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Frontiers in Oncology 2024The purpose of the study was to analyse the role of prognostic factors on the risk of recurrence and overall survival of patients with uterine adenosarcoma.
OBJECTIVE
The purpose of the study was to analyse the role of prognostic factors on the risk of recurrence and overall survival of patients with uterine adenosarcoma.
METHODS
A retrospective international multicentre study involving 46 centres collected 32 cases of uterine adenosarcoma, and these cases were included in the present subanalysis. Clinical and demographic features and tumour characteristics were gathered, as well as information on treatment and relapse. Disease-free and overall survival were analysed.
RESULTS
The 5-year disease-free survival (DFS) was 85.3% and the 5-year overall survival (OS) rate was 89.5%. The risk factors significantly associated with overall survival were age (HR 1.09, 95% CI 1.03-1.15; = 0.004) and FIGO stage II-III (HR 17.75, 95% CI 2.87-109.93; = 0.002). Patients who experienced early relapse (within 12 months) had a tumour size >30 mm and advanced stage. The majority of recurred cases were treated with radiotherapy or surgery and obtained a good response rate.
CONCLUSION
The most significant prognostic factors in uterine adenosarcoma were age and FIGO stage and, indirectly, tumour size at diagnosis. The use of secondary surgery and/or radiotherapy could help in prolonging the disease-free status of the patients.
PubMed: 38835369
DOI: 10.3389/fonc.2024.1393707 -
Ginekologia Polska Jul 2023Cervical adenofibroma is a rare form of mixed mesodermal tumor that can present as cervical polyps with a tendency for local recurrence and progression. Few cases...
OBJECTIVES
Cervical adenofibroma is a rare form of mixed mesodermal tumor that can present as cervical polyps with a tendency for local recurrence and progression. Few cases progressing to adenosarcoma have previously been reported. We report a case of cervical adenofibroma progressing to adenosarcoma, and we seek to remind clinicians of the method and importance of the differential diagnosis of this disease. A fertile woman was admitted in our department for the eighth recurrence of a cervical polypoidal mass which for the past 10 years. Recurrence of cervical adenofibroma was confirmed by ultrasound and MRI. A wide local excision under hysteroscopy was performed due to her strong desire to preserve the uterus. Surgical pathology and immunohistochemical interpretation revealed cervical adenosarcoma. A hysterectomy with conservation of the ovaries was recommended, with regular follow-ups for evidence of disease recurrence.
CONCLUSIONS
Differential diagnoses of cervical adenofibroma are hard to prove. Adenosarcoma should be ruled out, especially in women presenting with recurrent cervical polypoidal masses. A combined histological/immunohistochemical investigation is mandatory.
PubMed: 37435916
DOI: 10.5603/GP.a2023.0069 -
Revista Colombiana de Obstetricia Y... Sep 2023To report the case of a patient with a uterine collision tumor and to conduct a review of the literature.
OBJECTIVES
To report the case of a patient with a uterine collision tumor and to conduct a review of the literature.
MATERIAL AND METHODS
A 76-year-old patient who presented to the national cancer referral center in Bogota (Colombia), where she was diagnosed with a uterine collision tumor consisting of a seroustype endometrial adenocarcinoma and a cervical adenosarcoma. The patient underwent surgical treatment followed by chemotherapy and supplemental radiotherapy, and died 16 months later. A search was conducted in the Medline via PubMed and Embase databases, including reports and case series of women with a diagnosis of uterine collision tumor, with retrieval of information regarding diagnosis, treatment and prognosis. A narrative summary of the findings was made.
RESULTS
The search identified 36 titles, of which 14 studies with 17 patients were included. The most frequent histopathological diagnosis was endometrial adenocarcinoma and high and low grade endometrial sarcoma (47 %). Primary treatment was surgery and adjuvant treatment with chemotherapy and radiotherapy (15 %) was performed in close to 50 % of cases. One-year survival was 75 %.
CONCLUSIONS
No cases of uterine collision tumors with the histopathology or in the location of the reported case were found in the literature. If this reported case is taken into account, 2-year mortality is 28 %. Further studies to describe the immunohistochemistry, treatment and prognosis of this condition are needed.
