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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.

Herein we evaluated a series of 21 embryonal rhabdomyosarcomas of the uterine corpus (ucERMS), a rare neoplasm, to characterize their morphology, genomics, and behavior. Patients ranged from 27 to 73 (median 52) years and tumors from 4 to 15 (median 9) cm, with extrauterine disease noted in two. Follow-up (median 16 months) was available for 14/21 patients; nine were alive and well, four died of disease, and one died from other causes. Most tumors (16/21) showed predominantly classic morphology, comprised of alternating hyper- and hypocellular areas of primitive small cells and differentiating rhabdomyoblasts in a loose myxoid/edematous stroma. A cambium layer was noted in all; seven had heterologous elements (six with fetal-type cartilage) and eight displayed focal anaplasia. The remaining five neoplasms showed only a minor component (

A 32-year-old woman with chemorefractory mullerian adenosarcoma showed clinical benefit in response to administration of lenvatinib plus pembrolizumab. In this case report, we describe the course of her illness and her response to this treatment.

Summary Mullerian adenosarcoma is an uncommon biphasic malignant uterine tumor. It is composed of benign epithelial and malignant stromal elements. We present a case of a 45-year-old woman who presented with post-menopausal bleeding for three months. She had a significant past medical history of pelvic irradiation for squamous carcinoma of cervix 20 years ago. Pathology revealed adenosarcoma with sarcomatous overgrowth. The patient had a recurrence of pure sarcoma three months later and unfortunately succumbed to her disease. The role of radiation in the pathogenesis of adenosarcoma has been uncommonly described compared to its well established role in the development of carcinosarcoma. Our case fulfils the criteria for a radiation induced sarcoma. We review the salient clinical and pathological features of this uncommon lesion highlighting the importance of sarcomatous overgrowth in these lesions and the possible role of radiation in the development of these tumors.

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.

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

Extrauterine Mullerian adenosarcomas (MA) are rare and often associated with endometriosis. We report a 55-yr-old patient seen in consultation for abdominal pain and bloating. Imaging was suggestive of a left adnexal mass and "peritoneal carcinomatosis". Pathological examination of the specimen revealed a MA arising in the left fallopian tube, with sarcomatous overgrowth, diffuse peritoneal involvement and omental "caking". Next-generation sequencing identified a MEIS1-NCOA2 gene fusion, previously unreported in MA.

Mullerian adenosarcoma is a rare tumor. Around 200 cases have been reported till date. This tumor usually occurs in peri or postmenopausal women and asymptomatic presentation is rarely reported. Use of tamoxifen has been considered to be a risk factor for this tumor occurrence. We here report a woman who was referred due to detection of a suspected malignant mass on PET scan, which was done to follow up the patient after treatment of breast cancer. Patient had history of tamoxifen use for 5years. But she was asymptomatic. Total abdominal hysterectomy with bilateral salphingo-oophoectomy was done. Histological examination confirmed the diagnosis of mullerian adenosarcoma. Post-operative course was uneventful and radiotherapy was given later in a nuclear medicine centre. This article wishes to emphasize the importance of recognizing this tumor for both gynecologists and pathologists. Uterine adenosarcoma has variable survival rate depending on its sarcomatous component, lympho-invasion, and myoinvasion. This patient showed no lymph invasion and no myoinvasion, which account for her good prognosis. Oncologists and gynecologists should be aware that this tumor can occur in patients with history of tamoxifen use, as a positive correlation has been seen in reported cases.

Background Uterine adenosarcoma, a rare uterine tumor subtype, is a biphasic tumor consisting of epithelial and mesenchymal elements. To date, there is no research comparing the histopathological features and immunohistochemistry of primary and recurrent tumors; furthermore, the relationship between pathology and the clinical course remains unclear. We reviewed the pathology and immunohistochemical features of patients with adenosarcoma and investigated the relevance of the histomorphological features to the clinical course. We also compared the immunohistochemical features of the primary and recurrent tumors. Methods The data of seven patients with adenosarcoma who underwent surgery in our hospital were evaluated. We performed immunohistochemical staining for the progesterone receptor, estrogen receptor, p53, and two Switch/Sucrose Non-Fermentable chromatin remodeling proteins (SMARCA4, BCOR), which were recently developed for the undifferentiated sarcoma diagnosis in addition to conventional staining methods. Results All patients had International Federation of Gynecology and Obstetrics stage IB–IC diseases. All tumors were polypoid and every patient presented with abnormal uterine bleeding. Six patients aged over 50 years and were menopausal; one patient aged under 50 years and was non-menopausal (average age: 59.1 years). Histologically, the sarcomatous components were homologous and heterogenous in six and one patient, respectively. Four and three cases were recurrent and non-recurrent, respectively. The recurrent patients showed high-grade morphology with sarcomatous overgrowth and were negative for ER and PR. Three recurrences could be evaluated by imaging, showing recurrence only in a distant area; biopsy specimens from these tissues revealed the identical mesenchymal component found in the primary tumor without a benign epithelial component. Immunohistochemical staining results were also similar to the corresponding of the original tumor, except for the p53 expression in one patient. At the primary site, p53 was overexpressed in two recurrent patients and had a wild-type level in one recurrent patient; however, all three recurrent tissues showed p53 overexpression. None of our patients showed SMARCA4 loss, and BCOR expression was positive in one case. Conclusions Initial pathological adenosarcoma analysis with appropriate immunohistochemical staining is vital for prognostic assessment. p53 expression might increase at recurrence. SMARCA4 and BCOR might not be an index of malignancy.

