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1
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Ramineni M, Armbuster YC, Katerji H, Huang W, McDowell JL, Wang X. A locally aggressive pelvic MEIS1::NCOA1 fusion sarcoma in a young adult female: a case report and review of the literature. Diagn Pathol 2025; 20:65. [PMID: 40420135 DOI: 10.1186/s13000-025-01656-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2025] [Accepted: 04/21/2025] [Indexed: 05/28/2025] Open
Abstract
MEIS1::NCOA1/2 fusions have been identified in spindle cell tumors of the gynecologic and genitourinary tracts, as well as in two cases of intraosseous spindle cell rhabdomyosarcomas. These tumors typically exhibit an infiltrative growth pattern characterized by short fascicles of monomorphic, plump spindle cells. Their immunoprofile is nonspecific, often demonstrating focal and variable expression of ER, PR, CD10, and cyclin D1. Depending on their location, these tumors are frequently diagnosed as low-grade endometrial stromal sarcomas or undifferentiated uterine or renal sarcomas. While they generally exhibit low malignant potential with multiple local recurrences, two cases with high-grade morphology and lung metastases have been reported. Here, we describe a case of pelvic low-grade spindle cell sarcoma in a 19-year-old woman characterized by strong diffuse ER/PR expression and focal CD10 positivity. Next-generation sequencing revealed a MEIS1::NCOA1 fusion without additional genetic alterations. She presented with extensive local disease throughout the abdomen, while the uterus and adnexa appeared normal intraoperatively.
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Affiliation(s)
- Madhurya Ramineni
- Department of Pathology, University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, NY, 14642, USA
| | - Youngeun C Armbuster
- Department of Obstetrics and Gynecology, University of Rochester Medical Center, Rochester, NY, USA
| | - Hani Katerji
- Department of Pathology, University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, NY, 14642, USA
| | - Wei Huang
- Valley Pathologists, Inc, Wyoming Street, Dayton, OH, USA
| | - Jamie L McDowell
- Division of Gynecologic Oncology, Department of Obstetrics and Gynecology, University of Rochester Medical Center, Rochester, NY, USA
| | - Xi Wang
- Department of Pathology, University of Rochester Medical Center, 601 Elmwood Avenue, Rochester, NY, 14642, USA.
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Żołyniak-Brzuchacz A, Barnaś E, Bartusik-Aebisher D, Aebisher D. The Use of Photodynamic Therapy in the Treatment of Endometrial Cancer-A Review of the Literature. Int J Mol Sci 2024; 25:8772. [PMID: 39201458 PMCID: PMC11354903 DOI: 10.3390/ijms25168772] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2024] [Revised: 08/03/2024] [Accepted: 08/10/2024] [Indexed: 09/02/2024] Open
Abstract
Endometrial cancer is the most common malignant tumor of the female reproductive system. It develops in the mucous membrane lining the inside of the uterine body-the endometrium, through the abnormal and continuous growth of cancer cells originating from the uterine mucosa. In recent years, there has been a significant increase in the number of cases in European countries. Photodynamic therapy (PDT) is an innovative and dynamically developing medical procedure, useful in the treatment of cancer and non-cancer tissue conditions. The PDT reaction involves the activation of a photosensitizing substance with visible light, which in turn leads to the formation of free oxygen radicals, which contribute to the destruction of the cell. PDT is minimally invasive, has few side effects, and preserves organ anatomy and function. Both diagnostics and photodynamic therapy as modern methods of treatment are becoming more and more popular in many research units around the world. They are most often practiced and tested in in vitro experimental conditions. In clinical practice, the use of PDT is rare. Comprehensive cooperation between scientists contributes to taking steps towards obtaining new, synthetic photosensitizers, directing their physicochemical properties, and showing the impact on a given organism. This review examines the evidence for the potential and usefulness of PDT in the treatment of endometrial cancer. This review highlights that PDT is gaining popularity and is becoming a promising field of medical research.
