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Epithelioid angiosarcoma

Ana Ortins-Pina, Mar Llamas-Velasco, Sara Turpin, Luís Soares-de-Almeida, Paulo Filipe, Heinz Kutzner
BACKGROUND: Accurate distinction of epithelioid hemangioma (EH) from its malignant mimics is paramount but it remains challenging due to its wide morphological spectrum and lack of objective molecular markers. FOSB oncogenic activation was recently identified as a key event in endothelial proliferation. We sought to investigate the FOSB staining pattern in EH with angiolymphoid hyperplasia with eosinophilia morphology (EH-AHLE) and to evaluate its value in differential diagnosis of epithelioid vascular tumors...
March 12, 2018: Journal of Cutaneous Pathology
Muhammad Waqas, Waqas Rahim, Durre Shohab, Muhammad Athar Khawaja, Zafar Ali, Nadira Mamoon
Primary renal angiosarcoma is a rare tumor. Epithelioid variant of primary renal angiosarcoma is extremely rare and aggressive entity described in literature as a few case reports. It presents as solid looking renal mass as other renal cell carcinomas. Management is not well described due to scarcity of cases and aggressiveness of disease. We hereby report a case of epithelioid renal angiosarcoma in a 62-year female who presented with features of infected perinephric collection. Histopathological and immunohistochemical examinations of the renal specimen revealed lower polar renal tumor with atypical epithelioid cells having eccentric nuclei with coarse chromatin and eosinophilic cytoplasm, which were positive for vascular endothelial (FLI-1, CD 34, CD31 and factor VII) as well as epithelial (CKAE1/AE3) immune markers...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Jasmine Haller, Marjorie Parker David, Nathan E Lee, Sara C Shalin, Jerad M Gardner
CONTEXT: - Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. OBJECTIVE: - To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. DESIGN: - Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [×2], and desmoid fibromatosis), and 6 without "active" pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma)...
January 29, 2018: Archives of Pathology & Laboratory Medicine
Lynette L Studer, Dale M Selby
Epithelioid hemangioendothelioma is a rare vascular tumor, composed of epithelioid and histiocytoid vascular endothelial cells in myxoid or fibrotic stroma, which can arise in multiple locations throughout the body. In the liver, this neoplasm usually presents on imaging as an incidental finding of multifocal, heterogeneously enhancing nodules in both lobes or presents clinically with nonspecific abdominal symptoms. Histologically, the tumor has been mistaken for metastatic carcinoma, angiosarcoma, hepatocellular carcinoma, and cholangiocarcinoma...
February 2018: Archives of Pathology & Laboratory Medicine
Sarah Walcott-Sapp, Ephraim Tang, Sanjay Kakar, Jian Shen, Paul Hansen
Hepatic small vessel neoplasm (HSVN) is a recently described vascular neoplasm of the adult liver. The neoplastic cells are positive for markers of vascular lineage (CD31, CD34, FLI-1). The distinctive morphology and infiltrative borders separate HSVN from benign vascular tumors such as cavernous hemangioma, while lack of atypical morphologic features, low to absent mitotic activity and low proliferation index distinguish it from malignant vascular tumors such as epithelioid hemangioendothelioma and angiosarcoma...
January 20, 2018: Human Pathology
Ioannis T Konstantinidis, Carolijn Nota, Zeljka Jutric, Philip Ituarte, Warren Chow, Peiguo Chu, Gagandeep Singh, Susanne G Warner, Laleh G Melstrom, Yuman Fong
BACKGROUND AND OBJECTIVES: Primary liver sarcomas (PLS) are rare. Published series are limited by small numbers of patients. METHODS: We reviewed the National Cancer Database (2004-2014) for patients who underwent surgical resection of PLS. RESULTS: Of 237 patients identified, the majority were female (60.8%), with median age of 52 years. Histologies were: epithelioid hemangioendothelioma (n = 67), angiosarcoma (n = 64), leiomyosarcoma (n = 33), embryonal rhabdomyosarcoma (n = 31), carcinosarcoma (n = 16), giant cell sarcoma (n = 14), spindle cell sarcoma (n = 12)...
January 22, 2018: Journal of Surgical Oncology
Yin P Hung, Jason L Hornick
Mesenchymal neoplasms rarely present in or adjacent to endocrine organs. In this context, the recognition of these rare tumor types can be challenging, with significant potential for misdiagnosis as sarcomatoid carcinomas (i.e., anaplastic thyroid carcinoma and sarcomatoid adrenal cortical carcinoma) or neuroendocrine carcinomas, depending upon the dominant histologic patterns. In this review, we address potential pitfalls in diagnosing selected mesenchymal neoplasms arising within or near endocrine organs, including dedifferentiated liposarcoma, synovial sarcoma, angiosarcoma, PEComa, proximal-type epithelioid sarcoma, Ewing sarcoma, and neuroblastoma...