Highlights • A woman with a history of Stage IA low-risk uterine adenosarcoma presented with shortness of breath and rib pain. • She was found to have recurrent metastatic disease with resultant fatal SVC Syndrome. • Better methods to determine which patients with uterine adenosarcoma are at risk of recurrence and death are needed.

Mullerian adenosarcoma (MAS) is a biphasic tumor with malignant stroma. It is most commonly of endometrial origin but occasionally originates in the cervix, ovary, or other pelvic/peritoneal sites. The typical MAS is low grade with an indolent clinical course; however, tumors with sarcomatous overgrowth (SO) or a high-grade sarcoma tend to be aggressive. Tumor etiology is largely unknown. To better understand the global genome alterations and gene mutations in MAS, whole-genome sequencing (WGS) and target validation analysis were performed. MAS showed remarkable chromosome (chr) copy number variation (CNV), specifically, gains in chr 1q, 5p, 12p, 12q, and 17q and losses in chr 3p, 3q, 9p, and 11q. Gain of chr 12q13-15 was present in 50% of cases. The selected gene products in gain regions were upregulated as measured by immunohistochemistry. HMGA2 overexpression was significantly correlated with SO. While the structural variation (SV) rate was relatively low overall, a disproportionally high rate of break-ends at chr 7 was noted involving 6 in-frame rearrangement fusion genes. Among 40 frequently mutated genes detected by WGS and validated in 29 MAS by next generation sequencing (NGS), KMT2C, and BCOR were frequently seen in MAS both with and without SO, while MAGEC1 and KDM6B were strongly associated with SO. Overall, a higher rate of frequently mutated genes was found in MAS with SO (33%) than MAS without (11%). This study uncovers the complex and specific genetic alterations in this malignancy. The findings provide a tool for future investigation of these molecular changes in tumorigenesis and target therapies.

Müllerian adenosarcoma is a very rare gynecological disease, comprising 5% of uterine sarcomas. Extragenital localizations are even rarer. We report a very interesting case of a 27-year-old woman complaining of pelvic pain, with a subsequent diagnosis of extragenital Müllerian adenosarcoma. This is the first case reported in the literature with a complete and wide imaging description. Even if rare, Müllerian adenosarcoma should be hypothesized in case of young female patients presenting with suspicious pelvic mass.O adenosarcoma Mülleriano é uma doença ginecológica muito rara, compreendendo 5% dos sarcomas uterinos. Localizações extragenitais são ainda mais raras. Relatamos um caso muito interessante de uma mulher de 27 anos queixando-se de dor pélvica com diagnóstico subsequente de adenosarcoma Mülleriano extragenital. Este é o primeiro caso relatado na literatura com uma descrição completa e ampla de imagem. Mesmo que raro, o adenosarcoma Mülleriano deve ser hipotetizado no caso de pacientes jovens do sexo feminino com massa pélvica suspeita.

WOS: 000413787700013, PubMed ID: 29085712, The aim of this study is to present a rare case of Mullerian adenosarcoma of the cervix with ovarian metastasis and sarcomatous overgrowth. A gravida 2, para 2 woman aged 32 years with vaginal bleeding was admitted to the gynecology department. A 3-4 cm polypoid mass protruding from the cervix was detected in a pelvic examination. Total abdominal hysterectomy and bilateral salpingoopherectomy was performed because of metastatic implants on the right ovary. The pathologic evaluation revealed Mullerian adenosarcoma of the cervix with sarcomatous overgrowth and ovarian metastasis. After surgery, the patient was planned to undergo chemo- and radiotherapy. This is the first cervical Mullerian adenosarcoma case mentioned in the literature with metastasis to the ovary in a young woman. There is no optimal management option for cervical adenosarcomas due to the rarity of this phenomenon. Nevertheless, even if the patient is young and imaging techniques do not elucidate metastatic disease, surgeons should evaluate the ovaries for the spread of tumor, especially if histology reveals sarcomatous overgrowth.

