Multifocal gastrointestinal epithelioid angiosarcomas diagnosed by endoscopic mucosal resection:A case report

angiosarcoma is a vascular neoplasm that is among the most aggressive subtypes of sarcomas.Its involvement in the gastrointestinal tract is rare.We here report a case of multifocal gastrointestinal epithelioid angiosarcomas presenting with gastrointestinal bleeding.CASE SUMMARY A 77-year-old woman was admitted because of melena and dizziness for three months.Gastroscopy and colonoscopy were performed,revealing a centrally ulcerated hemorrhagic polypoid lesion in the gastric body and multiple polypoid lesions with blood clots and hemorrhagic tendency in the colon.Histopathological examination of routine endoscopic biopsy samples showed inflammation in the gastric mucosa and tubular adenomas in the colon.The polypoid lesions were removed by endoscopic mucosal resection.Immunohistochemistry suggested a final diagnosis of epithelioid angiosarcomas.The patient refused chemotherapy and died after three months.CONCLUSION Epithelioid angiosarcomas are characterized by highly vascular nature and tendency to cause gastrointestinal bleeding.Efforts to obtain histological findings using endoscopic mucosal resection are of great importance.

Primary malignant vascular tumors of the liver in children:Angiosarcoma and epithelioid hemangioendothelioma

Primary malignant vascular neoplasms of the liver,angiosarcoma and epithelioid hemangioendothelioma,are extremely rare entities in the pediatric population.International Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age group.In this article,we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric population.Hepatic angiosarcoma in children shows a slight female predominance with an average age of 40 mo at diagnosis.The distinct histologic features include whorls of atypical spindled cells and eosinophilic globules,in addition to the general findings of angiosarcoma.Histologic diagnosis of pediatric hepatic angiosarcoma is not always straightforward,and the diagnostic challenges are discussed in the article.Hepatic epithelioid hemangioendothelioma also demonstrates a female predominance,but is more commonly identified in adolescents(median age at diagnosis:12 years).Histologically,the lesion is characterized by epithelioid cells and occasional intracytoplasmic lumina with a background of fibromyxoid stroma.While WWTR1-CAMTA1 and YAP1-TFE3 fusions have been associated with epithelioid hemangioendothelioma,there are currently no known signature genetic alterations seen in pediatric hepatic angiosarcoma.Advancement in molecular pathology,particularly for pediatric hepatic angiosarcoma,is necessary for a better understanding of the disease biology,diagnosis,and development of targeted therapies.

Pelvic Multifocal Primitive Bone Epithelioid Angiosarcoma (Iliac Crest and Hip) about One Case and Literature Review

Epithelioid angiosarcoma is a rare vascular tumour with dark prognosis at short term. The primitive bone localization is exceptional and sometimes presents itself under a unifocal form. We report a clinical case of pelvic multifocal primitive bone epithelioid angiosarcoma (iliac crest and hip) diagnosed on a young male adult (45 years old). The radiological investigations put in evidence geographic osteolytic lesions (type IC) and, diagnostic was given thanks to the histological examination of the surgical biopsy pieces. The evolution was marked by the appearance of pulmonary and lymph node metastases with a death occurring in less than 4 months. Extremely rare tumour, bone epithelioid angiosarcoma is a variant of angiosarcoma characterised by an aggressive nature whose evolution is always fatal. Through this observation we report our experience on the diagnostic approach;and using a literature review we discuss the therapeutic modalities of this tumour with very bad prognosis.

Hepatic perivascular epithelioid cell tumors:The importance of preoperative diagnosis

Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors(PEComas)because PEComas are mainly benign tumors and may not require surgical intervention.By analyzing the causes,properties and clinical manifestations of PEComas,we summarize the challenges and solutions in the diagnosis of PEComas.

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.