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Affiliation(s)
| | - Edyta Barnaś
- Department of Midwifery, Medical College of the University of Rzeszów, 35-310 Rzeszów, Poland;
| | - Dorota Bartusik-Aebisher
- Department of Biochemistry and General Chemistry, Medical College of the University of Rzeszów, 35-310 Rzeszów, Poland;
| | - David Aebisher
- Department of Photomedicine and Physical Chemistry, Medical College of the University of Rzeszów, 35-310 Rzeszów, Poland
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Dai Q, Xu B, Wu H, You Y, Li L. Prognosis of uterine and extrauterine low-grade endometrial stromal sarcoma: an observational cohort study. Int J Surg 2024; 110:1919-1928. [PMID: 38329091 PMCID: PMC11020013 DOI: 10.1097/js9.0000000000001146] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/21/2023] [Accepted: 01/25/2024] [Indexed: 02/09/2024]
Abstract
OBJECTIVE Little is known about the survival differences between uterine and extrauterine low-grade endometrial stromal sarcoma (LGESS). Survival outcomes, consisting of disease-free survivals and overall survivals (OS), were compared in these two entities. METHODS From February 2012 to June 2019, all primary LGESS cases and LGESS cases with first recurrence in the study center were reviewed. The clinicopathological characteristics and survival outcomes of extrauterine and uterine LGESS patients were compared for both primary and recurrent diseases. RESULTS During the study period, 143 patients with primary LGESS and 56 patients with recurrent LGESS were included and followed up to 1 June 2020, among whom 8 (5.6%) and 10 (17.8%) patients were identified as having extrauterine LGESS. Patients with primary and recurrent extrauterine LGESS had similar clinicopathological characteristics to those of patients with uterine LGESS. In primary or in recurrent LGESS cases, in univariate analysis, patients with uterine and extrauterine LGESS had similar disease-free intervals after the last treatment, and they also had similar OSs after the diagnosis. Ovarian preservation led to significantly increased recurrence for primary LGESS [hazard ratio (HR) 4.9, 95% CI: 2.3-10.1, P <0.001) and repeated recurrence for recurrent LGESS (HR 3.1, 95% CI: 1.3-7.3, P =0.009). Surgical treatment for recurrent LGESS decreased repeated recurrence after the first recurrence (HR 0.2, 95% CI: 0.1-0.7, P =0.006). No factors were found to be associated with the OS of primary or recurrent LGESS. CONCLUSION The clinical characteristics and survival outcomes of extrauterine LGESS are similar to those of uterine LGESS. Surgery is the treatment of choice for recurrent LGESS. Ovarian preservation is detrimental to disease-free survival but not to OS in both uterine and extrauterine LGESS.
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Affiliation(s)
| | - Baolin Xu
- Department of Obstetrics and Gynecology
- Department of Obstetrics and Gynecology, the Second People’s Hospital of Jingdezhen, Jingdezhen, People’s Republic of China
| | - Huanwen Wu
- Department of Pathology, Peking Union Medical College Hospital
| | - Yan You
- Department of Pathology, Peking Union Medical College Hospital
| | - Lei Li
- Department of Obstetrics and Gynecology
- State Key Laboratory for Complex, Severe and Rare Diseases
- National Clinical Research Center for Obstetric and Gynecologic Diseases, Beijing
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4
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Etrusco A, Agrifoglio V, Laganà AS, Gliozheni E, Caringella A, Stanziano A, Cicinelli E, Chiantera V, Giannini A, Alsannan B, Barra F, D’Amato A. Reproductive and oncologic outcomes in young women with uterine sarcoma undergoing fertility-sparing treatment: a systematic review. Ther Adv Reprod Health 2024; 18:26334941241271563. [PMID: 39351375 PMCID: PMC11440568 DOI: 10.1177/26334941241271563] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2024] [Accepted: 07/04/2024] [Indexed: 10/04/2024] Open
Abstract
Background Uterine sarcomas (US) are rare cancer of possible occurrence even in women of childbearing age. To date, total hysterectomy is the standard treatment in the early stages. The possibilities of carrying out fertility-sparing treatments (FST) to save the fertility of women with unfulfilled reproductive desires are described in the literature, but to date, they can only be considered experimental. Objective The aim of this systematic review was to evaluate the oncological and reproductive outcomes of women with different histological types of US undergoing FST. Design Systematic review. Data sources and methods Electronic databases were searched for English-language studies describing FST for US until January 31, 2024. Results Forty-five papers which met the abovementioned inclusion criteria, were included in the qualitative analysis. Quantitative analysis was not possible because of the heterogeneity of the data. A descriptive summary of the results according to the histotype of US was provided. Six hundred forty-one patients of childbearing age with US and undergoing FST. After treatment with FST, 89 (13.9%) disease recurrences and 107 (16.7%) pregnancies were recorded. Conclusion In selected cases of early-stage US, FST may be proposed. However, the patient must be informed of the real possibility of recurrence and potentially difficult achievement of pregnancy. Additional well-designed prospective studies and clinical trials are needed to address the knowledge gaps and enhance clinical decision-making in this population. Trial registration PROSPERO ID: CRD42024509356.
