Magnetic resonance imaging findings of radiation-induced breast angiosarcoma:A case report

BACKGROUND Breast conservation surgery(BCS)with adjuvant radiotherapy has become a gold standard in the treatment of early-stage breast cancer,significantly reducing the risk of tumor recurrence.However,this treatment is associated with adverse effects,including the rare but aggressive radiation-induced angiosarcoma(RIAS).Despite its rarity and nonspecific initial presentation,RIAS presents a challenging diagnosis,emphasizing the importance of imaging techniques for early detection and accurate diagnosis.CASE SUMMARY We present a case of a 48-year-old post-menopausal woman who developed skin ecchymosis on the right breast seven years after receiving BCS and adjuvant radiotherapy for breast cancer.Initial mammography and ultrasound were inconclusive,showing post-treatment changes but failing to identify the underlying angiosarcoma.Contrast-enhanced breast magnetic resonance imaging(MRI)revealed diffuse skin thickening and nodularity with distinctive enhan-cement kinetics,leading to the diagnosis of RIAS.This case highlights the crucial role of MRI in diagnosing and determining the extent of RIAS,facilitating timely and appropriate surgical intervention.CONCLUSION Breast MRI is crucial for detecting RIAS,especially when mammography and ultrasound are inconclusive.

Small intestinal angiosarcoma on clinical presentation, diagnosis, management and prognosis: A case report and review of the literature

BACKGROUND Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues.Smallintestinal angiosarcomas are rare,and the prognosis is poor.CASE SUMMARY We reported a case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma.Small intestinal angiosarcoma is more common in elderly and male patients.Gastrointestinal bleeding,anemia,abdominal pain,weakness,and weight loss were the common symptoms.CD31,CD34,factor VIII-related antigen,ETS-related gene,friend leukemia integration 1,and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma.Small-intestinal angiosarcoma most commonly occurs in the jejunum,followed by the ileum and duodenum.Radiation and toxicant exposure are risk factors for angiosarcoma.After a definite diagnosis,the mean and median survival time was 8 mo and 3 mo,respectively.Kaplan-Meier survival analysis showed that age,infiltration depth,chemotherapy,and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma.Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis.CONCLUSION Angiosarcoma should be considered for unexplained melena and abdominal pain,especially in older men and patients with a history of radiation exposure.Prompt treatment,including surgery and adjuvant chemotherapy,is essential to prolonging patient survival.

A complementary comment on primary hepatic angiosarcoma:A case report

BACKGROUND This article examines primary hepatic angiosarcoma(PHA)and fat-poor angiomyolipoma(AML),two uncommon vascular cancers.Clinical decisions in these situations are frequently aided by pathology reports and imaging techniques.Uncommon malignant tumors of the vascular endothelium include PHA.Another diagnosis that should not be overlooked when employing contrastenhanced MR and contrast-enhanced computed tomography(CT)imaging techniques is fat-poor AML,one of the uncommon vascular tumors of the liver.In both conditions,biopsy is the primary means of diagnosis.CASE SUMMARY In our article,besides the diagnosis of PHA,fat-poor AML,one of the other rare vascular tumors of the liver,is mentioned.In the case,a 50-year-old female patient with VHL Syndrome was admitted to our hospital with nonspecific lesions such as right upper quadrant pain,weight loss,and nausea.Abdominal ultrasonography(US)revealed a hypoechoic heterogeneous lesion with occasional faint contours.In computed tomography,it was observed as a hyperdense nodular lesion in segment 4.Magnetic resonance imaging(MRI)revealed that the lesion did not contain fat.In connection with the known history of VHL Syndrome,we first evaluated the possibility of AML.Thereupon,a histopathological sample was taken and the diagnosis was made as fat-poor AML with 5%fat content.CONCLUSION In conclusion,PHA in our case report and fat-poor AML in our clinic are two uncommon liver vascular malignancies with comparable incidences.Important imaging techniques like contrast-enhanced US(CEUS),CECT,and CEMRI give us substantial advantages in both cases.However,a biopsy is used to provide the final diagnosis.

