Sample records for kaposiform hemangioendothelioma khe

  1. Kaposiform Hemangioendothelioma: Case Report and Literature Review

    Institute of Scientific and Technical Information of China (English)


    @@ Kaposiform hemangioendothelioma (KHE) is a rare locally aggressive vascular tumor that mainly occurs in children and early adolescents. It is often associated with the Kasabach-Meritt phenomenon which is marked by severe thrombocytopenia and a variable degree of anemia.

  2. Imaging findings of Kaposiform Hemangioendothelioma in children. (United States)

    Ryu, Young Jin; Choi, Young Hun; Cheon, Jung-Eun; Kim, Woo Sun; Kim, In-One; Park, Ji Eun; Kim, Yu Jin


    Kaposiform hemangioendothelioma (KHE) is a rare, aggressive vascular tumor that typically occurs during infancy or early childhood. Though several case reports have discussed the imaging findings of KHE, larger comprehensive studies are lacking. The purpose of this study was to evaluate the imaging findings of KHE in children. A total of twelve cases of pathologically proven KHE were collected by searching our institution's pathology database for children diagnosed between January 2004 and April 2016 (6 male, 6 female; median age: 3 months; age range 7days - 18 years). CT (n=7) and MRI (n=9) findings were retrospectively evaluated. The location, morphology, enhancement, and growth pattern were analyzed. KHEs involved various locations: superficial or deep soft tissue of the extremities (n=4); abdomen (n=3; 2 cases, pancreas; 1 case, small bowel), neck and mediastinum (n=1); chest wall, diaphragm, and pericardium (n=1); abdominal wall (n=1); and head (IAC, CP angle) (n=2). Eight of 10 cases (80%) with CT/MR findings were locally invasive and involved two or more adjacent organs. Three cases presented as well-defined solid masses, and seven were associated with infiltrative lesions of the surrounding areas with (n=4) or without definite solid regions (n=3). In nine patients with MRI, all KHEs were heterogeneous and hyperintense to muscles on T2-weighted images (T2WI), while four KHEs consisted of some regions that were nearly isointense to muscle. Eight of the 10 cases with imaging exhibited heterogeneous intense enhancement, while only one demonstrated mild enhancement. Signal voids (n=2), engorged vessels (n=1), calcification (n=3), hemorrhage (n=1), or bone changes (n=4) were infrequently observed. Four patients (33%) had Kasabach-Merritt phenomenon, and recurrence was observed in two cases. KHEs occurred in various locations, affected mostly infants, and generally exhibited intense heterogeneous enhancement. In more than half of the included cases, KHEs were

  3. Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood

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    Bhagyalakshmi Atla


    Full Text Available 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.

  4. Kaposiform hemangioendothelioma arising in the deltoid muscle without the Kasabach-Merritt phenomenon

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    Tamai, Noriyuki; Yoshikawa, Hideki [Osaka University Graduate School of Medicine, Department of Orthopaedics, Suita, Osaka (Japan); Hashii, Yoshiko [Osaka University Graduate School of Medicine, Department of Pediatrics, Suita, Osaka (Japan); Osuga, Keigo [Osaka University Graduate School of Medicine, Department of Diagnosis and Interventional Radiology, Suita, Osaka (Japan); Chihara, Tsuyoshi; Morii, Eiichi; Aozasa, Katsuyuki [Osaka University Graduate School of Medicine, Department of Pathology, Suita, Osaka (Japan)


    Kaposiform hemangioendothelioma (KHE) is a rare tumor that occurs nearly exclusively during infancy and childhood. It has features common to both capillary hemangioma and Kaposi sarcoma and for that reason many terms have been used for these tumors including ''Kaposi-like infantile hemangioendothelioma'' and ''hemangioma with Kaposi sarcoma-like features.'' KHE typically presents as an ill-defined, red to purple, indurated plaque and is often complicated by the Kasabach-Merritt phenomenon (KMP), a condition of severe thrombocytopenia and consumptive coagulopathy. Knowledge of the radiological findings of this uncommon tumor might be helpful for diagnosis. We present the MRI features of a case of KHE with neither typical skin lesions nor the Kasabach-Merritt phenomenon. (orig.)

  5. Adult-onset Kaposiform hemangioendothelioma of the tongue: case report and review of the literature (United States)

    Vashi, P.; Abboud, E.; Bier-Laning, C.; Gupta, D.


    We present here a very rare clinical case of a 38-year-old man with Kaposiform hemangioendothelioma (khe) of the tongue who presented to our institution with a growth under the left side of the tongue with no pain or discomfort. There were no enlarged lymph nodes and no significant neurologic findings. Diagnostic histopathology confirmed the lesion to be khe. The tumour was removed surgically, and the surgical specimen confirmed the diagnosis. Follow-up at 3 months shows no clinical evidence of recurrence. PMID:27803613

  6. The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon (United States)

    Vivas-Colmenares, Grecia V.; Ramirez-Villar, Gema L.; Bernabeu-Wittel, Jose; Matute de Cardenas, Jose A.; Fernandez-Pineda, Israel


    Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas. Management of vascular tumors complicated by KMP is challenging, and it is common for referral centers to receive patients in critical medical condition after multimodality treatment failure of vascular anomalies. Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP. A full-term male newborn with KHE complicated by KMP is reported. Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids. PMID:25692091

  7. Surgical treatment of a huge kaposiform hemangioendothelioma in the chest wall: A case study

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    Xiaonan Guo


    Full Text Available Kaposiform hemangioendothelioma, a rare vascular pediatric tumor often associated with Kasabach–Merritt phenomenon, is characterized by severe thrombocytopenia and consumptive coagulopathy. Kaposiform hemangioendothelioma is a severe disease and may progress quickly, resulting in a high mortality. However, standard treatment regimens for Kasabach–Merritt phenomenon have not yet been established. We reported here an infant with a large congenital kaposiform hemangioendothelioma in his chest wall who responded extremely well to surgical excision.

  8. [Role of master transcriptional factor Prox-1 in lymphatic endothelial differentiation of Kaposiform hemangioendothelioma]. (United States)

    Ke, Z Y; Yang, S J


    Objective: To analyze the clinical and pathological features of Kaposiform hemangioendothelioma (KHE), and to investigate the role of master transcriptional factor Prox-1 in the regulation of lymphatic differentiation. Methods: Nine cases of KHE (during the period from October 2009 to June 2016) were collected with clinical and pathological data. H&E stained section review and immunohistochemietry using the Dako EnVision method were performed. Results: There were 6 female and 3 male patients with age ranging from 2 months to 8 years (median 3 years and 4 months). The patients presented with either single subcutaneous soft tissue mass, or bone tumors, with the duration of disease onset ranging from 1 month to 1 year. The sites of involvement included the skins of neck (2 cases), nose root (1 case), inguinal (1 case), thigh root (1 case), humerus (2 cases), lumbar vertebrae(1 case), and mesentery (1 case). These tumors were histologically composed of nodules of densely packed spindle or ovoid cells and deformed small blood vessels in an invasive growth pattern. The tumor cells were immunohistochemically positive for both blood vessels and lymphatic endothelial markers, including Prox-1, the master transcriptional factor, and VEGFR-3. With followed-up from 1 to 60 months (median 26 months), two patients died of the disease, while the remaining patients were alive without recurrence. Conclusions: KHE is a rare vascular tumor with at least partial lymphatic endothelial differentiation, in which Prox-1 may act as a master regulator for such differentiation. KHE is an aggressive tumor of intermediate malignant potential, with local invasion and recurrence tendency, and long term follow-up is required.

  9. Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: ultrastructural observation and immunohistochemistry staining reveal the trapping of blood components. (United States)

    Yuan, Si-Ming; Hong, Zhi-Jian; Chen, Hai-Ni; Shen, Wei-Min; Zhou, Xiao-Jun


    Kaposiform hemangioendothelioma (KHE), a borderline tumor of endothelial origin, is associated with Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia and consumptive coagulopathy resulting from the localized intravascular coagulation (LIC) in the tumor. Previous studies have suggested that the trapping of blood components, including platelets, may underlie the LIC in KHE. However, more evidence is needed to support this hypothesis. In this study, one case of a Chinese infant with a KHE in the left arm was complicated by Kasabach-Merritt phenomenon. The tumor was partially resected and the sample was used for ultrastructural observation and immunohistochemistry staining of Glut-1. Ultrastructural observation found the trapping of erythrocytes, platelets, macrophages, and lymphocytes in the slit-like channels of the tumor nodules, and phagocytic vesicles in the cytoplasm of neoplastic cells. Immunohistochemistry staining further showed numerous Glut-1(+) erythrocytes in the channels. In conclusion, our results provided compelling morphological evidence of the trapping of blood components in KHE, which may interpret the LIC in the tumor and subsequent consumptive coagulopathy.

  10. Successful Propranolol Treatment of a Kaposiform Hemangioendothelioma Apparently Resistant to Propranolol. (United States)

    Filippi, Luca; Tamburini, Angela; Berti, Elettra; Perrone, Anna; Defilippi, Claudio; Favre, Claudio; Calvani, Maura; Della Bona, Maria Luisa; la Marca, Giancarlo; Donzelli, Gianpaolo


    A newborn with unresectable kaposiform hemangioendothelioma associated with Kasabach Merritt phenomenon, unresponsive to vincristine and prednisone, received second-line treatment with propranolol at a dose of 2 mg/kg/day, starting at 2 months of life and continued for 13 months. There was only slight reduction in tumor mass, but measurement of propranolol levels showed extremely low plasma concentrations. The propranolol dose was progressively increased to 3.5 mg/kg/day, leading to a substantial increase in plasma levels associated with clinically relevant tumor reduction. This case highlights the importance of relating propranolol dose to its plasma concentration before considering the treatment ineffective for this vascular tumor.

  11. Exome sequence analysis of Kaposiform hemangioendothelioma: identification of putative driver mutations* (United States)

    Egashira, Sho; Jinnin, Masatoshi; Harada, Miho; Masuguchi, Shinichi; Fukushima, Satoshi; Ihn, Hironobu


    BACKGROUND Kaposiform hemangioendothelioma is a rare, intermediate, malignant tumor. The tumor's etiology remains unknown and there are no specific treatments. OBJECTIVE In this study, we performed exome sequencing using DNA from a Kaposiform hemangioendothelioma patient, and found putative candidates for the responsible mutations. METHOD The genomic DNA for exome sequencing was obtained from the tumor tissue and matched normal tissue from the same individual. Exome sequencing was performed on HiSeq2000 sequencer platform. RESULTS Among oncogenes, germline missense single nucleotide variants were observed in the TP53 and APC genes in both the tumor and normal tissue. As tumor-specific somatic mutations, we identified 81 candidate genes, including 4 nonsense changes, 68 missense changes and 9 insertions/deletions. The mutations in ITGB2, IL-32 and DIDO1 were included in them. CONCLUSION This is a pilot study, and future analysis with more patients is needed to clarify: the detailed pathogenesis of this tumor, the novel diagnostic methods by detecting specific mutations, and the new therapeutic strategies targeting the mutation. PMID:28099595

  12. 伴Kasabach-Merritt现象卡波西形血管内皮瘤和丛状血管瘤的临床及病理特征%Clinical and pathological features of Kasabach-Merritt phenomenon associated with Kaposiform hemangioendothelioma and tufted angioma

    Institute of Scientific and Technical Information of China (English)

    陈辉; 林晓曦; 林梅绥; 李伟; 金云波; 马刚; 胡晓洁; 周兆平


    Objective To investigate the clinical and pathological features of Kasabach-Merritt phenomenon(KMP)associated with Kaposiform hemangioendothelioma(KHE)and tufted angioma(TA).Methods Clinical presentations and pathological features of seven patients with KHE or TA complicated by KMP were analyzed retrospectively and a literature review was made.Results Five patients were diagnosed with KHE and two with TA,and all of them exhibited a decrease in platelet count(less than 100×109/L).In all patients,lesions were present or developed on the trunk or limbs at birth or wimin one month after birth.The lesions showed a rapid growth in two patients,slow expansion in two patients,kept stable or regressed gradually in three patients.Lesion appearance varied in KHE and TA,and characteristic manifestation was dark-erythematous or prunosus tumors or patches involving the skin or subcutis.Histopathologically.both KHE and TA were composed of numerous fusiform endothellal cell clumps arranged in a lobular fashion.The nodules of KHE had an indistinct boundary usually with erythrocyte stasis or hemosiderin deposition,and TA was characterized by rounded,well-defined nodules distributed in a crater-like fashion within the dermis.A satisfactory outcome was achieved in two patients treated with oral dehydrocortisone and one patient with surgical resection plus skin grafting;the other four patients remained untreated and no progress was observed during follow-up.Conclusions KMP is pathologically associated with KHE or TA.KHE is an intermediate tumor with local aggressiveness while TA is a benign tumor.Both of them can regress spontaneously and should be difierentiated from other types of vascular tumors.%目的 探讨伴Kasabach-Merritt现象(KMP)的卡波西形血管内皮瘤(KHE)和丛状血管瘤(TA)临床病理特征.方法 对7例伴KMP的KHE和TA病例进行临床及病理学分析.结果 诊断为KHE 5例,TA 2例,均伴有血小板降低.7例均在出生后1月内发病,

  13. Neck Kaposiform haemangioendothelioma in a Fischer's lovebird (Agapornis fischeri). (United States)

    Rossi, Giacomo; Galosi, Livio; Berardi, Sara; Piano, Maria Assunta; Robino, Patrizia; Rose, Timothy; Calabrò, Maria Luisa


    A six-year-old female Fischer's lovebird (Agapornis fischeri) presented at necropsy with a cutaneous mass on the neck, 3.5cm in diameter, yielding and with blood content. Histopathological findings showed a neoplasm characterized by proliferation of vascular endothelial cells. The histology of the mass revealed a multinodular, focally infiltrating tumor. Deeper dermal nodules were made of spindle cells forming vascular slits reminiscent of the histology seen in Kaposi's sarcoma (KS). More superficially located dermal nodules consisted of small blood vessels, with histology resembling capillary hemangioma. The spindle cells and capillaries were strongly positive for Vimentin, endothelial cell marker CD31, and negative for sarcomeric α-smooth muscle actin (α-SMA). Intravascular platelet trapping and Periodic acid-Schiff (PAS)-positive hyaline globules were also observed. Differential diagnosis included Kaposi's sarcoma, capillary haemangioma, spindle cell haemangioendothelioma, and epithelioid haemangioendothelioma. Based on morphological and immunohistochemical findings, the tumor was diagnosed as a cutaneous Kaposiform haemangioendothelioma (KHE), a rare, low-grade malignant vascular neoplasm. Other organs showed no abnormalities. PCR amplifications, conducted using Kaposi's sarcoma-associated herpesvirus (KSHV)-specific primers and degenerate sets of primers designed to detect and characterize members of the Herpesviridae, on DNA extracted from tumor tissue and from whole blood failed to amplify any KSHV-related sequence. Moreover, no specific signal was obtained using primers for detection of psittacine herpesvirus, known to be linked to Pacheco's disease in parrots. To the best of our knowledge, this unusual case is the third report of KHE in a non-human animal species, the first described in a bird. Copyright © 2016 Elsevier Ltd. All rights reserved.

  14. Hemangioendotelioma kaposiforme e síndrome de Kasabach-Merritt Kaposiform haemangioendothelioma and Kasabach-Merritt syndrome

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    Adriana de Oliveira Mukai


    Full Text Available OBJETIVO: Descrever a apresentação e a evolução de um caso de hemangioendotelioma kaposiforme complicado por síndrome de Kasabach-Merritt. DESCRIÇÃO DO CASO: Lactente masculino de 45 dias, com febre, irritabilidade e choro, associados ao aparecimento de nodulações em tronco e membros. Evoluiu rapidamente com coagulopatia de consumo e queda do estado geral, necessitando de internação em Unidade de Terapia Intensiva Pediátrica (UTIP. Tomografia computadorizada revelou condensações parenquimatosas intra e extrapulmonares compatíveis com hemangiomas múltiplos e ausência de lesões em crânio e abdome. Houve complicação para síndrome Kasabach-Merritt em conseqüência de uma hemangiomatose kaposiforme. Optado por iniciar tratamento conservador, associando terapia de suporte com concentrado de glóbulos vermelhos, criopreciptado, plasma fresco, concentrado de plaquetas e uso das seguintes drogas: ácido tranexâmico e dexametasona. Após 20 dias em UTIP, já se encontrava em bom estado geral, em processo de remissão dos hemangiomas e normalização dos exames laboratoriais, sendo transferido para a enfermaria. Recebeu alta em tratamento com ácido tranexâmico e prednisona, com acompanhamento ambulatorial e laboratorial semanal. COMENTÁRIOS: A doença apresenta curso clínico rápido e grave, com necessidade de diagnóstico e tratamento precoces, para melhor prognóstico e sobrevida.To describe the clinical presentation of kaposiform hemangioendothelioma that was complicated by Kasabach-Merritt syndrome. CASE DESCRIPTION: A 45-day-old male infant presented fever, irritability and crying associated with the eruption of nodules in trunk and members. Soon after admission, the patient developed a consumptive coagulopathy, requiring admission in the Pediatric Intensive Care Unit (PICU. Computed tomography scans showed intra and extrapulmonary condensations suggestive of multiple thoracic hemangiomas, without the central nervous system

  15. Epitheloid hemangioendothelioma of urinary bladder

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    Narmada P Gupta


    Full Text Available Epitheloid hemangioendothelioma is an uncommon vascular neoplasm and has an unpredictable clinical behavior. It is characterized by round or spindle-shaped endothelial cells with cytoplasmic vacuolation. Most often, epitheloid hemangioendothelioma arise from the soft tissues of the upper and lower extremities and it has borderline malignant potential. We describe the first reported case of epitheloid hemangioendothelioma in the urinary bladder, which was treated by transurethral resection. The diagnosis was confirmed by immunohistochemistry.

  16. Medial canthus retiform hemangioendothelioma

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    Yasser H Al-Faky


    Full Text Available Retiform hemangioendothelioma (RH is a distinct entity in the spectrum of vascular tumors with a high local recurrence rate. It is considered a low-grade, well-differentiated cutaneous angiosarcoma with low metastatic potential. We report here for the first time a case of medial canthus recurrent RH. It may be helpful in our practice to include RH as a differential diagnosis of eyelid lesions. It is noteworthy that the progressive course and recurrence tendency of RH might be misdiagnosed as angiosarcoma or basal cell carcinoma (BCC, if not expected and carefully evaluated by the pathologist.

  17. Primary cardiac hemangioendothelioma: a case report

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    WANG Li-feng; LIU Ming; ZHU Hong; HAN Wei; HU Cheng-yi; QI Ji-ping; MEI Huan-lin; GE Re-le; ZHOU Min


    @@ Primary cardiac hemangioendothelioma is extremely rare.1-3 Up to now less than twenty cases have been reported in English literature, the data about this kind of cardiac tumors are scanty. In this report, a case of a huge hemangio-endothelioma that arose from the right atrium and was successfully resected is presented.

