Mullerian adenosarcoma of the uterus with MASO: a case report of cervical adenosarcoma in a young female that never had sexual behavior.

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.

Nonpuerperal uterine inversion caused by an adenosarcoma: A case report.

INTRODUCTION: Eighty-five per cent of uterine inversions are puerperal. Non-puerperal uterine inversion is usually caused by tumours that exert a traction force on the fundus of the uterus. This causes the uterus to be partially or completely inverted. It is commonly related to benign tumours like submucosal leiomyomas. Nevertheless, malignancies are an infrequent association. CASE PRESENTATION: We report a case of a 35-year-old female patient, medically and surgically free, gravida0 para0, complaining of menometrorrhagia associated with pelvic pain for 2 years. A suprapubic ultrasound scan showed an enlarged, globular uterus with a heterogeneous, undefined mass of 49 mm in size. MRI scan showed the appearance of a U-shaped uterine cavity and a thickened inverted uterine fundus with an endometrial infiltrating mass of 25 mm. Intraoperative exploration showed uterine inversion involving the ovaries; the fallopian tubes and the round ligaments and a necrotic intracavitary mass. The malignancy of the tumor was confirmed through anatomopathological examination as Adenosarcoma. CONCLUSIONS: Uterine inversion is rare outside the puerperal period, and malignant etiology must not be overlooked. Therefore, comprehensive care with meticulous etiological investigation is crucial.

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.

[Uterin adenosarcoma: French Guidelines of the French Sarcoma Group and the Rare Gynecologic Tumor Group]. / Adénosarcomes mullériens de l'utérus ­ référentiels de prise en charge du GSF-GETO/NETSARC+ et du groupe TMRG.

Uterine adenosarcoma is a very rare malignancy defined as a biphasic tumor composed of both benign epithelial component and a malignant sarcoma component. The stage of the disease is determined by the presence of myometrial invasion and the extent of extra-uterine disease. The most important histopronostic factors are the existence of a sarcomatous overgrowth defined by a sarcomatous contingent occupying more than 25 % of the volume of the tumor (directly correlated to the grade of the disease), the presence of a heterologous and/or a high-grade component. Stage I adenosarcomas without sarcomatous overgrowth have a good prognosis, with an overall 5-year survival of up to 80 %. In localized disease, complete surgical removal is recommended. The role of hormone therapy, chemotherapy and adjuvant radiotherapy is not established. If possible, relapses should be re-treated surgically, with the aim of achieving complete resection. In the advanced inoperable or metastatic setting, hormone therapy is an option for low-grade adenosarcomas with estrogen receptor (ER) and progesterone receptor (PR) overexpression. For high-grade tumors, the standard chemotherapies are doxorubicin-based combinations, although an integrated approach of surgery and medical treatment should also be considered in this setting.

Malignant Mixed Mullerian Tumor Arising from Endometriosis in the Groin: A Case Report and Literature Review.

This was a 57-year-old woman who presented with mild discomfort in the right groin. Physical examination revealed a mass in the right groin, and by ultrasound, the mass was hypoechoic and solid with some internal vascularity. The clinical differential diagnosis included lymphoma and others. The mass was excised for pathologic evaluation. Gross examination of the specimen revealed a 3 × 2.4 × 2 cm, solid and cystic mass. Microscopically, it was a biphasic tumor consisting of carcinomatous and sarcomatous components. The tumor was seen contiguous with endometriosis and atypical endometrioid hyperplasia. The histologic findings were consistent with malignant mixed Mullerian tumor (MMMT) arising from endometriosis in the right groin. The tumor involved the resection margin. Subsequent chest/abdominal/pelvic computed tomography did not reveal evidence of tumors, and diagnostic peritoneal/pelvic laparoscopy did not show diseases. Postoperatively, the patient received 6 cycles of chemotherapy consisting of carboplatin and paclitaxel, followed by radiation in the right groin. Malignant transformation from endometriosis occurs in less than 1% of endometriosis cases, and about 80% of the transformed tumors occur in the ovaries. The most commonly transformed malignant tumors are endometrioid and clear cell carcinomas, with rare adenosarcoma and endometrial stromal sarcoma reported. To our knowledge, we are reporting the first case of MMMT arising from endometriosis in the groin.

