Spontaneous splenic rupture is an extremely rare occurrence, often attributed to tumorous pathologies. Among these, primary splenic angiosarcoma stands as a malignancy arising from the endothelial cells within the spleen. While sporadic cases have been reported globally, there remains a lack of comprehensive consensus on standardized approaches for diagnosis and treatment. We report a case of an 83-year-old male who underwent emergency enhanced CT due to sudden shock, revealing significant intra-abdominal fluid accumulation. Emergency surgery revealed splenic rupture necessitating splenectomy. Histopathological examination confirmed the diagnosis of splenic angiosarcoma. Despite successful surgery, the patient succumbed to severe complications two weeks postoperatively.

Gastrointestinal angiosarcoma is an extremely rare malignant tumor of the digestive tract, characterized by a very poor prognosis, with few patients surviving more than 1 year after diagnosis. This case report describes a 71-year-old female patient with a 3-year history of intermittent abdominal pain and significant exacerbation of abdominal pain and bloating 2 weeks prior to treatment. After surgical treatment, the pathological and immunohistochemical diagnosis was primary epithelioid angiosarcoma of the jejunal mesentery. The patient refused postoperative adjuvant chemotherapy and died 4 months after diagnosis due to widespread systemic metastasis. In addition, this article reviews 38 previously reported cases of primary gastrointestinal angiosarcoma, aiming to further understand angiosarcoma and thus guide clinical practitioners in providing more comprehensive treatment approaches.

Primary angiosarcomas are a rare type of soft-tissue sarcomas that originate from endothelial cells. These sarcomas can develop in any part of the body and have a poor prognosis. However, they are commonly found in the skin of elderly white men, particularly on the scalp and head region. Primary angiosarcoma of the cervix is exceptionally rare. To date, only two cases of this disease have been reported worldwide. The diagnosis of the disease is difficult microscopically, requiring immunohistochemistry and genetic testing to distinguish. We report a recent case, in which the lesion was preoperatively considered a high-grade endometrial stromal sarcoma. A 35-year-old woman presented with vaginal bleeding and cervical erosions. A high-grade endometrial stromal sarcoma involving the cervix was considered and a modified radical hysterectomy was performed with bilateral salpingo-oophorectomy and sentinel lymph nodes resection. The gene diagnosis performed by fluorescence in situ hybridization for YWHAE translocation fusion was negative excluding a YWHAE-translocated high-grade endometrial stromal sarcoma. A primary angiosarcoma of the cervix was finally diagnosed. Primary angiosarcoma of the cervix is rare, and gynecologic pathologists do not know it well, so it is easy to be wrongly considered. Immunohistochemistry and genetic testing help confirm the diagnosis.

Angiosarcomas, originating from endothelial cells, are infrequent soft tissue sarcomas characterized by a high propensity for metastasis and an unfavorable prognosis. Splenic angiosarcoma, an exceedingly rare and aggressive neoplasm, exhibits variable clinical manifestations. The present case report describes a patient initially exhibiting anemia and bone marrow fibrosis, mimicking primary myelofibrosis, ultimately diagnosed with splenic angiosarcoma. The findings of the present case report underscore the importance of considering splenectomy for histopathological confirmation. Employing a panel of vascular differentiation markers is invaluable for establishing the diagnosis of angiosarcoma.

BACKGROUND: Angiosarcomas in the head and neck region are aggressive tumours associated with high local recurrence and metastatic rates. We present our 17-year experience at the North of England Bone and Soft Tissue Tumour Service.
METHODS: A retrospective review of our prospectively maintained database was undertaken, looking for patients diagnosed with angiosarcomas affecting the head and neck. Data were gathered using a pre-defined proforma to include demographics, histological characteristics, treatment modalities, adjuvant therapies, local recurrence, distant spread, and disease-specific survival.
RESULTS: A total of 23 patients (17 males, 6 females) were identified, with a mean age of 76 years at presentation. Fourteen presented with scalp lesions, whereas the remainder arose on the face. Eighteen patients underwent resection with curative intent, whereas three received palliative radiotherapy and two received comfort-based care only. Of the patients undergoing surgery, 12 had local flap reconstruction and 6 underwent free tissue transfer. Clear resection margins were obtained in nine cases (50%). Fourteen patients (78%) presented with local recurrence after surgery, and 11 (61%) developed distant metastases. The median disease-specific survival time for patients treated with curative intent was 38 months. Eight patients had mapping biopsies ahead of their resection; however, complete resection was achieved in only two cases.
CONCLUSIONS: Angiosarcomas of the head and neck are associated with a poor prognosis, with most patients rapidly developing local recurrence, resulting in adverse clinical outcomes. Mapping biopsies do not demonstrate a clear advantage for achieving complete surgical resection. A radical surgical approach is warranted, given the aggressiveness of the pathology. However, there remains no consensus on optimal surgical management; we recommend further synthesising studies to determine the most appropriate treatment pathway.