  18. [Epithelioid hemangioendothelioma: an uncommon liver tumor]. (United States)

    Pareja, Eugenia; Cortés, Miriam; Rayon, Miguel; Moya, Angel; Mir, Jose


    We report the case of a female patient who was referred to our unit because of a solid liver tumor, suggestive of metastasis. After biopsy, the patient was diagnosed with epithelioid hemangioendothelioma of the liver. Epithelioid hemangioendothelioma is a rare entity with an unpredictable, potentially fatal, clinical course and outcome. Due to its rarity, this entity should be considered when a solitary hepatic lesion is detected and should be included in the differential diagnosis with liver metastases. We highlight the infrequency of this tumor, its presentation as a solitary hepatic lesion and the indication of surgical treatment. We describe the clinical and pathological characteristics of epithelioid hemangioendothelioma of the liver and report a new case of this entity. The distinct therapeutic options are discussed. Copyright 2010 Elsevier España, S.L. All rights reserved.

  19. [Lung epithelioid hemangioendothelioma: Report of one case]. (United States)

    Quiroz, Manuel; Undurraga, Álvaro; Moya, Rafael; Fernández, Cristina; Bezares, Katiuska; Linacre, Virginia


    Epithelioid hemangioendothelioma is a multifocal tumor that rarely metastasizes. It is difficult to diagnose, most often it is an incidental finding in young asymptomatic women. The radiologic pattern is heterogeneous. Histologic confirmation of Weibel-Palade bodies or immunohistochemistry based on specific tumor markers such as factor VIII and CD34 are the most important finding to confirm the diagnosis. We report a 21 years old woman Presenting with cough and dyspnea. A chest X ray was suggestive of tuberculosis. Sputum smears were negative for acid fat bacilli and the tuberculin test was negative. A chest CAT scan showed multiple nodular lesions. A surgical biopsy of the lesions confirmed the presence of a hemangioendothelioma. The patient was initially treated with prednisone and azathioprine without response. Thereafter, the patient is without treatment and without evidence of disease progression.

  20. Liver Transplantation for Hepatic Epithelioid Hemangioendothelioma: The Canadian Multicentre Experience

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    Carmine G Nudo


    Full Text Available INTRODUCTION: Hepatic epithelioid hemangioendothelioma (HEHE is a rare entity. At the present time, there is no standardized effective therapy. Liver transplantation (LT has emerged as a treatment for this rare tumour.

  1. Ulcerated cutaneous epithelioid hemangioendothelioma in an 8-month old infant

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    Silonie Sachdeva


    Full Text Available Epithelioid hemangioendothelioma is an uncommon malignant vascular tumour usually involving soft tissue and rarely only skin. It is considered to be a borderline neoplasm between angiolymphoid hyperplasia with eosinophilia and an epithelioid angiosarcoma and is mostly found in adults with few cases reported in children. We herein report a case of ulcerated cutaneous epithelioid hemangioendothelioma in an infant of 8 months. This tumour has not been reported at such a young age in literature.

  2. Epithelioid Hemangioendothelioma of the Femur with Benign Cystic Appearance

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    Choi, Jung Ah; Kang, Heung Sik [Dept. of Radiology, Seoul National University Bundang Hospital, Seongnam (Korea, Republic of); Kim, Yeo Goon [Dept. of Radiology and Institute of Radiation Medicine, Seoul National University College of Medicine, Seoul (Korea, Republic of); Chung, Jin Haeng [Dept. of Patholgy, Seoul National University Bundang Hospital, Seongnam (Korea, Republic of); Oh, Joo Han [Dept. of Orthopedic Surgery, Seoul National University Bundang Hospital, Seongnam (Korea, Republic of)


    An epithelioid hemangioendothelioma is an intermediate grade tumor between hemangioma and angiosarcoma that frequently shows marked enhancement because it is a vascular tumor. Herein, we describe a rare case of a malignant epithelioid hemangioendothelioma of the femur that was mistaken as a benign lesion such as a simple bone cyst or fibrous dysplasia because the tumor had a benign cystic appearance on MRI and its imaging findings showed a histopathologic correlation.

  3. Hepatic epithelioid hemangioendothelioma: a report from three university centers* (United States)

    Giardino, Antonello; Miller, Frank H.; Kalb, Bobby; Ramalho, Miguel; Martin, Diego R.; Rodacki, Karina; Woosley, John T.; Semelka, Richard C.


    Objective To determine common imaging findings of hepatic epithelioid hemangioendothelioma on magnetic resonance images. Materials and Methods A search was made of three institutional databases between January 2000 and August 2012. Seven patients (mean age, 47 years; range, 21-66 years; 6 women) with pathology-confirmed diagnosis of hepatic epithelioid hemangioendothelioma who had undergone magnetic resonance imaging were identified. None of the patients had received any treatment for hepatic epithelioid hemangioendothelioma at the time of the initial magnetic resonance imaging examination. Results Hepatic epithelioid hemangioendothelioma tumors appeared as focal masses in 7/7 patients, greater than 5 in number, with a coalescing lesion in 1/5, and peripheral localization in 6/7. Capsular retraction was present in 4/7, and was associated with peripherally located lesions. Early ring enhancement was appreciated in the majority of lesions in 7/7 patients. Centripetal progressive enhancement was shown in 5/7 patients on venous phase that exhibited a distinctive thick inner border of low signal on venous phase images, and a central core of delayed enhancement. Small lesions did not show this. Conclusion The combination of multifocal round-configuration lesions that are predominantly peripheral and exhibit early peripheral ring enhancement and late appearance of an inner thick border of low signal and central core of high signal may represent an important feature for hepatic epithelioid hemangioendothelioma. PMID:27818541

  4. [Biologically borderline neoplasms: epithelial hemangioendothelioma. A case report]. (United States)

    Genovese, A M; Barbera, A; Fedele, F; Broccio, M; Lepore, V; Ciccolo, A


    The case of a patient affected by an epithelial hemangioendothelioma of the arm is described. A definitive diagnosis was possible only by histologic examination. The difficulty to reliably predict the biological behaviour of these tumors is emphasized and therefore the necessity of a follow-up of the patient for many years is stressed, even in the case of a histologically benign tumor.

  5. Low-Grade Uterine Epithelioid Hemangioendothelioma Presented as a Submucosal Leiomyoma during Labor

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    Anastasios V. Koutsopoulos


    Full Text Available With the exception of leiomyomas, soft tissue tumors of the uterine corpus are not common. This is particularly true for vascular neoplasms, with the epithelioid hemangioendothelioma being a curiosity; not more than twenty-two cases of malignant hemangioendotheliomas have been reported in the literature so far, all of which were high-grade hemangioendotheliomas (hemangiosarcomas. We present herewith a unique case of low-grade epithelioid hemangioendothelioma of the uterus in a pregnant woman aged 29 years. The clinical, histological, and immunohistochemical characteristics of this entity, together with its differential diagnosis, are discussed.

  6. Ischemic stroke as the first manifestation of hepatic epithelioid hemangioendothelioma. (United States)

    Zis, Panagiotis; Assi, Avraam; Kravaritis, Dimitrios; Sevastianos, Vassilios A


    A 38-year-old obese woman, with a past medical history of cholecystectomy and dyslipidaemia, presented with acute occipital headache, vomiting and rotational vertigo which lasted 8 hours. On admission neurological examination was unremarkable, however general physical examination revealed hepatomegaly. Routine blood tests showed abnormal liver function tests. MRI scan indicated an acute ischemic infarct in the right cerebellum. Extensive investigation was negative. However, liver MRI revealed multiple lesions in both liver lobes. Ultrasound guided liver biopsy and histopathological analysis confirmed the diagnosis of hepatic hemangioendothelioma. In conclusion, hypercoaguable state related to hepatic epithelioid hemangioendothelioma can lead to an ischemic stroke, as a rare first manifestation of the disease. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  7. Hemangioendothelioma of the sphenoid bone: a case report. (United States)

    Joo, M; Lee, G J; Koh, Y C; Park, Y K


    Hemangioendothelioma is borderline or intermediate type of vascular neoplasm. Hemangioendothelioma is rare lesion that constitutes less than 0.5% of the malignant tumors of bone. We present a case of low-grade hemagioendothelioma of the skull in a 29-yr-old woman. She had pain, diplopia and exophthalmos of the left eye. Radiographic images showed a relatively well-demarcated, expansile osteolytic lesion with irregularly thickened trabeculae and calcifications in the left greater wing of sphenoid bone. Histologically, the tumor was an infiltrative vasoformative lesion. The vessels are generally well-formed with open or compressed lumina surrounded by endothelial cells showing mild atypia. It lacked frequent mitotic figures and severe atypia. Although excessive bleeding occurred during the operation, the mass was totally resected. Postoperative radiation was not necessary. She is free of disease and well 6 months postoperatively.

  8. Rapid resolution of consumptive hypothyroidism in a child with hepatic hemangioendothelioma following liver transplantation (United States)

    We report a unique case of a 3-mo-old female with consumptive hypothyroidism and liver hemangioendothelioma who required pharmacological doses of thyroid hormones and was cured following liver transplantation. Liver hemangioendotheliomas are capable of producing an excess of the thyroid hormone inac...

  9. Multifocal and metastatic hepatic hemangioendothelioma: case report and literature review

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    Torres, Lucas Rios; Galvao Filho, Mario Melo [Hospital Sao Luiz, Sao Paulo, SP (Brazil); Timbo, Luciana Satiro; Verrastro, Carlos Gustavo Yuji; D' Ippolito, Giuseppe, E-mail: [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Medicina; Ribeiro, Cristiane Maria de Freitas [Laboratorio Diagnostica, Sao Paulo, SP (Brazil)


    Epithelioid hemangioendothelioma is a rare neoplasm of vascular origin whose involvement of organs such as lung, spleen, bone, breast and liver has already been reported. The clinical signs and the prognosis of this tumor are quite variable. From the present case report, the authors have sought to contribute with literature data, by reviewing clinical and radiological features which may be useful to include such a condition in the differential diagnosis of liver lesions. (author)

  10. Intracranial epithelioid hemangioendothelioma: two cases report and literature review

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    Jun-chao FANG


    Full Text Available Background Epithelioid hemangioendothelioma is an uncommon low-grade malignant tumor with various biological behaviors. This paper retrospectively analyzed the clinical data of 2 cases with epithelioid hemangioendothelioma which were confirmed by histopathological features, and reviewed relevant literatures, so as to summarize clinical and radiological features, diagnosis and differential diagnosis, treatment and prognosis of this disease.  Methods and Results The tumor in Case 1 was cystic accompanied with hemorrhage. Hematoma clearance, decompressive craniectomy and total removal of the tumor were performed. The tumor in Case 2 was misdiagosed as meningioma firstly, and craniotomy was implemented to remove the tumor. But 8 months later, the tumor relapsed. The tumor was vascularized in operation and subtotally removed with moderate blood loss. Postoperative pathological diagnosis revealed epithelioid hemangioendothelioma in both cases. Immunohistochemical staining showed the tumor cells were positive for CD34, FⅧ RAg in Case 1, while CD31, vimentin (Vim and Fli-1 in Case 2, and both negative for cytokeratin (CK and epithelial membrane antigen (EMA. Case 1 was followed up for one year, and no recurrence was found. Case 2 received postoperative auxiliary radiotherapy, but tumor metastasis was found 3 months later. Conclusions Epithelioid hemangioendothelioma is a unique vascular tumor characterized by proliferation of epithelioid or histiocytoid endothelial cells. The histopathological features and biological behaviors are intermediate between hemangioma and angioscarcoma, and its intracranial occurrence is extremely rare. A preoperative diagnosis is usually difficult because of lacking characteristic clinical and radiological features. Diagnosis mainly depends on histopathology and immunohistochemistry. Gross resection is currently the firstline treatment for these tumors. Otherwise, adjuvant therapies are required. The prognosis of this

  11. Comparative analyses of phenotypic methods and 16S rRNA, khe, rpoB genes sequencing for identification of clinical isolates of Klebsiella pneumoniae. (United States)

    He, Yanxia; Guo, Xianguang; Xiang, Shifei; Li, Jiao; Li, Xiaoqin; Xiang, Hui; He, Jinlei; Chen, Dali; Chen, Jianping


    The present work aimed to evaluate 16S rRNA, khe and rpoB gene sequencing for the identification of Klebsiella pneumoniae in comparison with phenotypic methods. Fifteen clinical isolates were examined, which were initially identified as K. pneumoniae subsp. pneumoniae using the automated VITEK 32 system in two hospitals in Enshi City, China. Their identity was further supported by conventional phenotypic methods on the basis of morphological and biochemical characteristics. Using Bayesian phylogenetic analyses and haplotypes network reconstruction, 13 isolates were identified as K. pneumoniae, whereas the other two isolates (K19, K24) were classified as Shigella sp. and Enterobacter sp., respectively. Of the three genes, 16S rRNA and khe gene could discriminate the clinical isolates at the genus level, whereas rpoB could discriminate Klebsiella at the species and even subspecies level. Overall, the gene tree based on rpoB is more compatible with the currently accepted classification of Klebsiella than those based on 16S rRNA and khe genes, showing that rpoB can be a powerful tool for identification of K. pneumoniae isolates. Above all, our study challenges the utility of khe as a species-specific marker for identification of K. pneumoniae.

  12. Epithelioid Hemangioendothelioma of the Liver: Etiology, Natural History and Treatment

    Institute of Scientific and Technical Information of China (English)

    Weirong Chen; Maogen Chen; Gaoyang Cai; Ziqun Liao


    @@ Introduction Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor, which also has been called histiocytoid haemangioma. This low-grade malignant tumor was first described as a distinctive entity in 1982 by Weiss and Enzinger[1]. Histopathologically, EHE is charac-terized by round or spindle-shaped endothelial cells with cytoplasmic vacuolation[2]. The tumor may occur in numerous organs, includingliver, lung, parotid grand, skin, pleura, thyroid, spleen, cardiac valve,radial artery, oral cavity and parapharyngeal space[2-11]. Many cases have been reported, but its etiology and natural history remain un-clear.

  13. Polymorphous hemangioendothelioma in a child with acquired immunodeficiency syndrome (AIDS). (United States)

    Paul, Stephan R; Hurford, Matthew T; Miettinen, Markku M; Aronoff, Stephen C; Delvecchio, Michael; Grewal, Harsh; Tuluc, Madalina


    Polymorphous hemangioendotheliomas (PH) are rare and borderline malignant tumors that are among the wide range of vascular tumors. We report here a 13-year-old male presenting with a history of weight loss, opportunistic infections, and lymphadenopathy. He was determined to be HIV positive and to have acquired immunodeficiency syndrome (AIDS). A biopsy of a femoral node was diagnostic of PH. His systemic lymphadenopathy appeared to resolve with anti-retroviral therapy. This tumor should be considered within the differential diagnoses of pediatric and immunocompromised patients.

  14. Hemangioendothelioma with an epithelioid phenotype arising in hemangioma of the fibula

    Energy Technology Data Exchange (ETDEWEB)

    Shah, Zarine K. [Changi General Hospital, Department of Diagnostic Radiology (Singapore); Peh, Wilfred C.G. [Changi General Hospital, Department of Diagnostic Radiology (Singapore); Singapore Health Services, Programme Office (Singapore); Shek, Tony W.H. [University of Hong Kong, Queen Mary Hospital, Department of Pathology, Hong Kong SAR (China); Wong, Jimmy W.K.; Chien, Eric P. [University of Hong Kong, Queen Mary Hospital, Department of Orthopaedic Surgery, Hong Kong SAR (China)


    The classification of certain vascular bone tumors that show an epithelioid cytologic appearance remains confusing, with overlap in features of epithelioid hemangioma, hemangioendothelioma and epithelioid hemangioendothelioma. Radiographs of a 27-year-old woman who presented with ankle pain showed an expanded lytic-sclerotic lesion in the distal left fibula. Magnetic resonance imaging showed an intramedullary lesion with a small lateral intracortical component. The lesion was hypo- to isointense to muscle on T1-weighted images and heterogeneously hyperintense on T2-weighted images. Initial incisional biopsy was inconclusive. Open biopsy showed hemangioendothelioma with epithelioid morphology, and the lesion was completely resected with reconstruction using a peroneal fibular rotation graft. Examination of the resected specimen showed focal hemangioendothelioma with an epithelioid phenotype arising in a hemangioma. This case illustrates the difficulty and pitfalls of making the correct diagnosis on the basis of a small biopsy specimen. (orig.)

  15. Epithelioid hemangioendothelioma of the spine. Report of two cases.

    LENUS (Irish Health Repository)

    Aquilina, Kristian


    Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin. The authors describe two cases of spinal EH, one involving the T-10 vertebra and the second involving the upper cervical spine. In the first case the patient underwent resection of the tumor; this case represents the longest reported follow-up period for spinal EH. In the second case, extensive involvement of C-2, C-3, and C-4 as well as encasement of both vertebral arteries precluded safe tumor resection, and posterior occipitocervical stabilization was performed. The patient subsequently died of metastatic disease. The findings in these two cases underscore the difficulty in predicting the clinical behavior of spinal EH based solely on histological and clinical features as well as the uncertainty of the roles of surgery, chemotherapy, and radiotherapy in the oncological management of a spinal tumor for which clinical data are very limited.

  16. Multifocal epithelioid hemangioendothelioma of the phalanges of the hand

    Energy Technology Data Exchange (ETDEWEB)

    Bruegel, Melanie; Waldt, Simone; Woertler, Klaus; Rummeny, Ernst J. [Klinikum Rechts der Isar der Technischen Universitaet Muenchen, Department of Radiology, Munich (Germany); Weirich, Gregor [Klinikum Rechts der Isar der Technischen Universitaet Muenchen, Institute of Pathology, Munich (Germany)


    Epithelioid hemangioendothelioma (EH) of bone is a rare vascular neoplasm characterized by epithelioid endothelial cells and a variable biologic behaviour. The principal sites of occurrence of this osteolytic tumor are the lower extremity and the axial skeleton. Approximately half of the cases present with multifocal disease. The latter feature can be helpful in suggesting the diagnosis of a vascular tumor; on the other hand, it strengthens the need for a skeletal survey or whole-body MRI/CT. We report on the clinical, histologic and radiologic features - including CT and MRI findings - of EH in a case of multifocal disease of the phalanges of the hand, a very uncommon anatomic site of affliction. (orig.)

  17. [Epithelioid hemangioendothelioma of bone complicated by femoral fracture]. (United States)

    Charfi, L; Mrad, K; Karray, S; Sassi, S; Driss, M; Abbes, I; Ben Romdhane, K


    A 54-year-old man was seen with a fracture of the left femur. Plain radiographs revealed a 40-mm lytic centromedullary lesion. Magnetic resonance T1- and T2-weighted sequences showed high and low signals. After stabilization of the fracture, the tumor was removed followed by reconstruction with a vascularized fibula. The pathological examination demonstrated proliferation of non atypical CD34 and CD31 positive epithelioid cells with few lumens, accompanied by abundant fibrous stroma, sometimes masking tumor cells. Satisfactory motion was achieved with no recurrence at 20 months follow-up. Bone hemangioendothelioma can simulate metastasis and must be distinguished by immunohistochemistry. Prognosis is a subject of debate as the tumor is considered to exhibit intermediate malignancy by some authors while other consider it to be a malignant tumor.

