparing treatments may be employed in selected cases of early stage uterine sarcoma.

scholarly journals Uterine sarcoma: a clinical case and a literature review

2019 ◽  
Vol 25 (4) ◽  
pp. 206-218
Author(s):  
Diana Bužinskienė ◽  
Saulius Mikėnas ◽  
Gražina Drąsutienė ◽  
Matas Mongirdas
Keyword(s):  
Clinical Case ◽  
Uterine Sarcoma ◽  
Imaging Modalities ◽  
Histological Evaluation ◽  
Low Grade ◽  
Undifferentiated Sarcoma ◽  
Uterine Sarcomas ◽  
Uterine Tumour ◽  
Presenting Symptoms ◽  
Power Morcellation

Background. Uterine sarcomas are rare gynaecologic tumours representing 3–7% of all uterine malignancies. The aetiology of sarcomas is still unclear: it is thought, that chromosomal translocations have influence on wide histological variety of sarcomas. Presenting symptoms are vague and nonspecific. Usually sarcoma causes abnormal vaginal bleeding, can cause abdominal or pelvic pain, or manifests as a rapidly growing uterine tumour. The diagnosis of sarcoma is often made retrospectively after surgical removal of a presumed benign uterine neoplasm, because imaging modalities such as ultrasound, computed tomography, or magnetic resonance imaging cannot yet accurately and reliably distinguish between benign leiomyoma and malignant pathology. If there are certain clinical features that raise a suspicion of malignancy in the uterus, it is recommended to avoid the use of power morcellation through laparoscopic surgery in order to prevent disease dissemination. Materials and methods. We present a clinical case of a 64-year-old patient, who was referred to hospital due to abdominal pain and tenesmus that lasted for two days. From a past medical history it was known that previously the patient had been diagnosed with uterine myoma. Transvaginal ultrasonography showed a 10.4 cm × 9.8 cm uterine tumour of nonhomogeneous structure with signs of necrosis and good vascularization. The patient refused urgent hysterectomy, that was advised to her. The patient was operated on one month later and total hysterectomy with bilateral salpingooforectomy was performed. Postoperative histological evaluation showed undifferentiated sarcoma uterus pT1b L/V0. Imaging modalities were made to evaluate possible dissemination of the disease. In the absence of signs of disease progression, the patient received radiotherapy and brachytherapy and was followed-up by doctors. Results and conclusions. Uterine sarcomas are highly malignant tumours that originate from smooth muscles and connective tissue elements of the uterus and make up 1% of all malignant gynaecological tumours and about 3–7% of all malignant uterine tumours. Imaging modalities cannot yet reliably distinguish benign myomas from malignant sarcomas. It is important not to damage the wholeness of uterus during operation in order to prevent dissemination of the disease in the abdominal cavity. The low-grade endometrial stromal sarcoma has the best survival prognosis, while carcinosarcoma and undifferentiated uterine sarcoma have the lowest survival rates.

scholarly journals Immunophenotype of Pediatric NTRK fusion mesenchymal tumors

2020 ◽  
Author(s):  
Jeongwan Kang ◽  
Jin Woo Park ◽  
Jae-Kyung Won ◽  
Jeong Mo Bae ◽  
Jaemoon Koh ◽  
...  
Keyword(s):  
S100 Protein ◽  
Protein A ◽  
Mitotic Rate ◽  
University Hospital ◽  
Low Grade ◽  
Fusion Partner ◽  
Pediatric Tumors ◽  
Mesenchymal Tumors ◽  
Undifferentiated Sarcoma ◽  
Infantile Fibrosarcoma

Abstract Background: While ETV6-NTRK3 fusion is common in infantile fibrosarcoma, NTRK1/3 fusion in pediatric tumors is scarce and, consequently, not well known. Herein, we evaluated NTRK1/3 fusion pediatric mesenchymal tumors clinicopathologically and immunophenotypically. Methods: We reviewed nine NTRK -fusion pediatric sarcomas confirmed by fluorescence in situ hybridization and/or next-generation sequencing from Seoul National University Hospital between 2002 and 2020. Results: One case of TPR - NTRK1 fusion-positive intracranial extra-axial high-grade undifferentiated sarcoma (12-year-old boy), one case of LMNA-NTRK1 fusion-positive low-grade infantile fibrosarcoma of the forehead (3-year-old boy), one case of ETV6-NTRK3 fusion-positive inflammatory myofibroblastic tumor (IMT) (3-months-old girl), and six cases of ETV6-NTRK3 fusion-positive infantile fibrosarcoma (median age: 2.6 months, range: 1.6–5.6 months, M: F = 5:1) were reviewed. The Trk immunopositive pattern was distinctive according to the fusion genes. We notified nuclear positivity in TPR-NTRK1 fusion sarcoma, nuclear membrane positivity in LMNA-NTRK1 fusion sarcoma, and both cytoplasmic and nuclear positivity in ETV6-NTRK3 fusion-positive IMT and infantile fibrosarcomas. Also, the TPR-NTRK1 fusion sarcoma showed robust positivity for CD34/nestin and high mitoses. The LMNA-NTRK1 fusion sarcoma revealed CD34/S100 protein/nestin/CD10 coexpression, and a low mitotic rate. The IMT with ETV6-NTRK3 fusion expressed SMA. Six infantile fibrosarcomas with ETV6-NTRK3 fusion showed variable coexpression of nestin (6/6)/CD10 (4/5)/ S100 protein (3/6). Conclusions: All cases of NTRK1 and NTRK3 fusion-positive pediatric tumors robustly expressed the Trk protein. A Trk immunopositive pattern and CD34/S100/nestin/CD10/SMA immunohistochemical phenotype may suggest NTRK fusion partner genes and diagnoses. LMNA-NTRK1 fusion sarcoma might be a low-grade subtype of infantile fibrosarcoma. Interestingly, more than half of the infantile fibrosarcoma cases were positive for S100 protein and CD10. The follow-up period of TPR-NTRK1 and LMNA-NTRK1 fusion tumors are not enough to predict prognosis. However, ETV6-NTRK3 fusion infantile fibrosarcomas showed an excellent prognosis with no evidence of disease for an average of 11.7 years after gross total resection of the tumor.

