Uterine adenosarcoma is a rare biphasic malignancy composed of benign epithelial and malignant stromal components. Adenosarcoma with sarcomatous overgrowth is an aggressive subtype associated with rapid progression and a poor prognosis. We report a case of uterine adenosarcoma with sarcomatous overgrowth diagnosed through the histopathological evaluation of a hysteroscopic polypectomy specimen. A 47-year-old woman presented to a local physician with abnormal uterine bleeding. Pelvic magnetic resonance imaging revealed a 5.5-cm polypoid lesion arising from the uterine corpus and protruding into the endometrial cavity, along with an intramyometrial mass. Hysteroscopic polypectomy was performed, and histopathological examination revealed uterine adenosarcoma, prompting referral to our institution. Positron emission tomography/computed tomography demonstrated fluorodeoxyglucose uptake in both the endometrial lesion and intramyometrial mass, without evidence of distant metastasis. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy. Histopathological examination of the resected uterus showed mildly atypical glandular epithelium surrounded by densely cellular, high-grade malignant stromal cells, together with extensive high-grade sarcomatous overgrowth involving the myometrium. Immunohistochemical staining demonstrated strong CD10 positivity and focal weak h-caldesmon positivity. The final diagnosis was uterine adenosarcoma with sarcomatous overgrowth, International Federation of Gynecology and Obstetrics stage IC. The patient received three cycles of adjuvant doxorubicin plus ifosfamide chemotherapy and remained free of recurrence 18 months after surgery. This case highlights the diagnostic value of hysteroscopic polypectomy in patients with intracavitary uterine lesions and underscores the importance of careful histopathological evaluation for the early recognition of sarcomatous overgrowth.

We report a case of a uterine mesenchymal tumor with myogenic differentiation and SRF::RELA fusion occurring in a 23-year-old woman manifesting as an endometrial polyp. The tumor consisted of bland spindle cells with a predominantly fascicular growth pattern and entrapment of the endometrial glands and vessels. The tumor was positive for smooth muscle markers and CD10. NGS RNA analysis revealed SRF::RELA fusion. NGS DNA analysis revealed no pathogenic variants or copy-number alterations. On follow-up 12 months after curettage, the patient showed no signs of the disease. To the best of our knowledge, only 3 cases of uterine tumors with SRF::RELA fusion arising in the gynecologic tract have been described previously, and all show benign behavior so far. Differential diagnosis includes both benign and potentially aggressive entities, such as leiomyoma, inflammatory myofibroblastic tumor, perivascular epithelioid cell tumor, uterine adenosarcoma, low-grade endometrial stromal sarcoma, and uterine sarcoma with KAT6B/A::KANSL1 fusion.

ObjectiveTo investigate the clinicopathological features, differential diagnosis, and prognosis of undifferentiated carcinoma of the pancreas with osteoclastic giant cells (UCOGCP).MethodsThe clinical histomorphological features and immunohistochemical results of 20 patients of UCOGCP were collected and analyzed. Sanger sequencing was performed on 3 tumor components to assess KRAS gene mutations.ResultsThe 20 patients ranged in age from 52 to 84 years, with a mean age of 62.8 years. The cohort included 12 male patients and 8 female patients. Of the 20 specimens, 9 were pancreaticoduodenectomy specimens, 7 were distal pancreatectomy specimens, and 4 were pancreatic puncture biopsy specimens. Tumor maximum diameters ranged from 1.2 to 11 cm. Microscopically, the tumor was composed of multinucleated giant cells with bland, uniform cytology, and mononuclear cells with significant nuclear atypia. The multinucleated giant cells were large, with abundant eosinophilic cytoplasm, containing several to dozens of uniformly distributed nuclei that exhibited fine chromatin and small, clear nucleoli. The mononuclear cells were round or short spindle-shaped, scattered, with a moderate amount of eosinophilic cytoplasm and marked nuclear atypia, characterized by heterochromatin, active mitotic figures, and easily identifiable pathological mitoses. Both multinucleated giant cells and mononuclear cells expressed vimentin. Multinucleated giant cells expressed CD68, leukocyte common antigen (LCA/C45), and MAC387 and showed a low Ki-67 proliferation index (<10%). Mononuclear cells showed aberrant p53 expression (suggestive of a mutant pattern) and a high Ki-67 proliferation index (>60%). They consistently expressed at least one epithelial marker, including keratin pan, EMA, CAM5.2, keratin 7, keratin 8/18, keratin 19, or keratin 20. Mononuclear cells were negative for S100 protein, Melan A, and HMB45. Molecular pathological analysis revealed KRAS gene mutations in all specimens.ConclusionUndifferentiated carcinoma of the pancreas with osteoclastic giant cell is a rare clinical entity with histomorphological features resembling those of malignant giant cell tumors of bone and soft tissue. It can be distinguished from pleomorphic giant cell carcinoma and pancreatic adenosarcoma through immunohistochemistry and molecular testing. Radical surgical resection remains the optimal treatment for patients with UCOGCP.

Uterine polypoid adenomyoma (UPA) is a rare focal form of adenomyosis that can present as an intracavitary lesion and pose a significant diagnostic challenge, often mimicking more common or concerning entities, such as endometrial polyps or uterine adenosarcoma. We describe the case of a 53-year-old woman who presented with profound anemia (hemoglobin 3.9 g/dL) secondary to acute on chronic heavy vaginal bleeding. Multimodal imaging, including ultrasound and MRI, prompted the initial diagnosis of UPA, later supported by surgical pathology. Ultrasound demonstrated diffusely heterogeneous myometrial echotexture and multifocal "venetian blind" artifact consistent with adenomyosis. MRI revealed an ovoid lesion within the endometrial cavity containing numerous T2 hyperintense cystic foci and imaging characteristics similar to the junctional zone, consistent with a submucosal UPA. Management included blood transfusions, hormone therapy, and ultimately hysterectomy. This case serves to underscore the importance of a combined approach, including both multimodal imaging and histopathology, in guiding the accurate diagnosis and optimal management of UPA. It is also intended to increase awareness of this rare lesion and encourage its consideration in the differential diagnosis of intracavitary uterine masses, as it may have implications for treatment planning.