Misdiagnosis of scalp angiosarcoma: A case report

BACKGROUND Angiosarcoma is a rare malignant tumor.Owing to the lack of specific clinical manifestations of this disease,it is difficult to achieve early diagnosis and start early treatment.CASE SUMMARY A 78-year-old male patient was admitted to the hospital because of a bump on his head that did not heal for 4 mo.The patient was diagnosed with a refractory head wound.The patient underwent neoplasm resection and skin grafting surgery in the Plastic Surgery.The neoplasm was sent for pathological examination during the operation.The final pathological results were confirmed scalp angiosarcoma.CONCLUSION Our research suggests that pathological examination should be performed for refractory ulcers of the scalp,and physical factor therapy should be used with caution before the diagnosis is clear.

Primary ovarian angiosarcoma:Two case reports and review of literature

BACKGROUND Angiosarcoma(AS)is a rare and highly aggressive soft tissue disease that most commonly arises in deep soft tissues.There are only a few reported cases of AS involving the ovary and even fewer reports of the underlying molecular abnormalities.Here,we briefly review two cases of primary ovarian AS(oAS)with specific molecular events and immune checkpoints.The clinical features and prognosis of the disease,diagnosis,differential diagnosis,and new treatment approaches are discussed based on a literature review.CASE SUMMARY Case 1:A 51-year-old female patient was admitted with right lower limb pain for 5 mo,and lower abdominal pain with hematuria for 1 mo.Partial removal of rectus abdominis muscle and fascia,partial hysterectomy,bilateral salpingooophorectomy,and inguinal and pelvic lymphadenectomy were performed.Pathology revealed primary oAS.Fluorescence in situ hybridization revealed c-MYC gene amplification.MESNA+ADM+IFO+DTIC(MAID)regimen was administered,but stable disease was achieved.The patient died 1 mo later.Case 2:A 41-year-old female patient presented with fatigue,nausea,decreased appetite,and diffuse abdominal pain.On physical examination,the abdomen was distended and a complex cystic mass was palpable in the right pelvic cavity.Pathology revealed primary oAS.MAID chemotherapy was administered and programmed death ligand 1(PD-L1)staining was performed on the tumor samples.The patient benefited from anti-PD-1 immunotherapy and is alive without any evidence of disease 27 mo off therapy in follow-up.CONCLUSION Long-term survival benefit for primary oAS can be achieved by alternative therapeutic strategies using pathological indicators to inform treatment.

Transcription factor ERG is a specific and sensitive diagnostic marker for hepatic angiosarcoma

AIM: To investigate the expression of ERG, CD34, CD31 (PECAM-1, platelet/endothelial cell adhesion molecule 1) and factor VIII-related antigen (FVIIIRAg) in the diagnosis of hepatic angiosarcoma patients.

Case report of primary splenic angiosarcoma with hepatic metastases

Primary splenic angiosarcoma(PSA) is the most unusual type of malignancy with early multifocal metastasis through hematogenous spread. PSA is generally believed to originate from splenic sinusoidal vascular endothelium with a high rate of metastasis and to have a poor prognosis. Its etiology and pathogenetic mechanisms have not yet been clearly described. Thus far, only approximately 200 cases have been reported. PSA has variable symptomatology with the potential to present with life-threatening complications. The diagnosis of PSA is challenging; and often late. PSA should be considered in the differential diagnosis of patients with splenomegaly and anemia of unknown etiology. Surgical treatment with splenectomy is considered the only curative intervention for potential long-term disease-free survival. Early diagnosis and treatment are very important. It is important that clinical doctors improve the understanding of PSA. Herein, we report one rare case of PSA with hepatic metastases, along with a review of the current literature.

