Case Of Angiosarcoma Research Articles

A Case of Angiosarcoma with Rapid course and death following a head injury

A Case of Angiosarcoma with Rapid course and death following a head injury

A Case of Angiosarcoma with a Chief Complaint of Facial Edema

A Case of Angiosarcoma with a Chief Complaint of Facial Edema

A case of hepatic angiosarcoma with Kasabach-Merritt syndrome

A case of hepatic angiosarcoma with Kasabach-Merritt syndrome

Pulmonary angiosarcoma presenting with diffuse alveolar hemorrhage: a case report

This study aimed to analyze the diagnosis and treatment of one case of pulmonary angiosarcoma (PPA) retrospectively. The main manifestation of this female patient was cough, hemoptysis and dyspnea. Computed tomography (CT) of the chest revealed multiple small nodules and ground-glass patches in both lungs suggesting of diffuse alveolar hemorrhage (DAH). Laboratory examination revealed decreased hemoglobin and platelet counting, normal coagulation function. Results of rheumatic markers testing including antinuclear antibody (ANA), anti-extractable nuclear antigen antibody (ENA), vasculitis marker, and antiphospholipid antibody were negative. Tumor markers were negative. Sputum smear, sputum culture, and alveolar lavage fluid culture showed negative results. The bone marrow smear was essentially normal. The patient received methylprednisolone pulse therapy (250 mg daily × 5 days) and immunoglobin (20 d daily × 7 days) treatment, but her hemoptysis persisted. Bilateral pleural effusion drainage found a large amount of bloody effusion, but cytology of the pleural fluid showed negative results. The clinical symptoms, laboratory results, imaging findings, and pathological features of the patient were summarized, and problems in diagnosis and treatment were discussed. A thoracoscopic lung biopsy was performed and the diagnosis of PPA was confirmed by pathology and immunohistochemistry (IHC) staining. This case suggested that the possibility of PPA should be considered in patients with DAH, but with negative findings in routine examinations, lung biopsy is usually required.

A Case of Primary Angiosarcoma of the Ascending Aorta Causing Recurrent Strokes

A Case of Primary Angiosarcoma of the Ascending Aorta Causing Recurrent Strokes

Reply of the article: Contribution of the Visante® OCT and B-scan ultrasonography in the diagnosis and follow up of a bilateral ciliochoroidal effusion syndrome induced by topiramate

Angiosarcoma is a rare complication of both functioning and nonfunctioning fistulas. It is an aggressive soft tissue sarcoma arising from vascular or lymphatic endothelial cells. We report a case of angiosarcoma from a nonfunctional fistula in a kidney transplantation patient receiving immunosuppressive drugs. The patient had presented with arm pain mimicking a thrombosed arteriovenous fistula.

Malignant Epithelioid Soft Tissue Tumours- A Pathologist's Perspective With Review of Literature.

BackgroundSoft tissue tumours with epithelioid morphology have many differential diagnoses, which include epithelioid sarcoma, malignant extrarenal rhabdoid tumour, epithelioid malignant peripheral nerve sheath tumour, epithelioid leiomyosarcoma, epithelioid angiosarcoma and sclerosing epithelioid fibrosarcoma. There are other rare entities also. They often express characteristic patterns of immunohistochemical markers that can be used to identify these tumours.Materials and methodsThis retrospective study comprises 22 cases of malignant soft tissue tumours with epithelioid differentiation diagnosed over a period of four years. Findings regarding clinical presentation, cytological findings, histopathological findings and immunohistochemical profile of the tumours were noted and analyzed.ResultsA total of 22 cases were included in the study including five cases of epithelioid sarcoma (conventional and proximal), three cases of epithelioid angiosarcoma and epithelioid myxofibrosarcoma, two cases of epithelioid malignant peripheral nerve sheath tumour, epithelioid gastrointestinal stromal tumour and clear cell sarcoma each, one case of epithelioid leiomyosarcoma, sclerosing epithelioid fibrosarcoma, malignant extrarenal rhabdoid tumour, monophasic synovial sarcoma and malignant and malignant perivascular epithelioid cell tumour each.ConclusionPathologists should be aware of the various differential diagnoses of soft tissue tumours with epithelioid morphology. Over and above the clinical findings and morphological features, ancillary methods like immunohistochemistry help to arrive at a definitive diagnosis in most cases.