Epidemiology of adenosarcoma and the inverse probability of treatment weighting (IPTW) adjusted survival analysis of lymph node dissection in uterine adenosarcoma.

The objective for the study was to analysis the epidemiology of adenosarcoma, and independent prognostic factors and impact of lymph node dissection (LND) of uterine adenosarcoma. Cases of patients with primary adenosarcoma were obtained from the Surveillance, Epidemiology, and End Results (SEER) database from 2000 to 2016. Overall survival was analyzed by the Kaplan-Meier method and log-rank test. The differences in baseline covariates between the 2 groups were adjusted by inverse probability of treatment weighting method. The prognostic factors were identified by univariate and multivariate Cox regression analysis and hazard ratio and 95% confidence interval (CI) of covariates were also estimated. 1129 patients with pathological primary adenosarcoma between 2000 and 2016 were identified from the surveillance, epidemiology, and end results database. The only 4 patients were male. 1027 patients with primary uterine adenosarcoma, and 53.1% underwent LND and only 3.5% patients were with positive lymph node. Age, marital status, largest tumor size, tumor grade, T stage and chemotherapy were significantly correlated with survival. Race, tumor number, LND, and radiotherapy did not affect overall survival in patients. Inverse probability of treatment weighting-adjusted K-M curve showed that LND did not improve survival and lymph node metastasis (LNM) did not affect survival. The majority of primary adenosarcoma patients are female with high incidence of uterus and rare incidence of distant metastasis. Age, marital status, tumor size, T stage, grade, and chemotherapy are independent prognostic factors of uterine adenosarcoma. LNM was not a significant prognostic risk factor, and LND did not benefit survival.

Uterine Adenosarcoma in a Teen Ager.

Uterine adenosarcoma is a rare tumor composed of benign epithelium in combination with malignant mesenchymal components. Misdiagnosis is common due to the lack of specific clinical manifestations and the rarity of such tumors in young age groups. The mean age at diagnosis is 58.6 years, and <10% of patients are younger than 40 years. Herein, we describe a case of a 19-year girl who presented with worsening menorrhagia. Her ultrasound revealed multiple heterogeneous masses in the uterine cavity. Intraoperatively, a small piece of grayish-white tumor tissue was sent for the frozen section, but the results did not confirm malignancy. She underwent hysteroscopy, laparoscopic hysterectomy, and bilateral salpingo-oophorectomy. Histopathologic examination finally confirmed uterine adenosarcoma. The patient had no evidence of residual tumor or recurrence during six months of follow-up. Key Words: Uterus, Adenosarcoma, Mixed tumor.

Adenosarcoma of Uterus- Rare Biphasic Malignant Tumor: A Case Report.

Uterine adenosarcoma is a rare variant of mixed Mullerian tumors comprised of neoplastic glands with the benign appearance and sarcomatous stroma. The epithelium most often consists of endometrium- like cells, while the sarcomatous component usually shows low-grade homologous uterine sarcoma. These tumors present as a pelvic mass or an enlarged uterus with abnormal vaginal bleeding. Here, we present a case of 61 years old postmenopausal female patient with chief complaints of excessive vaginal bleeding and urine retention.

Carcinosarcoma of the Cervix: A Case Report.