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Affiliation(s)
- Andrea Etrusco
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Piazza Marina, 61, Palermo 90133, Italy
- Unit of Obstetrics and Gynecology, “Paolo Giaccone” Hospital, Palermo, Italy
| | - Vittorio Agrifoglio
- Unit of Obstetrics and Gynecology, “Paolo Giaccone” Hospital, Palermo, Italy
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy
| | - Antonio Simone Laganà
- Unit of Obstetrics and Gynecology, “Paolo Giaccone” Hospital, Palermo, Italy
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy
| | - Elko Gliozheni
- Section of Obstetrics and Gynecology, Department of Medicine and Surgery, University of Perugia, Perugia, Italy
- Department of Obstetrics and Gynecology, University of Medicine of Tirana, Tirana, Albania
| | - Annamaria Caringella
- Department of Advanced Reproductive Risk Management and High-Risk Pregnancies, ASL Bari, Reproductive and IVF Unit, PTA Conversano, Conversano, Italy
| | - Antonio Stanziano
- Department of Advanced Reproductive Risk Management and High-Risk Pregnancies, ASL Bari, Reproductive and IVF Unit, PTA Conversano, Conversano, Italy
| | - Ettore Cicinelli
- Unit of Obstetrics and Gynecology, Department of Interdisciplinary Medicine, University of Bari, Bari, Italy
| | - Vito Chiantera
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy
- Unit of Gynecologic Oncology, National Cancer Institute—IRCCS—Fondazione “G. Pascale,” Naples, Italy
| | - Andrea Giannini
- Unit of Gynecology, Department of Surgical and Medical Sciences and Translational Medicine, Sant’Andrea Hospital, Sapienza University of Rome, Rome, Italy
| | - Baydaa Alsannan
- Department of Obstetrics and Gynecology, College of Medicine, Kuwait University, Safat, Kuwait
| | - Fabio Barra
- Unit of Obstetrics and Gynecology, P.O. “Ospedale del Tigullio”—ASL4, Chiavari, Genoa, Italy
- Department of Health Sciences (DISSAL), University of Genoa, Genoa, Italy
| | - Antonio D’Amato
- Unit of Obstetrics and Gynecology, Department of Interdisciplinary Medicine, University of Bari, Bari, Italy
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Lebreton C, Meeus P, Genestie C, Croce S, Guyon F, Moscardo CL, Taieb S, Blay JY, Bonvalot S, Bompas E, Chevreau C, Lécuru F, Rossi L, Joly F, Rios M, Chaigneau L, Duffaud F, Pautier P, Ray-Coquard I. Sarcomes du stroma endométrial de bas grade : référentiels de prise en charge du GSF-GETO/NETSARC+ et du groupe TMRG. Bull Cancer 2023:S0007-4551(23)00141-8. [PMID: 36990895 DOI: 10.1016/j.bulcan.2023.03.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/11/2022] [Revised: 02/08/2023] [Accepted: 03/05/2023] [Indexed: 03/29/2023]
Abstract
Low-grade endometrial stromal sarcoma (LG-ESS) accounts for approximately 15% of all uterine sarcomas. Median age of patients is around 50 years and half of the patients are premenopausal. In all, 60% of cases present with FIGO stage I disease. Preoperatively radiologic findings of ESS are not specific. Pathological diagnosis remains essential. This review aimed to present the French guidelines for low grade ESS treatment within the Groupe sarcome français - Groupe d'étude des tumeurs osseuse (GSF-GETO)/NETSARC+ and tumeur maligne rare gynécologique (TMRG) networks. Treatments should be validated in multidisciplinary team involved in sarcomas or rare gynecologic tumors. Hysterectomy is the cornerstone of treatment for localized ESS, and morcellation should be avoided. Systematic lymphadenectomy in ESS does not improve the outcome and is not recommended. Leaving the ovaries in situ in stage I tumors could be discussed for young women. Adjuvant hormonal treatment could be considered, for two years for stage I with morcellation or stage II and livelong for stages III or IV. Nevertheless, several questions remain, such as optimal doses, regimens (progestins or aromatase inhibitors) and duration of therapy. Tamoxifen is contraindicated. Secondary cytoreductive surgery if feasible for recurrent disease, appears to be an acceptable approach. Systemic treatment for recurrent or metastatic disease is mainly hormonal, with or without surgery.
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Affiliation(s)
- Coriolan Lebreton
- Institut Bergonié, département d'oncologie médicale, 33000 Bordeaux, France; Centre Léon-Bérard, département d'oncologie médicale, 69008 Lyon, France.