Müllerian adenosarcomas usually arise as polypoid masses in the endometrium of post-menopausal women. Occasionally, these tumors arise in the cervix, vagina, broad and round ligaments, ovaries and rarely in extragenital sites; these cases are generally associated with endometriosis. We experienced a rare case of extraendometrial, intramural adenosarcoma arising in a patient with adenomyosis. A 40-year-old woman presented with sudden-onset suprapubic pain. The imaging findings suggested leiomyoma with cystic degeneration in the uterine fundus. An ill-defined ovoid tumor with hemorrhagic degeneration, measuring 7.5 cm in diameter, was detected. The microscopic findings showed glandular cells without atypia and a sarcomatous component with pleomorphism and high mitotic rates. There was no evidence of endometrial origin. To recognize that adenosarcoma can, although rarely, arise from adenomyosis is important to avoid overstaging and inappropriate treatment.

Highlights • DICER1 mutations play a significant role in gynecologic malignancy. • DICER1 may be involved in the sarcomagenesis of endometrial adenosarcoma. • The knowledge of a genetic mutation can help clarify a patient's medical history.

Rationale: Uterine adenosarcoma (UA) with sarcomatous overgrowth (ASSO) is a rare and aggressive disease. Herein, wereported the case of a young patient with advanced uterine ASSO. Patients concerns: A 29-year-old woman with the diagnoses of endometrial polyp and adenomyosis underwent hysteroscopic endometrial polypectomy for the giant endometrial polyp. Postoperative regular ultrasound scan indicated thickened endometriumand an ill-defined mass with continuous enlargement in the myometrium of the posterior wall of the uterus, which was considered as an adenomyoma. Two years after hysteroscopy, she was re-admitted due to lower abdominal distension and large pelvic mass. At that time, she had taken oral short-acting contraceptives for 2.5 years. Diagnoses: Magnetic resonance imaging (MRI) of the pelvis revealed an irregular mass with the size of 12∗56∗107 mm3 in the right annex area, without distinct border with the rectum, moreover, an uneven intrauterine echo that has no obvious boundary with uterine wall. Right ovarian cancer and adenomyoma were initially considered. Interventions: The patient received transperitoneal retroperitoneal pelvic combined with total viscera resection, including uterus, bilateral appendages and rectum, omentectomy, appendectomy, lymphadenectomy, and ileostomy. Postoperative pathology confirmed ASSO in the uterine cavity and muscular layer, the whole cervical duct and the right adnexal. She underwent 2 systemic chemotherapy sessions after the surgery. The chemotherapy regimen was ifosfamide 2.5 g day 1 to 3, with liposomal doxorubicin 40 mg day 1. Outcomes: The final diagnosis was uterine ASSO, International Federation of Gynecology and Obstetrics stage IVa. The patient has been following-up so far, with no progression. Lessons: Review of the case indicated that history of long-term oral short-acting contraceptives and giant endometrial polyps may be the high-risk factors for UA. For patients with high-risk factors, the follow-up ultrasound scan should be more frequently conducted. Moreover, 3D-ultrasound, MRI and outpatient hysteroscopy are recommended for routine screening. Placement of levonorgestrel-releasing intra-uterine system after hysteroscopy may be an effective intervention for patients with a high risk of giant polyps. Cluster of Differentiation 10, Estrogen receptor, Progesterone receptor, and nuclear antigen may be predictors for prognosis and selection of individualized treatment program.

Mullerian adenosarcoma (MA) is a rare tumor variant with low malignancy potential and is reported to account for 8% of all uterine sarcomas. Cervical MAs are reported to occur in relatively younger patients with the mean age of 27 years, while those in the uterine corpus generally present in postmenopausal women. Due to the rarity of cervical MAs, optimal management for these patients (especially younger women) is still under exploration. Here, we describe a case of cervical MA in a woman of reproductive age who was treated by fertility-preserving surgery and successfully delivered a child 18 months later.

