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Revista Colombiana de Obstetricia Y... Jun 2019To present the case of a patient diagnosed with high grade adenosarcoma of the endocervix and the endometrial cavity, with a heterologous component, and to conduct a... (Review)
Review
OBJECTIVE
To present the case of a patient diagnosed with high grade adenosarcoma of the endocervix and the endometrial cavity, with a heterologous component, and to conduct a review of the literature focusing on the diagnosis and therapeutic management of this disease condition.
MATERIALS AND METHODS
We present the case of a 31-year-old female patient who came to Virgen Macarena University Hospital of Seville, a Level III regional institution, complaining of genital bleeding arising from an endocervical polypoid mass. The biopsy of the mass revealed a high grade, poorly differentiated leiomyosarcoma of the endocervix. The patient was taken later to total abdominal hysterectomy. The study of the surgical specimen provided the following result: adenosarcoma of the endocervix and endometrial cavity with a heterologous rhabdomyosarcoma component. A search was conducted in the Medline database via Pubmed using the terms "adenosarcoma," "endocervical," "cervix," "uterus," "heterologous." The search in- cluded literature review articles, case reports and clinical case series describing aspects of cervical adenosarcoma and the heterologous rhabdomyosar- coma component, published in English and Spanish since 1974.
RESULTS
Six articles corresponding to literature reviews, case reports or case series in which the most relevant aspects of the diagnosis and treatment of this disease condition are described were retrieved.
CONCLUSIONS
This condition is characterized, on occasions, by rapid and aggresive growth, hence the importance of early diagnosis and optimal treatment based on a combination of surgery, radiation therapy and chemotherapy. However, due to its low prevalence, further studies are needed in order to confirm these data.
Topics: Adenosarcoma; Adult; Endometrial Neoplasms; Female; Humans; Hysterectomy; Neoplasm Grading; Rhabdomyosarcoma; Uterine Cervical Neoplasms
PubMed: 31613077
DOI: 10.18597/rcog.3258 -
Modern Pathology : An Official Journal... Jun 2020Embryonal rhabdomyosarcomas (ERMS) account for 2-3% of cancers in pediatric and adolescent populations. They are rarer in adults. We and others have reported that ERMS...
Embryonal rhabdomyosarcomas (ERMS) account for 2-3% of cancers in pediatric and adolescent populations. They are rarer in adults. We and others have reported that ERMS arising in the uterine cervix may harbor mutations in the gene encoding the microRNA biogenesis enzyme, DICER1, but a large series of cases has not been published. In the uterus, distinguishing ERMS from adenosarcoma can be very challenging, even for expert pathologists, and DICER1 alterations have been identified in a variable subset of uterine adenosarcomas. We hypothesized that DICER1 genetic testing may be useful in distinguishing between ERMS and adenosarcoma. We conducted a central pathology review-based study of 64 tumors initially thought to be uterine ERMS or adenosarcoma; 19 neoplasms had a consensus diagnosis of ERMS, 27 of adenosarcoma and for 18, no consensus diagnosis was reached. The median age at diagnosis was 30 years (range 2.5-69) for ERMS, 57.5 years (range 27-82) for adenosarcoma, and 65.5 years (range 32-86) for no consensus cases. In our series, the DICER1 mutation prevalence differed between the three groups: DICER1 alterations were present in 18/19 (95%) ERMS, 7/27 (26%) adenosarcomas (p < 0.001), and 4/18 (22%) no consensus cases. A germline alteration was present in 6/12 ERMS patients tested versus 0/6 adenosarcoma patients. Thus, although DICER1 mutations are near ubiquitous in uterine ERMS and are significantly less common in uterine adenosarcoma, DICER1 testing is only of value in distinguishing between the two neoplasms when a DICER1 mutation is absent, as this is helpful in excluding ERMS. On review of the clinical and radiological features of the single DICER1 wild-type cervical ERMS, this was thought most likely to be of vaginal origin. Given the significant prevalence of DICER1 germline pathogenic variants in uterine ERMS, all patients with this diagnosis should be referred to a genetics service.