Cervical carcinoma is the most common cause of mortality due to cancer in Nepal. Carcinosarcoma is a very rare subtype of cervical cancer which is characterized by the presence of both epithelial and mesenchymal malignant component. It constitutes less than 1% of cervical carcinoma. Due to the low occurrence of the disease, most of the data on treatment and prognosis are based on case reports and series. Here, we report a case of 69 years, female with cervical cancer (FIGO IIA2). Histopathological and immunohistochemical analysis of cervical biopsy initially showed primary adenosarcoma of the cervix. The tumor was non-responsive to primary treatment with concurrent chemoradiation. Later she was treated with abdominal hysterectomy and bilateral salpingo-oophorectomy. The final histopathology of the resected specimen showed a sarcomatous component along with carcinomatous changes in the endocervical glands favouring the diagnosis of carcinosarcoma of the cervix.

Uterine Adenosarcoma Originating in Adenomyosis: Report of an Extremely Rare Phenomenon and Review of Published Literature.

Müllerian adenosarcoma is an uncommon biphasic malignant tumor most often occurring in the uterine corpus and derived from native surface endometrium. We report a case of intramural uterine adenosarcoma arising in association with adenomyosis, in the absence of tumor involving the surface endometrium. This is an extremely rare phenomenon, with only 8 other published cases of uterine corpus adenosarcoma in the absence of surface endometrial involvement, 5 originating in adenomyosis and 3 in adenomyomas. We review these cases. The current FIGO staging system for uterine adenosarcoma assumes origin from the surface endometrium and does not address the rare occurrence of intramural tumors without a surface endometrial component. Such tumors are problematic to stage and could potentially be overtreated, particularly if there is deep myometrial involvement.

Italian consensus conference on management of uterine sarcomas on behalf of S.I.G.O. (Societa' italiana di Ginecologia E Ostetricia).

BACKGROUND: Uterine sarcomas are very rare tumours with different histotypes, molecular features and clinical outcomes; therefore, it is difficult to carry out prospective clinical trials, and this often results in heterogeneous management of patients in the clinical practice. AIM: We planned to set up an Italian consensus conference on these diseases in order to provide recommendations on treatments and quality of care in our country. RESULTS: Early-stage uterine sarcomas are managed by hysterectomy + bilateral salpingo-oophorectomy according to menopausal status and histology; lymphadenectomy is not indicated in patients without bulky nodes, and morcellation must be avoided. The postoperative management is represented by observation, even though chemotherapy can be considered in some high-risk patients. In early-stage low-grade endometrial stromal sarcoma and adenosarcomas without sarcomatous overgrowth, hormonal adjuvant treatment can be offered based on hormone receptor expression. In selected cases, external beam radiotherapy ± brachytherapy can be considered to increase local control only. Patients with advanced disease involving the abdomen can be offered primary chemotherapy (or hormonal therapy in the case of low-grade endometrial stromal sarcoma and adenosarcoma without sarcomatous overgrowth), even if potentially resectable in the absence of residual disease in order to test the chemosensitivity (or hormonosensitivity); debulking surgery can be considered in patients with clinical and radiological response. Chemotherapy is based on anthracyclines ± ifosfamide or dacarbazine. Palliative radiotherapy can be offered for symptom control, and stereotactic radiotherapy can be used for up to five isolated metastatic lesions. CONCLUSIONS: Treatment of uterine sarcoma should be centralised at referral centres and managed in a multidisciplinary setting.

A case of three rare uterine neoplasms in the same surgical specimen.