ETMR-02. NOVEL CIC-LEUTX FUSION IN CNS EMBRYONAL TUMOR: A CASE REPORT AND REVIEW OF THE LITERATURE

INTRODUCTIONCentral nervous system (CNS) embryonal tumor is a group of rare, poorly differentiated neuroepithelial malignant neoplasm predominantly occurs in pediatrics. Herein, we firstly report a CNS embryonal tumor harboring pathogenic CIC-LEUTX fusion.METHODSImmunohistochemistry (IHC), Fluorescence in Situ Hybridization (FISH) and Next Generation sequencing (NGS).RESULTSA 2-year-old male was found to have solid and cystic mass in left temporal lobe-basal ganglia and left parietal lobe (maximum diameter=75mm). The pathological diagnosis was CNS embryonal tumor (NEC) after totally resection. The tumor was poorly differentiated embryonal neoplasms of neuroectodermal origin that lacked the specific features and rosettes. IHC showed Syn was strongly/diffusely positive and Ki67 proliferation index was high (50%+), and copy number at the 19q13.42 C19MC locus showed no alterations. NGS showed pathogenic mutations including a brand new CIC-LEUTX fusion, heterozygous germline NBN c.C127T mutation and somatic TSC2 c.G2714A mutation. One month after operation, intracranial tumor recurred (maximum diameter=55mm) and spinal cord implantation metastasis occurred, and then the patient received chemotherapy (CTX+CBP+VCR/DDP+VP-16) and had significant improvement in symptoms and tumor shrinkage (maximum diameter=31mm). Literature review revealed CIC fusion predominantly presented in sarcomas, such as CIC-NUTM1 fusion in rare CNS sarcoma, CIC-LEUTX fusion in epithelioid angiosarcoma and CIC-DUX4 fusion in Ewing-like sarcoma. Hitherto, apart from this case, there were only two cases which had CIC-LEUTX fusion in CNS, including a case of CNS angiosarcoma and a case of anaplastic ganglioglioma.CONCLUSIONSWe firstly found a specific new type in CNS embryonal tumor with distinct molecular-pathological characteristics of CIC-LEUTX fusion.

Bleeding \u201csarcomatosis\u201d as a rare presentation of vascular graft-related angiosarcoma: case report and review of the literature

BackgroundAngiosarcoma is a rare malignant tumor, originating from vascular endothelial cells, accounting for approximatively 1–2% of soft tissue sarcomas. It is characterized by a rapid proliferation and high metastatic potential. Some cases of angiosarcoma are described in association with vascular prosthesis, orthopedic devices and foreign bodies. Hereby, we report a case of a patient treated with the endovascular placement of a PTFE aorto bis-iliac prosthesis for aortic aneurysm, who developed a graft-related angiosarcoma with bone and peritoneal localizations. The peritoneal “sarcomatosis” led to an acute presentation with hemoperitoneum and anemia. We perform a thorough review of the literature summarizing the description of similar cases, their epidemiology and the possibilities for treatment.Case presentationAn 84-year-old male with a history of abdominal aortic aneurysm endovascular repair presented to our emergency department complaining with low back pain radiating to the left limb. He underwent a type II endoleak embolization of the aneurysmal sac nine days before. During hospitalization he underwent a spine MRI which documented a vertebral alteration of non-univocal interpretation. Vertebral biopsy was performed revealing groups of cells of uncertain nature. He lately underwent percutaneous L2–L4 arthrodesis. Forty-two days after admission, he developed acute anemia. Emergency laparotomy revealed a massive hemoperitoneum and actively bleeding peritoneal nodules. Abdominal packing was performed, and several nodules were sent for definitive histological examination. After surgery, he developed progressive and severe hypovolemic shock and expired on postoperative day 5.ConclusionsAngiosarcoma associated with foreign bodies, especially vascular prosthesis, is a very rare entity. In patients who have a history of prosthetic vascular graft placement that present with lumbar pain, osteolytic changes at radiologic imaging or the development of ascites, angiosarcoma should be considered in the differential diagnosis. Despite the poor prognosis, a prompt diagnosis might give access to an adequate treatment planning, with the aim for disease control and increased survival.