Müllerian adenosarcoma (MA) is an uncommon biphasic neoplasm of the female genital tract, composed of malignant stroma and benign epithelium. Little is known about the molecular and cytogenetic aberrations in MA pathogenesis, including those with progression to sarcomatous overgrowth (SO). Herein, we report all cases of MA in which karyotyping was attempted at our institution. Twenty-one samples from 20 subjects consisted of 15 primary (7 without SO, 8 with SO) and 6 metastatic MA, were cytogenetically investigated in our institution. Karyotypes were successfully obtained in 14/21 (67%) cases and 9 (45%) had cytogenetic aberrations. Two (1 MA with SO and 1 metastatic MA) were markedly complex, displaying extreme aneuploidy with numerous rearrangements. Seven (2 MA without SO, 3 MA with SO, and 2 metastatic MA) demonstrated noncomplex clonal aberrations, of which 5 (71%) included an abnormality involving chromosome 8. Two tumors had rearrangements at 8q13 and another 3 tumors had extra copies of chromosome 8. In 5 cases, a normal karyotype (46,XX) was obtained (2 MA without SO, 2 MA with SO, and 1 metastatic MA). Further study is warranted to explore the genetic mechanism by which chromosome abnormalities, particularly those at 8q13, contribute to MA tumorigenesis.

Uterine adenosarcomas are uncommon tumors. It is a biphasic tumor with both epithelial and mesenchymal component. The epithelial component is benign in nature, and the mesenchymal component is malignant. Metastasis is rare in adenosarcoma. We report a case of adenosarcoma with lymph nodal metastasis. A 20-year-old female presented with history of per vaginal bleeding for 1 month. Per vaginal examination revealed a fungating mass protruding through the cervical os. Ultrasonography and magnetic resonance imaging showed a large intrauterine mass. Biopsy of the mass done at an outside hospital was reported as rhabdomyosarcoma. Hence, she was given one cycle of neoadjuvant chemotherapy. Following this, she had profuse bleeding. Emergency hysterectomy with pelvic lymph nodal dissection was performed. The final histopathology was reported as adenosarcoma. One pelvic lymph node showed metastatic deposit of rhabdomyosarcomatous element. In young females presenting with polypoidal mass, uterine adenosarcoma can be considered in the differential diagnosis.

Uterine adenosarcomas are an uncommon gynecological neoplasm consisting of benign epithelial and malignant mesenchymal components and usually presenting as a polypoid mass. We report herein two cases of uterine adenosarcoma. Both of the patients were obese/overweight (body mass index 32 kg/m2 and 27 kg/m2, respectively), and controlling obesity and metabolic disorders seemed to be the key to prevent their post-operative tumor recurrence. You can submit your Manuscripts at: https://symbiosisonlinepublishing.com/submitManuscript.php

Dedifferentiated carcinoma is defined as undifferentiated carcinoma coexisting with a second component of FIGO grade 1 or 2 endometrioid carcinoma. It is a rare entity with highly aggressive behavior. Dedifferentiated carcinoma combined with another primary uterine tumor is even rarer. We describe a case containing 3 different morphologies comprised of a dedifferentiated carcinoma associated with a low-grade endometrioid carcinoma coexisting with a low-grade Mullerian adenosarcoma. We also used targeted genomic analysis to show all 3 components arise from the same founding clone and identify novel mutations that drive tumor progression.

Adenosarcomas are the rarest form of uterine sarcomas, and clinical experience with their management is still limited. Here, we reported 7 patients with uterine adenosarcoma referred to our institution, focusing on main pathologic features, their medical history, and long-term follow-up. Among these patients, we provided a detailed description of the medical history of a 49-year-old woman with advanced uterine adenosarcoma with sarcomatous overgrowth who presented a brilliant radiologic and pathologic response after 3 cycles of epirubicin and ifosfamide, ultimately achieving an extraordinary long-term outcome through an integrated surgical and medical approach. Our single-centre experience would suggest that aggressive uterine adenosarcomas with sarcomatous overgrowth are sensitive to standard epirubicin and ifosfamide and that an integrated approach, both medical and surgical, could be considered in clinical practice, again emphasizing the relevant role of multidisciplinary management for this extremely rare disease.

Background. A malignant mixed Müllerian tumor (MMMT) is a malignant neoplasm found in the uterus, the ovaries, the fallopian tubes, and other parts of the body that contains both carcinomatous (epithelial tissue) and sarcomatous (connective tissue) components. Outcome of MMMTs is determined primarily by depth of invasion and stage. The metastatic background of these lesions is controversial and unknown.Case Report. A 75-year-old woman was admitted to the hospital with anorexia, weakness, and persistent coughing. The imaging exams revealed a solid, promiscuous lesion of 16 × 14 cm in dimensions located into the small pelvis, surrounding the uterus and the ovaries. The patient underwent exploratory laparotomy. The mass was removed and the histological examination of the specimen revealed an advanced mesodermal adenocarcinoma of the ovary (MMMT). Nine days after the operation the patient presented with metastatic lesions in the mouth as well as the lungs. Within a month after the discharge from the hospital metastatic lesions of the MMMT were also depicted in the CT brain scan.Conclusion. Despite the fact that sarcomas have a long-term metastatic potential, to our knowledge this is the first case of Müllerian adenosarcoma presenting with such extraperitoneal metastases.