Topics: Female; Humans; Adenocarcinoma; Colombia
PubMed: 37937912
DOI: 10.18597/rcog.4011 -
Translational Cancer Research May 2024Endometrial adenosarcoma is an unusual type of uterine tumor that features a seemingly benign epithelial component, paired with a low-grade sarcomatous component,...
BACKGROUND
Endometrial adenosarcoma is an unusual type of uterine tumor that features a seemingly benign epithelial component, paired with a low-grade sarcomatous component, usually similar in appearance to endometrial stromal sarcoma. To our knowledge, no image of endometrial adenocarcinoma in the cesarean scar diverticulum has been reported previously.
CASE DESCRIPTION
We present a rare case of endometrial adenocarcinoma located in the cesarean scar diverticulum of a 44-year-old patient. The patient was admitted to our hospital complaining of irregular vaginal bleeding that had lasted for over two months. Both B-ultrasound and magnetic resonance imaging confirmed a mass at the junction of the corpus uteri and cervix. After the initial curettage failed to confirm the disease, a hysteroscopy was subsequently performed. Upon further pathological analysis, a diagnosis of endometrial adenosarcoma was confirmed. The patient underwent hysterectomy and salpingo-oophorectomy. The patient was discharged home four days after the surgery and remained recurrence-free for one year after follow-up.
CONCLUSIONS
Hysteroscopy can serve as a valuable diagnostic tool to identify the lesion in this unique scenario, particularly when curettage fails to diagnose this uncommon condition. We hope that this case would bring awareness of this potential scenario, enabling clinicians in the future to identify similar cases more readily.
PubMed: 38881921
DOI: 10.21037/tcr-23-2155 -
International Journal of Analytical... 2023Uterine adenosarcoma (UA) is an uncommon mixed tumor containing a benign to at most mildly atypical epithelial component and a sarcoma-like stroma, usually a low-grade,...
Uterine adenosarcoma (UA) is an uncommon mixed tumor containing a benign to at most mildly atypical epithelial component and a sarcoma-like stroma, usually a low-grade, stromal component, with rare heterogeneous elements. Currently, tumor etiology is largely unknown. To better understand the gene mutations in UA, next-generation sequencing (NGS) technology analysis was performed. This study showed that two low-grade UAs with heterologous components had ATRX gene frameshift mutation, and one patient had a MED12 missense mutation. Copy number amplification genes were mainly observed on chromosome 12q. In this study, PIK3/AKT/PTEN pathway mutations were found to be common in adenosarcoma. In addition, a rare BCORL1-PRR14L fusion mutation was also identified. These findings provide a basis for future research into these molecular changes in tumorigenesis and targeted therapy.
PubMed: 37810911
DOI: 10.1155/2023/7436368 -
BMC Women's Health May 2024Cervical mullerian adenosarcoma is a rare uterine sarcoma, especially in young women. Its pathological features are low-grade malignant tumors with bidirectional...
BACKGROUND
Cervical mullerian adenosarcoma is a rare uterine sarcoma, especially in young women. Its pathological features are low-grade malignant tumors with bidirectional differentiation, and the degree of malignancy is similar to that of low-grade endometrial stromal sarcoma. This paper reports the case of a young asexual patient who has been closely followed up after tumor resection and has not had any recurrences.
CASE PRESENTATION
A 20-year-old, young asexual woman was diagnosed with cervical mullerian adenosarcoma with sarcomatous overgrowth (MASO). Cervical tumor resection was performed after admission, and the resection margin was negative. After the operation, she refused to undergo secondary surgery due to fertility requirements and did not receive adjuvant treatment. The patient was closely followed up after the operation and has not yet relapsed.
CONCLUSION
A young woman with cervical MASO did not receive adjuvant treatment after cervical tumor resection. For women with fertility requirements, close follow-ups should be conducted after the operation to guard against tumor recurrence and radical tumor resection should be performed as early as possible after the patient no longer requires their fertility.