BACKGROUND: Uterine sarcomas are mesenchymal tumors; they are rare, representing less than 2-3% of all uterine malignancies. Among them, we can define four types: leiomyosarcoma (LMS), endometrial stromal sarcoma (ESS), Adenosarcoma and Carcinosarcoma. This last type was recently reclassified by FIGO as a Mullerian type of the endometrial adenocarcinoma. Therefore, today only the first three types are histologically considered. METHODS: In this paper, we reported a case of simultaneous presence of three different rare neoplasms in the same surgical specimen, resulting from a hysterectomy of a premenopausal woman. The woman presented to the ED with a six-months history of vaginal bleeding. Given the complexity of the clinical picture, we suggested hospitalization in our Department of Gynecology, to perform appropriate diagnostic tests. Because of the persistent hemorrhage and the absence of required fertility preservation, a laparotomic hysterectomy with bilateral annessiectomy was performed. RESULTS: The postoperative histology of the specimen described the myoma at the fundus as a leiomyosarcoma. The myoma of the uterine anterior wall appeared as an endometrial stromal sarcoma of low-grade. Moreover, an intramural cavernous hemangioma of 3 cm in diameter was reported at the uterine corpus. CONCLUSION: All these described pathologies have no specific clinic characteristics; the most common symptom is abnormal uterine bleeding. To date, hysterectomy and bilateral salpingo-oophorectomy are the standards of care in the management of all early stage uterine sarcomas. To our knowledge, cases of LMS, ESS and cavernous haemangioma coexisting in the same patient have not been reported in literature to date. The pathogenesis of this combination remains to be elucidated. Key words: Cavernous hemangioma, Endometrial stromal sarcoma, Leiomyosarcoma, Uterine sarcomas.

Adenosarcoma de alto grado de endocérvix y cavidad endometrial con componente heterólogo de rabdomiosarcoma. Reporte de caso y revisión de la literatura / High grade adenosarcoma of endocervix and endometrial cavity with a heterologous component of rhabdomyosarcoma. Case report and review of the literature

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.

Objetivo: presentar el caso de una paciente con diagnóstico de adenosarcoma de alto grado de en- docérvix y de cavidad endometrial, con componente heterólogo, y hacer una revisión de la literatura con especial atención al diagnóstico y manejo terapéutico de esa patología. Materiales y métodos: Se presenta el caso de una paciente de 31 años, que consulta al Hospital Universitario Virgen Macarena de Sevilla, institución regional de tercer nivel, por presentar san grado genital originado por una masa polipoidea endocervical que se llevó a biopsia con resultado de un leiomiosarcoma poco diferenciado de alto grado de endocérvix. Posteriormente fue llevada a histerectomía total abdominal. En el estudio de la pieza quirúrgica el resultado fue: adenosarcoma de endocérvix y cavidad endometrial, con componente heterólogo de rabdomiosarcoma. Con los términos: "adenosarcoma", "endocervical", "cérvix", "uterus", "heterologous", en las bases de datos Medline vía PubMed se realizó una búsqueda de artículos de revisión bibliográfica, reportes y series de casos clínicos que describían aspectos del adenosarcoma cervicouterino y del componente heterólogo de rabdomiosarcoma, en inglés y español, publicados desde 1974. Resultados: se hallaron seis artículos correspondientes a revisiones de la literatura, reportes o series de casos, donde se describen los aspectos más importantes referentes al diagnóstico y tratamiento de esta patología. Conclusiones: esta patología se caracteriza, en ocasiones, por un crecimiento rápido y agresivo, donde es importante el diagnóstico precoz y el tratamiento óptimo, basado en una combinación de cirugía, radioterapia y quimioterapia, aunque dada su baja prevalencia se necesitan aún más estudios para poder confirmar estos datos.

Uterine and Cervical Adenosarcoma: A Retrospective Study of Overall Oncologic Outcomes and Fertility Preservation in Early-Stage Disease.