Conservative treatment of a mullerian adenosarcoma of the uterine cervix Abstract Mullerian adenosarcoma of the cervix is a rare tumor that usually presents in young women as a bleeding cervical polyp. There is a tendency for local recurrence due to their low malignant potential. The optimal therapy for these tumors is uncertain but conservative treatment can be provided in selected patients to preserve female fertility. Long-term follow-up is of paramount importance. We present the case of a 19-year-old girl who was diagnosed with a Mullerian adenosarcoma of the uterine cervix treated with a wide cervical conization.

Background Mullerian adenosarcomas of the cervix are composed of benign epithelial and malignant stromal components. The purpose of this report is to describe the clinical and histologic difficulties in diagnosis and to propose fertility-preserving management of low-grade lesions. Case A 14-year-old girl presented with a friable lesion found to originate from the anterior cervical lip. Initially, clinical suspicion was for sarcoma botryoides, however, pathologic evaluation revealed a low-grade cervical Mullerian adenosarcoma. Cold knife conization was performed, and the mass was resected with clear margins. Summary and Conclusion Mullerian adenosarcoma of the cervix is difficult to diagnose in adolescents because of features more commonly associated with alternative diagnoses. For patients with low-grade lesions desiring future fertility, local excision with close follow-up is reasonable.

OBJECTIVE. Uterine adenosarcoma (UAS) is a rare gynecologic malignancy and the significance of lymph node metastasis on survival has not been well studied. METHODS. A retrospective study was performed utilizing the Surveillance, Epidemiology, End Results Program to examine UAS (n = 994), endometrial stromal sarcoma (ESS, n = 2910), and uterine leiomyosarcoma (LMS, n = 5506) diagnosed between 1973 and 2013. The impact of lymph node metastasis on cause-specific survival (CSS) was cross-compared by multivariable analysis. Systematic literature review was conducted to examine the impact of nodal metastasis on progression-free survival (PFS) in UAS. RESULTS. UAS had the lowest incidence of lymph node metastasis among the sarcoma subtypes examined (UAS 2.9%, LMS 3.4%, and ESS 6.6%, P < 0.001). Lymph node metastasis was independently associated with decreased CSS in all three tumor types (all, P < 0.01); however, magnitudes of statistical significance of lymph node metastasis for CSS were similar across the three tumor types: adjusted-hazard ratio (aHR) for UAS 2.34, ESS 2.43, and LMS 2.10. Systematic literature review identified 230 unique cases of surgically treated UAS. On multivariable analysis, lymph node metastasis (aHR 4.72) had the greatest degree of significance for PFS compared to other tumor factors including sarcomatous overgrowth (aHR 2.88), heterologous elements (aHR 2.08), and deep myometrial invasion (aHR 1.51). Large tumor, deep myometrial invasion, and sarcomatous overgrowth were associated with increased risk of lymph node metastasis (all, P < 0.05). CONCLUSION. While uterine adenosarcoma had a low incidence of lymph node metastasis, the impact of lymph node metastasis on survival was comparable to ESS or LMS.

Background. Mullerian adenosarcoma with sarcomatous overgrowth (MASO) is a very rare variant of uterine sarcomas first described by Clement et al. as early as 1974. The presence of heterologous sarcomatous components is associated with aggressive biological behavior.Case Presentation. This is a case report of a 62-year female (P2 + 0) presenting with postmenopausal vaginal bleeding. Her preoperative USG revealed subserosal fibroid with adherent omentum. She underwent abdominal hysterectomy with bilateral oophorectomy. Histopathological diagnosis of resected specimen was Mullerian adenosarcoma with sarcomatous overgrowth and presence of heterologous elements involving body of the uterus. The whole thickness of the myometrium was involved along with the presence of serosal nodules and omental deposits of sarcomatous component.Conclusion. MA is considered as a low-grade malignant tumor, but MASO is a high-grade tumor frequently associated with invasion and metastasis with poor treatment outcome. Because of its rarity, correct identification of these tumors and distinction from other uterine sarcomas are a challenging job and hence its morphological features merits attention.