| | - Pierre Meeus
- Centre Léon-Bérard, département de chirurgie, 69008 Lyon, France
| | - Catherine Genestie
- Gustave Roussy Cancer Campus, service de biopathologie, 94805 Villejuif, France
| | - Sabrina Croce
- Institut Bergonié, département de biopathologie, 33076 Bordeaux, France
| | - Frédéric Guyon
- Institut Bergonié, département de chirurgie, 33000 Bordeaux, France
| | - Carmen Llacer Moscardo
- Institut du cancer de Montpellier (ICM), département de radiothérapie oncologique, 208, avenue des Apothicaires, parc euromédecine, 34298 Montpellier cedex 5, France
| | - Sophie Taieb
- Centre Oscar Lambret, département de radiologie, 59000 Lille, France
| | - Jean-Yves Blay
- Centre Léon-Bérard, département d'oncologie médicale, 69008 Lyon, France; Université Claude Bernard Lyon 1, health services and performance research lab (EA 7425 HESPER), 69008 Lyon, France
| | - Sylvie Bonvalot
- Institut Curie, département de chirurgie oncologique, 75005 Paris, France
| | | | | | - Fabrice Lécuru
- Institut Curie, département de chirurgie oncologique, 75005 Paris, France
| | - Léa Rossi
- Centre Léon-Bérard, département de chirurgie, 69008 Lyon, France
| | - Florence Joly
- U1086 Anticipe, université Unicaen, Normandie, département oncologie médicale CLCC François Baclesse, Caen, France
| | - Maria Rios
- Institut de cancérologie de Lorraine Alexis Vautrin, département oncologie médicale, 54519 Vandœuvre-lès-Nancy, France
| | | | - Florence Duffaud
- AP-HM, hôpitaux universitaires de Marseille Timone, département d'oncologie médicale, 13005 Marseille, France
| | - Patricia Pautier
- Saclay université, Institut Gustave-Roussy, Cancer Campus, département de médecine, Villejuif, France
| | - Isabelle Ray-Coquard
- Centre Léon-Bérard, département d'oncologie médicale, 69008 Lyon, France; Université Claude Bernard Lyon 1, health services and performance research lab (EA 7425 HESPER), 69008 Lyon, France
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Piątek S, Szymusik I, Dańska-Bidzińska A, Ołtarzewski M, Trojan G, Bidziński M. Fertility-Sparing Management May Be Considered in Young Women with Uterine Sarcoma. J Clin Med 2022; 11:jcm11164761. [PMID: 36012998 PMCID: PMC9410102 DOI: 10.3390/jcm11164761] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/21/2022] [Revised: 08/11/2022] [Accepted: 08/12/2022] [Indexed: 11/16/2022] Open
Abstract
Uterine sarcomas occur very rarely in young women. Hysterectomy, which is a standard treatment, may not be acceptable for those patients, especially nulliparous women. Fertility-sparing management may be an alternative. The aim of the study was to assess fertility-sparing management in patients with uterine sarcoma. Eleven patients were eligible for the study. Histopathologic types of the tumor included: adenosarcoma (n = 3), low-grade endometrial stromal sarcoma (n = 3), low-grade myofibroblastic sarcoma (n = 1), leiomyosarcoma (n = 1), leiomyosarcoma myxoides (n = 1), rhabdomyosarcoma (n = 1), high grade endometrial stromal sarcoma (n = 1). The mean age of the patients at the time of diagnosis was 27.4 years (range: 17–35) and the average follow-up 61 months (range: 12–158). Six patients received adjuvant treatment: megestrol (n = 5) and chemotherapy (n = 1). Recurrence was diagnosed in five cases. Median time to recurrence was 35 months (range: 8–90). Three patients conceived spontaneously following treatment and gave at least one live birth. In total, five full-term pregnancies were recorded and five healthy children were born. Fertility-sparing management may be considered in some patients with uterine sarcoma; however, it may not be appropriate in high-grade endometrial stromal sarcoma. Patients with adenosarcoma may have a low chance of childbearing.
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Affiliation(s)
- Szymon Piątek
- Department of Gynecologic Oncology, the Maria Sklodowska-Curie National Research Institute of Oncology, 5 Roentgen Street, 02-781 Warsaw, Poland
- Correspondence:
| | - Iwona Szymusik
- 1st Department of Obstetrics and Gynecology, Medical University of Warsaw, 02-015 Warsaw, Poland
| | - Anna Dańska-Bidzińska
- 2nd Department of Obstetrics and Gynecology, Medical University of Warsaw, 02-091 Warsaw, Poland
| | | | - Gabriela Trojan
- Students’ Scientific Group, Kazimierz Pulaski University of Technology and Humanities in Radom, 26-600 Radom, Poland
| | - Mariusz Bidziński
- Department of Gynecologic Oncology, the Maria Sklodowska-Curie National Research Institute of Oncology, 5 Roentgen Street, 02-781 Warsaw, Poland
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Applications of Photodynamic Therapy in Endometrial Diseases. Bioengineering (Basel) 2022; 9:bioengineering9050226. [PMID: 35621504 PMCID: PMC9138084 DOI: 10.3390/bioengineering9050226] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2022] [Revised: 05/13/2022] [Accepted: 05/17/2022] [Indexed: 11/16/2022] Open
Abstract
Photodynamic therapy (PDT) is a medical procedure useful for several benign conditions (such as wound healing and infections) and cancer. PDT is minimally invasive, presents few side effects, good scaring, and is able to minimal tissue destruction maintaining organ anatomy and function. Endoscopic access to the uterus puts PDT in the spotlight for endometrial disease treatment. This work systematically reviews the current evidence of PDT’s potential and usefulness in endometrial diseases. Thus, this narrative review focused on PDT applications for endometrial disease, including reports regarding in vitro, ex vivo, animal, and clinical studies. Cell lines and primary samples were used as in vitro models of cancer, adenomyosis and endometrioses, while most animal studies focused the PDT outcomes on endometrial ablation. A few clinical attempts are known using PDT for endometrial ablation and cancer lesions. This review emphasises PDT as a promising field of research. This therapeutic approach has the potential to become an effective conservative treatment method for endometrial benign and malignant lesions. Further investigations with improved photosensitisers are highly expected.