Primary pure angiosarcoma of the testis: a vanishingly rare malignancy. Case report and literature review

BackgroundPrimary pure angiosarcoma of the testis is an exceptionally rare testicular malignancy, which is poorly understood. We present the fifth and youngest case in the current medical literature. Additionally, all cases of angiosarcoma of the testicle, both occurring with associated germ cell tumour and without, were compared in an extended tabular format.Case presentationA 56-year old man presented with unilateral scrotal pain, swelling and erythema. Ultrasonography revealed two testicular lesions with a high suspicion of malignancy but serum tumour markers were negative. A radical orchidectomy was performed with clear surgical margins. Diagnosis of primary pure angiosarcoma of the testis was confirmed on subsequent histopathology.ConclusionsPrimary pure angiosarcoma is a rare testicular neoplasm. We present the fifth case in the literature. Clinical and radiological features are non-specific. The diagnosis is purely histological, with the pathologist choosing immunohistochemistry based on abnormal morphology. Local invasiveness is variable but metastatic sites are typical for extra-gonadal angiosarcomas. Primary pure testicular angiosarcoma diagnosis confers a relatively better prognosis compared to angiosarcoma arising in the context of a testicular germ cell tumour. While extra-gonadal angiosarcomas are associated with high rates of local recurrence following resection, in all cases of testicular angiosarcoma there were no local recurrences following radical orchidectomy. Surgical resection remains the most effective treatment for both subtypes of testicular angiosarcoma.

Relapsing Haemothorax as an Unusual Presentation of Primary Angiosarcoma of the Spleen.

We describe a case of primary angiosarcoma of the spleen presenting as relapsing haemothorax. An 81-year-old woman was referred to our hospital because of a 2-month history of relapsing haemothorax after the performance of urgent splenectomy due to active bleeding from large spleen cysts. No evidence of neoplasm was seen after pathological examination of the spleen. On admission, left haemothorax and 2 new cystic masses in the thoracic wall were documented, both in close relation to the scars of previous surgery and chest tubes. After excision of 1 mass, histological examination revealed angiosarcoma, and a final diagnosis of primary angiosarcoma of the spleen with postsurgical metastatic dissemination to the thorax and pleura was made. Primary angiosarcoma should be included in the differential diagnosis of haemorrhagic spleen cysts. Clinical diagnosis and management usually requires splenectomy, but it should be carefully planned in order to avoid local metastatic dissemination or haematogenous spread of the tumour. The differential diagnosis of spleen cysts must include malignant conditions.Despite its absolute rarity, angiosarcoma is considered the most common primary non-haematolymphoid splenic malignancy and has a poor prognosis.Splenectomy is usually required for a prompt diagnosis of primary angiosarcoma of the spleen, but it needs to be performed with extreme caution to avoid metastatic dissemination.

Radiation-associated angiosarcoma of the brain with repeated intracerebral hemorrhage: A case report

BackgroundDelayed side effects of irradiation to the brain are now known to include the induction of new neoplasms. Radiation-induced angiosarcoma of the central nervous system is extremely rare. We report a case of radiation-associated angiosarcoma. Case descriptionA 40-year-old man was taken to our hospital by ambulance with loss of appetite and impaired consciousness. He had undergone radiotherapy for a germinoma of the left basal ganglia 30 years previously. Initial computed tomography of the head showed a large and well-defined mass in the right temporal lobe. This mass showed heterogeneous contrast enhancement at the periphery on gadolinium-enhanced T1-weighted imaging. We performed an initial operation to evacuate the hematoma and the mass. After that, recurrent bleeding occurred, so we performed evacuation of the hematoma and tumor. A final pathological diagnosis of angiosarcoma was made. ConclusionsThis is the third case of radiation-associated angiosarcoma in the literature. The authors describe this particular case and review the literature pertaining to angiosarcoma of the brain.

Primary epithelioid angiosarcoma originating from the mandibular gingiva: a case report of an extremely rare oral lesion

BackgroundAngiosarcoma occurs very rarely in the oral cavity, and the epithelioid type is even rarer. Here, we report a rare case involving an elderly man with a primary epithelioid angiosarcoma that originated from the mandibular gingiva and resembled a dentigerous cyst on radiographs.Case presentationA 66-year-old Japanese man visited our hospital with a chief complaint of gingival swelling in right mandibular third molar region. A panoramic radiograph showed bone resorption around the crown of right mandibular third molar, which was impacted. Incisional biopsy confirmed a diagnosis of epithelioid angiosarcoma. The lesion exhibited aggressive proliferation after biopsy resulting in uncontrolled bleeding and difficulty in closing the mouth. Mandibular segmental resection including the tumor was performed without reconstruction. Because of the aggressive preoperative course of the tumor, the patient received adjuvant chemotherapy. There were no signs of recurrence during a 2-year follow-up period.ConclusionsA review of the literature yielded only four reported cases of epithelioid angiosarcoma in the jaw region, with the lesions occurring in the maxilla in three cases. To our knowledge, this is the second case of primary epithelioid angiosarcoma in the mandible.

