Müllerian adenosarcoma with sarcomatous overgrowth and heterologous elements: A case report and literature review.

Uterine mullerian adenosarcoma (MA) is a rare biphasic tumour that accounts for less than 0.5% of uterine neoplasms. The age range of presentation is wide, with the median age in the 5th decade of life. It usually has a good prognosis; however, it worsens when it presents with sarcomatous overgrowth, heterologous elements or infiltrates the myometrium. We report the case of a 63-year-old woman presenting with abnormal vaginal bleeding and a sensation of solid material coming out of the cervical canal who was diagnosed with mullerian adenosarcoma with sarcomatous overgrowth (MASO) and presence of heterologous elements after performing a mass biopsy and subsequent hysterectomy. We reviewed the literature, focusing especially on the differential diagnoses to be evaluated, as well as the differences in prognosis and treatment according to whether or not they present histologic features of poor prognosis.

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.

Uterine adenosarcoma: Clinical significance of histological classification and SNP array analysis.

Mullerian adenosarcoma is a rare malignant biphasic tumor. The mesenchymal component may be low or high grade, with or without sarcomatous overgrowth (SO). Little is known about the molecular heterogeneity of these tumors. In this study, we aim to reclassify a large retrospective monocentric cohort of uterine adenosarcomas according to tumor grade and SO, to evaluate the clinical significance of pathological classification and to correlate with copy-number variations inferred from single nucleotide polymorphism array. Of the 67 uterine adenosarcomas, 18 (26.9%) were low grade without SO, 7 (10.4%) low grade with SO, 8 (11.9%) high grade without SO and 34 (50.7%) high grade with SO. SO, necrosis and RMS were more frequent in high grade than low grade adenosarcomas (p < 0.001). Low-rank test showed that recurrence-free survival was significantly shortened in high grade than low grade adenosarcomas (p = 0.035) and SO was associated with shortened overall and recurrence-free survival (p = 0.038 and p = 0.009, respectively). High-grade tumors displayed complex genomic profiles with multiple segmental losses including TP53, ATM and PTEN genes. The median genomic index was significantly higher in high grade than low grade tumors (27 [3-60] vs 5,3 [0-16], p < 0.0001) and was significantly higher in presence of SO in low grade tumors (12,8 [10-16] vs 2,6 [0-10], p = 0.0006). We propose to report sarcomatous overgrowth with the tumor grade for prognostication in adenosarcoma and representative sampling is crucial for evaluation of these histological criteria.

A Case Report of Uterine Müllerian Adenosarcoma With Sarcomatous Overgrowth.

Uterine adenosarcoma remains a highly aggressive tumor and is less described in the literature, with an unfavorable prognosis and an increased risk of local and distant recurrence. However, surgery, chemotherapy, and radiotherapy offer local control of the disease, and overall survival remains reduced. We report the case of a 79-year-old patient with stage IIIB uterine adenosarcoma, confirmed by immunohistochemistry and initially diagnosed with postmenopausal metrorrhagia. The patient was managed through a multimodal treatment by conducting a multidisciplinary consultation.

Ovarian adenosarcoma in a postmenopausal woman: Case report and review of literature.

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.

Uterine Adenosarcoma with Sarcomatous Overgrowth and Rhabdoid Features: A Rare Case.

Uterine adenosarcoma is usually a low-grade neoplasm with a mixed benign epithelial component and malignant stroma, commonly found in postmenopausal women. In the presence of sarcomatous overgrowth, it has been shown to have poor prognosis. Uterine adenosarcoma with sarcomatous overgrowth and rhabdoid features is extremely rare. We report here a case of a 28-year-old female who was found to have adenosarcoma with sarcomatous overgrowth with extensive rhabdoid features. The tumor had metastasized to the pelvis, omentum, iliac, and obturator lymph nodes. She was lost to follow-up for 10 months, after which she presented with recurrent tumor at the hysterectomy site. She was started on palliative chemoradiotherapy, on which she progressed but later experienced drug toxicity, became cachectic, and was unwilling to continue chemotherapy. There are a few cases of adenosarcoma with sarcomatous overgrowth reported in young women and only two cases with rhabdoid features. Based on this report, adenosarcoma with sarcomatous overgrowth and rhabdoid features appears to be an extremely aggressive tumor with poor prognosis.

High grade uterine adenosarcoma with sarcomatous overgrowth in a young woman amenable to primary surgical reduction: A case study and literature review.

Mullerian adenosarcomas are rare and often low-grade mixed tumors that typically respond well to optimal surgical resection. However, adenosarcoma with sarcomatous overgrowth (ASSO) is a high-grade mixed tumor commonly associated with invasion, metastasis, and a poor prognosis. The health care providers herein report a case study of a patient diagnosed with ASSO who has maintained remission status for 19 months following radical surgical resection alone. The patient, a 24-year-old Caucasian female without significant medical history, initially complained of abdominal fullness, pelvic pressure, altered menses, and unintentional weight loss. A necrotic cervical mass was present on the exam; mass biopsy revealed spindle cell sarcoma with rhabdomyosarcomatous differentiation. The patient underwent exploratory laparotomy, total abdominal hysterectomy, bilateral salpingectomy, radical tumor debulking, and pelvic and periaortic lymph node dissection. Histopathological diagnosis of the resected specimen was consistent with ASSO, limited to 0.7 cm out of 2.0 cm of myometrial thickness, with negative lymph node and parametrial tissue, consistent with Stage IB disease. She did not receive adjuvant chemotherapy or radiation and has remained disease-free to date. Because of the rarity of ASSO and lack of abundant case study reports, uniform clinical guidelines for treatment following surgical resection of a high-grade adenosarcoma remain unclear. However, the case study below may suggest that radical surgical debulking of this disease with negative margins in young patients with early-stage disease can be sufficient in treating high-grade ASSO, despite their typical aggressive nature.