Mullerian adenosarcoma is a rare biphasic malignant neoplasm of cervix characterized by an admixture of benign epithelial elements and a malignant sarcomatous stromal component, which may be either homologous or heterologous. Mullerian adenosarcoma with stromal overgrowth (MASO) in an aggressive variant of adenosarcoma, which is extremely rare with only two such cases reported till date. In this report, we present a case of MASO of cervix with heterologous elements in a 55/F presenting with postmenopausal bleeding. As it commonly simulates clinically and radiologically as benign cervical polyp, the gynecologists and pathologists should be aware of this extremely rare entity presenting with aggressive clinical course.

e17569Background: Surgery is potentially curative in patients with stage I Endometrial Stromal Sarcoma (ESS) and uterine adenosarcoma; however, 20-40% patients will develop recurrence, suggesting n...

Mullerian adenosarcoma is an uncommon, but not rare, mixed tumor containing a neoplastic but benign or mildly atypical epithelial element and a sarcomatous, usually low-grade, stromal component. The most common site is the uterine corpus but adenosarcoma also occurs in the cervix and ovary and more rarely in the vagina, fallopian tube, arising from peritoneal surfaces, or outside the female genital tract, for example in the intestine. Most uterine cases have a polypoid gross appearance, sometimes resulting in the formation of multiple polyps. Characteristic histologic features include a low power "phyllodes-like" architecture with leaf-like projections lined by a variety of benign Mullerian type epithelia, sometimes with squamous metaplasia. Intraglandular stromal protrusions are a characteristic feature. The stroma may be uniformly cellular but there is typically increased cellularity around the epithelial elements, resulting in the formation of a cambium layer. Using the World Health Organization definition, stromal mitotic activity of 2 or more per 10 high-power fields is required for a diagnosis of adenosarcoma but in practice the diagnosis is made with stromal mitotic activity less than this if the characteristic architecture and cambium layer is present. The stromal component is usually morphologically "low-grade" and of endometrial stromal or fibroblastic type (hormone receptor and CD10 positive). Sometimes it is high grade, resembling undifferentiated sarcoma. Additional features sometimes present include heterologous stromal elements or sex cord-like differentiation. Uterine adenosarcomas are, in general, low-grade neoplasms capable of local recurrence after polypectomy or hysterectomy and much less commonly distant metastasis. The 2 most important adverse prognostic factors, which sometimes coexist, are deep myometrial invasion and sarcomatous overgrowth; the latter is usually associated with morphologically "high-grade" stromal elements with loss of expression of hormone receptors and CD10. Adenosarcoma may be confused with a variety of lesions and one of the main differential diagnoses is adenofibroma in which the stromal component is, by definition, morphologically benign. However, occasional adenofibromas recur or even metastasize. As such, it has been suggested that all adenofibromas should be classified as adenosarcomas, albeit with low-malignant potential. Ovarian adenosarcomas are much more likely to exhibit malignant behavior than their uterine counterparts, probably due to the lack of an anatomic barrier to peritoneal dissemination.

Background Mullerian adenosarcoma is a rare mixed epithelial–mesenchymal tumor. An extragenital site of origin and sarcomatous overgrowth are associated with aggressive clinical behavior. Case We present a rare case of extragenital adenosarcoma with sarcomatous overgrowth and coexistent endometriosis. She was treated with initial cytoreductive surgery and chemotherapy. She underwent a second surgery for management of a high-grade bowel obstruction, due to pathologically confirmed recurrent intraperitoneal adenosarcoma. A complete clinical response was achieved with liposomal doxorubicin, and the patient remains disease-free eighteen months after completion of chemotherapy. Conclusion Liposomal doxorubicin appears to be an active agent for the treatment of adenosarcoma with sarcomatous overgrowth. In addition, we conclude from our review of all reported cases of extragenital adenosarcoma that concurrent endometriosis may represent a favorable prognostic factor.

A case of müllerian adenosarcoma arising in ovarian endometriosis is reported in which the whole of the mesenchymal component exhibited striking deciduoid morphology, a phenomenon that has not been previously described. The patient was not taking hormonal preparations. We discuss the differential diagnosis and the possible pathogenesis of the deciduoid stromal alteration.

Mullerian adenosarcoma (MS) is a rare neoplasm of uterine cervix composed of benign epithelial and malignant stromal components. An aggressive variant of adenosarcoma, mullerian adenosarcoma with sarcomatous overgrowth (MASO) is extremely rare. The difference between MS and MASO is the pure high grade sarcoma features in MASO. In this report we present a MASO case, derived from uterine cervix of a 60 year-old-female patient presenting as a cervical polypoid mass, to our knowledge the second case of the English literature. In spite of sarcomatous overgrowth, high mitotic activity and huge tumor size of 12,5 cms, it displayed no myometrial invasion, vascular invasion and heterologous elements. The patient has been clinically free of disease for 14 months of follow up after total abdominal hysterectomy and bilateralsalpingo-oopherectomy. The difficulties in diagnosis and treatment of this entity will be evaluated in this report.