A pooled analysis of treatment and prognosis of hepatic angiosarcomain adults

Background： Hepatic angiosarcoma is a rare malignant vascular tumor presenting unique treatment challenges.The aim of the present study was to determine the treatment and prognosis of this entity.Data sources： A systematic literature search was conducted using PubMed, Embase and Chinese BiomedicalLiterature database, to identify articles published from January 1980 to July 2017. Search termswere “hepatic angiosarcoma” and “liver angiosarcoma”. Additional articles were retrieved through manualsearch of bibliographies of the relevant articles. Pooled individual data concerning the prognosis followingvarious therapeutic modalities were analyzed.

Primary splenic angiosarcoma with liver metastasis: A case report and literature review

Primary splenic angiosarcoma(PSA) is an unusual and highly malignant vascular tumour with a high rate of metastatic. Moreover, the research on prognosis of the disease is poor. The epidemiology, etiology, clinical diagnosis and treatment of the disease remain challenging, because case reports of the disease are few in number. In accordance with other malignant tumors, PSA is very aggressive, and the majority of patients in which this disease is found are at an advanced stage. Almost all patients die within 12 mo of diagnosis irrespective of treatment. We report here a woman who had complained of upper bellyache and anorexia for 10 d. Magnetic resonance imaging showed enlargement of the spleen with multiple heterogeneous masses in the lower pole of the spleen. A hand-assisted laparoscopic splenectomy was performed which allowed histopathologic diagnosis. The patient was diagnosed with PSA and liver metastasis, and succumbed to the disease 35 d after surgery. The literature was finished combined with the clinical features, diagnosis and management of PSA.

Combination therapy for scalp angiosarcoma using bevacizumab and chemotherapy: a case report and review of literature

Bevacizumab, an angiogenesis inhibitor, is a recombined humanized monoclonal antibody against vascular endothelial growth factor and a promising therapeutic option for angiosarcoma management. This is a ease report and review of the literature using bevacizumab and combination chemotherapy for angiosarcoma. The understanding of the effectiveness of combined therapy of bevacizumab and chemotherapy agents is still limited. The benefits of bevacizumab treatment for angiosarcoma will need to be weighed against the risks of venous thromboembolism in this population.

Giant primary angiosarcoma of the small intestine showing severe sepsis

Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40  °C and marked abdominal distension. Her medical history was unremarkable, but blood examination showed elevated inflammatory markers. Abdominal computed tomography showed a giant tumor with central necrosis, extending from the epigastrium to the pelvic cavity. Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected. Because no improvement occurred in response to antibiotics, surgery was performed. Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity. The giant tumor was a solid tumor weighing 3490 g, measuring 24 cm × 17.5 cm × 18 cm and showing marked necrosis. Histologically, the tumor comprised spindle-shaped cells with anaplastic large nuclei. Immunohistochemical studies showed tumor cells positive for vimentin, CD31, and factor VIII-related antigen, but negative for c-kit and CD34. Angiosarcoma was diagnosed. Although no postoperative complications occurred, the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively. The prognosis of small intestinal angiosarcoma is very poor, even after volume-reducing palliative surgery.

Contrast uptake in primary hepatic angiosarcoma on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging in the hepatobiliary phase

Primary hepatic angiosarcoma is the most common malignant mesenchymal tumor of the liver. It has a poor prognosis and various appearances on magnetic resonance(MR) images. We report a case of hepatic angiosarcoma with a characteristic appearance on gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid(Gd-EOB-DTPA)-enhanced MR imaging in the hepatobiliary phase. A 72-year-old man was admitted with a complaint of abdominal pain. Gd-EOBDTPA-enhanced MR imaging revealed a liver tumor that showed slight hyperintensity in the hepatobiliary phase. These findings suggested Gd-EOB-DTPA uptake in the tumor. An autopsy revealed the solid proliferation and sinusoidal spreading of hepatic angiosarcoma cells. Immunohistochemistry indicated that the tumor was negative for OATP1B3. Gd-EOB-DTPA uptake in the liver tumor in the hepatobiliary phase suggested sinusoidal tumor invasion with residual normal hepatocytes.

Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome

Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome(KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.

Ruptured angiosarcoma of the liver treated by emergency catheter-directed embolization

Angiosarcoma is a rare primary malignant neoplasm of the liver with a poor prognosis. Here, we report a case of a patient with a ruptured hepatic angiosarcoma which was treated by emergency catheter-directed embolization, followed by leflc-sided hemihepatectomy.

Small intestine bleeding due to multifocal angiosarcoma

We report a case of an 84-year-old male patient with primary small intestinal angiosarcoma.The patient initially presented with anemia and melena.Consecutive endoscopy revealed no signs of upper or lower active gastrointestinal bleeding.The patient had been diagnosed 3 years previously with an aortic dilation,which was treated with a stent.Computed tomography suggested an aorto-intestinal fistula as the cause of the intestinal bleeding,leading to operative stent explantation and aortic replacement.However,an aorto-intestinal fistula was not found,and the intestinal bleeding did not arrest postoperatively.The constant need for blood transfusions made an exploratory laparotomy imperative,which showed multiple bleeding sites,predominately in the jejunal wall.A distal loop jejunostomy was conducted to contain the small intestinal bleeding and a segmental resection for histological evaluation was performed.The histological analysis revealed a lessdifferentiated tumor with characteristic CD31,cytokeratin,and vimentin expression,which led to the diagnosis of small intestinal angiosarcoma.Consequently,the infiltrated part of the jejunum was successfully resected in a subsequent operation,and adjuvant chemotherapy with paclitaxel was planned.Angiosarcoma of the small intestine is an extremely rare malignant neoplasm that presents with bleeding and high mortality.Early diagnosis and treatment are essential to improve outcome.A small intestinal angiosarcoma is a challenging diagnosis to make because of its rarity,nonspecific symptoms of altered intestinal function,nonspecific abdominal pain,severe melena,and acute abdominal signs.Therefore,a quick clinical and histological diagnosis and decisive measures including surgery and adjuvant chemotherapy should be the aim.

Angiosarcoma of the scalp diagnosed by the presence of neck inflammation:a case report

Angiosarcoma is an uncommon malignancy, which spread out from the endothelial cells of vessels. Scalp angiosarcoma with cervical lymph node metastasis is particularly rare. This article describes a rare case of angiosarcoma of the scalp, presenting as neck inflammation. Imaging procedures such as computed tomography （CT）, magnetic resonance image （MRI） and ultrasonography （US） were not sufficient to diagnose this case. A needle biopsy provided an effective and accurate diagnosis of cervical lymph node metastasis. Additional observation and physical examination was required to diagnose the origin of the primary cancerous lesion. Once the angiosarcoma diagnosis was confirmed histologically, sequential weekly and monthly docetaxel （DTX） treatment was effective in preventing reoccurrence. Nonetheless, the optimization of angiosarcoma treatment remains a future goal. Although patients generally describe pain and swelling at the primary lesion site, this patient complained only of painful neck inflammation, without any indication of pain or swelling of the scalp. A revised diagnostic protocol should note that cervical lymph node metastasis of unknown primary origin may result from angiosarcoma of the scalp.

Pneumonectomy of Primary Pulmonary Angiosarcoma with Malignant Effusion and Intrapleural Hypotonic Hyperthermic Chemotherapy: Case Report and Review of the Literature