Massive simultaneous hepatic and renal perivascular epithelioid cell tumor benefitted from surgery and everolimus treatment:A case report

BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus.The occurrence of this tumor in the liver,particularly with simultaneous involvement of the liver and kidney,is exceedingly uncommon.Pathological diagnosis is the gold standard.PEComas usually show positive immunohistochemical staining for melanocytic(HMB-45,Melan-A)and myoid(SMA,muscle-specific actin)markers.CASE SUMMARY We presented a noteworthy case of malignant PEComa affecting both the liver and kidney in a 53-year-old man with tuberous sclerosis complex(TSC).FAT2 and TP73 mutations in the kidney were identified and positive expression of diagnostic markers including HMB-45,Melan A,and TFE3 were detected.In addition,we demonstrated that hepatic artery perfusion chemotherapy was ineffective for hepatic PEComa,while surgery remained the most effective approach.Everolimus showed an excellent efficacy in the postoperative treatment of the tumor.CONCLUSION Surgical treatment is preferred for malignant PEComa affecting liver and kidney,especially with TSC;everolimus is effective postoperatively.

Uterine epithelioid trophoblastic tumor with the main manifestation of increased human chorionic gonadotropin:A case report

BACKGROUND Epithelioid trophoblastic tumor(ETT)is an extremely rare malignant gestational trophoblastic neoplasm commonly presenting with abnormal vaginal bleeding,abdominal pain,and increased human chorionic gonadotropin(hCG).This study reported a case of uterine ETT with the main manifestation being increased hCG.CASE SUMMARY A 39-year-old female was referred to the Ningbo Maternal and Child Hospital of China in December 2022,complaining of increased hCG levels for 1 month.Magnetic resonance imaging revealed gestational trophoblastic tumor,and hysteroscopic electrotomy and curettage of intrauterine hyperplasia were performed.The patient was diagnosed with uterine ETT through postoperative pathological examination and immunohistochemical results.Total laparoscopic hysterectomy and bilateral salpingectomy were performed,and hCG levels returned to normal.The patient was without recurrence during the postoperative 3-month follow-up.CONCLUSION This study reported a case of uterine ETT with the main manifestation being increased hCG,highlighting that ETT should be considered in the presence of abnormal hCG.A total laparoscopic hysterectomy is recommended.

Clinical review and literature analysis of hepatic epithelioid angiomyolipoma in alcoholic cirrhosis: A case report

BACKGROUND Hepatic epithelioid angiomyolipoma(HEA)has a low incidence and both clinical manifestations and imaging lack specificity.Thus,it is easy to misdiagnose HEA as other tumors of the liver,especially in the presence of liver diseases such as hepatitis cirrhosis.This article reviewed the diagnosis and treatment of a patient with HEA and alcoholic cirrhosis,and analyzed the literature,in order to improve the understanding of this disease.CASE SUMMARY A 67-year-old male patient with a history of alcoholic cirrhosis was admitted due to the discovery of a space-occupying lesion in the liver.Based on the patient’s history,laboratory examinations,and imaging examinations,a malignant liver tumor was considered and laparoscopic partial hepatectomy was performed.Postoperative pathology showed HEA.During outpatient follow-up,the patient showed no sign of recurrence.CONCLUSION HEA is difficult to make a definite diagnosis before surgery.HEA has the poten-tial for malignant degeneration.If conditions permit,surgical treatment is recom-mended.