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8
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Uterine Preservation Treatments in Sarcomas: Oncological Problems and Reproductive Results: A Systematic Review. Cancers (Basel) 2021; 13:cancers13225808. [PMID: 34830960 PMCID: PMC8616470 DOI: 10.3390/cancers13225808] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/27/2021] [Revised: 11/09/2021] [Accepted: 11/17/2021] [Indexed: 12/29/2022] Open
Abstract
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.
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Dai Q, Xu B, Wu H, You Y, Wu M, Li L. The prognosis of recurrent low-grade endometrial stromal sarcoma: a retrospective cohort study. Orphanet J Rare Dis 2021; 16:160. [PMID: 33827628 PMCID: PMC8028754 DOI: 10.1186/s13023-021-01802-8] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2020] [Accepted: 03/30/2021] [Indexed: 12/04/2022] Open
Abstract
Background The prognosis of recurrent low-grade endometrial stromal sarcoma (LGESS) is little known. This study was to investigate the survival outcomes of a cohort of patients with recurrent LGESS. Methods Patients with primary LGESS diagnosed and treated for first recurrence confirmed by histology in the study center from February 2012 to June 2019 were retrospectively included. The progression-free interval (PFI) after the last treatment for first recurrence and overall survival (OS) since the diagnosis of first recurrence, which were followed up to June 1, 2020, were compared between groups of various therapy modalities. Results Fifty-six patients were included, and 43 patients (76.8%) had definite follow-up outcomes. The 5-year PFI and OS rates were 30.0% (95% confidence interval [95% CI] 29.2–30.8) and 75.0% (68.0–82.0), respectively. In univariate analysis, only fertility-sparing treatment, ovarian preservation and surgical treatment had a significant impact on the PFI (hazard ratio [HR] 4.5, 3.1, and 0.2; 95% CI 1.5–13.1, 1.3–7.3, and 0.1–0.7; and p = 0.006, 0.009 and 0.006, respectively), but no factor was found to be associated with increased mortality risk. After adjusted with hormone treatment or chemotherapy, surgical treatment had significant effectiveness on OS (HR 0.3 and 0.3, 95% CI 0.1–1.0 and 0.1–1.0, p = 0.045 and 0.049, respectively). None of the patients with fertility-sparing treatment had successful conception, and all experienced repeated relapse. Conclusion For patients with recurrent LGESS, fertility-sparing treatment or ovarian preservation should not be provided. Surgery is the treatment of choice, and hormone treatment and/or chemotherapy was effective for the survival benefits of surgical treatment. Supplementary Information The online version contains supplementary material available at 10.1186/s13023-021-01802-8.
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Affiliation(s)
- Qianwen Dai
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Shuaifuyuan No. 1, Dongcheng District, Beijing, 100730, China
| | - Baolin Xu
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Shuaifuyuan No. 1, Dongcheng District, Beijing, 100730, China.,Department of Obstetrics and Gynecology, the Second People's Hospital of Jingdezhen, Jingdezhen, 333099, China
| | - Huanwen Wu
- Department of Pathology, Peking Union Medical College Hospital, Beijing, 100730, China
| | - Yan You
- Department of Pathology, Peking Union Medical College Hospital, Beijing, 100730, China
| | - Ming Wu
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Shuaifuyuan No. 1, Dongcheng District, Beijing, 100730, China.
| | - Lei Li
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Shuaifuyuan No. 1, Dongcheng District, Beijing, 100730, China.
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10
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Gu YZ, Duan NY, Cheng HX, Xu LQ, Meng JL. Fertility-sparing surgeries without adjuvant therapy through term pregnancies in a patient with low-grade endometrial stromal sarcoma: A case report. World J Clin Cases 2021; 9:983-991. [PMID: 33585648 PMCID: PMC7852628 DOI: 10.12998/wjcc.v9.i4.983] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/10/2020] [Revised: 11/30/2020] [Accepted: 12/10/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Low-grade endometrial stromal sarcoma (LGESS) is a rare indolent tumor with a favorable prognosis. With the importance of improving quality of life recognized, fertility-sparing surgery may be an option for those young women. However, most of the reports suggested that stage IA patients might be candidates for fertility-sparing surgery, and adjuvant hormonal treatment was considered a feasible adjuvant therapy for reducing the recurrence risk of patients with LGESS and hysterectomy was recommended after the completion of pregnancy and delivery.