Topics: Humans; Female; Adenosarcoma; Young Adult; Uterine Cervical Neoplasms; Uterine Neoplasms; Sexual Behavior
PubMed: 38783282
DOI: 10.1186/s12905-024-03140-w -
World Journal of Surgical Oncology Apr 2024To investigate the clinicopathological characteristics and prognosis of patients with primary sarcoma of the uterine cervix.
OBJECTIVES
To investigate the clinicopathological characteristics and prognosis of patients with primary sarcoma of the uterine cervix.
METHODS
We identified all patients with primary cervical sarcomas treated at our institution from 2002 to 2020 and analyzed the clinicopathological characteristics and prognosis.
RESULTS
34 patients were identified, 7 (20.6%) patients had leiomyosarcoma, 6 (17.6%) had carcinosarcoma, 5 (14.7%) had Ewing sarcoma, 4 (11.8%) had rhabdomyosarcoma, 4 (11.8%) had undifferentiated sarcoma, 2 (5.9%) had adenosarcoma, 2 (5.9%) had endometrial stromal sarcoma, 1 (2.9%) had dermatofibrosarcoma protuberans, 1 (2.9%) had alveolar soft tissue sarcoma and 2 (5.9%) had sarcoma not otherwise specified. The median age of the whole patients was 43.5 years (range, 13-63). The median age of patients with Ewing sarcoma or rhabdomyosarcoma was 22 years (range, 13-39) and 17 years (range, 13-36 years), respectively. The distribution by stage was: stage I in 21 (61.8%) patients, stage II in 4 (11.8%), stage III in 6 (17.6%) and stage IV in 3 (8.8%). Overall, 30 patients (88.2%) received surgical treatment. The median follow-up was 33.3 months (range 3.6-187.3 months). 11 patients died within 2 years after diagnosis, most of them were patients with carcinosarcoma or undifferentiated sarcoma (45.5%, 5/11). In the entire cohort, 2- and 5-year OS were 67.2% and 56.9%, respectively. 5-year OS was 25.0% for undifferentiated sarcoma, 50.0% for rhabdomyosarcoma, 50.0% for carcinosarcoma, 53.3% for Ewing sarcoma, 57.1% for leiomyosarcoma.
CONCLUSION
Cervical sarcomas are rare neoplasms with multiple histological subtypes and follow an aggressive course. Prognosis may be associated with tumor histology and stage.
Topics: Female; Humans; Adolescent; Young Adult; Adult; Middle Aged; Leiomyosarcoma; Sarcoma, Ewing; Uterine Cervical Neoplasms; Uterine Neoplasms; Sarcoma; Carcinosarcoma; Rhabdomyosarcoma; Prognosis
PubMed: 38637834
DOI: 10.1186/s12957-024-03376-8 -
Cancer Reports (Hoboken, N.J.) Oct 2023In patients with uterine adenosarcoma, a total abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO) is typically recommended as an initial treatment.... (Review)
Review
The uterine adenosarcoma with postoperative residual in a woman treated by total abdominal hysterectomy/bilateral salpingo-oophorectomy: A case report and review of literature.
BACKGROUND
In patients with uterine adenosarcoma, a total abdominal hysterectomy (TAH) with bilateral salpingo-oophorectomy (BSO) is typically recommended as an initial treatment. There is no consensus on adjuvant therapies.
CASE
We report the case of a patient with uterine adenosarcoma with postoperative residual disease. We performed four courses of adjuvant chemotherapy, including Ifosfamide, Mesna, and Adriamycin, and whole pelvic radiation with a dose of 50.4 Gy/28 Fr.
CONCLUSION
A combination of chemotherapy and radiotherapy may be a promising treatment option for uterine adenosarcoma with postoperative residual disease.
Topics: Female; Humans; Salpingo-oophorectomy; Uterine Neoplasms; Hysterectomy; Adenosarcoma
PubMed: 37592402
DOI: 10.1002/cnr2.1891 -
Diagnostic Pathology Apr 2024Müllerian adenosarcoma, a rare malignancy, presents diagnostic and therapeutic challenges. In this study, we conducted an analysis of the clinicopathological...