Müllerian adenosarcoma is a distinctive type of mixed Müllerian tumor of the female genital tract. To our knowledge, no cytogenetic data have been documented on Müllerian adenosarcoma in the literature so far. We report here the chromosomal findings of a Müllerian adenosarcoma in a 15-year-old female. Cytogenetic and molecular cytogenetic analysis revealed a complex karyotype involving chromosomes 2, 8, 10, 13, 19, and 21. These numerical and structural abnormalities may be of etiologic significance. This report may highlight the potential value of molecular cytogenetic analysis in differential diagnosis of Müllerian tumors. More cases are warranted to further genetically characterize this type of neoplasm.

We report four cases of mixed mullerian tumors of the endometrium arising in postmenopausal women taking tamoxifen for breast carcinoma. Various histopathologic features were encountered: one mullerian adenosarcoma and three malignant mixed mullerian tumors so called carcinosarcomas (in one case the sarcomatous component was homologous composed of tissues normally found in the uterus, while the others were heterologous because they were containing some elements normally not found in the uterus). Concern has been raised about prolonged tamoxifen treatment and subsequent occurrence of endometrial adenocarcinoma. Then, attention has been drawn through high-risk histologic subtypes including poorly differentiated patterns, uterine sarcomas and such mixed mullerian tumors. There is no consensus regarding internal surveillance of women receiving tamoxifen. The usefulness of pelvic sonography has not been demonstrated.

Background. Mullerianadenosarcoma is a rare pelvic malignancy that most commonly arises from the endometrium. These tumors are relatively insensitive to chemotherapy and radiation and are primarily treated by surgical resection. We report a case of mullerian adenosarcoma arising outside of the uterus from a background of endometriosis treated with a combination of surgical resection and medroxyprogesterone acetate. Case. A 43-year-old woman with a history of endometriosis was diagnosed with advanced extrauterine mullerian adenosarcoma. After suboptimal tumor dubulking surgery she was treated with medroxyprogesterone acetate. Ten months postoperatively she remains without evidence of disease. Conclusion. Medroxyprogesterone acetate may be a useful drug in the treatment of advanced mullerian adenosarcoma.

We report a primary peritoneal mullerian adenosarcoma with sarcomatous overgrowth associated with endometriosis in a 50-year-old female. The tumor formed multiple peritoneal masses occupying the pelvis and subdiaphragmatic space. Histologically, the tumor was composed of benign-appearing mullerian glands surrounded by a sarcomatous stroma. In addition, one perisplenic mass was largely devoid of the epithelial component and was of higher grade than the remaining lesions. Multiple foci of endometriosis were associated with the pelvic masses. To our knowledge, primary peritoneal mullerian adenosarcoma with sarcomatous overgrowth associated with endometriosis has not been previously reported.

Mullerian adenosarcomas of the female genital tract are rare malignancies, originally described in the uterus, the most common site of origin, but they may also arise in extrauterine locations. Uterine adenosarcomas make up 5% of uterine sarcomas and tend to occur in postmenopausal women. They are usually low-grade tumors and are characterized by a benign epithelial component with a malignant mesenchymal component, which is typically a low-grade endometrial stromal sarcoma but can also be a high-grade sarcoma. Tumors that exhibit a high-grade sarcomatous overgrowth have a worse outcome. Adenosarcomas have been described as being midway along the spectrum between benign adenofibromas and carcinosarcomas. They generally have a good prognosis with the exception of deeply invasive tumors or those with high-grade sarcomatous overgrowth. Extrauterine adenosarcomas also have a higher risk for recurrence. In view of their rarity, there have not been any clinical trials in mullerian adenosarcomas and relatively little research. This article reviews the current knowledge and provides recommendation for the management of mullerian adenosarcomas.

A case of extrauterine Müllerian adenosarcoma of the peritoneum in a 20-year-old woman is reported. The tumor was widely based on the abdominopelvic wall and there were no unusual features in the genital organs. The cut surface of the tumor showed a marked gelatinous appearance. The tumor was composed of an admixture of benign Müllerian-type epithelium and sarcomatous stroma. The predominant element of the sarcomatous area was rhabdomyosarcoma, which showed a close resemblance to well-differentiated embryonal rhabdomyosarcoma. In another sarcomatous area, fibroblastic cells without myoblastic properties diffusely proliferated in a marked myxoid background with some collagen bundles. Both the mitotic count and Ki-67 proliferative index of these cells were lower than those of rhabdomyoblastic cells. On follow up, the patient was disease free for 1 year postoperatively, without any subsequent treatment. The present case indicates that extrauterine adenosarcoma can also show histological heterogeneity as do uterine adenosarcomas. The remarkable myxoid change of this tumor seemed to be more largely due to a fibromyxoid element than a rhabdomyosarcomatous element, and the coexistence of the former may be related to the less aggressive behavior of this tumor.