  18. A spindle cell hemangioendothelioma on the head resembling an arteriovenous malformation. (United States)

    Higashino, Takuya; Hirai, Rintaro


    A spindle cell hemangioendothelioma is a relatively uncommon lesion, especially on the head and neck. Recurrence occurs after local excision of 50% to 60% of these lesions; therefore, it is important to recognize this unusual neoplasm and avoid misdiagnosis. Here, we report a rare case of a spindle cell hemangioendothelioma of the head. A 37-year-old woman presented with a soft subcutaneous mass, 2.5 cm in size, on her right occipital region. The mass pulsated strongly and a thrill was present. Magnetic resonance imaging showed that some dilated feeding arteries flowed into the mass and that a flow-void sign was present. The lesion looked like an arteriovenous malformation, and a marginal resection was performed. Histologically, there was a mix of cavernous vascular cavities and Kaposi sarcomalike spindle cell vascular zones, which is compatible with a spindle cell hemangioendothelioma.

  19. Epithelioid hemangioendothelioma of right innominate vein mimics a teratoma (United States)

    Wan, Qi; Zhou, Jiaxuan; Yu, Yudong; Sun, Qingyu; Bao, Yingying; Lei, Qiang; Zou, Qiao; Deng, Yingshi; Li, Xinchun


    Abstract Rational: Epithelioid hemangioendothelioma (EHE) is a rare neoplasm commonly known to arise from the soft tissue, lung, and liver. EHE arising from right innominate vein (RIV) has scarcely been reported in English literature. Patient concerns: Herein, we present a rare case of EHE of RIV in a 51-year-old woman with right-lower chest pain for 4 days. Computed tomography of the chest revealed a spherical mass with calcification and fatty foci located in the anterior mediastinum, thus a presumptive diagnosis of teratoma was made. Diagnoses, interventions, and outcomes: Video-assisted thoracoscopic explorations and resection of mediastinal tumor were then performed. The pathological examination showed that the tumor was EHE. Postoperative radiotherapy was delivered to the patient. Pulmonary metastases were found by chest CT a year after surgery. Lessons: A diagnosis of EHE might be considered, when a mediastinal tumor closely related to veins showing intratumoral calcification and obvious enhancement, despite the presence of a clear boundary and visible fat content. PMID:28248868

  20. Hepatic epithelioid hemangioendothelioma: A diagnostic pitfall in aspiration cytology

    Directory of Open Access Journals (Sweden)

    Gupta Ruchika


    Full Text Available Hepatic epithelioid hemangioendothelioma (EH is a rare vascular neoplasm. An accurate radiologic diagnosis is usually difficult due to the presence of multiple nodules, simulating metastatic carcinoma. Though histologic features of this tumor are well described, cytologic reports of hepatic EH are very few in the available literature. We describe a case of a young healthy adult male who was found to have multiple hepatic masses on radiologic investigations. A guided fine needle aspiration demonstrated a poorly differentiated neoplasm. The diagnosis was made on core biopsy assisted by immunohistochemistry, which showed characteristic features of EH. He is doing well 14 months after diagnosis, without surgical excision or chemotherapy. An accurate diagnosis of hepatic EH on aspiration cytology requires an adequate specimen and awareness of its cytologic features, including discohesive atypical cells with intracytoplasmic lumina and intranuclear inclusions. Since this tumor is usually unresectable but has a favorable prognosis as compared to hepatocellular carcinoma, a correct diagnosis is essential for appropriate management and prognostication.

  1. Common Presentation with Uncommon Diagnosis: Multifocal Epithelioid Hemangioendothelioma

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    Susmita Kundu


    Full Text Available A young female patient presenting with recurrent hemoptysis, neck swelling, and mediastinal mass mimicking lymphadenopathy was admitted to the Institute of Post Graduate Medical Education and Research and SSKM hospital, Kolkata, India. Clinical features, radiological studies, fibre optic bronchoscopy, and fine needle aspiration cytology from the neck swelling created a diagnostic dilemma until surgical resection and immunohistochemistry reports confirmed the diagnosis of multifocal epithelioid hemangioendothelioma, a rare vascular tumor with intermediate malignancy potential. Because it is a slow-progressing disease and due to the non-availability of standard chemotherapy, the patient, and her legal guardian, opted for palliative care only. She was asymptomatic for four years but again presented with hemoptysis, reappearance of the neck swelling on the same side, and a mediastinal mass compressing the superior vena cava and right pulmonary artery. This report describes the diagnostic problems and therapeutic challenges in the management of this rare tumor over a four-year follow-up period. The clinical course emphasizes the highly unpredictable nature of this tumor.

  2. Epithelioid Hemangioendothelioma : clinicopathologic, immunhistochemical, and molecular genetic analysis of 39 cases

    NARCIS (Netherlands)

    Flucke, Uta; Vogels, Rob J. C.; Somerhausen, Nicolas de Saint Aubain; Creytens, David H.; Riedl, Robert G.; van Gorp, Joost M.; Milne, Anya N.; Huysentruyt, Clement J.; Verdijk, Marian A. J.; van Asseldonk, Monique M.; Suurmeijer, Albert J. H.; Bras, Johannes; Palmedo, Gabriele; Groenen, Patricia J. T. A.; Mentzel, Thomas


    Background: Epithelioid hemangioendothelioma is a malignant, often indolent vascular tumor which occurs at various anatomic sites. Based on a reciprocal translocation t (1;3)(p36;q25), a consistent WWTR1-CAMTA1 fusion gene has been found. An alternate YAP1-TFE3 fusion has been detected in a small an

  3. Hemangioendothelioma of bone in a patient with a constitutional supernumerary marker

    DEFF Research Database (Denmark)

    Rogatto, S R; Rainho, C A; Zhang, Z M


    A 13-year old girl was diagnosed as having a bone hemangioendothelioma. Cytogenetic studies identified the presence of a small supernumerary marker chromosome in this patient. Classical cytogenetic methods using G-, C-, Ag-NOR-banding were supplemented by spectral karyotyping (SKY) and fluorescen...

  4. CAMTA1 Immunostaining is not Useful in Differentiating Epithelioid Hemangioendothelioma from its Potential Mimickers

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    Zarifa YUSİFLİ


    Full Text Available Objective: Epithelioid hemangioendothelioma is a rare member of vascular tumors of intermediate malignancy. Recently, presence of t(1;3 translocation and WWTR1/CAMTA1 gene fusion, which enhances CAMTA1 expression, are found to be specific to this tumor. We investigated the CAMTA1 immune expression profile of epithelioid hemangioendothelioma and its potential mimickers using a commercially available CAMTA1 antibody. Material and Method: Standard whole sections from the formalin fixed, paraffin embedded blocks of 12 epithelioid hemangioendotheliomas, 10 angiosarcomas, 9 epithelioid sarcomas, 8 malignant melanomas, 8 signet ring carcinomas, 7 lobular carcinomas of breast, 2 epithelioid mesotheliomas, 2 rhabdoid tumors and 12 miscellaneous hemangiomas were immunostained for anti-CAMTA1 (ab64119, 1:200; Abcam after pretreatment with citrate pH 6.0 for 20 minutes using Leica Bond detection kit with DAB chromogen. Strong nuclear CAMTA1 expression was scored for its extent as ‘negative’ (50% positive. Results: In 60 out of 70 cases (86% either 2+ or 3+ strong nuclear staining was seen. Eighty-three % of epithelioid hemangioendotheliomas, 100% of angiosarcomas, 89% of epithelioid sarcomas, 89% of malignant melanomas, 63% of signet ring carcinomas, 71% of lobular carcinomas of breast, 100% of epithelioid mesotheliomas, 50% of rhabdoid tumors and 100% of hemangiomas were stained. Besides neurons, CAMTA1 expression was also observed in squamous epithelium, skin adnexa, breast lobules, prostate glands, bile ducts, colonic mucosa and gastric pits. Conclusion: Epithelioid hemangioendothelioma, its potential morphological mimickers and other benign or malignant vascular tumors showed strong and diffuse CAMTA1 expression, nullifying the potential use of CAMTA1 immunohistochemistry as an adjunct in the differential diagnosis.

  5. Multifocal hemangioendothelioma of the lumbar spine and response to surgical resection and radiation. (United States)

    Kelahan, Linda C; Sandhu, Faheem A; Sayah, Anousheh


    Epithelioid hemangioendothelioma rarely occurs in the lumbosacral spine, with very few case reports of spinal hemangioendothelioma in the literature. There is variability in aggressiveness of these lesions without established treatment guidelines. The aim was to present a case of epithelioid hemangioendothelioma in the lumbar spine, including magnetic resonance imaging (MRI) findings, which rapidly progressed over a 2-month period as regional multifocal lumbosacral spinal lesions with epidural extension causing severe spinal canal stenosis. This was a case report in a university hospital setting. The sample included an otherwise healthy adult male with low back pain. Multimodality imaging was performed to help with diagnosis and management including computed tomography, MRI, and positron emission tomography (PET). The patient was treated by embolization, L5 corpectomy and L4-S1 stabilization, and radiation therapy. The diagnosis was confirmed by tissue biopsy. The patient initially presented with severe back and leg pain after a vertebroplasty for an L5 compression fracture at an outside hospital where biopsy was negative for malignancy. Magnetic resonance imaging showed diffuse abnormality of L5 with several smaller lesions in the sacrum. Due to progressive pain 2 weeks after the vertebroplasty, the patient underwent an L5 laminectomy, L4-S1 instrumented posterior fusion, and attempted partial corpectomy for stenosis. At this surgery, the L5 corpectomy was aborted owing to profound bleeding. Pathology was again negative for malignancy. Presumed to be an atypical hemangioma, the lesion was embolized before repeat surgery where the thecal sac was decompressed by partial L5 corpectomy. Biopsy at this time revealed a vascular neoplasm, with hemangioendothelioma not excluded. Approximately 2 months after the stabilization procedure, the patient had increasing pain and bilateral lower extremity weakness. Magnetic resonance imaging was performed and demonstrated marked

  6. Hepatic Mesenchymal Hamartoma Combined with Infantile Hepatic Hemangioendothelioma in an Infant

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    Kuang-Hung Hsiao


    Full Text Available Hepatic mesenchymal hamartoma is a rare benign tumor in children, and infantile hepatic hemangioendothelioma is also a rare liver neoplasm. We report a female newborn with an abdominal mass noted by the regular maternal ultrasound at 32 weeks of gestation. After birth, a liver mass was detected by computed tomography and magnetic resonance cholangiopancreatography. Frequent postprandial vomiting and progressive abdominal distension occurred 4 months later. Three tumor masses were detected this time, and the serum alpha-fetoprotein (AFP was 6700 ng/mL. Segmental resection was performed initially and complete resection of these tumors and left lobectomy were performed 21 days later. Pathologic examination of these liver masses revealed mesenchymal hamartoma combined with infantile hepatic hemangioendothelioma. After half a year of regular follow-up, the AFP level decreased gradually to 79.5 ng/mL, without evidence of tumor recurrence. [J Formos Med Assoc 2007;106(3 Suppl:S1-S4

  7. Hemangioendothelioma of the Bladder: The Youngest Case Report in A Child

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    Jupiter Sibarani


    Full Text Available Hemangioendothelioma is a vascular tumor that commonly found in soft tissue, lungs, heart, liver, and bones, but it is very rare in bladder. We report a case of pediatric hemangioendothelioma of the bladder in a 2 years 7 months-old boy that treated with total excision of the bladder and bilateral ureterocutaneoustomy. This case is the second case in pediatric patients, and the youngest case that reported in a child. Our patient is doing well post operatively. But unfortunately interferon a-2b is not available in our hospital. At 3 months follow up, there was progressive progression of the tumor. Recurrent Abdominal mass was confirmed by Abdominal CT-Scan. The patient was died one week later. Interferon a-2b is might be an effective regimen on this tumor but further study is needed to confirm this statement.

  8. A Multidisciplinary Approach to the Management of Atypical Osseous Epithelioid Hemangioendothelioma

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    J. K. Ma


    Full Text Available Hemangioendothelioma is a rare vascular tumor of intermediate malignant potential. Though epithelioid hemangioendothelioma (EH is commonly found in soft tissues, it has been known to be present in skeletal tissues. The authors present a case of a 50-year-old woman diagnosed with EH of the iliac bone and acetabulum, who experienced pathological fracture at presentation. This report describes a multidisciplinary approach to the management that includes initial incisional biopsy, curettage, and bone grafting, followed by Intensity Modulated Radiation Therapy. The patient finally underwent hemipelvic resection with allograft reconstruction after recurrence. Histopathological study revealed osseous EH of low mitotic activity that stained positively for CD31, CD34, vimentin, and Factor VIII. Herein, the authors discuss the imaging characteristics, histopathological aspects, cytogenetic findings, and the radiobiological behavior of osseous EH. After an aggressive multidisciplinary intervention, the patient is able to achieve local control with no evidence of distal metastatic disease.

  9. Pulmonary Epithelioid Hemangioendothelioma in a Patient with Crohn’s Disease

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    Nanda Ramchandar


    Full Text Available Pulmonary epithelioid hemangioendothelioma (PEH is a rare neoplasm, largely unresponsive to chemotherapeutic medications, and with varied prognosis. Imaging on computerized tomography may demonstrate perivascular nodules, but diagnosis is ultimately made on biopsy with immunohistochemical analysis. Here we describe a case of PEH in a 14-year-old male with Crohn’s disease, which, to our knowledge, has not previously been described in the literature.

  10. Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules

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    Riccardo Cazzuffi


    Full Text Available We report here a case of primary pulmonary epithelioid hemangioendothelioma diagnosed in a 67-year-old Caucasian man, presenting with exertion dyspnoea, dry cough, and multiple bilateral pulmonary nodules revealed by computed tomography. At the 18F-fluorodeoxyglucose positron emission tomography, these nodules were negative. The histopathological diagnosis was made on a pulmonary wedge resection (performed during video-thoracoscopic surgery.

  11. Primary Pulmonary Epithelioid Hemangioendothelioma: A Rare Cause of PET-Negative Pulmonary Nodules (United States)

    Cazzuffi, Riccardo; Calia, Nunzio; Ravenna, Franco; Pasquini, Claudio; Saturni, Sara; Cavallesco, Giorgio Narciso; Quarantotto, Francesco; Rinaldi, Rosa; Cogo, Annaluisa; Caramori, Gaetano; Papi, Alberto


    We report here a case of primary pulmonary epithelioid hemangioendothelioma diagnosed in a 67-year-old Caucasian man, presenting with exertion dyspnoea, dry cough, and multiple bilateral pulmonary nodules revealed by computed tomography. At the 18F-fluorodeoxyglucose positron emission tomography, these nodules were negative. The histopathological diagnosis was made on a pulmonary wedge resection (performed during video-thoracoscopic surgery). PMID:21869893

  12. Epithelioid hemangioendothelioma of the proximal phalanx of the left second toe – a rare occurence

    Directory of Open Access Journals (Sweden)

    Bharath Raju G


    Full Text Available Introduction: Epithelioid hemangioendothelioma (EHE of the bone is extremely rare and occurs predominantly in males. It most frequently occurs during the second and third decades of life. The lower extremities are most commonly involved. We describe a diagnostically challenging case of epithelioid hemangioendothelioma of proximal phalanx of 2nd toe of left foot with histological features reminiscent of osteoblastomatosis. Case Report: A 52 year old man presented with history of intermittent pain with swelling in second toe since 6 months. Radiograph showed a lytic lesion in proximal phalanx of the great toe. CT and MRI reported non specific lesion in the toe. Pain was quite severe and as the patient was a labourer and wanted to get back to his work as soon as possible a decision of disarticulation of the second toe at metatarsophalnageal joint was taken. Histopathology confirmed the diagnosis of Epithelioid hemangioendothelioma and patient was called for regular follow up. There are no complications and recurrence at two year follow up. Conclusion: EHE of the bone is extremely rare vascular tumor. To our knowledge, this is the first case of EHE with such features. EHE should be kept as one of the important differential diagnosis while diagnosing vascular tumors. Careful attention to the histopathological features is necessary for the confirmation of the diagnosis. Keywords: Epitheloid haemangioma; epitheloid haemangioendothelioma; rare occurrence; vascular tumor.

  13. Retiform hemangioendothelioma over forehead: A rare tumor treated with chemoradiation and a review of literature

    Directory of Open Access Journals (Sweden)

    Anup Sunil Tamhankar


    Full Text Available Retiform hemangioendothelioma (RH is low grade tumor of skin and subcutaneous tissue. It needs to be differentiated from angiosarcoma as RH has excellent prognosis. It is usually seen in young adults on extremities. Sometimes it may mimic benign conditions and can delay treatment. Surgery has been mainstay of its treatment with or without adjuvant radiation. We present first case of RH on face. This is only second case being treated with definitive chemoradiation. So it′s important to distinguish RH from angiosarcoma due to treatment implications as well.

  14. Role of FDG-PET scan in staging of pulmonary epithelioid hemangioendothelioma

    Directory of Open Access Journals (Sweden)

    Calabrese Cecilia


    Full Text Available In this report we describe a case of pulmonary epithelioid hemangioendothelioma (PEH in a young woman. The neoplasm manifested with dry cough, chest pain, finger clubbing, and multiple bilateral pulmonary nodules on chest x-ray and computed tomographic (CT scan. She underwent thoracoscopy, and the histological features of the lung biopsies were initially interpreted as consistent with a not-well-defined interstitial lung disease. Our patient was clinically and radiologically stable over a period of four years, after which the disease progressed to involve not only the lung but also mediastinal lymph nodes, liver and bone. Fiberoptic bronchoscopy showed subtotal occlusion of the right middle and lower lobe bronchi. The histologic examination of bronchial biopsies revealed a poorly differentiated neoplasm immunohistochemically positive for vimentin and vascular markers CD31, CD34 and Factor VIII. A diagnosis of malignant hemangioendothelioma was made. Positron emission tomography (PET is more sensitive than CT scan and bone scintigraphy in detecting PEH metastases. Furthermore, 18-fluorodeoxyglucose (FDG uptake seems to be related to the grade of malignancy of PEH lesions. Therefore, we suggest that FDG-PET should be included in the staging system and follow-up of PEH.

  15. Epithelioid hemangioendothelioma of the oral cavity: report of two cases and review of the literature. (United States)

    Chi, Angela C; Weathers, Dwight R; Folpe, Andrew L; Dunlap, Derek T; Rasenberger, Kenneth; Neville, Brad W


    The epithelioid hemangioendothelioma is an uncommon vascular neoplasm of borderline or intermediate malignant potential. Although numerous sites of involvement are possible, these tumors most commonly arise in soft tissue, liver, and lung. Involvement of the oral cavity is rare. Only 12 cases of intraoral epithelioid hemangioendothelioma have been reported in the English language literature. We review the salient features of these previously reported cases and present 2 additional intraoral cases--one presenting as an asymptomatic radiolucency in the posterior mandible of a 23-year-old female, and the other presenting as an asymptomatic, erythematous to purplish gingival nodule in a 28-year-old female. Intraoral tumors most commonly involve the gingival soft tissues and often are associated with adjacent alveolar bone resorption. Although it is not possible to estimate with accuracy the potential for recurrence and metastasis among intraoral tumors given the small number of previously reported cases, wide local excision with close clinical follow-up appears to be the treatment of choice for these tumors because of their unpredictable clinical behavior.