CASE SUMMARY A 28-year-old pregnant woman diagnosed with stage IB LGESS was treated by fertility-sparing surgery when term cesarean section delivery was performed. Without any adjuvant treatment, she had the other successful term pregnancy and cesarean section 45 mo after first fertility-sparing surgery. Moreover, only hysteroscopic resection was performed to retain fertility again even when the tumor recurred after 6 years. So far the patient’s fertility and disease-free status have remained for more than 8 years without any adjuvant therapy despite local resection of the sarcoma. And the two babies were in good health.
CONCLUSION For young patients with stage I LGESS, it seems that repeated fertility-sparing surgeries could be performed even after two term deliveries and the tumor recurrence, and it might be attempted without adjuvant therapy but the counseling should be considered as mandatory.
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Affiliation(s)
- Yong-Zhong Gu
- Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
- Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong University, Jinan 250021, Shandong Province, China
| | - Ning-Ya Duan
- Department of Obstetrics and Gynecology, Union Hospital Affiliated to Tongji Medical College of Huazhong University of Science and Technology, Wuhan 430022, Hubei Province, China
| | - Hong-Xia Cheng
- Department of Pathology, Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
| | - Lian-Qiong Xu
- Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong University, Jinan 250021, Shandong Province, China
| | - Jin-Lai Meng
- Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong First Medical University, Jinan 250021, Shandong Province, China
- Department of Obstetrics and Gynecology, Provincial Hospital Affiliated to Shandong University, Jinan 250021, Shandong Province, China
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Zheng Y, Yin Q, Yang X, Dong R. Fertility-sparing management of low-grade endometrial stromal sarcoma: analysis of an institutional series, a population-based analysis and review of the literature. ANNALS OF TRANSLATIONAL MEDICINE 2020; 8:1358. [PMID: 33313103 PMCID: PMC7723593 DOI: 10.21037/atm-20-2180] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Background Low-grade endometrial stromal sarcoma (LGESS) is the second most common malignant mesenchymal tumor of the uterus which usually affects young women. However, the researches on the safety and feasibility of the fertility-sparing management of it are limited. Methods A retrospective analysis was performed including 5 women diagnosed with LGESS treated with fertility-sparing management at Qilu Hospital of Shandong University from 2010 to 2019. Besides that, 1,070 patients diagnosed with LGESS in SEER database from 1973 to 2016 were examined. By using the Kaplan-Meier method, survival curves were estimated, and comparisons of statistical significance were performed with the stratified log-rank test within each group. Results Five patients with LGESS were enrolled in this study. All patients were submitted to fertility-sparing surgeries, after surgery, they all continued hormonal therapy for one year. Four out of the 5 patients recurred, to be more exact, 3 of them recurred in uterus and the other one in the uterus and iliac vascular region. They all suffered further surgery and all 5 patients were alive at the time of last contact. Besides, among these patients, two conceived naturally and delivered a healthy baby by cesarean section. Among 1,070 patients in SEER database, only 28 (2.6%) patients underwent local tumor excision, including excisional biopsy (39%), myomectomy (25%), laser ablation or excision (4%) and polypectomy (4%). There was no statistical significance was observed among TH±BSO, radical hysterectomy, subtotal hysterectomy and local tumor excision (P=0.29). Conclusions Our analysis indicated that for those young LGESS patients who wish to preserve their fertility, the feasibility and safety of fertility-sparing management should be considered after gynecological oncologist and gynecological pathologist making professional decisions.