UNASSIGNED: Angiosarcoma, a rare endothelial-origin tumor, can develop throughout the body, with the head and neck skin being the most commonly affected areas. It can also originate in other sites such as the breast, iliac artery, and visceral organs including the liver, spleen, and kidneys. Angiosarcoma of the bone is remarkably rare, presenting as either unifocal or multifocal bone lesions and often leading to a grim prognosis. Diagnosing bone angiosarcoma poses a significant challenge. 18F-FDG PET/CT serves as a reliable and indispensable imaging modality for evaluating distant metastases and clinically staging angiosarcomas.
UNASSIGNED: A 57-year-old woman presented with a 10-day history of dizziness and headaches. Cranial CT scan revealed bone destruction of the parietal bone, accompanied by soft tissue lesions, protruding into the epidural space. MRI examination demonstrated lesions with slightly elevated signal intensity on T2FLAIR, showing moderate enhancement. Furthermore, multiple foci were observed within the T12, L1-5, and S1-2 vertebrae, as well as in the bilateral iliac bones. For staging, 18F-FDG PET/CT was performed. The MIP PET showed multifocal FDG-avid lesions in the sternum, bilateral clavicles, bilateral scapulae, multiple ribs, and pelvic bones. Heterogeneous FDG uptake was observed in multiple bone lesions, including intracranial (SUVmax = 11.3), right transverse process of the T10 vertebra (SUVmax = 5.8), ilium (SUVmax = 3.3), and pubis (SUVmax = 4.7). The patient underwent surgical resection of the cranial lesion. The pathological diagnosis was made with a highly differentiated angiosarcoma.
UNASSIGNED: Angiosarcoma of bone on FDG PET/CT scans is characterized by abnormal FDG uptake along with osteolytic destruction. This case highlights that angiosarcoma of bone can manifest as multicentric FDG uptake, resembling the pattern seen in multiple myeloma. FDG PET/CT can be a useful tool for staging this rare malignant tumor, offering the potential to guide biopsy procedures toward the most metabolically active site. And it should be considered in the differential diagnosis of multiple osteolytic lesions, including metastatic carcinoma, multiple myeloma, and lymphoma of bone.

Scalp angiosarcoma (SA) is rare, accounting for <1% of soft tissue sarcomas, with a high degree of malignancy, a high recurrence rate and a poor prognosis. The best treatment strategy is uncertain. Therefore, it is essential to continuously refine treatment strategies and improve the prognosis of patients. Curative-intent surgery increases overall survival in patients with primary cutaneous angiosarcoma of the scalp and face, and radiation therapy combined with chemotherapy is now recommended for the curative treatment of patients who both can or cannot undergo surgery. The present case report is of an 87-year-old man hospitalised for the fifth time with SA. He had experienced four recurrences and previously underwent curative-intent surgery four times. However, the patient did not undergo radiotherapy or chemotherapy after any of the surgeries. A detailed report of the management of this case is presented along with a review of the relevant literature. It is hypothesised that patients with SA should receive a combination of radiotherapy and chemotherapy after surgery whenever possible, which may improve patient prognosis.

Benign lymphangioendothelioma (BL) is a rare, poorly identified, slow-growing benign vascular lesion characterized by asymptomatic, solitary, well-demarcated macules, or by mildly infiltrated plaque. We report a case of an atypical BL that arose as a tender, protuberant, flesh-colored mass with cyanotic vesicles, and then progressed to a persistent exudative wound after two incomplete excisions. The patient was also diagnosed with thoracic duct narrowing. Although the stenosis was removed by surgery, the right lower extremity ulceration and exudation did not improve. Thus, we performed a thorough excision and split-thickness skin graft transplant following vacuum sealing drainage, and eventually the patient had a favorable functional and cosmetic outcome. A biopsy revealed irregular, dilated vascular spaces lined with a single layer of flat endothelial cells extending from the superficial dermis to the subcutis that did not reach the striated muscles. Additionally, by reviewing the literature on BL, in this paper we summarize the diverse pathogenic, morphological, and immunohistochemical presentations for this rare disease, as well as the histopathological differential diagnosis of lymphangiomatosis, Kaposi\'s sarcoma, and angiosarcoma.