January 16, 2018: Endocrine Pathology
Yunus Oc, Bekir Eray Kilinc, Rodi Ertugrul, Hasan Basri Sezer, Osman Tugrul Eren
Primary malignant vascular tumors which constitute less than 1% of the primary bone tumors are very rarely encountered. Epithelioid angiosarcoma is a very uncommon subtype of angiosarcomas presenting with an epithelioid phenotype and mimicking carcinomas clinically. These tumors select mostly extra-skeletal locations but rarely bones as their medium. A 31-year-old male patient applied to our clinic with a nonspecific left hip pain. X-ray evaluation revealed a cystic expansile mass in the trochanteric region of the femur extending to the femoral neck...
December 2017: World Journal of Oncology
Jalal A Khan, Robert G Maki, Vinod Ravi
Angiosarcoma, epithelioid hemangioendothelioma, and Kaposi sarcoma are classified according to the line of differentiation that these neoplastic cells most closely resemble: the endothelial cell. Although these malignant vascular sarcomas demonstrate immunohistochemical and ultrastructural features typical of this lineage, they vary dramatically in presentation and behavior, reflecting oncologic mechanisms unique to each. Antineoplastic therapies offer significant benefit, but because of the rarity of these cancers, novel therapies are slow to develop, and treatment options for these cancers remain limited...
January 10, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Shi-Long Zhang, Li Liang, Yuan Ji, Zhi-Ming Wang, Yu-Hong Zhou
Epithelioid angiosarcoma (EA) is a kind of rare malignant soft tissue sarcoma, with high recurrence/metastatic rate and poor prognosis. To date, no effective standardized treatment regimen has been available for patients with recurrence/metastatic EA. Everolimus is an oral rapamycin derivative that highly inhibits the mechanistic target of rapamycin(mTOR) signal pathway. Previous studies have suggested that everolimus is effective and safe in some soft tissue sarcoma. We reported two cases with recurrence/metastatic EA, who received everolimus after failure of surgery, radiotherapy, chemotherapy or interventional therapy...
November 7, 2017: Oncotarget
Emily Z Keung, Yi-Ju Chiang, Rachel K Voss, Janice N Cormier, Keila E Torres, Kelly K Hunt, Barry W Feig, Christina L Roland
INTRODUCTION: The incidence and clinical significance of lymph node metastasis (LNM, N1) in soft tissue sarcoma (STS) is unclear. Recent studies have focused on extremity/trunk STS (ETSTS). We sought to define the subgroup of patients with LNM at sarcoma diagnosis across all disease sites and histologies. METHODS: We identified and categorized 89,870 STS patients from the National Cancer Data Base (1998-2012) by nodal stage. Pathologically confirmed LNM (pN1) were identified in 1404 patients; 1750 had clinically suspicious but not pathologically confirmed LNM (cN1)...
January 2018: European Journal of Surgical Oncology
Manish M Subramaniam, Nur L Salleh, Bingcheng Wu, Michelle A Rozario, HueyJin Lim, Mark E Puhaindran, Richie Soong, Victor K Lee
AIMS: We report an unusual case of epithelioid angiosarcoma (AS) mimicking an epithelioid hemangioma (EH) and analyze mutational patterns in EHs and ASs. METHODS AND RESULTS: A 58-year-old woman presented with a finger lump and metastatic lung nodules. Initial needle biopsies showed an EH, with only focal atypical histologic features. The patient underwent finger amputation and resection of lung nodules. The amputation specimen and lung nodules revealed features of AS...
January 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
Eric C Ehman, Michael S Torbenson, Michael L Wells, Brian T Welch, Scott M Thompson, Ishan Garg, Sudhakar K Venkatesh
A number of benign and malignant neoplasms may arise from the vascular elements within the liver parenchyma. Lesions discussed in this article include angiosarcoma, epithelioid hemangioendothelioma, solitary fibrous tumor (hemangiopericytoma), infantile, and cavernous hemangiomas. Despite a common theme of vascular origin, the pathologic and imaging appearance of these entities can be heterogeneous. Angiosarcomas are bizarrely enhancing, highly aggressive tumors, which often present with metastatic disease...