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Affiliation(s)
- Yawen Zheng
- Department of Obstetrics and Gynecology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Qihui Yin
- Department of Obstetrics and Gynecology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Xingsheng Yang
- Department of Obstetrics and Gynecology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Ruiying Dong
- Department of Obstetrics and Gynecology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
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Zang Y, Dong M, Zhang K, Gao C, Guo F, Wang Y, Xue F. Hormonal therapy in uterine sarcomas. Cancer Med 2019; 8:1339-1349. [PMID: 30897294 PMCID: PMC6488133 DOI: 10.1002/cam4.2044] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/16/2018] [Revised: 01/10/2019] [Accepted: 02/04/2019] [Indexed: 12/20/2022] Open
Abstract
Uterine sarcomas (USs) are a group of rare but aggressive uterine malignancies, accounting for only 1% of the malignant tumors of female reproductive organs. Due to the high rate of recurrence and metastasis, the prognosis of USs is poor. Given the high mortality rate and limited clinical benefit of surgery and adjuvant chemoradiotherapy, hormonal therapy has shown good prospects in recent years. Hormonal agents include progestins, aromatase inhibitors (AIs), and gonadotropin‐releasing hormone analogue (GnRH‐a). According to the literature, hormonal therapy has been confirmed effective for recurrent, metastatic or unresectable low‐grade endometrial stromal sarcoma (LGESS) and hormone receptor positive (ER+/PR+) uterine leiomyosarcoma (uLMS) with favorable tolerance and compliance. Besides, hormonal therapy can also be used in patients with early‐staged disease who desire to preserve fertility. However, due to the rarity of USs, the rationale of hormonal therapy is generally extrapolated from data of hormone‐sensitive breast cancer, and present studies of hormonal therapy in USs were almost limited to case reports and small‐sized retrospective studies. Therefore, further systematic researches and standardized clinical trials are needed to establish the optimal hormonal therapy regimen of USs. Herein, we reviewed the existing studies related to the hormonal therapy in USs in order to provide reference for clinical management in specific settings.
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Affiliation(s)
- Yuqin Zang
- Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin, China
| | - Mengting Dong
- Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin, China
| | - Kai Zhang
- Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin, China
| | - Chao Gao
- Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin, China
| | - Fei Guo
- Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin, China
| | - Yingmei Wang
- Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin, China
| | - Fengxia Xue
- Department of Gynecology and Obstetrics, Tianjin Medical University General Hospital, Tianjin, China
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Clinical application of photodynamic diagnosis and photodynamic therapy for gynecologic malignant diseases: A review. Photodiagnosis Photodyn Ther 2018; 24:52-57. [DOI: 10.1016/j.pdpdt.2018.08.014] [Citation(s) in RCA: 34] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/23/2018] [Revised: 08/25/2018] [Accepted: 08/27/2018] [Indexed: 12/22/2022]
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Fertility-sparing surgery for patients with low-grade endometrial stromal sarcoma. Oncotarget 2018; 8:10602-10608. [PMID: 27736798 PMCID: PMC5354684 DOI: 10.18632/oncotarget.12491] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2016] [Accepted: 09/29/2016] [Indexed: 02/06/2023] Open
Abstract
Purpose To assess the clinical outcomes and fertility of young women with stage I low-grade endometrial stromal sarcoma (ESS) treated with fertility-sparing surgery. Results Seventeen patients with stage I low-grade ESS (stage IA, n = 6; stage IB, n = 11) were entered into this study. Adjuvant hormone therapy was administered to 15 (88.2%) patients. At a median follow-up of 39 months (range, 4106 months), 10 (58.8%) patients developed recurrence. All 10 patients had stage IB disease; among them, the first recurrence limited to the uterus was observed in 6 patients. All 17 patients were alive and disease-free at the time of last contact. After treatment, five of eight (62.5%) patients who attempted pregnancy conceived. No offspring had congenital anomalies. Methods Patients with stage I low-grade ESS who underwent fertility-sparing surgery between April 2001 and November 2015 were retrospectively reviewed. Conclusions Fertility-sparing surgery may be considered for young patients with stage IA low-grade ESS who wish to preserve their fertility.
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Laurelli G, Falcone F, Scaffa C, Messalli EM, Del Giudice M, Losito S, Greggi S. Fertility-sparing management of low-grade endometrial stromal sarcoma: analysis of an institutional series and review of the literature. Eur J Obstet Gynecol Reprod Biol 2015; 195:61-66. [PMID: 26476800 DOI: 10.1016/j.ejogrb.2015.09.041] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2015] [Revised: 09/20/2015] [Accepted: 09/28/2015] [Indexed: 12/21/2022]
Abstract
OBJECTIVE Low-grade endometrial stromal sarcoma (LG-ESS) is a rare malignancy, often occurring before menopause. There is no consensus regarding its optimal management. Total hysterectomy and bilateral salpingo-oophorectomy precludes future fertility and may thus be undesirable by women wishing to maintain their reproductive potential. However, experience of fertility-sparing management in LG-ESS is very limited. In this paper, the disease outcome is presented in six young women with LG-ESS conservatively treated by combined hysteroscopic resection and hormonal therapy. STUDY DESIGN From October 2009 to February 2013, at the Gynecologic Oncology Department of the National Cancer Institute of Naples, six women, with early-stage LG-ESS aged 18-40 years who desired childbearing and/or retaining their fertility, were enrolled into a pilot study of fertility-sparing management. Diagnosis of LG-ESS was made on specimens from hysteroscopic resection performed on a presumed benign lesion. All patients were planned to be treated with adjuvant megestrol acetate for two years. Hormonal therapy was started within 6 weeks from the hysteroscopic resection, with orally megestrol acetate at 40mg daily, increasing gradually according to patient's tolerance to the recommended total dose of 160mg daily. RESULTS All patients were submitted to hysteroscopic resection in a one-step procedure. Five patients started megestrol acetate within 6 weeks from the hysteroscopic resection (one patient did not start hormonal therapy because of early pregnancy after the hysteroscopic resection). Hormonal therapy was well tolerated; one patient stopped megestrol acetate after 12 months because of self-supporting strong desire to conceive; the other four patients regularly completed the hormonal therapy. To date, all patients show no evidence of disease. CONCLUSIONS Although fertility-sparing management is not the current standard of care for young women with early-stage LG-ESS, our preliminary data are promising. Larger series with a longer follow-up are needed to further assess safety and efficacy of combined hysteroscopic resection and hormonal therapy.