scholarly journals Clinicopathological findings of pediatric NTRK fusion mesenchymal tumors

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Jeongwan Kang ◽  
Jin Woo Park ◽  
Jae-Kyung Won ◽  
Jeong Mo Bae ◽  
Jaemoon Koh ◽  
...  
Keyword(s):  
S100 Protein ◽  
Mitotic Rate ◽  
University Hospital ◽  
Low Grade ◽  
Fusion Partner ◽  
Pediatric Tumors ◽  
Mesenchymal Tumors ◽  
Undifferentiated Sarcoma ◽  
Infantile Fibrosarcoma ◽  
Clinicopathological Findings

Abstract Background While ETV6- NTRK3 fusion is common in infantile fibrosarcoma, NTRK1/3 fusion in pediatric tumors is scarce and, consequently, not well known. Herein, we evaluated for the presence of NTRK1/3 fusion in pediatric mesenchymal tumors, clinicopathologically and immunophenotypically. Methods We reviewed nine NTRK fusion-positive pediatric sarcomas confirmed by fluorescence in situ hybridization and/or next-generation sequencing from Seoul National University Hospital between 2002 and 2020. Results One case of TPR-NTRK1 fusion-positive intracranial, extra-axial, high-grade undifferentiated sarcoma (12-year-old boy), one case of LMNA-NTRK1 fusion-positive low-grade infantile fibrosarcoma of the forehead (3-year-old boy), one case of ETV6-NTRK3 fusion-positive inflammatory myofibroblastic tumor (IMT) (3-months-old girl), and six cases of ETV6-NTRK3 fusion-positive infantile fibrosarcoma (median age: 2.6 months, range: 1.6–5.6 months, M: F = 5:1) were reviewed. The Trk immunopositivity patterns were distinct, depending on what fusion genes were present. We observed nuclear positivity in TPR-NTRK1 fusion-positive sarcoma, nuclear membrane positivity in LMNA-NTRK1 fusion-positive sarcoma, and both cytoplasmic and nuclear positivity in ETV6-NTRK3 fusion-positive IMT and infantile fibrosarcomas. Also, the TPR-NTRK1 fusion-positive sarcoma showed robust positivity for CD34/nestin, and also showed high mitotic rate. The LMNA-NTRK1 fusion-positive sarcoma revealed CD34/S100 protein/nestin/CD10 coexpression, and a low mitotic rate. The IMT with ETV6-NTRK3 fusion expressed SMA. Six infantile fibrosarcomas with ETV6-NTRK3 fusion showed variable coexpression of nestin (6/6)/CD10 (4/5)/ S100 protein (3/6). Conclusions All cases of NTRK1 and NTRK3 fusion-positive pediatric tumors robustly expressed the Trk protein. A Trk immunopositive pattern and CD34/S100/nestin/CD10/SMA immunohistochemical expression may suggest the presence of NTRK fusion partner genes. LMNA-NTRK1 fusion sarcoma might be a low-grade subtype of infantile fibrosarcoma. Interestingly, more than half of the infantile fibrosarcoma cases were positive for S100 protein and CD10. The follow-up period of TPR-NTRK1 and LMNA-NTRK1 fusion-positive tumors are not enough to predict prognosis. However, ETV6-NTRK3 fusion-positive infantile fibrosarcomas showed an excellent prognosis with no evidence of disease for an average of 11.7 years, after gross total resection of the tumor.

Morphological and Molecular Classification of Human Cancer

2017 ◽  
Author(s):  
Mark E. Sherman ◽  
Melissa A. Troester ◽  
Katherine A. Hoadley ◽  
William F. Anderson
Keyword(s):  
Human Cancer ◽  
Molecular Classification ◽  
Tumor Stage ◽  
Cancer Surveillance ◽  
Molecular Profile ◽  
Malignant Neoplasms ◽  
Anatomic Site ◽  
Molecular Events ◽  
Classification Of Tumors

Accurate and reproducible classification of tumors is essential for clinical management, cancer surveillance, and studies of pathogenesis and etiology. Tumor classification has historically been based on the primary anatomic site or organ in which the tumor occurs and on its morphologic and histologic phenotype. While pathologic criteria are useful in predicting the average behavior of a group of tumors, histopathology alone cannot accurately predict the prognosis and treatment response of individual cancers. Traditional measures such as tumor stage and grade do not take into account molecular events that influence tumor aggressiveness or changes in the tumor composition during treatment. This chapter provides a primer on approaches that use pathology and molecular biology to classify and subclassify cancers. It describes the features of carcinomas, sarcomas, and malignant neoplasms of the immune system and blood, as well as various high-throughput genomic platforms that characterize the molecular profile of tumors.

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