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Aileen Grace P Arriola, Laura A Taylor, Eseosa Asemota, Markus D Boos, David E Elder, Kristy L Weber, Robert G Micheletti, Paul J Zhang
Retiform hemangioendothelioma (RH) is a rare vascular neoplasm with a high rate of local recurrence and low metastatic potential. We describe an unusual case of RH in a 45-year-old patient with Milroy disease, with a prominent solid component diffusely involving a chronic lymphedematous leg. This case is consistent with the postulated relationship between lymphedema and vascular neoplasms developing as a result of local immune dysfunction, and highlights the need to closely monitor patients with Milroy disease for pathologic changes...
October 11, 2016: Journal of Cutaneous Pathology
Paloma Triana, Mariela Dore, Vanesa Nuñez Cerezo, Manuel Cervantes, Alejandra Vilanova Sánchez, Miriam Miguel Ferrero, Mercedes Díaz González, Juan Carlos Lopez-Gutierrez
Aim of the Study mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Patients and Methods A retrospective review of 41 patients treated with sirolimus between January 2011 and December 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendotheliomas, 1 PTEN) and 85% (n = 35) had malformations (13 generalized lymphatic anomalies/Gorham-Stout diseases [GSD], 1 kaposiform lymphangiomatosis [KLA], 11 large lymphatic malformations (LMs) in critical areas, 2 lymphedemas, 4 venous malformations, and 4 aggressive arteriovenous malformations [AVM])...
October 10, 2016: European Journal of Pediatric Surgery
José Maurício Mota, Mariana Scaranti, Leonardo G Fonseca, Diego Araújo Tolói, Veridiana Pires de Camargo, Rodrigo Ramella Munhoz, Olavo Feher, Paulo M Hoff
BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a rare neoplasm of vascular origin that typically arises from the skin or soft tissues as a solitary tumor. The optimal therapy for this disease is still unknown. We report the case of an adult patient presenting with metastatic KHE of the spleen, who had a partial response after treatment with paclitaxel. CASE PRESENTATION: A 36-year-old man presented in November 2012 with a nontraumatic rupture of the spleen...
May 2016: Case Reports in Oncology
Ayan Biswas, Emma C Clark, Chandan K Sen, Gayle Gordillo
: Hemangiomas are endothelial cell tumors and the most common soft tissue tumors in infants. They frequently cause deformity and can cause death. Current pharmacologic therapies have high-risk side effect profiles, which limit the number of children that receive treatment. AIMS: The objectives of this work were to identify the mechanisms through which standardized berry extracts can inhibit endothelial cell tumor growth and test these findings in vivo. RESULTS: EOMA cells are a validated model that generates endothelial cell tumors when injected subcutaneously into syngeneic (129P/3) mice...
October 5, 2016: Antioxidants & Redox Signaling
Beverly A Schaefer, Dehua Wang, Arnold C Merrow, Belinda H Dickie, Denise M Adams
Kaposiform hemangioendothelioma (KHE) is a rare aggressive vascular tumor of skin and deep soft tissues that typically presents in infancy and may be associated with a potentially life-threatening coagulopathy known as Kasabach-Merrit phenomenon (KMP). Recent advances in medical therapy have successfully treated many patients. However, our knowledge regarding the natural history of these lesions and optimum surveillance strategies remains rudimentary. We report two young women who had KHE with KMP treated in infancy and presented in adolescence with comorbidities related to their KHE tumor...
October 4, 2016: Pediatric Blood & Cancer
T Moga, A Popescu, S C Ivascu, M Cornianu, I Sporea
No abstract text is available yet for this article.
May 2016: Ultrasound Int Open
Chia-Hung Wu, Nai-Chi Chiu, Yi-Chen Yeh, Yu Kuo, Sz-Shian Yu, Ching-Yao Weng, Chien-An Liu, Yi-Hong Chou, Yi-You Chiou
BACKGROUND: Beside hepatocellular carcinoma, metastasis, and cholangiocarcinoma, the imaging findings of other relatively uncommon hepatic lesions are less discussed in the literature. Imaging diagnosis of these lesions is a daily challenge. In this article, we review the imaging characteristics of these neoplasms. METHODS: From January 2003 to December 2014, 4746 patients underwent liver biopsy or hepatic surgical resection in our hospital. We reviewed the pathological database retrospectively...
September 2016: Medicine (Baltimore)
Raquel Rojas-Vigott, Cinthya Monge Castro, Steffi Romero Méndez
No abstract text is available yet for this article.
October 2016: Journal of Thoracic Oncology
Z Y Ding, R S Zhang, B Yu, X T Wang, Z F Lu, Q L Shi, X J Zhou, Q Shen
Objective: To investigate the clinicopathologic characteristics, differential diagnosis and prognosis of pulmonary epithelioid hemangioendotheliomas (PEHs). Methods: The clinical symptoms and imaging findings of 6 cases of PEHs were investigated and pathologic analyses including histomorphologic and immunohistochemical studies were performed. Results: Clinical symptoms of the patients were nonspecific and insidious. The typical radiological manifestation was characterized by multiple small pulmonary nodules...
September 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Fatima Zahra Nasreddine, Bouchra Baghad, Soumiya Chiheb
Spindle cell hemangioma, formerly known as spindle cell hemangioendothelioma, was described by Weiss and Enzinger in 1986. Since the advent of immunohistochemical studies it is no longer considered as low grade angiosarcoma. It is a benign vascular tumor It almost exclusively affects the dermis at the distal ends. We report the first case of a patient with spindle cell hemangioma located in the scapular, breast, thighs and mandibular area. According to the literature, only 9 cases located in the head and neck were reported...