Sclerosing epithelioid fibrosarcoma of the pancreas:A case report

BACKGROUND A sclerosing epithelioid fibrosarcoma(SEF)is a rare malignant fibroblastic soft tissue tumor that rarely occurs in intra-abdominal organs.A case of a SEF in the pancreatic head is reported herein,including its clinical manifestations,preoperative imaging features,gross specimen and pathological findings.CASE SUMMARY A 33-year-old male patient was admitted to Peking Union Medical College Hospital in December 2023 due to a one-year history of intermittent upper abdominal pain and the discovery of a pancreatic mass.The patient underwent an enhanced computed tomography scan of the abdomen,which revealed a welldefined,round mass with clear borders and calcifications in the pancreatic head.The mass exhibited progressive,uneven mild enhancement,measuring approximately 6.6 cm×6.3 cm.The patient underwent laparoscopic pylorus-preserving pancreaticoduodenectomy.Postoperative pathological examination revealed that the lesion was consistent with a SEF.At the 3-month postoperative follow-up,the patient did not report any short-term complications,and there were no signs of tumor recurrence.CONCLUSION SEFs are rare malignant fibrous soft tissue tumors.SEFs rarely develop in the pancreas,and its preoperative diagnosis depends on imaging findings,with confirmation depending on pathological examination and immunohistochemistry.Currently,only four cases of pancreatic SEF have been reported in studies written in English.This case is the first reported case of a pancreatic SEF by a clinical physician.

Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma:Three case reports

BACKGROUND Hepatic perivascular epithelioid cell neoplasms(PEComas)are rare.Diagnostic and treatment experience with hepatic PEComa remains insufficient.CASE SUMMARY Three hepatic PEComa cases are reported in this paper:One case of primary malignant hepatic PEComa,one case of benign hepatic PEComa,and one case of hepatic PEComa with an ovarian mature cystic teratoma.During preoperative imaging and pathological assessment of intraoperative frozen samples,patients were diagnosed with hepatocellular carcinoma(HCC),while postoperative pathology and immunohistochemistry subsequently revealed hepatic PEComa.Patients with hepatic PEComa which is misdiagnosed as HCC often require a wider surgical resection.It is easy to mistake them for distant metastases of hepatic PEComa and misdiagnosed as HCC,especially when it’s combined with tumors in other organs.Three patients eventually underwent partial hepatectomy.After 1-4 years of follow-up,none of the patients experienced recurrence or metastases.CONCLUSION A clear preoperative diagnosis of hepatic PEComa can reduce the scope of resection and prevent unnecessary injuries during surgery.

Epithelioid Angiosarcoma of Bone: A Neoplasm with Potential Pitfalls in Diagnosis

Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. This subset can radiologically mimic non-vascular neoplasms and impose serious challenges in reaching the correct diagnosis. Meanwhile histological diagnosis can be extremely challenging too, as the pathological features often resemble that of aneurysmal bone cysts. We present an unusual case of a 22-year-old woman who presented with a rapidly growing humeral tumor of 8 months’ duration. The case of intraosseous angiosarcoma presented as a diagnostic dilemma and the relevant radiological and pathologic findings were discussed. We describe the clinical, radiological and pathological features of this unique case, and review the literature concerning Angiosarcoma of bone. Our case highlights the diagnostic difficulties for such very rare tumours and clinico-pathological correlation is of paramount importance to differential diagnosis.

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.

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.

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.

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.

Long-term durable response to lenalidomide in a patient with hepatic epithelioid hemangioendothelioma

Epithelioid hemangioendothelioma(EH)is a rare tumor arising from the vascular endothelial cells of soft tissue or visceral organs.The most common visceral site is the liver,where it is often involved in a multifocal man-ner known as hepatic EH(HEH).Surgical resection with curative intent represents the gold standard therapy.When surgery is not feasible,or in cases of metastatic disease,no standard medical treatment is currently indicated.In small series,drugs with anti-angiogenic activity(such as bevacizumab,sorafenib,thalidomide,and lenalidomide)have been proposed with promising results.We describe a 73-year-old man with multifocal non-resectable HEH treated with lenalidomide.Disease status was evaluated by abdominal ultrasound and magnetic resonance every four months.The patient was treated for a total of 39 mo with prolonged disease stabilization and,at the time of writing,is still under treatment with a good tolerance profile.During a short period of treatment discontinuation,the disease showed slight progression that immediately resolved after the reintroduction of lenalidomide.Lenalidomide may represent a valid treatment option for HEH due to its anti-angiogenic and antineoplastic activities.This preliminary result merits further study in a large series.

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.

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.