Background: Primary pulmonary angiosarcoma is extremely rare and the prognosis is very poor. We report a combination therapy of pneumonectomy and intrapleural hypotonic hyperthermic chemotherapy (IPHHC). Case report: A 48-year-old male with exertional dyspnea was found to have a left massive pleural effusion. Bronchoscopic examination displayed endobronchial stenosis of the left lower bronchus B8,9,10?and diagnosed with pulmonary angiosarcoma. Chest computed-tomographic scanning revealed a 5-cm mass in the left inferior lobe, which invaded the left upperbronchus. Intrapleural dissemination and malignant pleural effusion were also suspected (cT3N1M1a,?c-stage IV). As a palliative initial therapy, we performed a pneumonectomy. On the 7th?postoperative day, under general anesthesia, we performed video-assisted IPHHC (43°C, 60 min, 200 mg/m2?of CDDP). After the IPHHC, there was no major adverse event (more than Grade 3) during the postoperative course. Histological examination of the resected specimen revealed a highly-cellular growth of atypical spindle cells with a storiform pattern. By immunohistochemical testing, the tumor cells stained positive for markers including CD31 and the factor VIII related antigen, and the diagnosis of pulmonary angiosarcoma was made. No adjuvant chemoradiotherapy was given, and the postoperative clinical course was uneventful. Although there had been a recurrence in the chest wall, the patient eventually died twenty-one months later. Conclusion: As a new therapeutic option, we performed IPHHC after the pneumonectomy for an advanced pulmonary angiosarcoma with malignant pleural effusion. Although the patient had a recurrence in the chest wall, he had a reasonable postoperative outcome, that is, he returned to a work, retained a good quality of life and had a longer survival in spite of the poor prognosis of the pulmonary angiosarcoma.

Primary hepatic angiosarcoma manifesting as hepatic sinusoidal obstruction syndrome:A case report

BACKGROUND Primary hepatic angiosarcoma(PHA)is a rare malignancy with a poor prognosis.It is difficult to diagnose PHA because of the lack of specific symptoms or tumour markers,and it rapidly progresses and has a high mortality.To our knowledge,PHA has not been reported to mimic hepatic sinusoidal obstruction syndrome.Herein,we present a case of PHA manifesting as hepatic sinusoidal obstruction syndrome,diagnosed using transjugular liver biopsy,that resulted in the death of the patient.CASE SUMMARY A 71-year-old man was admitted with the primary complaint of abdominal distension,decreased appetite,fatigue in the previous month,and loss of 10 kg of weight in the past 2 years.Both the liver and spleen were enlarged,and the liver had a medium-hard texture on percussion.Laboratory examinations were performed,and abdominal plain computed tomography(CT)and contrastenhanced CT showed hepatomegaly and splenomegaly,as well as diffuse lowdensity shadows distributed in the liver and spleen.Contrast-enhanced CT revealed diffuse,hypodense,nodular or flake shadows in the liver and heterogeneous enhancement in the spleen.A transjugular liver biopsy was performed.Based on the pathology results,the patient was diagnosed with hepatic sinusoidal obstruction syndrome secondary to PHA.The patient’s status further deteriorated and he developed serious hepatic failure.The patient was discharged,and died 3 d later.CONCLUSION PHA is rare and has a poor prognosis;however,transjugular liver biopsy can be safely performed to aid in diagnosis.

Splenic inflammatory pseudotumor mimicking angiosarcoma

Splenic tumors are rare. Differentiation of the tumors before operation is of great value regarding the outcome. A case of a 32-year-old man with a splenic inflammatory pseudotumor (IPT) mimicking splenic angiosarcoma is described. The tumor was highly suspected of being splenic angiosarcoma based on radiological findings preoperatively. However, after splenectomy, histopathological examinations revealed splenic IPT. Splenic IPT and angiosarcoma are rare and often pose diagnostic difficulties because the clinical and radiological fi ndings are obscure. Due to large differences in prognosis, we briefl y reviewed the clinical, radiological, and pathological features of both of the tumors.

Hepatic angiosarcoma manifested as recurrent hemoperitoneum

Angiosarcoma is a rare tumor that account for less than 1% of all sarcomas. Although hepatic angiosarcoma usually presents with unspecific symptoms, it rapidly progresses and has a high mortality. We report a rare case of primary hepatic angiosarcoma manifested as recurrent hemoperitoneum.