  16. Continued application of Endostar combined with chemotherapy in advanced hemangioendothelioma of bone

    Institute of Scientific and Technical Information of China (English)

    Ningrong Yang; Lin Wang; Xun Chen; Shukui Qin


    By one case of hemangioendothelioma of bone accompanying pulmonary metastasis was treated with rh-enostatin injection (Endostar) combined with chemotherapy. The patient got partial response (PR) for 3 years after the plication of Endostar maintenance therapy and Endostar combined with taxane-based chemotherapy. During the periousing Endostar as monotherapy, the patient got long-term disease control and good quality of life. There was no drug relaadverse event during the therapy of Endostar. Suggested continued using of Endostar combined with chemotherapy coachieve an convinced therapeutic effect. Then using Endostar as maintenance treatment after patient got the optimal efficwas feasible and profitable. This treatment strategy of long-term administration of Endostar was worthy of further observato explore the feasibility for long-term administration of combined with chemotherapy in the treatment of hemangioendoioma of bone accompanying pulmonary metastasis.

  17. Multifocal Epithelioid Hemangioendothelioma Derived from the Spine Region: Case Report and Literature Review

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    G. Kerry


    Full Text Available Background: Epithelioid hemangioendothelioma (EHE is a rare vascular tumor with malignant biological behavior. It arises from endothelial cells, usually within soft tissues, and can occur in almost all locations. Case Report: We report a unique case of a 25-year-old man who presented with sudden attacks of severe back pain followed by acute non-traumatic paraplegia. Emergency diagnostics revealed a pathologic fracture of the T7 vertebra with tumor tissue invasion of the spinal canal. Furthermore, multifocal metastases were found. Results: To achieve en bloc resection, interdisciplinary surgical approaches were indicated. Despite multimodal therapy concepts, including radiotherapy and chemotherapy as well as endovascular embolization, the patient died within 8 weeks. Conclusion: Prognosis of EHE is unpredictable and mainly determined by its location. The lesions are potentially aggressive; therefore, en bloc resection should be attempted whenever possible. However, as shown in the literature, only 15% of patients are suitable for total resection.

  18. Epithelioid hemangioendothelioma of the spine treated with RapidArc volumetric-modulated radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Guy, Jean-Baptiste; Trone, Jane-Chloé [Department of Radiotherapy, Institut de Cancérologie de la Loire, St Priest en Jarez (France); Chargari, Cyrus [Department of Radiation Oncology, HIA du Val de Grâce, Paris (France); Falk, Alexander Tuan [Department of Radiation Oncology, Centre Antoine Lacassagne, Nice (France); Khodri, Mustapha [Department of Physics, Institut de Cancérologie de la Loire, St Priest en Jarez (France); Magné, Nicolas, E-mail: [Department of Radiotherapy, Institut de Cancérologie de la Loire, St Priest en Jarez (France)


    Radiotherapy for epithelioid hemangioendothelioma (EHE) using volumetric intensity-modulated arc radiotherapy (VMAT). A 48-year-old woman was referred for curative irradiation of a vertebral EHE after failure of surgery. A comparison between VMAT and conventional conformal tridimensional (3D) dosimetry was performed and potential advantage of VMAT for sparing critical organs from irradiation's side effects was discussed. The total delivered dose on the planning target volume was 54 Gy in 27 fractions. The patient was finally treated with VMAT. The tolerance was excellent. There was no acute toxicity, including no increase in pain. With a follow-up of 18 months, no delayed toxicity was reported. The clinical response consisted of a decrease in the dorsal pain. The D{sub max} for the spinal cord was reduced from 55 Gy (3D-radiotherapy [RT]) (which would be an unacceptable dose to the spine because of the risk of myelopathy) to 42.8 Gy (VMAT), which remains below the recommended dose threshold (45 Gy). The dose delivered to 20% of organ volume (D{sub 20}) was reduced from 47 Gy (3D-RT) to 3 Gy (VMAT) for the spinal cord. The study shows that VMAT allows the delivery of curative treatment for vertebral EHEs because of critical organ sparing.

  19. Infantile Hepatic Hemangioendothelioma Associated With Congestive Heart Failure: Two Case Reports With Different Outcomes. (United States)

    Wang, Tao; Wang, Yibin; Liang, Yun; Lu, Guoyan


    Infantile hepatic hemangioendothelioma (IHH) is rare which can regress spontaneously. Arteriovenous shunts within hemangiomas, however, may result in pulmonary artery hypertension (PAH) and congestive heart failure (CHF).The authors report 2 young infants suffering from multifocal IHH associated with CHF were both treated with glucocorticoid and transcatheter arterial embolization (TAE), but had different outcomes. The PAH decreased immediately and the symptoms of CHF were alleviated after TAE for both of them. For the Tibetan infant, the development was normal with tumor regression by follow-up. For the Han ethnic neonate, PAH increased again in the seventh day with progressive cardiovascular insufficiency. Ultrasound showed a persisting perfusion caused by collateralization around occluded main feeders. Furthermore, a pulmonary infection occurred and ventilation was performed. As a result, the infant died from multiorgan failure caused by CHF and infection.TAE is a treatment of reducing shunting for hemangiomas. Fistula recanalization in multifocal IHH, however, might be an important risk factor affecting the outcome of TAE. TAE should be further evaluated with special attention to anatomy of feeding and draining vessels, and cardiopulmonary conditions. In addition, the patients were susceptible to secondary pulmonary infection because of lung congestion. As well, the infant from the high altitude area showed better adaptability to hypoxia.

  20. Bone multicentric epithelioid hemangioendothelioma of the lower and upper extremities with pulmonary metastases: A case report (United States)



    The present study reports a rare case of bone multicentric epithelioid hemangioendothelioma (EH) involving the upper and lower extremities simultaneously, with visceral involvement of the lung. Osteolytic lesions were first identified in the right distal femur and proximal tibia. Slight increased radionuclide uptake was observed in the right shoulder joint on bone scintigraphy, however, this was ignored, as no clinical symptoms were present. The patient was initially misdiagnosed with multifocal chondroblastoma, and an extra-articular curettage of lesions was performed in the proximal tibia and medial femoral condyle, which was filled with bone cement. The histopathological diagnosis was corrected post-operatively following immunohistochemical analysis, which indicated EH, and subsequently, an amputation of the right leg at thigh level was performed. In addition, multiple lytic lesions in the right shoulder joint and pulmonary metastases were identified on whole-body radiological examination. Radiotherapy was administered to the right shoulder joint, however, the patient refused chemotherapy or further surgery. At 15 months after the initial surgery, the patient currently remains alive. This case indicates that an improved understanding with regard to the clinical features of this disease may prevent misdiagnosis and improve EH treatment. PMID:26137035

  1. Diffuse Hepatic Epithelioid Hemangioendothelioma Developed in a Patient with Hepatitis C Cirrhosis

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    Pedro W. Baron


    Full Text Available Hepatic epithelioid hemangioendothelioma (HEHE is an infrequent vascular tumor of endothelial origin that primarily occurs in women in the mid-fifth decade of life without underlying chronic liver disease or cirrhosis. Liver transplant should be the first-line of therapy in patients with large or diffuse unresectable tumors even in the presence of metastatic disease due to the favorable long-term outcome. We report the case of a 48-year-old female who complained of abdominal pain and weight loss. She has a history of cirrhosis secondary to chronic hepatitis C (HCV and was treated with interferon and ribavirin with sustained virological response. Her work-up revealed multiple confluent infiltrating bilobar liver masses diagnosed as HEHE. She underwent a successful liver transplant without evidence of recurrent HCV infection. She developed cervical spine (C4-C6 HEHE metastases 4 years after transplant. She underwent surgical resection and local radiotherapy after resection with good clinical response. To the best of our knowledge, this is the first report of HEHE that developed in a patient with HCV cirrhosis successfully treated with antiviral therapy before transplant and liver transplant with good allograft function without evidence of recurrent liver tumor or HCV infection but developed metastases to the cervical spine 4 years after transplant.

  2. The Battle for Khe Sanh (United States)


    1Apr68) Capt Henry J. M. Radcliffe (Uan68-31Mar68) Capt Henry D. Banks (lApr68) Capt Robert T. Bruner (Uan68-26Jan68) IstLt Arthur N. Mangham, Jr...15Mar68) Capt Jerome P. Rogers (16Mar68-2lMar68) IstLt Jacob W. Hughes, Jr (22Mar68-lApr68) Capt Dennis L. Pardee (Aug67-29Feb68) Capt Victor B

  3. Hepatic hemangio-endothelioma: radiological findings and clinical evolution of a case; Hemangioendotelioma hepatico: aspectos radiologicos e evolucao clinica de um caso

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    Carvalho Neto, Arnolfo de; Souza, Carolina Althoff; Yanaga, Ricardo Hideki [Parana Univ., Curitiba, PR (Brazil). Hospital de Clinicas. Servico de Radiologia; Albonei, Mara [Parana Univ., Curitiba, PR (Brazil). Hospital de Clinicas. Dept. de Pediatria]. E-mail:


    Vascular tumors encompass a spectrum of lesions, which may vary from benign hemangiomas to malignant angio sarcomas. Hemangio-endotheliomas can be considered intermediary between these two types of lesions. We report a case of hepatic hemangio endothelioma in a newborn female and review the most important aspects of this disease. (author)

  4. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

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    Angela Sardaro


    Full Text Available Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers, and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.

  5. Contrast-enhanced multiple-phase imaging features in hepatic epithelioid hemangioendothelioma

    Institute of Scientific and Technical Information of China (English)

    Ying Chen; Ri-Sheng Yu; Ling-Ling Qiu; Ding-Yao Jiang; Yan-Bin Tan; Yan-Biao Fu


    AIM: To investigate and review the contrast-enhanced multiple-phase computed tomography (CEMP CT) and magnetic resonance imaging (MRI) findings in patients with pathologically confirmed hepatic epithelioid hemangioendothelioma (HEHE). METHODS: Findings from imaging examinations in 8 patients (5 women and 3 men) with pathologically confirmed HEHE were retrospectively reviewed (CT images obtained from 7 patients and MR images obtained from 6 patients). The age of presentation varied from 27 years to 60 years (average age 39.8 years). RESULTS: There were two types of HEHE: multifocal type (n = 7) and diffuse type (n = 1). In the multifocal- type cases, there were 74 lesions on CT and 28 lesions on MRI with 7 lesions found with diffusion weighted imaging; 18 (24.3%) of 74 lesions on plain CT and 26 (92.9%) of 28 lesions on pre-contrast MRI showed the target sign. On CEMP CT, 28 (37.8%) of 74 lesions appeared with the target sign and a progressive-enhancement rim and 9 (12.2%) of 74 lesions displayed progressive enhancement, maintaining a state of persistent enhancement. On CEMP MRI, 27 (96.4%) of 28 lesions appeared with the target sign with a progressive-enhancement rim and 28 (100%) of 28 lesions displayed progressive-enhancement, maintaining a state of persistent enhancement. In the diffuse-type cases, an enlarged liver was observed with a large nodule appearing with persistent enhancement on CEMP CT and MRI. CONCLUSION: The most important imaging features of HEHE are the target sign and/or progressive enhancement with persistent enhancement on CEMP CT and MRI. MRI is advantageous over CT in displaying these imaging features.

  6. Hemangioendotelioma epitelióide de pleura Epithelioid hemangioendothelioma of the pleura

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    Patrícia K. Vitório


    Full Text Available Relata-se o caso de um paciente exposto profissionalmente a asbesto por dez anos e portador de um tumor pleural muito raro, o hemangioendotelioma epitelióde. O paciente apresentava derrame pleural serohemorrágico, sem evidência de células neoplásicas e com predomínio de linfócitos. A biópsia pleural por agulha revelou processo inflamatório crônico inespecífico, com áreas de tecido mixóide. A videotoracoscopia mostrou nódulos difusos nas pleuras parietal e visceral. A biópsia revelou neoplasia mesenquimal e eram semelhantes às áreas focais observadas na primeira biópsia. O estudo imunohistoquímico demonstrou a presença dos marcadores vasculares CD31, CD34 e Fator VIII, caracterizando a origem vascular do tumor. O paciente foi tratado com cisplatina e ectoposide, tendo o óbito ocorrido três meses após o diagnóstico.Epithelioid hemangioendothelioma (EHE, a very uncommon pleural tumor, was diagnosed in a 61-year-old man with work-related exposure to asbestos. Serohemorrhagic pleural effusion was diagnosed in the work-up of this patient, whose complaints were chest pain and weight loss. A lymphocytic predominance was present in the effusion, but no malignant cells were seen; pleural needle biopsy disclosed only a non-specific inflammatory process. Video thoracoscopy revealed nodules in parietal and visceral pleurae. A biopsy revealed a mesenchymal neoplasm; vascular markers CD 31, CD 34 and VIII factor were present; therefore, diagnosis of HE was accepted. The tumor was not responsive to cisplatin or etoposide and the patient died 3 months after the diagnosis.

  7. Hemangioendotelioma: tumor raro de mediastino Hemangioendothelioma: a rare tumor of the mediastinum

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    Marcelo Loze de Queiroz


    Full Text Available Apresentou-se para atendimento um homem de 30 anos, branco, natural e procedente de São Paulo, com quadro de dor em hemitórax esquerdo, na região anterior e lateral, constante e de leve intensidade havia três meses, associado a dispnéia aos grandes esforços havia um mês. Durante a investigação foi visualizada em radiograma, tomografia e ressonância magnética de tórax grande tumoração em mediastino anterior e médio, com possível invasão dos vasos da base. Ele foi submetido à mediastinotomia paraesternal esquerda com biópsia da massa mediastinal, a qual complicou por sangramento intenso. Optou-se pela esternotomia mediana total e toracotomia ântero-lateral esquerda de urgência, com controle do sangramento e ressecção completa do tumor. Houve boa evolução, com alta hospitalar no nono dia pós-operatório. O exame anatomopatológico mostrou tratar-se de hemangioendotelioma de mediastino.A 30-year-old Caucasian male from São Paulo was admited to the hospital. He had been complaining about constant, moderate pain in the anterior and lateral left hemi-thoracic region for the last three mouths as well as associatede great effort dyspnea over the last mounth. Investigation with chest X-rays, CT scans and MRI revealed an large vessel invasion. The patient was submitted to a left side parasternal madiastinostomy and a biopsy of the mediastinal mass which was complicated by severe bleeding. An immediate median full sternotomy was elected in addition to a left anterior-lateral thoracotomy for total tumor resection and control of the bleeding. Evolution was good, with hospital discharge on the ninth postoperative day. The anatomical-pathological essay disclosed a hemangioendothelioma of the mediastinum.

  8. Epithelioid hemangioendothelioma of the liver with metastatic coeliac lymph nodes in an 11-year-old boy

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    Ines, David da; Petitcolin, Virginie; Garcier, Jean-Marc [CHU Clermont-Ferrand, Service de Radiologie et Imagerie Medicale, Hotel Dieu, Clermont-Ferrand (France); Joubert-Zakeyh, Juliette [CHU Clermont-Ferrand, Service d' Anatomopathologie, Hotel Dieu, Clermont-Ferrand (France); Demeocq, Francois [CHU Clermont-Ferrand, Service de Pediatrie Generale et Multidisciplinaire, Hotel Dieu, Clermont-Ferrand (France)


    Epithelioid hemangioendothelioma of the liver is a very rare vascular tumour in children with intermediate malignant potential. We present a case in which the typical imaging appearances of coalescent peripheral hepatic masses with capsular retraction contributed to the diagnosis. A positron emission tomography-CT (PET-CT) procedure was performed in staging the disease with a strong suspicion of coeliac nodal involvement confirmed after laparotomy and histological analysis. Our case is unique because of the rarity of the disease, the young age of the child, and proven nodal metastases at initial diagnosis. The use of PET-CT allows better staging at initial diagnosis and thus better management with improved follow-up in these patients. (orig.)

  9. Malignant hepatic epithelioid hemangioendothelioma with high-output heart failure: successful management of heart failure with transcatheter arterial chemoembolization. (United States)

    Hsu, Chien-Yi; Liu, Yao-Chung; Li, Chung-Pin; Huang, Po-Hsun; Lin, Chin-Hsuan; Chao, Yee


    A 73-year-old woman was admitted to hospital because of progressive dyspnea on exertion. Computed tomography revealed a large hepatic tumor, which was proved to be a hepatic epithelioid hemangioendothelioma (EHE). Echocardiography demonstrated high cardiac output, for which the tumor was considered to be the leading cause. A transcatheter arterial chemoembolization (TACE) was performed sequentially at 1-month intervals to reduce the size of the hepatic tumor, and this temporarily improved the patient's cardiac condition and quality of life. In this case, we successfully used TACE in the treatment of hepatic EHE with high-output heart failure. TACE is a reasonable choice of treatment both for managing malignant hepatic tumors and resolving low systemic vascular resistance by embolization of the abnormal neoangiogenic vessels. Nevertheless, clinicians should be aware of the potential adverse effect of hepatic decompensation induced by TACE, especially when the tumor involvement is widespread and poorly preserved hepatic function is encountered.

  10. Targeting VEGF-VEGFR Pathway by Sunitinib in Peripheral Primitive Neuroectodermal Tumor, Paraganglioma and Epithelioid Hemangioendothelioma: Three Case Reports

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    Tiziana Prochilo


    Full Text Available Sunitinib malate (Sutent™; Pfizer Inc., New York, N.Y., USA is a small molecule kinase inhibitor with activity against a number of tyrosine kinase receptors, including vascular endothelial growth factor receptors, stem-cell factor receptor, and platelet-derived growth factor receptors alpha and beta. Sunitinib, registered for the treatment of renal cell carcinoma and gastrointestinal stromal tumors, has recently been approved for the treatment of patients with advanced pancreatic neuroendocrine tumors. Peripheral primitive neuroectodermal tumor (pPNET, paraganglioma (PGL and epithelioid hemangioendothelioma (EHE are rare tumors in which there is an overexpression of pro-angiogenic factors and in which a high intratumoral microvessel density is a significant poor prognostic factor. On the basis of this preclinical rationale and the lack of effective treatments in pre-treated advanced stages of these rare diseases, we report our interesting experience of pPNET, PGL and EHE treatment with sunitinib.

  11. Epithelioid hemangioendothelioma and multiple thoraco-lumbar lateral meningoceles: two rare pathological entities in a patient with NF-1

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    Reis, C.; Carneiro, E.; Fonseca, J.; Salgado, A. [Hospital S. Joao, Departments of Neuroradiology, Porto (Portugal); Pereira, P.; Vaz, R. [Hospital S. Joao, Department of Neurosurgery, Porto (Portugal); Pinto, R. [Hospital S. Joao, Department of Orthopaedics, Porto (Portugal); Capelinha, A.F.; Lopes, J.M. [Hospital S. Joao, Department of Pathology, Porto (Portugal)


    Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the Neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. Biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles. (orig.)