BACKGROUND: Primary cardiac tumors are rare but have the potential to cause significant morbidity if not treated in an appropriate and timely manner. To date, however, there have been no studies examining the survival characteristics of patients who did not undergo surgical resection.
METHODS: We are presenting a case of a 61-year-old male admitted to our department due to \"heart tumor\'\'. He had 5+years previous history of type 2 diabetes was, and he took Metformin orally for a long time. Under extracorporeal circulation and general anesthesia, the heart lesion was removed under thoracoscopy. The tumor was about 5 cm ∗ 4 cm pale yellow color and sent for biopsy. Pathology report showed tumor like hyperplasia of fat and striated muscle tissues, some fat cells had atypical hyperplasia.
CONCLUSIONS: For patients with malignant tumor, most of them had local invasion or had distant metastasis when the diagnosis was clear, and only conservative management could be done. Moreover, the prognosis of malignant tumor is poor, and the pathological morphology is diverse. The causes of death are due to widespread tumor metastasis, refractory heart failure, and various arrhythmias.

Objective: To investigate the clinicopathological features, treatment, and prognosis of hepatic angiosarcoma. Methods: Clinicopathological data and prognostic conditions of 18 cases with hepatic angiosarcoma were collected retrospectively. The recurrence-free survival rate and overall survival rate were calculated by the Kaplan-Meier method. A Cox regression analysis was used to explore the survival-related risk factors. Results: There were 12 male and 6 female patients, with an average age of 57 (37 ~ 70) years. The tumor\'s average diameter was 8.40 (2.00 ~ 18.00) cm. Seven cases had multiple tumors, while two cases had large vessel tumor thrombuses. Microscopically, the tumor tissues were irregularly anastomosed, with vascular lacunar or solid bundle-like weaving, and the tissue morphology mimicked capillary hemangioma, cavernous hemangioma, or angioepithelioma, while tumor cells were spindle-shaped or epithelioid, lined with hobnails in the lumen, or formed papillary structures in the lumen. The proportion of highly, moderately, and poorly differentiated tumors was 4:8:6, with six cases having clear tumor boundaries, eight having microvascular tumor thrombi, and sixteen having blood lake formation. Different levels of expression of CD31, CD34, erythroblast transformation-specific related genes, and Fli-1 markers were demonstrated in all of the cases. Four cases had a P53 mutation, and six cases had Ki-67 > 10%. During the follow-up period of 0.23-114.20 months, the five-year recurrence-free survival rate and overall survival rate were 16.7% and 37.2%, respectively. Cox regression multivariate analysis showed that preoperative symptoms and multiple tumors were significant risk factors for recurrence-free survival, while preoperative symptoms and Ki-67 > 10% were significant risk factors for overall survival. Conclusion: Hepatic angiosarcoma is a rare hepatic mesenchymal tumor with high malignancy and a poor prognosis. Pathological morphology and immunohistochemical marker combinations are needed for a definite diagnosis. However, the complexity of angiosarcomas\' histological and cytological conformations and the overlap of pathological features with benign vascular tumors, sarcomas, and carcinomas pose difficulties in the differential diagnosis. Thus, the only effective ways to prolong survival are early detection and radical surgical resection.
目的： 探讨肝血管肉瘤的临床病理特征和治疗预后。 方法： 回顾性收集18例肝血管肉瘤患者的临床病理信息和预后情况，Kaplan-Meier法计算无复发生存率和总生存率，Cox回归分析探索生存相关风险因素。 结果： 患者男性12例，女性6例，平均年龄57（37~70）岁。肿瘤平均直径8.40 （2.00~18.00）cm，多发肿瘤7例，2例有大血管瘤栓。镜下肿瘤组织排列呈不规则吻合状血管腔隙样或实性束状编织状，组织形态上可模拟毛细血管瘤、海绵状血管瘤或血管外皮瘤。瘤细胞可呈梭形或上皮样，呈靴钉样衬覆于管腔或在管腔内形成乳头状结构。高、中、低分化肿瘤占比为4∶8∶6，6例肿瘤边界清晰，8例有微血管瘤栓，16例见血湖形成。全部病例不同程度表达CD31、CD34、成红细胞转化特异性相关基因、Fli-1标志物，P53突变病例4例，Ki-67 > 10%病例6例。随访0.23~114.20个月，5年无复发生存率为16.7%，5年总生存率为37.2%。Cox回归多因素分析提示，术前有症状、多发肿瘤为无复发生存的显著风险因素，术前有症状、Ki-67 > 10%为总生存的显著风险因素。 结论： 肝血管肉瘤是一种罕见的肝脏间叶源性肿瘤，恶性程度高，预后差，确诊需结合病理形态和免疫组织化学标志物情况。复杂的组织和细胞学构象以及与良性血管源性肿瘤、肉瘤、癌在病理特征上的重叠是血管肉瘤鉴别诊断的难点。早期发现和根治性手术切除是延长生存期仅有的有效方法。.