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Affiliation(s)
- Giuseppe Laurelli
- Gynecologic Oncology Surgery, Istituto Nazionale Tumori "Fondazione G. Pascale" IRCCS, Naples, Italy
| | - Francesca Falcone
- Department of Woman, Child, and General and Specialized Surgery, Seconda Università degli Studi di Napoli, Naples, Italy
| | - Cono Scaffa
- Gynecologic Oncology Surgery, Istituto Nazionale Tumori "Fondazione G. Pascale" IRCCS, Naples, Italy
| | - Enrico M Messalli
- Department of Woman, Child, and General and Specialized Surgery, Seconda Università degli Studi di Napoli, Naples, Italy
| | - Maurizio Del Giudice
- Anesthesiology and Intensive Care, Istituto Nazionale Tumori "Fondazione G. Pascale" IRCCS, Naples, Italy
| | - Simona Losito
- Surgical Pathology, Istituto Nazionale Tumori "Fondazione G. Pascale" IRCCS, Naples, Italy
| | - Stefano Greggi
- Gynecologic Oncology Surgery, Istituto Nazionale Tumori "Fondazione G. Pascale" IRCCS, Naples, Italy.
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Endometrial Stromal Nodule: A Rarity and a Pathological Challenge. Case Rep Obstet Gynecol 2015. [PMID: 26221551 PMCID: PMC4499397 DOI: 10.1155/2015/376817] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
Endometrial stromal tumors are rare, and endometrial stromal nodule is the least common. In the region of Middle Jutland, Denmark, only two cases are reported since 1995. The nodules are benign; nevertheless, hysterectomy is the treatment of choice. Tumor margins are required for diagnosis and essential to differentiate it from an invasive stromal sarcoma whose prognosis is very different. We report a rare case of a 38-year-old woman, with presurgical diagnosis of a uterine tumor/polyp. She presented with nausea and changes in bleeding pattern and initially had a transcervical polyp resection performed. Histopathological examination showed the presence of an endometrial stromal tumor with unclear margins, and an invasive malignant endometrial sarcoma could not be excluded. Pathological examination revealed an endometrial stromal nodule with invasion, not exceeding three mm. Endometrial stromal tumors are interesting due to their rare existence and difficulties in establishing a histological diagnosis. Although endometrial stromal nodules are benign entities, they must be differentiated from the other invasive malignant stromal tumors, which may change the final prognosis. No preoperative diagnostic tools are at hand, and benign as well as malignant tumors are treated with hysterectomy.
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Ali RH, Rouzbahman M. Endometrial stromal tumours revisited: an update based on the 2014 WHO classification. J Clin Pathol 2015; 68:325-32. [PMID: 25595274 DOI: 10.1136/jclinpath-2014-202829] [Citation(s) in RCA: 79] [Impact Index Per Article: 7.9] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2014] [Accepted: 12/20/2014] [Indexed: 12/26/2022]
Abstract
Endometrial stromal tumours (EST) are rare tumours of endometrial stromal origin that account for less than 2% of all uterine tumours. Recent cytogenetic and molecular advances in this area have improved our understanding of ESTs and helped refine their classification into more meaningful categories. Accordingly, the newly released 2014 WHO classification system recognises four categories: endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LGESS), high-grade endometrial stromal sarcoma (HGESS) and undifferentiated uterine sarcoma (UUS). At the molecular level, these tumours may demonstrate a relatively simple karyotype with a defining chromosomal rearrangement (as in the majority of ESNs, LGESSs and YWHAE-rearranged HGESS) or demonstrate complex cytogenetic aberrations lacking specific rearrangements (as in UUSs). Herein we provide an update on this topic aimed at the practicing pathologist.
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Affiliation(s)
- Rola H Ali
- Department of Clinical Pathology, Faculty of Medicine, Health Sciences Center, Kuwait University, Kuwait, Kuwait
| | - Marjan Rouzbahman
- Department of Anatomical Pathology, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada
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