  12. A rare case of multifocal pseudomyogenic hemangioendothelioma, involving soft tissues and bone, misdiagnosed as a rhabdomyosarcoma: Diagnostic and treatment implications

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    Bharat Rekhi


    Full Text Available 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. Whole-body positron emission tomography–computed tomography (PET-CT scan revealed multiple, metabolically active, subcutaneous nodular lesions over the left lower leg and in the distal tibia. Subsequently, resection specimens from the various lesions and bone curettage also revealed features of PHE. Three months later, the patient developed multiple lesions over his fourth toe and left foot, for which he underwent tumor resections. At present, he is disease-free. PHE is a locally aggressive soft tissue tumor characterized by multifocality, rarely bony involvement and can be misdiagnosed as a high-grade sarcoma.

  13. Diffuse Infantile Hepatic Hemangioendothelioma With Early Central Enhancement in an Adult: A Case Report of CT and MRI Findings. (United States)

    Dong, Aisheng; Dong, Hui; Zuo, Changjing; He, Tianlin


    Infantile hepatic hemangioendothelioma (IHH) is the most common vascular tumor of the liver in infancy. Adult with IHH is extremely rare. We presented a diffuse IHH in an adult patient with computed tomography (CT) and magnetic resonance image (MRI) findings.A 39-year-old man was admitted to our hospital because of a 2-year history of abnormal liver function tests and a 7-day history of jaundice. Physical examination revealed enlarged liver. Unenhanced abdominal CT showed enlargement of the liver with diffuse hypodensity. Enhanced CT on the arterial phase revealed multiple centrally enhanced lesions diffusely involved the enlarged liver. The enhanced areas of the lesions became larger on the portal phase and all the lesions became homogeneous enhanced on the delayed phase. These lesions showed heterogeneously hyperintense on T2-weighted image, hypointense on T1-weighted image, and early centrally enhanced on dynamic gadolinium-enhanced MRI, with complete tumor enhancement after 180 s. The patient underwent orthotopic liver transplantation. IHH type 2 was confirmed by pathology. The patient died of tumor recurrence in the liver 4 months after transplantation.Unlike the previously described imaging appearances of IHH, this case showed diffuse nodules with early central enhancement on CT and MRI. Considering the importance of the ability to differentiate IHH from other hepatic tumors, radiologists should be aware of these imaging appearances to establish knowledge of the entire spectrum of IHH.

  14. Kasabach-Merritt Phenomenon: Classic Presentation and Management Options. (United States)

    Mahajan, Priya; Margolin, Judith; Iacobas, Ionela


    Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy associated with specific vascular tumors, kaposiform hemangioendothelioma, and tufted angioma. Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia, hypofibrinogenemia, elevated fibrin split products, and rapid tumor growth, can be life-threatening. Severe symptomatic anemia may also be present. With prompt diagnosis and management, KMP can resolve and vascular tumors have been shown to regress. This review highlights the clinical presentation, histopathology, management, and treatment of KMP associated with kaposiform hemangioendothelioma, and less frequently tufted angioma. A classic clinical case is described to illustrate the presentation and our management of a patient with KMP.

  15. Spindle Cell Hemangioendothelioma of the Temporal Muscle Resected with Zygomatic Osteotomy: A Case Report of an Unusual Intramuscular Lesion Mimicking Sarcoma

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    Tomohiro Minagawa


    Full Text Available Spindle cell hemangioendothelioma (SCH was originally described by Weiss and Enzinger (1986 as a low-grade angiosarcoma resembling both cavernous hemangioma and Kaposi's sarcoma. Recent studies suggest that SCH is a benign neoplasm or reactive lesion accompanying a congenital or acquired vascular malformation. Most SCHs present as one or more nodules affecting the dermis or subcutis of the distal extremities. Few reports describe SCH of the head and neck region; even fewer note intramuscular SCH. Here, we describe a case of SCH involving the temporal muscle mimicking soft tissue sarcoma, who had a successful surgical treatment with a coronal approach and zygomatic osteotomy.

  16. Hemangioendotelioma maligno do crânio: relato de um caso em criança Malignant hemangioendothelioma of the skull in a child

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    Walter C. Pereira


    Full Text Available Os hemangioendoteliomas primitivamente ósseos são neoplasias muito raras e de diagnósticos clínico e radiológico difíceis. Em virtude de seu crescimento rápido, grande capacidade infiltrativa e metastatização precoce, o prognóstico é geralmente sombrio, mesmo com cirurgia radical seguida de radioterapia. Relatamos um caso de hemangioendotelioma maligno, localizado no crânio, em criança de 6 anos de idade, cujo diagnóstico radiológico foi de displasia fibrosa óssea. O doente foi operado, sendo feita exérese aparentemente total da neoplasia e, a seguir, irradiado com cobaltoterapia em doses elevadas; tais medidas, contudo, não impediram a progressão do processo neoplásico. A quimioterapia, com Enduxan intravenoso, determinou a cura clínica e radiológica do paciente. São discutidos varios aspectos referentes ao diagnóstico do hemangioendotelioma maligno, tendo em vista a grande confusão existente na literatura em relação à nomenclatura e classificação desses tumores.The primary localization of hemangioendothelioma in the bone is very rare and the clinical and radiological diagnosis is often difficult. Due to the rapid growth, invasive properties and early metastatization, the evolution of these neoplasms is often progressive, despite the radical removal followed by radiotherapy. A case of a malignant hemangioendothelioma of the skull in a 6-year-old male patient is reported. The radiological aspects supported the diagnosis of fibrous dysplasia of bone. The patient was operated on, and an apparently total removal of the tumor was performed, followed by cobaltotherapy; however, the growth of the neoplasm was not arrested. Thus, chemotherapy with intravenous Enduxan (50 mg per day was employed during a month, with clinical recovery and radiological cure of the process. Some clinical features on the differential diagnosis of hemangioendothelioma are discussed due to the confusion on the terminology and classification of

  17. Vincristine: A new treatment option for Kasabach-Merritt Syndrome

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    Shalini Avasthi


    Full Text Available Kasabach-Merritt syndrome (KMS is characterized by a rapidly enlarging hemangioma, thrombocytopenia, microangiopathic hemolytic anemia and consumption coagulopathy as a result of platelet and red blood cell trapping and activation of clotting system within the vasculature of hemangioma. This syndrome is shown to be associated with kaposiform hemangioendotheliomas or tufted hemangioma.

  18. The place of liver transplantation in the treatment of hepatic epitheloid hemangioendothelioma: report of the European liver transplant registry. (United States)

    Lerut, Jan P; Orlando, Giuseppe; Adam, Rene; Schiavo, Marcello; Klempnauer, Jurgen; Mirza, Darius; Boleslawski, Emmanuel; Burroughs, Andrew; Sellés, Carlos Fernandez; Jaeck, Daniel; Pfitzmann, Robert; Salizzoni, Mauro; Söderdahl, Gunner; Steininger, Rudi; Wettergren, Andre; Mazzaferro, Vincenzo; Le Treut, Yves Patrice; Karam, Vincent


    Hepatic epitheloid hemangioendothelioma (HEHE) is a rare low-grade vascular tumor. Its treatment algorithm is still unclear mainly due to a lack of larger clinical experiences with detailed long-term follow-up. Fifty-nine patients, reported to the European Liver Transplant Registry, were analyzed to define the role of liver transplantation (LT) in the treatment of this disease. Eleven (19%) patients were asymptomatic. Eighteen (30.5%) patients had pre-LT surgical [hepatic (7 patients) and extrahepatic (3 patients)] and/or systemic or locoregional (10 patients) medical therapy. Ten (16.9%) patients had extrahepatic disease localization before or at the time of LT. Follow-up was complete for all patients with a median of 92.5 (range, 7-369) from moment of diagnosis and a median of 78.5 (range, 1-245) from the moment of LT. HEHE was bilobar in 96% of patients; 86% of patients had more than 15 nodules in the liver specimen. Early (3 months) post-LT mortality was 1.7% (1 patient) and 22% (14 patients). Fourteen (23.7%) patients developed disease recurrence after a median time of 49 months (range, 6-98). Nine (15.3%) patients died of recurrent disease and 5 are surviving with recurrent disease. One-, 5-, and 10- year patient survival rates from moment of transplantation for the whole series are 93%, 83%, 72%. Pre-LT tumor treatment (n = 18) (89%, 89%, and 68% 1-, 5-, and 10-year survival rates from moment of LT vs. 95%, 80%, and 73% in case of absence of pre-LT treatment), lymph node (LN) invasion (n = 18) (96%, 81%, and 71% 1-, 5-, and 10-year survival rates vs. 83%, 78%, and 67% in node negative patients) and extrahepatic disease localization (n = 10) (90%, 80%, and 80% 1-, 5-, and 10-year survival rates vs. 94%, 83%, and 70% in case of absence of extrahepatic disease) did not significantly influence patient survival whereas microvascular (n = 24) (96%, 75%, 52% 1-, 5-, and 10-year survival vs. 96%, 92%, 85% in case of absence of microvascular invasion) and combined

  19. Epithelioid hemangioendothelioma in parotid gland misdiagnosed as parotid gland cyst with hemorrhage in 1 case%腮腺上皮样血管内皮瘤误诊为腮腺囊肿伴出血1例

    Institute of Scientific and Technical Information of China (English)

    夏超; 谭颖徽


    回顾1例被误诊为腮腺囊肿伴出血的上皮样血管内皮瘤的患者的病历资料,结合文献讨论该疾病的临床特点、诊断、治疗。%The report presents a case of epithelioid hemangioendothelioma in parotid gland misdiagnosed as parotid gland cyst with hemor-rhage.Based on the literature review,clinical characteristics,diagnosis and treatment of the disease are discussed.

  20. Kasabach-Merritt Phenomenon: Classic Presentation and Management Options


    Priya Mahajan; Judith Margolin; Ionela Iacobas


    Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy associated with specific vascular tumors, kaposiform hemangioendothelioma, and tufted angioma. Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia, hypofibrinogenemia, elevated fibrin split products, and rapid tumor growth, can be life-threatening. Severe symptomatic anemia may also be present. With prompt diagnosis and management, KMP can resolve and vascular tumors have been shown to regress. This revie...

  1. Subperiosteal hemangioendothelioma of the femur

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Ajay [Royal National Orthopaedic Hospital NHS Trust, Departments of Orthopaedic Oncology, Stanmore, Middlesex (United Kingdom); Saifuddin, Asif [Royal National Orthopaedic Hospital NHS Trust, Departments of Radiology, Stanmore, Middlesex (United Kingdom); London Bone and Soft Tissue Tumour Service, London (United Kingdom); University College London, Institute of Orthopaedics and Musculoskeletal Sciences, London (United Kingdom); Briggs, Tim W.R. [Royal National Orthopaedic Hospital NHS Trust, Departments of Orthopaedic Oncology, Stanmore, Middlesex (United Kingdom); London Bone and Soft Tissue Tumour Service, London (United Kingdom); University College London, Institute of Orthopaedics and Musculoskeletal Sciences, London (United Kingdom); Flanagan, Adrienne M. [Royal National Orthopaedic Hospital NHS Trust, Departments of Histopathology, Stanmore, Middlesex (United Kingdom); London Bone and Soft Tissue Tumour Service, London (United Kingdom); University College London, Institute of Orthopaedics and Musculoskeletal Sciences, London (United Kingdom)


    Primary neoplastic vascular lesions of bone are rare, and include haemangiomas, haemangioendothelioma, epithelioid haemangioendothelioma and angiosarcoma. These lesions may be multicentric, and when they involve bone are typically intraosseous and lytic. Radiological findings are not always specific. We report a case of haemangioendothelioma localised to the subperiosteum of the femur, a site not previously described for this lesion. The nomenclature for vascular neoplasms in bone is discussed. (orig.)

  2. Imaging evaluation of fetal vascular anomalies

    Energy Technology Data Exchange (ETDEWEB)

    Calvo-Garcia, Maria A.; Kline-Fath, Beth M.; Koch, Bernadette L.; Laor, Tal [MLC 5031 Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Adams, Denise M. [Cincinnati Children' s Hospital Medical Center, Department of Pediatrics and Hemangioma and Vascular Malformation Center, Cincinnati, OH (United States); Gupta, Anita [Cincinnati Children' s Hospital Medical Center, Department of Pathology, Cincinnati, OH (United States); Lim, Foong-Yen [Cincinnati Children' s Hospital Medical Center, Pediatric Surgery and Fetal Center of Cincinnati, Cincinnati, OH (United States)


    Vascular anomalies can be detected in utero and should be considered in the setting of solid, mixed or cystic lesions in the fetus. Evaluation of the gray-scale and color Doppler US and MRI characteristics can guide diagnosis. We present a case-based pictorial essay to illustrate the prenatal imaging characteristics in 11 pregnancies with vascular malformations (5 lymphatic malformations, 2 Klippel-Trenaunay syndrome, 1 venous-lymphatic malformation, 1 Parkes-Weber syndrome) and vascular tumors (1 congenital hemangioma, 1 kaposiform hemangioendothelioma). Concordance between prenatal and postnatal diagnoses is analyzed, with further discussion regarding potential pitfalls in identification. (orig.)

  3. Formation of K*He Exciplexes on the Surface of Helium Nanodroplets Studied in Real Time (United States)

    Schulz, C. P.; Claas, P.; Stienkemeier, F.


    Superfluid helium nanodroplets are doped with potassium atoms to form complexes with the alkali atom residing on the surface of the droplets. Dispersed laser-induced fluorescence spectra of such systems already revealed the formation of M*He ( M = Na,K) exciplexes upon electronic excitation [Reho et al., Faraday Discuss. 108, 161 (1997)]. By means of femtosecond pump-probe spectroscopy, this formation process now is followed in real time. We find K*Hen = 1 to be formed within 180 fs. Furthermore, the existence of exciplexes with n>1 is quantified suggesting that the first ring around the potassium atom contains four helium atoms.

  4. Project CHECO Southeast Asia Report. Khe Sanh (Operation NIAGARA) 22 January - 31 March 68 (United States)


    reported trucks moving down Route 9 toward their 44/3 positions, As Covey 252 reported: "Of course, we fcresaw a reenactment of the disaster at Elephant,, We...aerial reconnaissance flown during Operation NIAGARA U almost doubled the film footage normally processed by 7AF facilities. As 3 the workload passed...100,000 feet of film per day, a 70 per cent augmentation I 48 (This , page is II F4 41 IA 44F AT4 IX I,v -r4. ijIUR 8" Iv wp - in Photo Interpreter

  5. Victualing Victory: Logistics from Lake George to KHE SANH, 1755-1968 (United States)


    time with a smaller corps of vehicles—leaning more heavily on animals and men.34 After the experience of the mud in 1941, the Germans adjusted their...copying one’s past experience . . . To repeat exactly what belongs to history in the face of reality is adventurism . . . If we fight the from the USAFA history department. In 1998, he attended Joint Specialized Undergraduate Pilot Training at Laughlin AFB, TX; and, upon graduation

  6. A Case of Patch Stage of Kaposi’s Sarcoma and Discussion of the Differential Diagnosis (United States)

    Kak, Ipshita; Salama, Samih; Gohla, Gabriella; Naqvi, Asghar; Alowami, Salem


    A 55 year old HIV positive male had a skin lesion biopsy which showed atypical vascular proliferation within the superficial and deep dermis with mild atypia of lining endothelial cells. A sparse lymphoplasmacytic infiltrate surrounding the irregular vascular channels was noted. Immunohistochemistry highlighted the atypical blood vessels with the vascular markers CD31, CD34 and Factor VIII. The differential diagnosis included unusual vascular or lymphatic proliferations, stasis dermatitis, kaposiform hemangioendothelioma, progressive lymphangioma and angiosarcoma with focal Kaposi’s sarcoma features. Characteristic human herpes virus-8 positive staining helped support the diagnosis of patch stage of Kaposi’s sarcoma. Herein, we discuss the case findings, differential diagnosis and characteristic histological findings associated with the patch stage of Kaposi’s sarcoma which can be an elusive diagnosis. PMID:27134709

  7. Contrast-enhanced ultrasound of histologically proven hepatic epithelioid hemangioendothelioma

    DEFF Research Database (Denmark)

    Dong, Yi; Wang, Wen-Ping; Cantisani, Vito


    %) in the arterial phase and hypoenhancement (25/25, 100%) in the portal venous and late phases (PVLP), a sign of malignancy. Eighteen patients showed central unenhanced areas (18/25, 72%); in seven patients (7/25, 28%), more lesions were detected in the PVLP. In contrast, all patients with hemangioma and focal...

  8. [Primary cardiac hemangioendothelioma: early diagnosis and surgical resection]. (United States)

    Gaytán-Cortés, Fernando Celso; Arteaga-Adame, Juan; Careaga-Reyna, Guillermo; Lezama-Urtecho, Carlos; Álvarez-Sánchez, Luis


    Introducción: el hemangioendotelioma engloba todos los tumores que derivan del endotelio de los vasos sanguíneos. Es de pronóstico incierto y siempre se considera como una lesión maligna de bajo grado. Caso clínico: paciente masculino de 23 años que ingresó a urgencias con dolor retroesternal intermitente, disnea paroxística nocturna y ataque al estado general. Negó antecedentes crónico-degenerativos y tuvo pérdida ponderal de 6 kg en dos meses. Las pruebas sugirieron derrame pericárdico. Presentó tendencia a la hipotensión por tamponade, resuelta con punción subxifoidea, y drenaje de 800 mL de líquido de características hemáticas antiguas. El paciente presentó un tumor de la aurícula derecha de 8 x 4 cm, dependiente de la pared anterior de la aurícula, sin involucro de septum interatrial. Al someter al paciente a cirugía, se identificó lesión tumoral en la pared anterior de orejuela derecha. Se hizo resección del tumor y del 70 % de la orejuela derecha, sustituida con parche de pericardio bovino. El paciente presentó buena evolución; egresó a los 10 días de la cirugía con seguimiento por seis meses en la consulta externa. Conclusión: estamos ante un tumor vascular oncológicamente impredecible y que rara vez se origina en el corazón. Una detección oportuna y la resección quirúrgica radical es el manejo más aceptado ante la poca experiencia que hay a nivel mundial para lidiar con esta entidad. Sugerimos un abordaje clínico agresivo y la extirpación quirúrgica dentro de las primeras horas de la sospecha diagnóstica para obtener la mayor posibilidad de resección total y la reducción del riesgo de recidiva.

  9. The utility of GLUT1 as a diagnostic marker in cutaneous vascular anomalies: A review of literature and recommendations for daily practice. (United States)

    van Vugt, Lieke J; van der Vleuten, Carine J M; Flucke, Uta; Blokx, Willeke A M


    To assess the utility of GLUT1 as an immunohistochemical marker in the diagnostics of cutaneous vascular anomalies. A systematic literature search was conducted for studies on GLUT1 staining patterns in cutaneous vascular lesions. Data was grouped according to the latest ISSVA classification for vascular anomalies. Vascular tumors: GLUT1 staining was positive in 368/386 (95%) of infantile hemangiomas. Congenital hemangiomas (16 cases) and kaposiform hemangioendotheliomas (62 cases) were all negative for GLUT1. Angiosarcomas were GLUT1 positive in 12/39 (31%) and epithelioid hemangioendotheliomas in 2/27 (7%) of cases. Vascular malformations: All vascular malformations (33 arteriovenous malformations, 16 capillary malformations, 64 lymphatic malformations, 54 venous malformations, 3 venous-lymphatic malformations and 3 capillary venous-lymphatic malformations) were negative for GLUT1 staining. Unclassified vascular anomalies: Angiokeratomas were GLUT1 positive in 1/15 (7%) and verrucous hemangiomas in 71/100 (71%) of cases. Microvenular hemangiomas were negative for GLUT1 in all 9 cases. GLUT1 can be used as an additional diagnostic tool in cutaneous vascular lesions. A negative GLUT1 stain renders a diagnosis of infantile hemangioma unlikely. A positive GLUT1 stain excludes vascular malformations and is suggestive of infantile hemangioma. One must be cautious, however, that the final diagnosis is made through interpretation of all clinical and diagnostic features, and not based on GLUT1 staining alone. Copyright © 2017 Elsevier GmbH. All rights reserved.