S2508 Primary Hepatic Angiosarcoma Presenting as Decompensated Cirrhosis

INTRODUCTION: Angiosarcomas are uncommon and aggressive endothelial cell tumors which arise from vessels or lymphatic tissue. The common sites are the head, neck, extremities and breast. It can spread hematogenously to lung, liver, lymph nodes, bones and rarely bowel (Lancet oncol 2010; 11:984). Primary hepatic angiosarcoma (HAS) is a rare and aggressive soft tissue tumor with dismal outcome. The known etiologies include exposure to vinyl chloride monomers, thorium oxide, arsenic, anabolic steroids, history of neurofibromatosis and hemochromatosis. CASE DESCRIPTION/METHODS: An 83-year-old male with a history of myasthenia gravis presented with abdominal distention, peripheral edema and intermittent encephalopathy for one month. Myasthenia gravis was treated with prednisone and azathioprine. Labs showed anemia, thrombocytopenia and elevated transaminases (ALT 33, AST 66, ALP 82, total bilirubin 0.7), INR was 1.2. Further testing for viral hepatitis and autoimmune panel were negative. A CT abdomen showed a nodular liver, ascites, innumerable hypoattenuating liver masses with a peripherally enhancing lesion (2.8 cm) and a hyper-vascular 2 cm mass in the right lobe. The head neck and chest imaging were negative for primary malignancy or metastatic disease. Ascites fluid analysis was consistent with portal hypertension. The liver biopsy suggested primary hepatic angiosarcoma which was confirmed on immunohistochemical staining. The patient decided not to opt for treatment and hospice was initiated. DISCUSSION: The only known risk factors in our case were advanced age and concurrent use of immunosuppressive agents. There is no specific tumor marker and a British study had shown negative alpha feto-protein for all the angiosarcoma cases (Gut 1987; 28:669). On immunohistochemistry the tumor cells express CD31, CD34, von Willebrand factor, vascular endothelial growth factor, and Ulex europaeus agglutinin 1. HAS manifests clinically at an advanced stage and life expectancy is usually 6 months. The (AASLD) considers angiosarcoma as a contraindication to liver transplantation (Hepatology 2014; 59:1147). Tumor resection is favorable only in subjects with a solitary tumor involving one hepatic lobe. Chemotherapy or locoregional therapy in surgically non-resectable tumors has shown no survival benefit. Hepatic angiosarcoma can develop in elderly, immunosuppressed individual. It can present as incidental hepatic mass or decompensated liver disease. Liver biopsy with immunohistochemical staining is the best diagnostic test.Figure 1.: CT abdomen axial section showing peripherally enhancing mass (red arrow) in right hepatic lobe.Figure 2.: CT abdomen axial section showing right hepatic lobe hypervascular lesion (red arrow) and diffuse hypoattenuating lesions.Figure 3a.: Liver biopsy Hematoxylin-eosin (H&E) staining stain of left hepatic lobe showing endothelial cells with enlarged hyperchromatic nuclei lining the vascular channels; 3b: Immunohistochemical stain shows that the tumor cells are positive for vascular antigen CD34.

PropAngio study protocol: a neoadjuvant trial on the efficacy of propranolol monotherapy in cutaneous angiosarcoma—a proof of principle study

IntroductionAngiosarcoma is a rare and aggressive malignancy with a high metastatic potential and recurrence rate. Despite optimal treatment with surgery, with or without radiation, the prognosis remains poor and, therefore,...

Male Breast Cancer in Togo: Imaging and Clinicopathological Findings.