Müllerian adenosarcoma with sarcomatous overgrowth presented by a 52-year-old female patient after adjuvant tamoxifen treatment for breast carcinoma is described. The diagnosis was made on histological basis after curettage and complementary total hysterectomy with bilateral salpingo-oophorectomy. The immunohistochemical study showed high expression of estrogen receptors in the epithelial component of the lesion and irregularly positive findings in the stroma. The proliferative activity evaluated by Ki-67 immunoexpression was higher in the stroma than the epithelium. Some of the stromal cells showed rhabdomyoblastic differentiation. The association of tamoxifen use and development of mesenchymal neoplasms is discussed. É descrito o caso de uma paciente do sexo feminino, 52 anos, com adenossarcoma Mülleriano com componente sarcomatoso predominante, que se apresentou após tratamento adjuvante com tamoxifeno para câncer de mama. O diagnóstico foi feito em bases histológicas após curetagem uterina e histerectomia total complementar com anexectomia bilateral. O estudo imuno-histoquímico mostrou alta expressão de receptores de estrogênio no componente epitelial da lesão e positividade irregular no estroma. A atividade proliferativa avaliada através da imunoexpressão do Ki-67 foi maior no estroma do que no epitélio. Algumas células estromais mostraram diferenciação rabdomioblástica. A associação entre uso de tamoxifeno e desenvolvimento de neoplasias mesenquimais é discutida.

Müllerian adenosarcoma is a rare neoplasm that can arise in both uterine and extrauterine locations. This report describes the ultrasound and magnetic resonance imaging findings of one case of ovarian adenosarcoma and reviews the literature as to the previously described imaging findings. Adenosarcoma should be considered in patients with a predominantly solid pelvic mass on imaging, particularly in those with a history of endometriosis or findings compatible with endometriosis on ultrasound or magnetic resonance imaging. A very low resistive index on ultrasound may also be suggestive of this diagnosis.

Highlights • Two cases of large cervical mullerian adenosarcoma with sarcomatous overgrowth or heterologous elements and contrasting survival outcomes are reported. • When the diagnosis of mullerian adenosarcoma is uncertain or suspected, review of pathology by a national expert may be considered. • Rhabdomyoblastic differentiation of mullerian adenosarcoma may be a more aggressive histologic type.

Background: Extragenital Müllerian adenosarcomas are extremely rare tumours characterised by a stromal component of low-grade malignancy and by a benign glandular epithelial component. Case Report: A 26-year-old woman was admitted to our clinic because of lower abdominal distension and left lower quadrant pain. Clinical and radiological examinations suggested an ovarian malignancy. Laparotomy revealed a cystic mass in the pouch of Douglas, originating from the left sacrouterine ligament. A total excision of the tumour was performed and showed low-grade adenosarcoma without sarcomatous overgrowth. Follow-up at 24 months after the surgery showed no evidence of recurrence. Conclusion: Mullerian adenosarcoma located in the pouch of Douglas is rare. For treatment, success may be achieved with only excision of the tumour if there is no sarcomatous overgrowth or spread to adjacent tissues.

Background Endometrial stromal sarcoma (ESS) is a term used to define a rare neoplasm that accounts for approximately 0.2%-1% of all uterine malignancies; it is, however, implicated in an estimated 10%-15% of those malignancies with a mesenchymal component. Recent evidence suggests that while the preservation of the ovaries may be considered appropriate in premenopausal women, hysterectomy and bilateral salpingo-oophorectomy remains the recommended treatment in postmenopausal women. Currently, only a few case series reporting the treatment of ESS in young women with a desire to preserve fertility and thus subjected to a fertility-sparing surgery are available in the literature. Case presentation We report a peculiar case of early stage ESS treated by laparoscopic fertility-sparing surgery and a strict follow-up program (every 3 months) of imaging and clinical evaluation. The patient remained disease free 1 year after primary treatment. Three months after completing oncological follow-up, the patient conceived spontaneously and is, to date, pregnant at 11 weeks of gestation without evidence of recurrent disease or obstetric complications. Conclusion Based on our case report and in accordance with the data available, we suggest that in young patients affected by early stage ESS who wish to preserve reproductive function, fertility-sparing surgery could represent a valid option, though strict oncological follow-up remains mandatory.