  10. K-He3 and K+K- interactions in the pd→ He3 K+K- reaction (United States)

    Grishina, V. Yu.; Büscher, M.; Kondratyuk, L. A.


    We investigate the K- He3 and K+K- interactions in the reaction pd→ He3 K+K- near threshold and compare our model calculations with data from the MOMO experiment at COSY-Jülich. A large attractive effective K-p amplitude would give a significant K- He3 final-state interaction effect, which is not supported by the experimental data. We also estimate upper limits for the a0(980) and f0(980) contributions to the produced K+K- pairs.

  11. Infantile hepatic hemangioendothelioma with heart failure as the main manifestation: a report of 4 cases and literature review%以心力衰竭为主要表现的婴儿型肝血管内皮细胞瘤4例并文献复习

    Institute of Scientific and Technical Information of China (English)

    汪周平; 黄萍; 张丽; 于明华; 谢小斐


    Objective To summarize the clinical features,treatment and prognosis of infantile hepatic hemangioendothelioma(IHHE) combined with congestive heart failure(CHF) in children.Methods A retrospective analysis was made with the clinical data from 4 children with IHHE combined with CHF in Department of Pediatric Cardiology,Affiliated Guangzhou Women and Children's Medical Center of Guangzhou Medical University from May 2013 to December 2015.Results Among 4 infants,there were 3 male and 1 female,mean aged of 109 days (21-219 days),the average weight of 4 350 g (2 750-6 500 g),and 1 case was admitted because of abdominal swelling and respiratory distress,1 case of abdominal swelling,jaundice,shortness of breath admission,1 case of fever and shortness of breath admission,and 1 case of cyanosis and shortness of breath admission.Two patients were associated with hemangioma and Kasabach-Merritt syndrome.Three cases had multiple liver hemangioma scattered in the left lobe and right lobe of the liver,and 1 case had solitary liver hemangioma scattered in the right lobe.Tumor size diameters were ranged from 2-10 mm.Four children had been used Prednisone [1-2 mg/(kg · d)] and Digoxin,Dopamine,Furosemide,Spironolactone.Two cases responsed well to the therapy and were followed up for 2 years or 1 year and 6 months respectively,whose heart size,heart function and pulmonary hypertension became normal.One case with Kasabach-Merritt syndrome responsed poorly to the therapy.Two cases experienced transcatheter hepatic arteriovenous fistula embolization by using coil occlusion of hepatic artery-venous fistula.The postoperative hepatic tumor volume was significantly decreased,and the heart function was improved.One patient was followed up for 1 year and 6 months,and the heart size,pulmonary hypertension and heart function returned to normal.One case was still under follow-up.One case with Kasabach-Merritt syndrome died from heart failure and severe infection before surgery.The mortality

  12. Successful Treatment of Mild Pediatric Kasabach-Merritt Phenomenon with Propranolol Monotherapy

    Directory of Open Access Journals (Sweden)

    Worawut Choeyprasert


    Full Text Available Kasabach-Merritt phenomenon (KMP is relatively rare in childhood and adolescents with high mortality rate because of its hemorrhagic complications and unresponsiveness to treatments such as corticosteroids, vincristine, intravascular embolization, and/or surgery. Propranolol, a β-adrenergic receptor blocker, has a promising efficacy against vascular tumors such as infantile hemangiomas. But limited and variable data has been reported regarding the role of propranolol in treatment of KMP. We herein reported the successful treatment of mild pediatric KMP with propranolol monotherapy in a case of a five-week-old child with kaposiform hemangioendothelioma with successful treatment of both clinical and hematologic responses. After eight months of follow-up, patient still had stable cutaneous lesion while receiving propranolol monotherapy. Regular hematologic monitoring was done in order to detect any late relapse of the disease. Six months after discontinuation of propranolol, patient has still remained free of hematologic relapse, and primary cutaneous lesion has become a pale pink, 1 cm sized skin lesion.

  13. A trimodality approach in the management of metastatic low-grade epithelioid hemangioendothelioma of the bone. (United States)

    Saste, Abhijit; Cabrera Fernandez, Diego Felipe; Gulati, Rohit; Gamalski, Steven


    A 29-year-old man presented with a 2-week history of severe pain in the left foot with no preceding history of trauma. A left foot radiograph demonstrated a cortical lucency in the mid-distal shaft of the third metatarsal bone. MRI of the left foot showed an expansile lesion in the same location. A staging bone scan showed a focal uptake in the known lucency in the left third metatarsal and in the proximal left femur. A subsequent left hip radiograph demonstrated a lucency in the intertrochanteric region. CT scan of the chest, abdomen and pelvis was unremarkable. A biopsy of the left third metatarsal expansile lesion performed during an incision and curettage procedure revealed an epithelioid haemangioma (EHE) of the bone. MRI of the left hip performed in response to the findings on the bone scan showed metastatic disease in the left intertrochanteric region. A prophylactic left hip fixation surgery with an interlocking intramedullary femoral nail was therefore undertaken to avoid a pathological fracture of the left hip from the metastatic disease. Simultaneously, a left hip biopsy was performed, which also revealed an EHE. The patient underwent external beam radiation to the left femoral head and neck. This was followed by fractionated radiosurgery to the left third metatarsal. Once the left foot wound had healed, the patient subsequently received four cycles of doxorubicin and ifosfamide. A restaging positron emission tomography CT carried out after completion of therapy showed no metabolic evidence of residual primary tumour or metastasis. More than 2 years after completing his trimodality therapy, the patient remains fully functional and symptom free.

  14. Primary Pleural Epithelioid Hemangioendothelioma (EHE) - Two Cases and Review of the Literature (United States)


    major fissure associated with a pseudotumor (Fig. 2). Initial evaluation included two CT guided fine needle aspirations of the mass that showed...was done in both of them revealed an exudative effusion that did not reveal any infection or malignancy. Both cases underwent surgical biopsy and the...revealed an exudative effusion that did not reveal any infection or malignancy. Both cases underwent surgical biopsy and the diagnosis of primary

  15. 1st Cavalry Division’s Effectiveness In Conducting Airmobile Operations During Operation Pegasus (United States)


    assault, OH-6 Cayuse scout helicopters and AH­ 1G Cobra attack helicopters from the cavalry scoured the area at low altitude , collected intelligence...Lieutenant General John Tolson, the division commander, drew-up initial plans to relieve the Marines at Khe Sanh on January 25, 1968.13 When he briefed...Cavalry Division to relieve Khe Sanh. 64 The Rogers Board began the development of the aircraft that would make 1st Cavalry Division successful by

  16. 儿童脉管异常117例临床病理学观察%Clinicopathologic study of pediatric vascular anomalies:a report of 117 cases

    Institute of Scientific and Technical Information of China (English)

    牛会林; 曾荣新; 周少毅; 林雀卿; 伊鹏; 王凤华; 高秋; 陈峥嵘; 夏建清; 郑汉城


    of ISSVA classification. Methods The clinical features, histopathologic findings and immunohistochemical results were analyzed in 117 cases of pediatric vascular anomalies encountered during the period from May 2014 to May 2015.Results A total of 117 cases of vascular anomalies were studied. The age of patients ranged from 18 hours after birth to 11 years ( mean age =34 months and median age =27 months) .There were 73 male patients and 44 female patients, with the male-to-female ratio being 1.7∶1.0.Congenital skin lesions were found in 37 cases ( 31.6%) .The common sites of involvement included head and neck region (46 cases, 39.3%), trunk (28 cases, 23.9%), extremities (14 cases, 12.0%) and internal viscera (31 cases, 26.5%).According to the new ISSVA classification, there were 74 cases of vascular malformations and 43 cases of vascular neoplasms ( ratio=1.7∶1.0 ) .The commonest vascular tumor encountered was infantile hemangioma ( 21 cases, 48.8%) , including 17 cases in proliferative phase and 4 cases in involutive phase.Thirteen cases (23.3%) of congenital hemangioma were found, with 8 cases of rapidly involuting congenital hemangioma and 5 cases of non-involutive congenital hemangioma.Three of the congenital hemangioma occurred in liver. There were 5 cases ( 11.6%) of pyogenic granuloma, 3 cases ( 7.0%) of tufted angioma and 1 case ( 2.3%) of Kaposiform hemangioendothelioma.Amongst the 74 cases of vascular malformations encountered, lymphatic malformation was found in 47 cases ( 63.5%) , venous malformation in 15 cases ( 20.2%) , lymphatic-venous malformation in 11 cases (14.9%) and arteriovenous malformation in 1 case (1.4%).All cases of vascular anomalies were all positive for CD31 on immunostaining.Glut1 and CD15 were positive both in proliferative and involutive phases of the 21 cases of infantile hemangioma, while other vascular tumors and vascular malformations were negative.Forty-seven cases of lymphatic malformation and 11 cases of lymphatic

  17. Epithelioid hemangioendothelioma of the brain: a case report and literature re-view%脑上皮样血管内皮瘤1例及文献复习

    Institute of Scientific and Technical Information of China (English)

    丁敏; 蚁国铮; 王晓秋; 胡闻


    目的:探讨脑上皮样血管内皮瘤(EHE)的诊断及鉴别诊断.方法:通过HE、免疫组化及电镜观察1例脑原发性多灶性上皮样血管内皮瘤、并复习文献.结果:肿瘤由排列呈巢状、索状的上皮样细胞组成,胞浆丰富、嗜酸性,内含明显空泡呈"印戒"状,有的空泡内可见红血球,间质含粘液样基质.免疫组化染色显示FⅧ、CD34、CD31、UEA-1和vimentin阳性;而CK、CEA、EMA、GFAP、S-100蛋白及Mac 387阴性;组织化学染色AB/PAS阴性,网状纤维显示无数血管腔隙结构.电镜观察胞质内可见特征性Weibel-Palade小体.结论:EHE是一种不常见的生物学行为介于良、恶性之间的血管肿瘤,发生在脑内很罕见.组织形态需与转移癌、粘液性软骨肉瘤、脊索样脑膜瘤及转移性心房粘液瘤等鉴别.

  18. Preliminary Experience with Prenatal Magnetic Resonance Imaging in Evaluation of Fetal Hepatic Hemangioendotheliomas%胎儿肝脏血管内皮瘤MRI诊断的初步探讨

    Institute of Scientific and Technical Information of China (English)

    董素贞; 朱铭; 李奋; 钟玉敏; 张弘; 潘慧红


    目的:探讨胎儿肝脏血管内皮瘤的产前MRI表现及诊断价值.方法:5例孕妇,孕龄22~ 37周.产前常规行超声(US)检查后24 ~ 48h内行MR检查,采用二维快速平衡稳态采集(2D FIESTA)序列、单次激发快速自旋回波(SSFSE)序列以及快速翻转恢复运动抑制(FIRM)序列,行胎儿颅脑胸腹部常规及肝脏重点冠状面、矢状面及横断面扫描,将产前MRI、US表现与出生后影像表现或手术病理结果对照.结果:5例肝脏血管内皮瘤均表现为肝脏内边界清晰的单个肿块,其中1例合并皮下多发血管瘤.在FIESTA、SSFSE序列上表现为不均匀稍高信号,在FIRM序列上表现为不均匀低信号.所有病例FIESTA序列肝脏横断面可见包块周边扩张的肝血管和远端腹主动脉变细.结论:产前MRI能提供诊断胎儿肝脏血管内皮瘤的有用信息,能提供产前US以外的补充诊断信息.%Purpose: To explore the value of combining high field MRI dynamic enhancement and MRCP in the diagnosis of extrahepatic cancerous obstructive jaundice. Methods: Thirty cases of obstructive jaundice were included in this study, MRCP and multi - phase dynamic enhancement were performed following conventional MRI scans. The manifestions were reviewed and correlated with pathology. Results: The pathological diagnosis of the patients were cholangiocarcinoma in 15 cases, ampullary carcinoma in 5 cases, carcinomas of pancreas head in 7 cases, duodenal carcinoma in 1 case, carcinomas of gallbladder in 2 cases. One case of carcinoma of pancreas head was missed. Two cases of cholangiocarcinoma and 1 case of ampullary carcinoma were misdiagnosed as inflammations. The diagnostic accuracy rate of qualitation and location was 86. 7% and 93.3% .Conclusion: High field MRI dynamic enhancement combined with MRCP can play an important role in diagnosis and differential diagnosis of extrahepatic cancerous obstructive jaundice.

  19. A New Swarm Intelligence Approach for Clustering Based on Krill Herd with Elitism Strategy

    Directory of Open Access Journals (Sweden)

    Zhi-Yong Li


    Full Text Available As one of the most popular and well-recognized clustering methods, fuzzy C-means (FCM clustering algorithm is the basis of other fuzzy clustering analysis methods in theory and application respects. However, FCM algorithm is essentially a local search optimization algorithm. Therefore, sometimes, it may fail to find the global optimum. For the purpose of getting over the disadvantages of FCM algorithm, a new version of the krill herd (KH algorithm with elitism strategy, called KHE, is proposed to solve the clustering problem. Elitism tragedy has a strong ability of preventing the krill population from degrading. In addition, the well-selected parameters are used in the KHE method instead of originating from nature. Through an array of simulation experiments, the results show that the KHE is indeed a good choice for solving general benchmark problems and fuzzy clustering analyses.

  20. Close Air Support: Why All the Fuss (United States)


    column, and hamlet defense in Indochina and South Vietnam; and siege-breaking at Dien Bien Phu and Khe Sanh. In all of these cases, CAS substituted...the Dien Bien Phu experience, and, indeed, enabled Khe Sanh to accomplish what the French at Dien Blien Phu had tried and failed to achieve: create a...technological frontiers on which the Air Force has always sought leadership. It fits nicely their service " persona ." But, is it in the Army’s interest to take

  1. An Analysis of Tactical Military Airlift (United States)


    Major William Scott of AFIT; MSGT (Ret.) Gaye Summitt and MSGT (Ret.) Vern Moore, who took a fledgling Lieutenant under their wings and molded him into...Decision: The Siege of Khe Sanh. New York: Houghton Mifflin Company. Roy, Jules . 1965. The Battle of Dienbienphu. New York: Harper & Row. Shore, Moyers

  2. Close Air Support: Which Way Do We Go? (United States)


    Reservoir fighting in 1950; outpost, column, and Parameters hamlet defense in Indochina and South Vietnam; and siege-breaking at Dien Bien Phu and Khe...Air Force has always sought leadership. It fits nicely their service persona . But is it in the Army’s interest to take over CAS, much less all of

  3. Joint Force Quarterly. Number 17, Autumn/Winter 1997-98 (United States)


    paths fifty miles from Khe Sanh past Tchepone, where the northern runner peters out. Its companion, which plunges on for another fifty miles, is a...natural barrier breached in just four places. The Lang Vei cleft farthest east expires south of Route 9 in a maze of serrated highlands. A second portal

  4. 75 FR 32085 - Prayer for Peace, Memorial Day, 2010 (United States)


    ...#0;#0; ] Proclamation 8532 of May 28, 2010 Prayer for Peace, Memorial Day, 2010 By the President of..., from Inchon and Khe Sanh to Baghdad and Kandahar, they departed our world as heroes and gave their... their memory and pray for the peace for which they laid down their lives. We mourn with the families and...

  5. "White cranes fly over black cranes"

    DEFF Research Database (Denmark)

    Vu, Thi Thao; Agergaard, Jytte


    by the ’migration as resistance’ approach, this paper examines the changes in rural–urban migration dynamics in Vietnam from the 1950s to 2010 in relation to the country’s political and economic reforms (пi M¿i). Drawing on a case study of female migration between Phu Khe and Hanoi, it analyses how migrants resist...

  6. Cloning of a gene encoding a unique haemolysin from Klebsiella pneumoniae and its potential use as a species-specific gene probe. (United States)

    Yin-Ching, Chuang; Jer-Horng, Su; Ching-Nan, Lin; Ming-Chung, Chang


    A gene, designated khe, that encodes a haemolysin of Klebsiella pneumoniae CMC-1 has been cloned and sequenced. When expressed in Escherichia coli, a unique peptide of approximately 20kDa was identified. Nucleotide sequence analysis predicted a single open reading frame (ORF) of 486bp encoding a 162 amino acid polypeptide with an estimated pI of 6.77. No extensive sequence homology could be identified between khe and any reported sequence at either the nucleotide or amino acid level. Furthermore, DNA hybridizations under high stringency conditions failed to show any cross hybridizations to several bacteria including K. oxytoca, K. planticola, K. terrigena and K. ornithinolytica. These data indicate that we have cloned a unique gene, which is highly conserved among tested K. pneumoniae isolates.

  7. Yangliuqing New Year Pictures

    Institute of Scientific and Technical Information of China (English)


    HANGING up New Yearpictures during khe SpringFestival to symbolize goodluck in the coming year has longbeen a tradition in China,The pic-tures are both colorful and jubilantin theme and composition so as toattract everything good in the com-ing year and drive away evil.Four main“schools” of folk artdealing with New Year pictures ex-ist.Among the best known is thewoodblocK print from Yangliuqing,

  8. Flexibility, Reach, and the Vietnam Fire Base (United States)


    48 v ACRONYMS AVRN Army of the Republic of Vietnam COIN Counterinsurgency DRV Democratic Republic of Vietnam DOD Department of...insect-transmitted diseases like malaria, dengue, and encephalitis. Cholera , hepatitis, and typhoid were common in the countryside because of v =Q-m96RoxwrI (accessed 18 January 2014). 75PBS, “Battlefield: Vietnam; The Siege at Khe Sanh,” Battlefield Vietnam Web Site, Adobe Shockwave

  9. American Leadership and Decision-Making Failures in the Tet Offensive (United States)


    1980. Chomsky, Noam, and Howard Zinn , eds. The Pentagon Papers. The Senator Gravel Edition. Boston: Beacon Press, 1972. Clausewitz, Carl von. On War...12Robert Pisor, The End of the Line: The Siege of Khe Sanh (New York: W. W. Norton and Company, 1982), 170-177. 13Ibid., 235; and Naom Chomsky and Howard ... Zinn , eds. The Pentagon Papers, The Senator Gravel Edition (Boston: Beacon Press, 1972), 548. 14Gilbert and Head, 84. 15Douglas Pike, PAVN: People’s Army

  10. Rao Quan Feasibility Study - Phase 1: Toxic Substances and Contamination (Final Report)

    Energy Technology Data Exchange (ETDEWEB)

    Berge, Dag; Knutzen, Jon


    Due to wartime activity in the Khe Sanh area, it was expected that damming of the river Rao Quan could wash out diffuse pollutants from the ground and from possible military dump sites. As discussed in this report, the rivers Rao Quan, Khe Nghi and Quang Tri contain relatively soft water. The buffer capacity against acidification is good. The sulphate concentration is low. The water will not be aggressive against concrete. The maximum particulate content in the wet season is low. The nutrient content of the rivers is relatively low. The phosphorus content correlated well with the turbidity, which indicates that the phosphorus is bound to particles. Khe Nghi has significantly higher nitrogen content than the other rivers, which may indicate some slight impact from animal husbandry. However, there was no eutrophication. The content of heavy metals is low and indicates no pollution from the military activities in the area. The water is well suited for irrigation. There was no indication from the analysed fish , the river sediments from Rao Quan or the top soil of the rice fields of any significant dioxin contamination. The content was similar to normal back ground levels caused by diffuse atmospheric fallout. The herbicide Agent Orange in 1962-1975 was applied mostly in the southernmost part of the country and contamination from it in the Quang Tri Province is not expected. 13 refs., 4 figs., 4 tabs.