BACKGROUND: The management of adenosarcoma is based on the limited available data. This study aimed to explore the characteristics and oncologic outcomes of patients with uterine and cervical adenosarcoma. MATERIALS AND METHODS: A total of 21 and 32 cases of cervical and uterine adenosarcoma, respectively, were retrospectively reviewed in Peking Union Medical College Hospital between April 2006 and March 2019. RESULTS: The median follow-up time was 37.5 months (range, 1-153 months). The disease progression rate (DPR) was significantly higher in patients with uterine adenosarcoma compared with those with cervical adenosarcoma (28.1% vs. 4.8%). The curve of progression-free survival significantly differed. For those with cervical adenosarcoma, the presence of a tumor stalk was a protective factor, whereas heterologous elements (HE) presented a risk factor for disease progression. For those with uterine adenosarcoma, the presence of a tumor stalk was an independent protective factor, whereas lymphovascular space invasion (LVSI) was an independent risk factor for disease progression. Moreover, HE was an independent risk factor for mortality. Fertility-sparing surgery (FSS) was performed in four and five patients with cervical and uterine adenosarcoma, respectively. Regarding FSS, combined with cases in previous studies, the DPR of patients with uterine adenosarcoma was relatively higher compared with those with cervical adenosarcoma. CONCLUSION: We found that cervical adenosarcoma had a better prognosis than uterine adenosarcoma. The presence of a tumor stalk was a protective factor, whereas HE and LVSI were risk factors for prognosis. For those with uterine adenosarcoma, if FSS was administered, robust evaluation would be necessary. The small sample size limits the ability to make any strong conclusions about FSS. IMPLICATIONS FOR PRACTICE: Uterine cervical adenosarcoma had a better prognosis than uterine adenosarcoma. For patients with cervical adenosarcoma, the presence of a tumor stalk was a protective factor and the presence of heterologous elements (HE) was a risk factor for disease progression. For those with uterine adenosarcoma, the presence of a tumor stalk was a protective factor and lymphovascular space invasion was a risk factor for disease progression. Moreover, HE was a risk factor for mortality. Regarding fertility-sparing surgery (FSS), the disease progression rate was higher in patients with uterine adenosarcoma compared with those with cervical adenosarcoma. For patients with uterine adenosarcoma, if FSS was administered, hysteroscopy and robust imaging evaluation would be necessary.

Mullerian adenosarcomas of the uterine cervix with sarcomatous overgrowth.

Mullerian adenosarcoma with sarcomatous overgrowth (MASO) of the uterine cervix is an extremely rare variant of adenosarcoma of the genital tract associated with aggressive clinical course. We searched the PubMed and Medline databases for MASO of the cervix and we identified and reviewed eleven cases published between years 2004 and 2017. The most common clinical picture includes abnormal vaginal bleeding, postcoital bleeding, pelvic pain and foul-smelling vaginal discharge. Therapeutic options for MASO are still undefined. Radical hysterectomy with sufficient tumour-free margins combined with adjuvant chemotherapy and radiotherapy should serve as an effective treatment tool with favourable outcome.

Clinical management of uterine cervical mullerian adenosarcoma: A clinicopathological study of six cases and review of the literature.

OBJECTIVE: To assess the clinicopathological characteristics and clinical management of patients diagnosed with mullerian adenosarcoma of the uterine cervix. MATERIALS AND METHODS: Records of six patients surgically treated for cervical mullerian adenosarcoma were reviewed. RESULTS: The median age of the patients was 50 years (range, 17-74). Four patients presented with vaginal bleeding and two of them had watery discharge as the primary symptoms. Three nulliparous patients who hoped to preserve their uterus were included in this study and only one of the three patients received fertility-preservation surgery. Five patients underwent hysterectomy (simple in 3, modified radical in 2) with bilateral salpingo-oophorectomy or bilateral salpingectomy. One patient underwent conization of the cervix to preserve her fertility as there was no sarcomatous overgrowth, heterologous elements, or deep cervical stromal invasion. A recurrence of cervical mullerian adenosarcoma with sarcomatous overgrowth was observed in one patient who underwent simple hysterectomy. CONCLUSION: The presence of sarcomatous overgrowth and deep cervical stromal invasion are associated with poor prognosis. Along with adequate counseling, fertility-preservation surgery may be an acceptable option for exophytic cervical mullerian adenosarcoma in the cases that do not show sarcomatous overgrowth and deep cervical stromal invasion.

Efficacy of Hyperthermic Intraperitoneal Chemotherapy and Cytoreductive Surgery in the Treatment of Recurrent Uterine Sarcoma.