2016: Pan African Medical Journal
Stacy E Croteau, Deepti Gupta
Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and long-term outcomes have been hindered by lesion misidentification, imprecise nomenclature, and lack of prospective, randomized clinical trials to assess therapeutic efficacy...
September 2016: Seminars in Cutaneous Medicine and Surgery
Haimin Zhao, Jiankui Han, Lei Qin, Changming Zhang
Bone epithelioid angiosarcoma is an extremely rare osseous malignancy with an aggressive and destructive biological behavior. We present FDG PET/CT findings of a 59-year-old woman with left lower limb pain for 1 month. The images revealed abnormal activity in both left fibula and left tibia. In addition, abnormal activity in the sacrum and enlarged retroperitoneal lymph nodes was also noted. Pathological examination demonstrated bone epithelioid angiosarcoma.
November 2016: Clinical Nuclear Medicine
Arianeb Mehrabi, Katrin Hoffmann, Karl Heinz Weiss, Carolin Mogler, Peter Schemmer, Markus W Büchler, Ingo Alldinger
BACKGROUND: Primary Hepatic Epithelioid Hemangioendothelioma is an extremely rare tumor of vascular origin. PATIENTS: In this work we present clinical data and long term results of eight patients who were surgically treated in our institution. Liver transplantation was performed in five patients (four cadaveric and one living related) and major liver resection in three patients. Three patients are alive with recurrent tumor. After a median follow-up of 100 months (48-266) all patients are alive...
November 2016: Annals of Medicine and Surgery
Mainak Dutta, Kuntal Roy
No abstract text is available yet for this article.
August 2016: Ear, Nose, & Throat Journal
Shintaro Sugita, Hiroshi Hirano, Noriaki Kikuchi, Terufumi Kubo, Hiroko Asanuma, Tomoyuki Aoyama, Makoto Emori, Tadashi Hasegawa
BACKGROUND: Pseudomyogenic hemangioendothelioma (PHE) is an unusual vascular tumor of intermediate malignancy that rarely metastasizes and tends to arise in the lower limbs of young adults and children. Histologically, PHE shows fascicular proliferation of eosinophilic spindle cells and/or epithelioid cells showing "pseudomyogenic" morphology. Immunohistochemically, PHE is usually positive for vimentin, cytokeratin, CD31 and ERG. METHOD: We examined FOSB immunohistochemistry (IHC) in 27 cases consisting of 4 PHE and its histologic mimics including 6 epithelioid hemangioendotheliomas (EHE), 8 angiosarcomas (AS), 4 Kaposi sarcomas (KS) and 5 epithelioid sarcomas (ES)...
August 11, 2016: Diagnostic Pathology
Shraddha A Adamane, Kedar K Deodhar, Amit M Gupta, George Karimundackal, Sangeeta B Desai
Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular neoplasm, predominantly encountered in women, more often in the age group of 40 years and below. It is a tumor of borderline malignant potential with a clinical course intermediate between hemangioma and angiosarcoma. The tumor has variable prognosis, and treatment options include surgical excision in operable cases and chemotherapy in disseminated ones. The present report describes complete clinical, radiological, and histopathological features of PEH with osteoclast-like giant cells and metaplastic ossification in a 20-year-old boy who presented with dyspnea and episodes of hemoptysis with review of literature...
July 2016: Indian Journal of Pathology & Microbiology
Bhagyalakshmi Atla, P V Sudhakar, Nagarjun Rao, Uma Prasad
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin) cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach-Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.
July 2016: Indian Journal of Pathology & Microbiology
Bharat Rekhi, Ashish Gulia, Venkatesh Rangarajan
Pseudomyogenic hemangioendothelioma (PHE) is an uncommon, but distinctive soft tissue tumor, characterized by multifocality. A 17-year-old male referred to us with progressively increasing multiple subcutaneous nodular lesions over his left leg and foot, reported elsewhere as a spindle cell rhabdomyosarcoma. On review, microscopy showed a cellular tumor comprising plump spindle cells arranged in loose fascicles with interspersed inflammatory cells. Tumor cells exhibited mild nuclear variation. Immunohistochemically, tumor cells expressed AE1/AE3, CD31, Fli-1, and smooth muscle actin (SMA), confirming diagnosis of PHE...
July 2016: Indian Journal of Pathology & Microbiology
Norifumi Tsubokawa, Hiroaki Harada, Daiki Taniyama, Takahiro Uemura, Kazuya Kuraoka, Yoshinori Yamashita
Epithelioid sarcoma-like hemangioendothelioma is a rare vascular neoplasm that usually occurs in the distal extremities of young adults. Although the overall behavior of this neoplasm is usually indolent with a low risk of distant metastasis, the risk of local recurrence is significant. Therefore, initial surgical treatment with an adequate margin is important to improve the prognosis. However, epithelioid sarcoma-like hemangioendothelioma is frequently misdiagnosed. A preoperative misdiagnosis could result in resection without a sufficient margin...
August 4, 2016: Asian Cardiovascular & Thoracic Annals
Chun Ian Soo, Boon Hau Ng, Eng Liang Tan, Faisal Abdul Hamid
Pulmonary epithelioid hemangioendothelioma is an uncommon lung malignancy of endothelial origin. Besides demonstrating unpredictable presentation features and prognosis, the paucity of established treatment guidelines remains a challenge in managing these patients. We present two patients. The first patient presented with chronic productive cough over 1-year duration. He was initially diagnosed and showed partial response to treatment for cardiac failure. A persistent right upper zone consolidation on chest radiograph prompted further investigations which revealed the diagnosis of pulmonary epithelioid hemangioendothelioma...
2016: SAGE Open Med Case Rep
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