  11. Solvation of Na+, K+, and Their Dimers in Helium


    An der Lan, Lukas; Bartl, Peter; Leidlmair, Christian; Jochum, Roland; Denifl, Stephan; Echt, Olof; Scheier, Paul


    Helium atoms bind strongly to alkali cations which, when embedded in liquid helium, form so-called snowballs. Calculations suggest that helium atoms in the first solvation layer of these snowballs form rigid structures and that their number (n) is well defined, especially for the lighter alkalis. However, experiments have so far failed to accurately determine values of n. We present high-resolution mass spectra of Na+He n , K+He n , Na2 +He n and K2 +He n , formed by electron ionization of do...

  12. Parameters: US Army War College Quarterly. Volume 20. Number 4 (United States)


    South Vietnam; and siege-breaking at Dien Bien Phu and Khe Sanh. In all of these cases, CAS substituted for a lack of available artillery assets, and...service persona . But is it in the Army’s interest to take over CAS, much less all of tactical aviation? No. The Army cannot afford the force structure...of a Former Iron Curtain Official." 10 (September, 1980), 8-15. Radvanyi, Janos. "Dien Bien Phu: Tiirt ’ears After." 15 (Summer, 1985), 63-68. Raho

  13. Histiocytoid hemangioma of bone: A benign lesion which may mimic angiosarcoma

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    Cone, R.O.; Nguyen, V.; Hudkins, P.; Merriwether, W.A.


    Histiocytoid hemangioma is a related family of endothelial cell neoplasms, which was described by Rosai at al. in 1979 and which encompasses a group of osseous, vascular and cutaneous neoplasms. These neoplasms bear a close resemblance to the angiosarcoma/hemangioendothelioma family of malignant neoplasms. A case of histiocytoid hemangioma is reported that presented as a multicentric osseous lesion and was initially diagnosed as angiosarcoma. The patient has had a remarkably benign course and at a later data developed skin and bone lesions diagnosed as angiolymphoid hyperplasia with eosinophilia identical to the initial lesion. This represents the first reported case of identical osseous and cutaneous lesions of the histiocytoid hemangioma group in the same patient and lends credence to Rosai's concept. A review of the literature concerning angiosarcoma/hemangioendothelioma of the bone is presented with the conclusion that some lesions considered to represent multicentric angiosarcoma may represent the more benign histiocytoid hemangioma.

  14. Stewart-Bluefarb acroangiodermatitis in a case of Parkes-Weber syndrome

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    Deepti H Ghia


    Full Text Available Stewart-Bluefarb acroangiodermatitis is the occurrence of pseudo-kaposiform changes with an underlying arterio-venous malformation. Parkes-Weber syndrome is a triad of arterio-venous malformation with varicose veins with bony and soft-tissue hypertrophy involving the extremity. A 13 year-old-female presented with ulcerated growth with bleeding episodes on right leg since past four years. A reddish discoloration over right leg was noticed at four years of age which remained unchanged until nine years of age, after which it showed rapid increase in size along with disproportionate increase in length and girth of right lower limb. Examination revealed hyperpigmented soft verrucous plaque over the right lower one-third of leg, along with presence of ulcers and dilated tortuous veins in the right lower leg with thrill and bruit over the right popliteal and inguinal region. A differential diagnosis of verrucous hemangioma and arterio-venous malformation with pseudo-kaposiform change was considered. Skin biopsy showed multiple fenestrated capillaries with perivascular lymphocyte infiltrate suggestive of capillary malformations. X-ray showed osteolytic defect in right tibia. Duplex ultrasound and magnetic resonance imaging of right leg showed multiple fast flowing small and medium sized arterio-venous malformations of small to moderate size. Thus, on the basis of clinical and radiological features, we made a diagnosis of Stewart-Bluefarb type of acroangiodermatitis with Parkes-Weber syndrome. She was managed conservatively with compression stockings.

  15. Bulky abdominal masses in pediatrics: iconographic essay; Massas abdominais volumosas em pediatria: ensaio iconografico

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    Reis, Fabiano; Faria, Andreia V.; Kluge, Patricia D.; Volpato, Ricardo G.; Santos, Sergio L.M. dos; Caserta, Nelson M.G. [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas]. E-mail:


    The ultrasound, computerized tomography and magnetic resonance findings of 19 patients with abdominal bulky masses diagnosed as hydronephrosis, Wilms' tumor, neuroblastoma, adrenal carcinoma, sarcoma, hemangioendothelioma, hepatoblastoma, mesenchymal hamartoma, hepatocellular carcinoma, choledochal cyst, splenic cyst, lymphoma, enteric cyst, teratoma, hydrometrocolpos and lipoma are presented. Imaging findings (including ultrasound, computerized tomography and magnetic resonance imaging) are important tools for the evaluation of abdominal masses in pediatric patients and can contribute to the diagnosis and evaluation of the extension of these diseases. (author)

  16. Multiple calcified large nodules or masses with calcifications: computed tomography findings; Massas e nodulos pulmonares multiplos com calcificacoes: aspectos na tomografia computadorizada

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    Marchiori, Edson [Universidade Federal Fluminense, RJ (Brazil). Dept. de Radiologia]. E-mail:; Damato, Simone; Koch, Melissa; Gasparetto, Emerson L. [Universidade Federal do Rio de Janeiro, RJ (Brazil); Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (Famerp), SP (Brazil). Hospital de Base; Escuissato, Dante L. [Parana Univ., Curitiba, PR (Brazil). Dept. de Radiologia; Nobre, Luiz Felipe [Santa Catarina Univ., Florianopolis, SC (Brazil); Zanetti, Glaucia [Faculdade de Medicina de Petropolis, RJ (Brazil)


    Objective: to describe the main tomographic patterns of the diseases that show multiple nodules or masses, with calcification. Material and method: we evaluated 31 patients showing multiple nodules or masses with calcification at computed tomography. Results: the diseases that cause this pattern were calcified metastases, amyloidosis, hyalinising granulomas, epithelioid hemangioendothelioma and the conglomerate masses of silicosis and talcosis. Conclusion: the identification of the pattern of is very important in the differential diagnostic of pulmonary multiple nodules or masses. (author)

  17. Engineer exchanging project on coal mine technology field in fiscal 1999. International information exchanging project (the overseas workshop in Vietnam); 1999 nendo gijutsusha koryu jigyo (tanko gijutsu bun'ya) kokusai koryu jigyo. Kaigai workshop (Vietnam)

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    Technical information exchange was performed with Vietnamese engineers related to coal mine technologies through the overseas workshop and the technical tour. At the same time, investigations were executed on the production technology levels and needs in Vietnam, and on possibilities of transfer of Japanese technologies. The 'Overseas workshop (Vietnam)' was held on September 28, 1999 with the 'trends and problems in coal production technologies' as the main theme. Lectures were given in the session related to coal production technologies by Vietnamese coal technology experts, each from VINACOAL and IMSAT, and by two Japanese coal technology experts. A comprehensive discussion was also executed, and items of technical information were exchanged on the coal production technologies. The number of persons actually participated in the workshop counted 58, with the proceedings presented by them distributed. The Mao Khe and Duong Huy coal mines in Quang Ninh Province were visited as the technical tour, whereas the coal mine sites were inspected and opinions were exchanged with the site engineers. Gas analysis was performed in the Mao Khe mine from the viewpoint of a gas control technology, and site investigation was carried out on lock bolts in the Duong Huy mine from the viewpoint of a roof control technology. (NEDO)

  18. Emission spectra of alkali-metal (K,Na,Li)-He exciplexes in cold helium gas (United States)

    Enomoto, K.; Hirano, K.; Kumakura, M.; Takahashi, Y.; Yabuzaki, T.


    We have observed emission spectra of excimers and exciplexes composed of a light alkali-metal atom in the first excited state and 4He atoms [K*Hen (n=1-6), Na*Hen (n=1-4), and Li*Hen (n=1,2)] in cryogenic He gas (the temperature 2 KK*He6. To confirm the assignment, we have also carried out ab initio calculation of adiabatic potential curves and peak positions of the emission spectra of the exciplexes.

  19. Alternative lengthening of telomeres phenotype in malignant vascular tumors is highly associated with loss of ATRX expression and is frequently observed in hepatic angiosarcomas. (United States)

    Liau, Jau-Yu; Tsai, Jia-Huei; Yang, Ching-Yao; Lee, Jen-Chieh; Liang, Cher-Wei; Hsu, Hung-Han; Jeng, Yung-Ming


    Alternative lengthening of telomeres (ALT) is a mechanism using homologous recombination to maintain telomere length and sustain limitless replicability of cancer cells. Recently, ALT has been found to be associated with inactivation of either α-thalassemia/mental retardation syndrome X-linked (ATRX) or death domain-associated (DAXX) protein. In this study, 119 tumors (88 angiosarcomas, 11 epithelioid hemangioendotheliomas, and 20 Kaposi sarcomas) were analyzed to determine the ALT status, its relationship to loss of ATRX/DAXX expression, and the clinicopathological features. In addition, the mutation status in the telomerase reverse transcriptase gene (TERT) promoter was also studied. Loss of ATRX expression was observed in 21% (16/77) of the primary angiosarcomas and 9% (1/11) of epithelioid hemangioendotheliomas. DAXX expression was intact in all but 2 ATRX-deficient angiosarcomas. Telomere-specific fluorescence in situ hybridization assay showed 28% (17/61) of the primary angiosarcomas were ALT positive. Remarkably, ALT was highly associated with loss of ATRX expression: all but 2 ALT-positive angiosarcomas were ATRX deficient. Notably, hepatic angiosarcomas were frequently ATRX deficient (8/13) and/or ALT positive (8/12). None of the secondary angiosarcomas were ATRX/DAXX deficient or ALT positive. The only ATRX-deficient epithelioid hemangioendothelioma was positive for ALT. Forty-seven angiosarcomas were tested for TERT promoter mutation. Despite the fact that angiosarcoma occurs most commonly in sun-damaged skin, mutation was detected in only 1 radiation-associated angiosarcoma (2%). We conclude that ALT is an important telomere maintenance mechanism in primary angiosarcomas. This feature is highly associated with loss of ATRX expression and is frequently observed in hepatic angiosarcomas.


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    Full Text Available ABSTRACT: INTRODUCTION : Retiform hemangioendothelioma is a rare variant of lo w - grade angiosarcoma with an indolent clinical behaviour, with predilection for young adults. Mostly is seen in the extremities, especially the distal lower limbs. CASE REPORT: An eighty year old lady, presented with complaints of a solitary, well defined swelling below the nape of the neck since two years. The swelling was clinically diagnosed as cavernous haemangioma. However the colour of the lesion s was looking like a melanocytic naevus. Excision was done under local anaesthesia and was sent for histopa tho lo gical examination. Histopathological diagnosis was confirmed as “Cavernous haemangioma with Retiform Haemangioendothelioma”

  1. Primary tumors of the patella. A review of 42 cases

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    Kransdorf, M.J. (Walter Reed Army Medical Center, Washington, DC (USA). Dept. of Radiology; University of the Health Sciences, Bethesda, MD (USA). Dept. of Radiology and Nuclear Medicine); Moser, R.P. Jr. (Armed Forces Inst. of Pathology, Washington, DC (USA). Dept. of Radiologic Pathology; University of the Health Sciences, Bethesda, MD (USA). Dept. of Radiology and Nuclear Medicine); Vinh, T.N. (Armed Forces Inst. of Pathology, Washington, DC (USA). Dept. of Orthopaedic Surgery); Aoki, J. (Armed Forces Inst. of Pathology, Washington, DC (USA). Dept. of Radiologic Pathology); Callaghan, J.J. (Duke Univ., Durham, NC (USA). Dept. of Orthopaedic Surgery)


    This study reports 42 cases of histologically proven and radiographically correlated primary patellar tumors. Despite diverse histologic diagnoses, the radiographic appearanaces of benign as opposed to malignant patellar neoplasms are essentially indistinguishable. Although the literature suggests that giant cell tumor is the most frequent benign tumor of the patella, the most common benign neoplasm in this series is chondroblastoma (16 cases). Only four primary malignant lesions were encountered, three cases of lymphoma and one case of hemangioendothelioma. Since 38 (90%) of the 42 cases were benign, a benign etiology should be strongly favored, notwithstanding the radiographic appearance, whenever a primary patellar tumor is encountered. (orig.).

  2. Contributions of Dr. Juan Rosai to the pathology of cutaneous vascular proliferations: A review of selected lesions. (United States)

    Wick, Mark R


    Vascular proliferations in the skin have been thoroughly studied over the past 35 years, and a great deal of knowledge has been accrued regarding their pathobiological features. Dr. Juan Rosai has been a consistent contributor to the literature on this topic throughout most of his career, and this article reviews selected cutaneous endothelial lesions that he helped to characterize. They include histiocytoid-epithelioid hemangioma, targetoid-hobnail hemangioma, acquired tufted hemangioma, glomeruloid hemangioma, spindle cell hemangioma, retiform hemangioendothelioma, and angiosarcoma. Two potential simulants of angiosarcoma-papillary intravascular endothelial hyperplasia and cutaneous rudimentary meningocele-meningothelial hamartoma-are also considered.

  3. An Unusual Case of Hepatic Tumor in an Elderly Patient

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    Huan-Lin Chen


    Full Text Available Malignant epithelioid hemangioendothelioma is a rare hepatic tumor of vascular origin. It is most commonly found in young to middle-aged women, and the tumors vary in their reported potential for malignancy. The etiologic factors are not yet clear, and some investigators have suggested an association with oral contraceptives, whereas others have noted an association with exposure to vinyl chloride, asbestos, thorotrast, major trauma to the liver, viral hepatitis, primary biliary cirrhosis, and alcohol consumption. The clinical manifestations are nonspecific, and most are asymptomatic. Among symptomatic patients, the most common symptom is right upper quadrant pain, followed by jaundice, weight loss, fatigue, ascites, hepatomegaly, and fever. The only definitive diagnosis requires immunohistochemical evidence of endothelial differentiation, which is demonstrated by the presence of factor VIII-related antigen and cytokeratins. As with most mesenchymal tumors, surgical resection is the most effective means of controlling local disease and preventing distant metastasis, although adjuvant therapies have been offered for patients with unresectable tumors or who are not transplant candidates. We present the case of an elderly man with a hepatic malignant epithelioid hemangioendothelioma, and we reviewed the English-language literature.

  4. Phase II Trial of Angiotensin-(1-7 for the Treatment of Patients with Metastatic Sarcoma

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    Paul D. Savage


    Full Text Available Background. Angiotensin-(1-7 [Ang-(1-7] is an endogenous antiangiogenic hormone with anticancer activity. In a phase I study of Ang-(1-7, two of three patients with metastatic sarcoma experienced disease stabilization. This phase II study examined clinical and biomarker outcomes for patients with metastatic sarcoma. Methods. Ang-(1-7 was administered by subcutaneous injection at a dose of 20 mg daily. If excessive toxicities occurred in the first cohort, a dose deescalation cohort was allowed. Blood samples were obtained to measure changes in biomarkers. Results. Treatment was well-tolerated and the dose deescalation cohort was not required. Plasma PlGF concentrations following treatment were not statistically significantly changed. A significant increase in plasma Ang-(1-7 was observed at 4 hours after injection. The median progression-free survival was 2.7 months (95% CI; 1.4 to 4.1 months, and the median overall survival was 10.2 months (95% CI; 5.3 to 18.3 months. Two patients with vascular sarcomas demonstrated prolonged disease stabilization of 10 months (hemangiopericytoma and 19 months (epithelioid hemangioendothelioma. Conclusions. Ang-(1-7 at a dose of 20 mg daily was well-tolerated. This prospective phase II study failed to confirm the PlGF biomarker effect identified in the prior phase I study. Prolonged disease stabilization in hemangiopericytoma and epithelioid hemangioendothelioma may warrant further investigation.

  5. Tumores vasculares como marcadores sindrómicos Vascular tumors as syndromic indicators

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    M. Hervella


    Full Text Available Los hemangiomas pueden ser marcadores o signos guía de síndromes graves. Aunque menos conocidos que los relacionados con malformaciones vasculares, existen algunos síndromes de importante valor diagnóstico que se asocian a hemangiomas. El reconocimiento precoz de los hemangiomas de alto riesgo, junto a una rápida actuación, puede ayudar a minimizar su morbilidad futura. Los hemangiomas en neonatos requieren un seguimiento especial, debido a que su patrón de crecimiento puede ser imprevisible a una edad tan temprana. Diversas presentaciones clínicas son relevantes por su riesgo de asociación sindrómica: los hemangiomas cervicofaciales, especialmente los de gran tamaño, pueden ser marcadores de trastornos dismórficos graves como el síndrome PHACE(S. Los que se distribuyen en el área de la barba se asocian en ocasiones a hemangiomas de la vía aérea. Los hemangiomas lumbosacros suelen ocultar un disrafismo espinal o malformaciones anorrectales y urogenitales. Los hemangiomas cutáneos múltiples pueden ser signo de una hemangiomatosis visceral, con más frecuencia hepática, que se complique con insuficiencia cardíaca o enfermedad tiroidea. Por último, existen dos neoplasias vasculares de crecimiento rápido e invasor, distintas del hemangioma del lactante (el hemangioendotelioma kaposiforme y el angioblastoma o hemangioma "en penachos", que son marcadores del síndrome de Kasabach-Merritt.Haemangiomas can be indicators or clue signs for serious syndromes. Although less well known than those related to vascular malformations, there are some syndromes of important diagnostic value that are associated with haemangomias. Early recognition of problematic haemangiomas, together with a prompt intervention, may help to minimise their future morbidity. Neonatal haemangiomas warrant a special follow-up, since their growth pattern might be unpredictable at such an early age. Several clinical presentations are relevant because of their risk

  6. Primary hepatic sarcomas: CT findings

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    Yu, Ri-Sheng; Chen, Ying; Jiang, Biao; Wang, Liu-Hong [Zhejiang University School of Medicine, Department of Radiology, Hangzhou (China); Xu, Xiu-Fang [Zhejiang Medical College, Teaching and Research Group of Radiology, Hangzhou (China)


    Primary hepatic sarcomas are rare tumors that are difficult to diagnose clinically. Different primary hepatic sarcomas may have different clinical, morphologic, and radiological features. In this pictorial review, we summarized computed tomography (CT) findings of some relatively common types of hepatic sarcomas, including angiosarcoma, epithelioid hemangioendothelioma (EHE), liposarcoma, undifferentiated embryonal sarcoma (UES), leiomyosarcoma, malignant fibrous histiocytoma (MFH), and carcinosarcoma (including cystadenocarcinosarcoma). To our knowledge, hepatic cystadenocarcinosarcoma has not been described in the English literature. The CT findings in our case are similar to that of cystadenocarcinoma, a huge, multilocular cystic mass with a large mural nodule and solid portion. The advent of CT has allowed earlier detection of primary hepatic sarcomas as well as more accurate diagnosis and characterization. In addition, we briefly discuss the MRI findings and diagnostic value of primary hepatic sarcomas. (orig.)