Extrauterine presentation of Mullerian adenosarcoma is rare and thought to either arise from endometriosis or from spread from a primary uterine or ovarian origin. When accompanied by sarcomatous overgrowth, particularly when the epithelial components are limited or obscured, these tumors can be challenging to recognize as adenosarcomas and may be misdiagnosed as primary pelvic or retroperitoneal sarcomas. Diagnostic molecular testing could be valuable but also misleading, if not interpreted in the right context, as some retroperitoneal sarcomas exhibit alterations that can have overlapping molecular alterations with Mullerian adenosarcoma, such as MDM2 amplification in liposarcomas. In this report, we describe three Mullerian adenosarcomas with extrauterine presentation, all exhibiting extensive sarcomatous overgrowth and MDM2 amplification. These tumors underscore the critical importance of integrating clinical history, comprehensive tissue sampling, detailed morphologic evaluation, and molecular analysis to establish an accurate diagnosis.

Mullerian adenosarcomas are biphasic tumors with benign epithelium and malignant stroma, typically found in the uterine corpus but rarely in the cervix, ovaries and other locations. A diagnostic pitfall is misdiagnosis as benign polyps due to bland cytology. Sarcomatous overgrowth (SO) and myometrial invasion are poor prognosticators. A positive prognosticator may be stalked tumors. Surgery alone can yield favorable outcomes. Here, we present a rare case of Mullerian adenosarcoma of the cervix in a 74-yr-old patient with p53 wild-type, high-grade SO that presented as a cervical polyp with a stalk.

Uterine sarcomas are rare heterogeneous neoplasms, comprising only a small percentage of uterine malignancies. There is a diverse array of uterine sarcoma sub-types, each with distinct molecular, clinical, and behavioral features. In recent years, there has been significant advancement in all facets of the multi-disciplinary management of uterine sarcomas. Despite this, several challenges regarding optimization of treatment remain. While early-stage uterine sarcomas are potentially curable with surgery, there remains a high risk of recurrence, with the lungs and liver being common sites for distant spread. In the case of oligometastatic disease, local treatment may be offered in select patients and may improve survival. In the metastatic setting, advances in our histopathologic understanding of these rare cancers have allowed for the development and utilization of more targeted treatments; however, it is evident that there is a need for more efficacious and clinically meaningful therapeutic options. These patients often experience significant morbidity as a consequence of their disease and/or treatment, and palliative care support early in the disease course may improve quality of life and goal-concordant care. Additional large-scale, international multi-center collaboration is needed to further optimize multi-disciplinary management for these patients. In this review, we provide a comprehensive update on the management of advanced uterine sarcomas, and discuss novel therapeutic and supportive strategies in the multi-disciplinary management of these rare malignancies.

Uterine adenofibroma is an extremely rare, benign, mixed epithelial and mesenchymal tumor, and its imaging characteristics remain poorly characterized because only a small number of cases have been reported. Accurate preoperative diagnosis can be difficult, as the clinical and radiological features of uterine adenofibroma often overlap with those of endometrial carcinoma, adenosarcoma, and other lesions of the endometrial cavity. Very few studies have documented detailed MRI findings, and further case reports are needed to establish distinctive imaging patterns. We report the case of an 84-year-old woman who presented with genital bleeding and a uterine mass on imaging. MRI revealed a mainly solid tumor with small cystic components occupying the endometrial cavity. Dynamic contrast-enhanced MRI demonstrated linear surface enhancement in the early phase and papillary projections with gradually increasing enhancement in the delayed phase. These imaging findings were consistent with the histopathological features of the tumor, which showed surface papillations and widespread squamous metaplasia. The presence of prominent squamous metaplasia likely contributed to the marked and progressive surface enhancement, a feature that distinguished this case from previously reported adenofibromas. This report highlights the importance of recognizing unique MRI characteristics, particularly papillary surface enhancement patterns, when evaluating endometrial cavity tumors. Further accumulation of imaging-pathology correlated reports may assist in improving the diagnostic accuracy of this rare tumor type.

Uterine sarcomas are rare heterogeneous neoplasms, comprising only a small percentage of uterine malignancies. There is a diverse array of uterine sarcoma sub-types, each with distinct molecular, clinical, and behavioral features. In recent years, there has been significant advancement in all facets of the multi-disciplinary management of uterine sarcomas. Despite this, several challenges regarding optimization of treatment remain. Pre-operative diagnosis can be difficult, as imaging and biopsy may not reproducibly distinguish uterine sarcomas from benign entities. Early-stage uterine sarcomas are potentially curable with surgery; however, there remains a high risk of recurrence, and the use of adjuvant treatment is controversial. There is also a lack of high-quality data to guide best practices for fertility-sparing surgery in younger patients. Optimal management requires multi-disciplinary discussion by clinicians experienced in sarcoma management at specialized sarcoma centers, as initial management may have a profound impact on patient outcomes. In this review, we summarize and address many of these challenges facing patients with early-stage uterine sarcoma, and provide a comprehensive update on the clinical features, the molecular and genomic pathogenesis, and the current diagnostic and treatment landscape for these patients.