Background Breast cancer in men is a rare condition, often diagnosed late. The purpose of this study was to describe its epidemiological, histopathological, and radiographic aspects in Togo. Materials and Methods This was a descriptive retrospective study on cases of breast cancer in humans diagnosed histologically at the Laboratory of Anatomy Pathological and Imagery of the University Hospital in Lomé, over a period of 25 years (1995 to 2019). The parameters studied were epidemiological, anatomopathological, and imaging. Results Eighty-two (82) cases were diagnosed, an annual frequency of 3.28 cases. The mean age was 45 ± 2.5 years; the range was 27-63 years. The family history of 47 patients (57.32%) was known. Carcinomas represented the predominant histological group with predominantly nonspecific invasive carcinoma (87.5%). These cancers were diagnosed at late stages (75.71% grade II). They were mainly of luminal B profile (38.75%) and associated with mutations of the BRCA2 and BRCA1 genes in 14.63% of the cases. The lesions were classified ACR 5 in 61.5% (11/18). Two cases of breast angiosarcoma were diagnosed by the identification of CD31 markers and factor VIII in immunohistochemistry. Hormone therapy such as tamoxifen was prescribed in all luminal patients (43 patients). Radiotherapy was administered to 15 patients (18.3%), with acute toxicity in 20% of the cases. After a median follow-up of 36 months, the evolution was complete remission in 27 patients (32.93%). Conclusion Breast cancer in men is rare, often diagnosed late with a poor prognosis.

Radiation-induced angiosarcoma masquerading as recurrent carcinoma cervix - A diagnostic challenge

Angiosarcomas are malignant mesenchymal tumours that develop from the endothelium of a blood vessel. It is an uncommon cancer that occurs among men and women, more often among older adults and usually in the skin, soft tissue, breast, or liver. Angiosarcoma is a rare but well recognised complication after radiation therapy. Most post radiation angiosarcomas are seen in the breast and very few cases of angiosarcoma have been reported following radiotherapy for cervical cancer.We report one such case of post radiation angiosarcoma which developed years after radiation therapy for carcinoma cervix.

Angiosarcoma radioinducido de mama. Revisión bibliográfica a propósito de dos casos en nuestro centro

ObjectiveLiterature review of the diagnosis and treatment of radiotherapy related angiosarcoma of the breast. Case reportWe present two cases of radiotherapy-related angiosarcoma of the breast treated in our hospital and describe the clinical, diagnostic, and therapeutic features. ConclusionRadiotherapy-related angiosarcoma of the breast is an uncommon tumour that usually presents as a late complication in patients who have undergone surgery and radiotherapy as conservative treatment for breast cancer. Surgery is the only curative treatment. However, because of its high rate of local recurrence, multimodal therapy is required to optimise prevention of recurrence.

Angiosarcoma Ovary and Kasabach–Merritt Syndrome in a Pre-pubertal Girl: An Exceedingly Rare Case Report

Angiosarcoma is an aggressive rare tumour developing from endothelial cells that lines blood vessels (Weiss and Goldblum in Enzinger and Weiss’s soft tissue tumors, Mosby, St. Louis, 2001). They constitutes around 1–2% of soft tissue sarcomas (Mark et al. in Cancer Interdiscip Int J Am Cancer Soc 77(11):2400–2406, 1996; Cafiero et al. in Cancer 77:2496–2502, 1996; Aust et al. in Ann Otol Rhinol Laryngol 106(11):943–951, 1997; Fury et al. in Cancer J 11(3):241–247, 2005). Angiosarcomas usually occur in adult and elderly patients. Reported cases of angiosarcomas in children are of in the head and neck region and mediastinum (Ayadi and Khabir in Arch Pathol Lab Med 134(3):481–485, 2010). Female genital tract angiosarcomas are rarely reported. Here we are reporting a rare case of angiosarcoma ovary in a 12-year-old girl associated with Kasabach–Merritt syndrome. Kasabach–Merritt syndrome describes a consumptive coagulopathy seen in association with vascular tumours. Platelets get trapped in these highly vascular tumours leading to increased consumption of clotting factors and subsequent bleeding episodes (Ohayi et al. in Niger J Med J Natl Assoc Resid Dr Niger 22(4):362–364, 2013).

Imaging features of pathologically proven pediatric splenic masses

Splenic masses present a diagnostic challenge to radiologists and clinicians alike, with a relative paucity of data correlating radiologic findings to pathological diagnosis in the pediatric population. To illustrate splenic mass imaging findings and approximate lesion prevalence, we retrospectively reviewed all splenectomies and splenic biopsies for splenic masses at a single academic pediatric hospital over a 10-year period in patients 18years and younger. A total of 31 splenic masses were analyzed. Lesion prevalence, pathology and imaging features associated with sampled splenic masses are described. The lesions encountered include benign splenic cysts (9), vascular anomalies (7), hamartoma (3), leukemia/lymphoma (3), granulomata (3) and metastasis (2). We also identified single cases of angiosarcoma, splenic cord capillary hemangioma, congestive hemorrhage, and benign smooth muscle neoplasm.