Imaging findings of uterine adenosarcoma with sarcomatous overgrowth: two case reports, emphasizing restricted diffusion on diffusion weighted imaging.

BACKGROUND: Adenosarcoma is classified as a mixed epithelial and mesenchymal tumor composed of a benign epithelial component and a malignant stromal component. The stromal component in adenosarcoma is usually low grade, and consequently the prognosis is relatively favorable. While, adenosarcoma with sarcomatous overgrowth (SO) is defined as an adenosarcoma in which the sarcomatous component constitutes more than 25% of the tumor. The stromal component is also high-grade sarcoma showing greater nuclear pleomorphism and mitotic activity, thus, it is associated with worse prognosis. MRI findings of adenosarcoma without SO have been described in previous literatures but the imaging findings in adenosarcoma with SO may be poorly defined. Therefore we present two cases of uterine adenosarcoma with SO. CASE PRESENTATION: Patient 1 was a 76-year-old woman referred to our hospital with complaint of abdominal distension and postmenopausal bleeding. Patient 2 was a 57-year-old woman with complaint of lower abdominal pain and abnormal uterine bleeding. On magnetic resonance imaging (MRI), T2 weighted imaging showed a large, heterogeneous high-intensity mass with hyperintense tiny cysts that expanded the uterine cavity and extended into the cervical canal for both patients. On diffusion-weighted imaging (DWI), both masses appeared as high signal intensity. Patient 2 also had a right ovarian adult granulosa cell tumor that may have contributed to development of the adenosarcoma. Patient 1 recurred with peritoneal sarcomatosis 6 months after surgery and died of the disease. Patient 2 also recurred with a left upper lung metastasis 3 months after surgery. CONCLUSIONS: DWI may depict pathological changes produced by SO of adenosarcoma as high signal intensity, even though SO does not seem to change MRI findings of adenosarcoma on other sequences. Therefore, DWI could potentially predict SO in presumptive adenosarcoma on MRI and the patient's prognosis. It is also important for pathologists to know if SO can arise in adenosarcoma because they need to examine the tumor thoroughly to determine the percentage of SO component in the tumor volume when SO is present.

Müllerian Adenosarcoma: A Single-Centre Experience of 59 Cases of This Rare Entity.

Background and objective Müllerian adenosarcomas (MA) are rare biphasic tumors with benign epithelial and sarcomatous stromal components. There is very limited cohort study data on MA in the South Asian countries and no such study has been attempted in Pakistan. Our aim was to evaluate the clinicopathological characteristics of MA and to review the published literature on the condition. Additionally, we also analyzed the impact of various prognostic factors on the overall survival (OS) of patients with MA. Materials and methods This was a retrospective observational study performed at the Shaukat Khanum Memorial Hospital and Research Centre, Lahore from 2003 to 2020. A total of 59 histologically confirmed cases of MA were included in the study and critically reviewed. Results The mean age of the patients was 54 ±16 years, and the most common tumor location was the uterine corpus (48, 81.4%), followed by the cervix (eight, 13.6%), ovary (two, 3.4%), and vagina (one, 1.7%). Sarcomatous overgrowth (SO) was seen in 22 (37.3%) patients, and high-grade cytology was observed in 18 (30.5%) patients. Furthermore, lymphovascular invasion (LVI) was present in six (10.2%) patients, and myometrial invasion was noted in 25 (42.4%) patients. The follow-up details of 29 patients were available, and death was recorded in 13 (44.8%) patients with a median OS of three years. Conclusion MA is a rare and diagnostically challenging entity due to its wide differential diagnosis. It is essential to take note of different morphological features such as SO, cytological features, LVI, and heterologous differentiation because of their significant prognostic impact.

Immunohistochemical markers and the clinical course of adenosarcoma: a series of seven 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.

Post-radiation Mullerian adenosarcoma with sarcomatous overgrowth: rare presentation of an uncommon malignancy.

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.

Mullerian adenosarcoma with sarcomatous overgrowth in a premenopausal patient / Philippine Journal of Obstetrics and Gynecology

@#We report a case of a 33-years old, nulligravid, diagnosed with mullerian adenosarcoma with sarcomatous overgrowth (MASO), who presented with vaginal bleeding and recurrent endometrial polyp. MASO is a rare type of uterine sarcomas, it is a variant of adenosarcomas with poor prognosis. The index patient underwent primary surgical management with lymphadenectomy with a final stage of IC. Histologic diagnosis was Mulllerian adenosarcoma with sarcomatous overgrowth. A panel of immunostaining for estrogen receptors, progesterone receptors and CD 10 showed diffused positivity for the hormones with loss of CD 10 which is consistent with MASO. The rarity of MASO has a distinctive histologic features which merits meticulous sectioning as the clinical course and management vary. It has a poor prognosis due to its short and fast course of the disease.