Topics: Adenosarcoma; Adolescent; Adult; Aged; Aged, 80 and over; Child; Child, Preschool; DEAD-box RNA Helicases; Female; Genetic Predisposition to Disease; Humans; Middle Aged; Mutation; Rhabdomyosarcoma; Ribonuclease III; Uterine Neoplasms; Young Adult
PubMed: 31900434
DOI: 10.1038/s41379-019-0436-0 -
International Journal of Surgery Case... May 2023Mullerian adenosarcoma is a rare malignancy that generally occurs in the uterine corpus but uncommonly, it may be found extrauterine. Ovarian adenosarcoma is extremely...
INTRODUCTION AND IMPORTANCE
Mullerian adenosarcoma is a rare malignancy that generally occurs in the uterine corpus but uncommonly, it may be found extrauterine. Ovarian adenosarcoma is extremely rare and often is presented in reproductive age women. Most of them are low grade and have à good prognosis except for adenosarcoma with sarcomatous overgrowth.
CASE PRESENTATION
A 77-year-old menopausal woman presented with abdominal discomfort. She had severe ascites and elevated levels of CA-125, CA 19-9, and HE4 tumor markers. Adenosarcoma with sarcomatous overgrowth was diagnosed after the histopathological examination of the surgical biopsy.
CONCLUSION
The possibility of endometriosis transformation to malignancy even in postmenopausal women may warrant continuous follow-up for early diagnosis of ovarian cancer, this potentially fatal disease. More studies are needed to find the best therapeutic approach to adenosarcoma with sarcomatous overgrowth.
PubMed: 37148726
DOI: 10.1016/j.ijscr.2023.108244 -
World Journal of Surgical Oncology Oct 2016Uterine adenosarcomas are rare malignant gynaecological tumours. Due to its submucous localization, they can be easily confound with benign tumours like endometrial...
BACKGROUND
Uterine adenosarcomas are rare malignant gynaecological tumours. Due to its submucous localization, they can be easily confound with benign tumours like endometrial polyps or submucous myomas. However, the treatment of uterine adenosarcomas requires an oncologic surgical approach.
CASE PRESENTATION
In the following case report, we present the minimally invasive treatment of a uterine adenosarcoma by hysteroscopy and laparoscopy in a 37-year-old patient and discuss the special role of hysteroscopy in such cases.
CONCLUSIONS
In case of unknown or suspect intrauterine tumours, a diagnostic and operative hysteroscopy with biopsy could be realized prior to laparoscopic hysterectomy especially when the use of a laparoscopic electric morcellation is planned. Thus, a correct oncologic approach can be guaranteed if an adenosarcoma is diagnosed.
TRIAL REGISTRATION
ISRCTN.
Topics: Actins; Adenosarcoma; Adult; Biopsy; Female; Humans; Hysterectomy; Hysteroscopy; Keratins; Neoplasm Staging; Neprilysin; Ovariectomy; Ovary; Salpingectomy; Ultrasonography; Uterine Neoplasms
PubMed: 27769260
DOI: 10.1186/s12957-016-1015-1 -
International Journal of Gynecological... Mar 2018This study aimed to identify the hormonal receptor status in uterine adenosarcoma (AS) and uterine AS with sarcomatous overgrowth (AS + SO), including those with...
OBJECTIVE
This study aimed to identify the hormonal receptor status in uterine adenosarcoma (AS) and uterine AS with sarcomatous overgrowth (AS + SO), including those with high-grade histologic features (nuclear pleomorphism, atypical mitoses, necrosis), with or without heterologous elements. Estrogen receptor (ER) status, including estrogen receptor α (ERα), estrogen receptor β (ERβ), and G protein-coupled estrogen receptor (GPER), and progesterone receptor (PgR) status were examined.
METHODS
From August 2001 to November 2013, 11 patients with histologic diagnosis of uterine AS were identified. Tumor tissue sections were stained for ERα, ERβ, GPER, and PgR and examined both for percentage of overall cells stained and for intensity of staining. Descriptive statistics were calculated using clinicopathologic data abstracted from the medical record.
RESULTS
Eight cases of AS and 3 cases of AS with high-grade features were identified. Seven of 8 tumor samples of AS showed strong or moderate intensity immunostaining for ERα; all AS + SO tumor samples showed minimal to no immunoreactivity for ERα. There was a significant decrease in ERα H scores in high-grade tumors when compared with AS (P = 0.01). Lower PgR H scores were observed in high-grade tumors compared with those in AS (P = 0.04). Estrogen receptor β immunostaining was variable, and GPER immunostaining was absent in the majority of tumor samples.