OBJECTIVE: Uterine sarcomas (USs) are characterized by poor response to systemic chemotherapy and high recurrence rates. This study evaluates whether the use of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) confers survival benefit in comparison with conventional treatment modalities in patients with recurrent US. METHODS/MATERIALS: A retrospective analysis of patients with recurrent US at a single institution for an 11-year study period was performed. All women with a pathologic diagnosis of leiomyosarcoma, adenosarcoma, endometrial stromal sarcoma, or undifferentiated US were identified. Overall and disease-free survival was estimated using Kaplan-Meier method. Comparisons between the study groups were performed with the log-rank test and Cox regression. RESULTS: A total of 26 patients were identified. Five patients received chemotherapy and/or radiotherapy without surgical intervention, 14 patients underwent surgery alone or a combination of surgery and adjuvant systemic chemotherapy, and 7 patients received cytoreductive surgery with HIPEC. There was no treatment-related mortality in any group, and only 1 patient had grade III-IV surgical complications. Median disease-free survival was 2.4 months for patients with nonsurgical treatments, 5.3 months for patients treated with conventional surgery, and 11.3 months for patients treated with HIPEC. Median overall survival was 35.9 months for patients treated with conventional surgery and 43.8 months for patients treated with HIPEC. CONCLUSIONS: Our study is the first to compare survival outcomes of HIPEC versus conventional therapies for recurrent US and is suggestive of treatment benefit. Further studies with more patients and longer follow-up to evaluate the role of HIPEC in management of this disease are warranted.

Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review.

BACKGROUND: Extra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours' histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes. CASE PRESENTATION: A 79-year-old woman was referred to our Department with an abdominal mass resembling a fibroid with a haemorrhage. Her gynaecological history was negative. A transvaginal and transabdominal ultrasound examination revealed a multicystic mass resembling an ovarian tumour arising from the pelvis and extending up to the abdomen. At laparotomy a peritoneal mass arising from Douglas peritoneum was resected. The uterus and adnexa appeared normal, and a supra-cervical hysterectomy with bilateral salpingo-oophorectomy was performed. No macroscopic residual disease was present. Final pathology diagnosed a malignant peripheral nerve sheath tumors with divergent differentiation. Four weeks later a new, multicystic mass was found. Due to the progressive poor condition, the patient died four months after diagnosis. Histological slides were reviewed by external expert pathologists and the final diagnosis was of extra-genital adenosarcoma with sarcomatous overgrowth. Furthermore, we also collected and analysed articles written in English regarding extra-uterine and extra-ovarian adenosarcomas published between January 1974 and October 2016. PubMed was used as a database for this search. Clinical and pathological characteristics, treatments and outcomes were assessed. CONCLUSIONS: Only 41 cases has been reported in literature. Previous endometriosis and sarcomatous overgrowth showed an inverse effect on prognosis. Endometriosis was confirmed to have a positive effect on disease free survival Complete surgical resection is the mainstay of treatment. A worldwide registry is urgently required to collect data to standardize treatment and to obtain reliable data on prognosis.

Uterine Adenosarcoma with Sarcomatous Overgrowth: A Case Report of Aggressive Disease in a 16-Year-Old Girl and a Literature Review.

BACKGROUND: Uterine adenosarcoma with sarcomatous overgrowth (ASSO) is a rare and aggressive disease. A case of a 16-year-old girl with uterine ASSO is reported herein. The patient received surgical resection and chemotherapy and remained alive without disease 11 months after the surgery. CASE: A 16-year-old girl was diagnosed with uterine ASSO, International Federation of Gynecology and Obstetrics (2009) stage I c. She underwent total abdominal hysterectomy, bilateral salpingectomy, and chemotherapy. She remains alive and there was no evidence of tumor recurrence on follow-up physical, laboratory, and ultrasound scan examinations. SUMMARY AND CONCLUSION: Surgery is the primary treatment for uterine ASSO, total abdominal or laparoscopic-assisted vaginal hysterectomy with or without bilateral salpingo-oophorectomy are recommended. Early surgical resection might increase survival of uterine adenosarcoma. Long-term follow-up of the patients is recommended because of the high chance of recurrence.