BACKGROUND
Müllerian adenosarcoma, a rare malignancy, presents diagnostic and therapeutic challenges. In this study, we conducted an analysis of the clinicopathological characteristics of 22 adenosarcomas, with a particular focus on screening for DICER1 hot mutations.
METHODS
The cohort consisted of patients with adenosarcoma who were registered at the West China Second Hospital between the years 2020 and June 2022. Sanger sequencing was employed to screen for somatic Hotspot mutations in the RNase IIIb domain of DICER1 in the 22 adenosarcomas.
RESULTS
Only one patient exhibited a DICER1 mutation that was not a DICER1 Hotspot mutation. Among the 22 patients, all underwent total hysterectomy with bilateral salpingo-oophorectomy, and 14 out of these 22 patients received adjuvant treatment.
CONCLUSION
In summary, our study of 22 Müllerian adenosarcomas focused on the clinicopathological features and the presence of DICER1 Hotspot mutations. Although our findings did not reveal any DICER1 mutations in the studied samples, this negative result provides valuable information for the field by narrowing down the genetic landscape of adenosarcomas and highlighting the need for further research into alternative molecular pathways driving this malignancy.
Topics: Female; Humans; Adenosarcoma; Mutation; China; Ribonuclease III; DEAD-box RNA Helicases
PubMed: 38570882
DOI: 10.1186/s13000-024-01477-2 -
Cancer Reports (Hoboken, N.J.) Feb 2024Mesenchymal neoplasms of the uterus encompass a diverse group of tumors, with varying characteristics and origins, collectively accounting for 8% of uterine... (Review)
Review
BACKGROUND
Mesenchymal neoplasms of the uterus encompass a diverse group of tumors, with varying characteristics and origins, collectively accounting for 8% of uterine malignancies. The most common variants include uterine leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, adenosarcoma, and undifferentiated sarcoma. Clinical presentation is often nonspecific and can lead to delayed diagnosis. Uterine sarcomas are generally aggressive, resulting in poorer prognosis compared to carcinomas. Recent advances in molecular techniques, such as next-generation sequencing (NGS), have led to the identification of new subtypes of uterine sarcomas, including COL1A1::PDGFB fusion-associated fibrosarcoma, which has a specific chromosomal translocation t(17;22)(q22;q13). Imatinib, a tyrosine kinase inhibitor (TKI), is an effective treatment for dermatofibrosarcoma protuberans (DFSP), marked by this translocation.
CASE
We present the case of a 42-year-old woman diagnosed with COL1A1::PDGFB fusion-associated uterine fibrosarcoma. The patient underwent total hysterectomy and excision of the tumor, initially misdiagnosed as a low-grade leiomyosarcoma. Subsequent histological examination, immunohistochemistry, and fluorescence in situ hybridization (FISH) confirmed the diagnosis. After 10 months, disease recurrence was detected, and Imatinib therapy was initiated at a dose of 400 mg daily. An allergic reaction led to a temporary discontinuation, but upon resumption with appropriate medication, a positive radiological response was observed. The patient achieved a complete remission after 2 years and is still on Imatinib treatment.
CONCLUSIONS
COL1A1::PDGFB fusion-associated uterine fibrosarcoma is an extremely rare mesenchymal neoplasm. In a case we present herein, we treated a patient with imatinib as first-line medical therapy. The patient is currently in complete remission after 37 months from treatment start. To the best of our knowledge, this represents a unique observation. We also provide a detailed literature review of the published cases so far. Prospective case series are needed to further understand the natural history of these tumors and optimize treatment strategies.
Topics: Female; Humans; Adult; Proto-Oncogene Proteins c-sis; Imatinib Mesylate; Dermatofibrosarcoma; In Situ Hybridization, Fluorescence; Leiomyosarcoma; Skin Neoplasms; Neoplasm Recurrence, Local; Fibrosarcoma; Soft Tissue Neoplasms; Translocation, Genetic; Uterus
PubMed: 38279510
DOI: 10.1002/cnr2.1969