CONCLUSIONS
Higher expression of ERα and PgR was observed in AS when compared with those with AS + SO and high-grade features. Both tumor subtypes showed similar levels of ERβ and GPER expression, although significant differences in ERβ and GPER expression were not detected. In contrast to our previous findings in uterine carcinosarcoma, ERs ERβ and GPER do not seem to play a significant role in AS in this study.
Topics: Adenosarcoma; Estrogen Receptor alpha; Estrogen Receptor beta; Female; Humans; Middle Aged; Receptors, Estrogen; Receptors, G-Protein-Coupled; Receptors, Progesterone; Uterine Neoplasms
PubMed: 29303935
DOI: 10.1097/IGC.0000000000001183 -
Molecular and Clinical Oncology Dec 2020The present study aimed to assess the diagnosis, treatment and follow-up of uterine sarcoma cases. A retrospective cohort study with 122 women recruited between 2001 and...
The present study aimed to assess the diagnosis, treatment and follow-up of uterine sarcoma cases. A retrospective cohort study with 122 women recruited between 2001 and 2016 was performed. The data regarding epidemiology, clinical presentation, treatment and follow-up were analyzed based on the following histological types: Carcinosarcoma, leiomyosarcoma, endometrial stromal sarcoma (ESS) and adenosarcoma. Statistical analysis included descriptive statistics, logistic regression and survival curves. The diagnosis of uterine sarcoma exhibited an increasing trend of +1.2 new cases every 2 years (P=0.044) and comprised 10% of all uterine cancer diagnoses. There were 47% carcinosarcomas, 22% leiomyosarcomas, 16% ESS and 14% adenosarcomas. The majority of the women was ≥60 years old (62%). Among the subjects, 77% were postmenopausal, 61% had a body mass index up to 29.9 kg/m and 71% presented with a comorbidity. Regression analysis exhibited an association between post menopause and the histological type associated with lower overall survival (OS), namely leiomyosarcoma or carcinosarcoma (odds ratio, 5.45, P<0.001). Stage I malignancy was present in 44% and Stage IV in 22%. The treatment included primary surgery in 78% of the cases, whereas 79% received adjuvant therapy. Only 55 cases achieved disease control and 20 relapsed (36%) with a 5-year OS rate of 33%. The OS was lower for carcinosarcoma and leiomyosarcoma (20%; P=0.003). In summary, the present study indicated that the number of uterine sarcoma cases had increased between 2001 and 2016. The majority of the women were >60 years old and diagnosed in advanced stages. The postmenopausal status was associated with histological types of poor prognosis. The OS was low and worse for patients with carcinosarcoma and leiomyosarcoma.
PubMed: 33093955
DOI: 10.3892/mco.2020.2151 -
Cancers Nov 2021Uterine sarcomas are rare cancers, sometimes diagnosed in women of childbearing age. Hysterectomy is the standard treatment in early stages. The option of lesion removal... (Review)
Review
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.
PubMed: 34830960
DOI: 10.3390/cancers13225808 -
Modern Pathology : An Official Journal... Jan 2022A subset of clinically benign uterine polyps shows atypical morphologic features worrisome for, but not diagnostic of, Mullerian adenosarcoma. We report...
A subset of clinically benign uterine polyps shows atypical morphologic features worrisome for, but not diagnostic of, Mullerian adenosarcoma. We report clinicopathologic data for 63 polyps from 58 women with atypical morphologic features including abnormal architecture, abnormal periglandular stroma, stromal atypia, and mitoses >2 per 10 hpf. Four (11%) of 36 women with follow-up tissue sampling had residual/recurrent atypical polyp. Twelve (27%) of 44 women underwent hysterectomy subsequent to a diagnosis of atypical polyp. No patient developed adenosarcoma over median follow-up of 150 months. Twenty-one primary atypical polyps underwent molecular profiling. Five (24%) harbored chr 12q13-15 gain or amplification, 9/20 (45%) harbored chr 6q25.1 gain, and 7/21 (33%) had no significant copy number alterations. Gains of chr 1q, chr 8p12, and chr 10q11.21-23, amplifications of chr 12q24.12-13, chr 15p24.1-26.1, and chr 18q21.33, and loss of chr 7 and chr 11q21 were each seen in a single polyp. Mean tumor mutational burden was 3.1 (range, 0.76-8.365) mutations/Mb. Pathogenic point mutations were identified in 12/20 (60%) primary atypical polyps. We propose the term "atypical uterine polyps" for these lesions, which show biologic overlap with early Mullerian adenosarcoma but lack molecular alterations characteristic of clinically aggressive adenosarcoma and appear to follow a benign clinical course. Conservative management with close clinical follow-up and repeat sampling can be considered for these lesions, when clinically appropriate.