Mullerian adenosarcoma (heterologous) of uterine cervix with sarcomatous overgrowth: A case report with review of the literature.

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.

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.

Prognostic factors for uterine adenosarcoma: a review.

INTRODUCTION: Uterine adenosarcoma is a rare tumor with both epithelial and stromal components. Standard treatment is total abdominal hysterectomy and bilateral salpingo-oophorectomy. There is no defined role for adjuvant or neoadjuvant chemotherapy or radiation. There is a misconception that this is an indolent, low-grade sarcoma. In fact, at least 50% of patients will develop disease recurrence. Establishing prognostic factors is of paramount importance. Areas covered: This article reviews the current literature regarding adenosarcoma prognostic factors from case reports, case series, and retrospective series. An extensive review of the literature was undertaken via PubMed and Medline searches, relevant articles are included in this review. Expert commentary: The most important prognostic factors of uterine adenosarcoma are age, presence of sarcomatous overgrowth, presence of myometrial invasion, presence of lymphovascular invasion, and lymph node involvement. These factors can be used to accurately prognosticate for uterine adenosarcoma patients. Patients at low risk of disease recurrence can be identified. These patients require observation alone. Patients at high risk of disease recurrence can be identified and are candidates for aggressive therapy with adjuvant chemotherapy to reduce the risk of disease recurrence.

Low grade Mullerian adenosarcoma of pouch of Douglas recurring as bilateral ovarian high grade Mullerian adenosarcoma with rhabdomyosarcomatous overgrowth after 11 years.

Mullerian adenosarcoma (MA) of ovary with sarcomatous (rhabdomyoblastic) overgrowth is an extremely rare malignant type of female genital tract neoplasm. These tumours are highly aggressive and presence of heterologous elements is associated with worse prognosis. A 44 year old female presented with lower abdominal pain and distension. She had history of removal of tumour from pouch of Douglas in 2006 for which she did not receive any additional treatment nor did she keep continuous follow up. Current preoperative radiological examination revealed bilateral ovarian masses. She underwent abdominal hysterectomy with bilateral oophorectomy. Microscopic examination revealed biphasic tumours exhibiting sarcomatous overgrowth with rhabdomyoblastic differentiation. Review of the previous biopsy revealed low grade Mullerian adenosarcoma without sarcomatous overgrowth. Hence the current tumour was considered recurrent. This report highlights the aggressive nature of MA even with low grade morphological features and emphasizes the importance of continuous follow up and additional treatment.

A Single-Centre Experience on the Management of Adenosarcoma: A Successful Report of an Integrated Medical and Surgical Approach.

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.

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.

Clinicopathologic study of adenosarcoma with sarcomatous overgrowth of the uterine / 临床与实验病理学杂志

Purpose To investigate the clinicopathologic characteristtics, immunophenotype and differential diagnosis of uterine adenosarcoma with sarcomatous overgrowth. Metheds The clinicopathological data of 4 cases of uterine adenosarcoma with sarcomatous overgrowth were collected, the histopathologic and immunohistochemical features were investigated, and the rele-vant literatures were also reviewed. Results All of tumors were arised from the endometrium with complains of postmenopausal vaginal bleeding or prolonged menstrual period. There is a poly-poid nodular in the uterine cavity with a pedicle or no pedicel, or rough endometrium. On the cut surface, the tumor was fish-like without distinct from the surrounding tissue. Light microsco-py show the tumors were composed of benign glands and malignant mesenchymal components, the sarcomatouscomponents ac-counted for over 25％. In 4 cases, 2 cases had heterologous com-ponent of rhabdomyosarcoma. The component of sarcomatous were positive for vimentin and CD10. The heterologous component of rhabdomyosarcoma were positive for desmin, MyoDl, and Myogenin.3 cases were died at in 5, 10, and 19 months after operation, 1 patient was disease free survival for 3 months. Conclusion Uterine adenosarcoma with sarcomatous overgrowth has a bad prognosis.

Uterine adenosarcomas: A dual-institution experience.

There has been limited literature about treatment and follow-up strategies of uterine adenosarcomas because of their rare nature. For this study we retrospectively investigated the medical database of the two major womens' health hospitals in Turkey. A total of 15 patients were identified from the hospital's database. Median follow-up was 86.43 months for all patients. Seven out of 15 patients had recurrences during their follow-up. Among these 7 patients, 4 of them had stage IA disease. Median Disease Free Survival (DFS) and Overall Survival (OS) were calculated as 41.47 and 57.21 months, respectively. According to our study, polypoid tumours confined to the uterus with superficial myometrial invasion can be treated without comprehensive surgical staging. We believe that, publishing all the data in an organised manner even though they are small in size, gives us an opportunity to design meta-analysis for the development of more appropriate treatment strategies.