November 20, 2017: Abdominal Radiology
Z B Wang, X J An, J F Deng, J H Liu, H Y Shi
Objective: To investigate the expression of ERG, Fli-1, CD34, CD31 and factor Ⅷ-related antigen(FⅧRAg) in hepatic malignant vascular tumors. Methods: A retrospective analysis was conducted on 63 cases of primary hepatic malignant vascular tumors and 31 cases of hepatic other malignant spindle cell tumors collected during January 1986 to January 2014. EnVision method was used to detect the expression of ERG, Fli-1, CD34, CD31, FⅧRAg. Results: Sixty-three cases of malignant vascular tumors, including 24 cases of angiosarcoma, 38 cases of epithelioid hemangioendothelioma and 1 case of hepatic Kaposi's sarcoma...
November 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Dinesh Pradhan, Karen Schoedel, Richard L McGough, Sarangarajan Ranganathan, Uma N M Rao
Pseudomyogenic hemangioendothelioma (PHE) is an uncommon neoplasm with propensity for local recurrence. The tumor mimics epithelioid hemangioendothelioma and epithelioid sarcoma, representing a possible diagnostic pitfall. We investigated the clinicopathological, immunohistochemical and fluorescence in situ hybridization features of PHEs. Eight cases of PHE were retrieved from our pathology archives. The clinical and outcome information was available in six patients. In six cases the tumors were located in the lower limb while the upper limb was involved in two cases...
November 2, 2017: Human Pathology
Belle Vivica van Rosmalen, Joanne Verheij, Saffire S K S Phoa, Thomas Matthijs van Gulik
We present a case of a 77-year-old male patient with a liver tumour diagnosed as hepatic epithelioid haemangioendothelioma (HEHE), a potentially malignant tumour treated with liver resection. The patient is disease-free 3 years after resection. Imaging features using fludeoxyglucose F 18 positron emission tomography CT and MRI with gadoxic acid as well as histopathological findings are discussed.
November 3, 2017: BMJ Case Reports
Richard C Semelka, Nadesan Nimojan, Saman Chandana, Miguel Ramalho, Suzanne L Palmer, Danielle DeMulder, Carolina Parada Villavicencio, John Woosley, Bonnie L Garon, Reena C Jha, Frank H Miller, Ersan Altun
PURPOSE: To determine if rare primary malignancies of the liver may have consistent features on magnetic resonance imaging (MRI). MATERIALS AND METHODS: This IRB-compliant retrospective study reviewed the records from the pathology departments of four university centres over an 11-year period from 2005-2016 to identify rare primary malignant tumours, which were cross-referenced with MRI records. MRI studies of these patients were reviewed to determine if these tumours exhibited consistent and distinctive features...
October 27, 2017: European Radiology
M A Ennouhi, A Guerrouani, A Moussaoui
Epithelioid hemangioendothelioma (EHE) is an infrequent vascular tumor that has an intermediate prognosis between the prognosis of the angioma and that of the angiosarcoma. Its evolution is characterized by a tendency to recurrence and metastases. EHE has been reported in locations such as: lungs, liver, bone and soft tissues. There are very few reports of cases involving the head and neck region. The occurrence of EHE in the orbital region and/or the eyelid is extremely rare. The authors report a case of EHE in the right upper eyelid...
October 11, 2017: Journal of Stomatology, Oral and Maxillofacial Surgery
Suya Hori, Motoko Tachihara, Daisuke Tamura, Kazuyuki Kobayashi, Kyosuke Nakata, Hiroshi Kamiryo, Yasuhiro Sakai, Tomoo Itoh, Takanori Hirose, Yoshihiro Nishimura
A 20-year-old Japanese woman with a history of pulmonary atresia was referred to our hospital after the detection of an abnormal mass in the right lung and mediastinal lymphadenopathy. A cytological specimen obtained by transbronchial brushing indicated that the pathological diagnosis was non-small cell lung cancer. During the follow-up period, the tumor spontaneously regressed. At four months after the diagnosis, she experienced sudden bleeding from the small intestine. The histological characteristics of the small intestine tumor were compatible with the cytological characteristics of the lung tumor...
October 11, 2017: Internal Medicine
Lauren F Alexander, Peter Harri, Brent Little, Courtney C Moreno, Pardeep K Mittal
Primary hepatic malignancies are less common than metastatic diseases, but a recognition of these lesions is important for diagnosis and treatment planning. Magnetic resonance imaging (MRI) provides the most imaging information to diagnose lesions noninvasively and to narrow differential diagnoses. This paper reviews the imaging findings of chronic liver disease and primary hepatic malignancies, including hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (CCA), epithelioid hemangioendothelioma, hepatic angiosarcoma, and primary hepatic lymphoma...
August 4, 2017: Curēus
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