Topics: Adenosarcoma; Adult; Aged; Cohort Studies; Female; Follow-Up Studies; Humans; Middle Aged; Mitosis; Polyps; Uterine Diseases; Young Adult
PubMed: 34675347
DOI: 10.1038/s41379-021-00946-z -
Zhong Nan Da Xue Xue Bao. Yi Xue Ban =... Nov 2022Currently, whole uterus and bilateral tubal resection and oophorectomy is the main treatment of cervical mullerian adenosarcoma. However, young patients generally wish... (Review)
Review
Currently, whole uterus and bilateral tubal resection and oophorectomy is the main treatment of cervical mullerian adenosarcoma. However, young patients generally wish to retain reproductive function. The clinical data of a patient with cervical mullerian adenosarcoma, who underwent fertility preservation surgery were collected. A 13-year-old girl with abnormal vaginal bleeding and a 1.0 cm flocculent echogenicity in the lower part of the uterine cavity to the cervical canal and a cervical mass of about 61 mm×37 mm was found in the pelvic MRI. After initial diagnosis of dysfunctional uterine bleeding in adolescence and cervical blood clot, the patient was treated with artificial cycle treatment, but her symptoms did not improve. Then she was transferred to the Third Xiangya Hospital of Central South University for uninjured virgin membrane hysteroscopy and cervical mass electrotomy, but a few pedicles remained after the operation, and the pathology suggested a cervical mullerian adenosarcoma. Because the patient was young and had not yet given birth, she was treated with primary IAP regimen of chemotherapy and subcutaneously injected with gonadotropin-releasing hormone analogue (GNRH-A) once every 28 days (6 times in total) to protect the ovarian function. After the chemotherapy, she was treated with uninjured virgin membrane hysteroscopy and pedicle electrotomy of cervical mullerian adenosarcoma. After the operation, she received chemotherapy with IAP regimen for 5 times. After discharge, she was treated with megestrol 200 mg per day for 3 years. During 5 years of regular follow-up, no abnormality was seen. Cervical mullerian adenosarcoma in non-sexual women is easily misdiagnosed as ovulation dysfunction abnormal uterine bleeding. The necessity of hysteroscopy should be emphasized, and for patients with low-grade early-stage lesions who wish to retain fertility, local resection could be chosen, but attention is paid to lifelong follow-up to exclude long-term recurrence.
Topics: Humans; Female; Adolescent
PubMed: 36481642
DOI: 10.11817/j.issn.1672-7347.2022.220453 -
Journal of Medical Cases Aug 2022Adenosarcomas are biphasic neoplasms that usually originate in the uterine corpus and comprise a benign epithelial component and a malignant stromal component. Uterine...
Adenosarcomas are biphasic neoplasms that usually originate in the uterine corpus and comprise a benign epithelial component and a malignant stromal component. Uterine adenosarcomas typically present with abnormal genital bleeding, an enlarged uterus, and a tumor that protrudes into the endometrial cavity. These tumors rarely protrude through the cervical os and are often misdiagnosed as cervical polyps. We present the case of a patient with cervical adenosarcoma with characteristics different from those reported in previous cases. This tumor showed endophytic growth, which is rare in cervical adenosarcomas. No watery discharge or obvious genital bleeding was noted. Although the tumor measured 4 cm, vaginal bleeding was noted only once at 6 months before diagnosis and was in the form of faint brown discharge.
PubMed: 36128067
DOI: 10.14740/jmc3952