  7. Cutaneous epithelioid cell histiocytoma: immunohistochemical and ultrastructural findings suggesting endothelial origin. (United States)

    Manente, L; Schmitt, I; Onetti, A M; Peris, K; Caracciolo, E; Chimenti, S


    A 13-year-old boy presented with a polypoid nodule localized in the groin. Although the clinical and histopathological features corresponded to previously described diagnostic criteria of epithelioid cell histiocytoma, immunohistochemical and ultrastructural findings suggested vascular differentiation of the epithelioid cells. In particular, the immunohistochemical negativity of the epithelioid cell elements for Factor XIIIa failed to substantiate the previously forwarded hypothesis that this lesion represents a dendrocytoma. Instead, the presence of histiocytoid, vacuolated cells occasionally containing sparse red blood cells, positive staining for Factor VIII-related antigen, and ultrastructural evidence of endothelial characteristics in epithelioid neoplastic cells favor the hypothesis that "epithelioid cell histiocytoma" is of endothelial origin. We suggest the descriptive term cutaneous histiocytoid hemangioendothelioma for this lesion.

  8. Tumor diagnosis in the adult liver transplant candidate

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    Mahfouz, A.E. [Department of Radiology, Humboldt Univ. (Germany)]|[Department of Radiology, Cairo University Hospital, Cairo (Egypt); Vogl, T. [Department of Radiology, Humboldt Univ., Berlin (Germany).; Hamm, B. [Department of Radiology, Humboldt Univ. (Germany)


    Hepatic transplantation has emerged as a potentially curative treatment of certain malignant hepatic neoplasms such as hepatocellular carcinoma, bile duct carcinoma, fibrolamellar hepatocellular carcinoma, metastases from neuroendocrine tumors, and epithelioid hemangioendothelioma. In the early years of hepatic transplantation, there was great enthusiasm to cure patients with unresectable hepatobiliary malignancy. This early enthusiasm was tempered by the unfavorable outcome of transplantation in advanced cases of malignancy and the organ-donor shortage. Presently, patients have to be selected with predictable likelihood for long-term survival. Pre-transplantation imaging is indispensable for detection, characterization, staging, and surgical road-mapping before the procedure. The present article focuses on the role of imaging modalities in these different aspects of preoperative assessment. (orig.) With 12 figs., 2 tabs., 66 refs.

  9. Angiosarcoma of the pericardium: a case report. (United States)

    Ma, Guo-Tao; Liu, Jian-Zhou; Miao, Qi; Cui, Quan-Cai


    Primary malignant tumors of the pericardium are rare, and most primary malignant pericardium tumors are mesotheliomas. Primary pericardial angiosarcoma is extremely rare, and it is associated with a poor prognosis. We report of a 47-year-old woman who complained of activity-related chest tightness and shortness of breath. Computed tomography, magnetic resonance imaging, and transesophageal echocardiography revealed an enlarged pericardium with hematic and solid components. An exploratory pericardiotomy was performed, and the results of the histological examination were suggestive of spindle cell hemangioendothelioma. She survived for 9 months after surgery without chemotherapy and radiotherapy, and she had a relatively good quality of life. Primary pericardial angiosarcoma is difficult to diagnose, and it has a poor prognosis. Pericardiotomy, radiation therapy, and chemotherapy were associated with a prolongation of survival.

  10. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma (United States)


    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Unclassified Pleomorphic Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult Unclassified Pleomorphic Sarcoma of Bone; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Corpus Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Unclassified Pleomorphic Sarcoma of Bone

  11. Complicaciones en la evolución de los hemangiomas y malformaciones vasculares Complications in the evolution of haemangiomas and vascular malformations

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    A. Belzunce


    Full Text Available La diferenciación de los hemangiomas y las malformaciones vasculares es histológica, clínica y pronóstica. Aunque la mayoría de los hemangiomas evolucionan a la resolución espontanea, pueden complicarse hasta en un 10% de los casos con ulceración, dolor y hemorragia. Además, la propia localización de los hemangiomas en la cabeza y cuello, junto a estructuras vitales pueden comprometer sus funciones. Así pues, la compresión de vía aérea puede ser una urgencia vital. Los hemangiomas periorbitarios pueden producir ambliopía por deprivación sensorial o por un estrabismo restrictivo. Los hemangiomas lumbosacros deben estudiarse con RMN por su frecuente asociación con alteraciones de la línea media a nivel espinal, anal, genital o renal. Entre los hemangiomas viscerales, los hepáticos son los más graves por su asociación con insuficiencia cardiaca congestiva. La asociación de hemangiomas faciales extensos con anomalías del SNC, anomalías vasculares, cardiacas, oculares y esternales se denomina síndrome PHACE y con frecuencia se complica con deficiencia mental, convulsiones o ictus. Las malformaciones vasculares de localización trigeminal se asocian hasta en un 15% con glaucoma o hemangiomas coroideos o leptomeníngeos (síndrome de Sturge-Weber. Las malformaciones vasculares combinadas localizadas en extremidades pueden complicarse con tromboflebitis, osteolisis regional e incluso tromboembolismos a distancia (Síndrome de Klippel-Treneaunay . Por otro lado, existe una coagulopatía por consumo (Síndrome de Kassabach-Merrit que puede complicar algunos tumores vasculares como el hemangiomatoendotelioma kaposiforme y el angioma en penacho. Se revisan por último las complicaciones de los tratamientos empleados.The differentiation of haemangiomas and vascular malformations is histological, clinical and prognostic. Although the majority of haemangiomas evolve towards spontaneous resolution, as many as 10% of cases can develop

  12. Treatment of bone pain secondary to metastases using samarium-153-EDTMP

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    Elba Cristina Sá de Camargo Etchebehere

    Full Text Available CONTEXT: More than 50% of patients with prostate, breast or lung cancer will develop painful bone metastases. The purpose of treating bone metastases is to relieve pain, reduce the use of steroids and to maintain motion. OBJECTIVE: To evaluate the use of samarium-153-EDTMP (153Sm-EDTMP for the treatment of bone pain secondary to metastases that is refractory to clinical management. TYPE OF STUDY: Retrospective. SETTING: Division of Nuclear Medicine, Universidade Estadual de Campinas (Unicamp. METHODS: Fifty-eight patients were studied (34 males with mean age 62 years; 31 patients had prostate cancer, 20 had breast cancer, three had lung cancer, one had lung hemangioendothelioma, one had parathyroid adenocarcinoma, one had osteosarcoma and one had an unknown primary tumor. All patients had multiple bone metastases demonstrated by bone scintigraphy using 99mTc-MDP,and were treated with 153Sm-EDTMP. Response to treatment was graded as good (pain reduction of 50-100%, intermediate (25-49% and poor (0-24%. RESULTS: All patients showed good uptake of 153Sm-EDTMP by bone metastases. Among the patients with prostate cancer, intermediate or good response to therapy occurred in 80.6% (25 patients and poor response in 19.4% (6. Among the patients with breast cancer, 85% (17 showed intermediate or good response to therapy while 15% (3 showed poor response. All three patients with lung cancer showed poor response to treatment. The lung hemangioendothelioma and unknown primary lesion patients showed intermediate response to treatment; the osteosarcoma and parathyroid adenocarcinoma patients showed good response to treatment. No significant myelotoxicity occurred. DISCUSSION: Pain control is important for improving the quality of life of patients with advanced cancers. The mechanism by which pain is relieved with the use of radionuclides is still not yet completely understood, however, the treatment is simple and provides a low risk of mielotoxicity

  13. Consumptive Coagulopathy in Angiosarcoma: A Recurrent Phenomenon?

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    Mohamad Farid


    Full Text Available Objectives. To report the prevalence of consumptive coagulopathy in angiosarcoma patients seen at a single center. Methods. We retrospectively reviewed case records of 42 patients diagnosed with angiosarcoma at Mount Sinai Hospital between 2000 and 2013. Results. Seven patients (17% met clinical criteria for disseminated intravascular coagulation (DIC in absence of concomitant clinical states known to cause coagulopathy or myelosuppression. In all patients who received systemic antineoplastic therapy with resultant disease response or stability, DIC resolved in tandem with clinical improvement. DIC recurred at time of disease progression in all cases. Two patients had bulky disease, defined as diameter of largest single or contiguous tumor mass measuring 5 cm or more. All patients demonstrated an aggressive clinical course with short duration of disease control and demise within 1 year. In contrast, evaluation over the same period of 17 epithelioid hemangioendothelioma patients serving as a clinical control group revealed no evidence of DIC. Conclusion. Angiosarcomas can be associated with a consumptive coagulopathy arising in tandem with disease activity. Vigilance for this complication will be needed in the course of often aggressive multimodality therapy. The potential utility of coagulopathy as a prognostic biomarker will need to be explored in future studies.

  14. Ultrasound-guided core needle biopsy in diagnosis of abdominal and pelvic neoplasm in pediatric patients. (United States)

    Wang, Hailing; Li, Fangxuan; Liu, Juntian; Zhang, Sheng


    Ultrasound-guided core needle biopsy of abdominal and pelvic masses in adults has gained tremendous popularity. However, the application of the same treatment in children is not as popular because of apprehensions regarding inadequate tissues for the biopsy and accidental puncture of vital organs. Data of the application of ultrasound-guided core needle biopsy in 105 pediatric patients with clinically or ultrasound-diagnosed abdominopelvic masses were reviewed. Diagnostic procedures were conducted in our institution from May 2011 to May 2013. The biopsies were conducted on 86 malignant lesions and 19 benign lesions. 86 malignant tumors comprised neuroblastomas (30 cases), hepatoblastomas (15 cases), nephroblastomas (11 cases), and primitive neuroectodermal tumors/malignant small round cells (6 cases). Among malignant tumor cases, only a pelvic primitive neuroectodermal tumor did not receive a pathological diagnosis. Therefore, the biopsy accuracy was 98.8 % in malignant tumor. However, the biopsies for one neuroblastomas and one malignant small round cell tumor were inadequate for cytogenetic analysis. Therefore, 96.5 % of the malignant tumor patients received complete diagnosis via biopsy. 19 benign tumors comprised mature teratoma (10 cases), hemangioendothelioma (3 cases), paraganglioma (2 cases), and infection (2 cases). The diagnostic accuracy for benign neoplasm was 100 %. Five patients experienced postoperative complications, including pain (2 patients), bleeding from the biopsy site (2 patients), and wound infection (1 patient). Ultrasound-guided core needle biopsy is an efficient, minimally invasive, accurate, and safe diagnostic method that can be applied in the management of abdominal or pelvic mass of pediatric patients.

  15. Vascular bone tumors: a proposal of a classification based on clinicopathological, radiographic and genetic features

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    Errani, Costantino [Istituto Ortopedico Rizzoli, Ortopedia Generale, Orthopaedic Service, Bagheria (Italy); Struttura Complessa Ortopedia Generale, Dipartimento Rizzoli-Sicilia, Bagheria, PA (Italy); Vanel, Daniel; Gambarotti, Marco; Alberghini, Marco [Istituto Ortopedico Rizzoli, Pathology Service, Bologna (Italy); Picci, Piero [Istituto Ortopedico Rizzoli, Laboratory for Cancer Research, Bologna (Italy); Faldini, Cesare [Istituto Ortopedico Rizzoli, Ortopedia Generale, Orthopaedic Service, Bagheria (Italy)


    The classification of vascular bone tumors remains challenging, with considerable morphological overlap spanning across benign to malignant categories. The vast majority of both benign and malignant vascular tumors are readily diagnosed based on their characteristic histological features, such as the formation of vascular spaces and the expression of endothelial markers. However, some vascular tumors have atypical histological features, such as a solid growth pattern, epithelioid change, or spindle cell morphology, which complicates their diagnosis. Pathologically, these tumors are remarkably similar, which makes differentiating them from each other very difficult. For this rare subset of vascular bone tumors, there remains considerable controversy with regard to the terminology and the classification that should be used. Moreover, one of the most confusing issues related to vascular bone tumors is the myriad of names that are used to describe them. Because the clinical behavior and, consequently, treatment and prognosis of vascular bone tumors can vary significantly, it is important to effectively and accurately distinguish them from each other. Upon review of the nomenclature and the characteristic clinicopathological, radiographic and genetic features of vascular bone tumors, we propose a classification scheme that includes hemangioma, hemangioendothelioma, angiosarcoma, and their epithelioid variants. (orig.)

  16. The association of consumptive hypothyroidism secondary to hepatic hemangioma and severe heart failure in infancy. (United States)

    Emir, Suna; Ekici, Filiz; İkiz, Mehmet Alper; Vidinlisan, Sadi


    Although hemangiomas are the most common vascular tumors of the liver in infancy, data regarding hypothyroidism and heart failure related to hepatic hemangiomas are limited. Here, we present a 15- day -old girl who presented with prolonged jaundice at the age of 15 days. Because her TSH level was found to be 74 μIU/mL, she was initially diagnosed with congenital hypothyroidism and L-Thyroxine replacement therapy was initiated. On follow-up examination performed two months later, it was observed that her TSH level was not suppressed and a mass was noticed in the right upper abdomen on physical examination. Abdominal ultrasonography revealed multiple masses with sizes of about 3-3,5 cm covering the whole liver. When evaluated with clinical and radiological appearance, oral methylprednisolone at a dose of 5 mg/kg/day and propranolol at a dose of 2 mg/kg were initiated with a diagnosis of hepatic hemagioma/hemangioendothelioma. Consumptive hypothyroidism due to hepatic hemangioma and congestive heart failure were considered in the patient who had findings of heart failure. The dose of L-Thyroxine was increased 2-fold. The patient received intensive care treatment for severe heart failure. Because his findings resolved, he was started to be followed up with propranolol, steroid and L-Thyroxine treatment.

  17. August 2012 Arizona thoracic society notes

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    Robbins RA


    Full Text Available No abstract available. Article truncated at 150 words. A dinner meeting was held on 8/29/2012 at Scottsdale Shea beginning at 6:30 PM. There were 23 in attendance representing the pulmonary, critical care, sleep, pathology, radiology, and thoracic surgery communities. Four cases were presented:1.Lewis Wesselius and Thomas Colby presented a 39 yo female with cough and small amounts of hemoptysis for over a year. Chest x-ray was interpreted as perhaps showing some small nodules in the lower lobes which were more easily seen with CT scan. The scattered nodules were lower lobe predominant, non-calcified and surrounded by ground glass haloes. Coccidioidomycosis serology was negative and rheumatologic serologies were negative. Bronchoscopy showed blood in the airway but other than blood, bronchoalveolar lavage was negative. A video-assisted thorascopic (VATS biopsy showed a hemangioendothelioma, a malignant neoplasm that falls between a hemangioma and angiosarcoma. These vascular tumors can originate in the heart and often metastasize to the lung and pleura…

  18. Pediatric multifocal liver lesions evaluated by MRI

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    Majed Almotairi


    Full Text Available Objective: The purpose of this study is to present our experience with MRI evaluation of multifocal liver lesions in children and describe the MRI characteristics of these lesions. Patients and Methods: A retrospective review of consecutive MRI exams performed for the evaluation of multiple liver lesions between 1 January 2007 and 31 December 2012 was done to note the number of lesions, the size of the largest lesion, MR signal characteristics, and background liver. Final diagnosis was assigned to each case based on pathology in the available cases and a combination of clinical features, imaging features, and follow-up in the remaining cases. Results: A total of 48 children (22 boys, 26 girls; age between 3 months and 18 years with average age 10.58 years and median age 11 years were included in the study. Totally 51 lesion diagnoses were seen in 48 children that included 17 focal nodular hyperplasia (FNH, 8 hemangiomas, 7 metastases, 6 regenerative nodules, 3 adenomas, 3 abscesses, and one each of angiomyolipoma, epithelioid hemangioendothelioma, focal fatty infiltration, hepatocellular carcinoma, hepatic infarction, nodular regenerative hyperplasia, and hepatic cyst. Background liver was normal in 33, cirrhotic in 10, fatty in 3, and siderotic in 2 children. Most FNH, hemangiomas, and regenerative nodules showed characteristic MRI features, while metastases were variable in signal pattern. Conclusion: Many commonly seen multifocal liver lesions in children have characteristic MRI features. MRI can help to arrive at reasonable differential diagnoses for multifocal liver lesions in children and guide further investigation and management.

  19. Imaging tumors of the patella

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    Casadei, R., E-mail: [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Kreshak, J., E-mail: [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rinaldi, R. [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rimondi, E., E-mail: [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Bianchi, G., E-mail: [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Alberghini, M., E-mail: [Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Ruggieri, P. [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Vanel, D., E-mail: [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy)


    Background: Patellar tumors are rare; only a few series have been described in the literature and radiographic diagnosis can be challenging. We reviewed all patellar tumors at one institution and reviewed the literature. Materials and methods: In an evaluation of the database at one institution from 1916 to 2009, 23,000 bone tumors were found. Of these, 41 involved the patella. All had imaging studies and microscopic diagnostic confirmation. All medical records, imaging studies, and pathology were reviewed. Results: There were 15 females and 26 males, ranging from 8 to 68 years old (average 30). There were 30 benign tumors; eight giant cell tumors, eight chondroblastomas, seven osteoid osteomas, two aneurysmal bone cysts, two ganglions, one each of chondroma, exostosis, and hemangioma. There were 11 malignant tumors: five hemangioendotheliomas, three metastases, one lymphoma, one plasmacytoma, and one angiosarcoma. Conclusion: Patellar tumors are rare and usually benign. As the patella is an apophysis, the most frequent lesions are giant cell tumor in the adult and chondroblastoma in children. Osteoid osteomas were frequent in our series and easily diagnosed. Metastases are the most frequent malignant diagnoses in the literature; in our series malignant vascular tumors were more common. These lesions are often easily analyzed on radiographs. CT and MR define better the cortex, soft tissue extension, and fluid levels. This study presents the imaging patterns of the more common patellar tumors in order to help the radiologist when confronted with a lesion in this location.

  20. Analysis and Optimization on Configuration Scheme of New High Efficient 220 MW Subcritical Turbine%高效新型220MW亚临界汽轮机优化选型分析

    Institute of Scientific and Technical Information of China (English)



    Based on the demand of EPC contract for MAO KHE 2 ×220 MW thermal power plant project in VIETNAM, after analy-zing the technology and economy in detail, comparing with the different characters between 200 MW superhigh pressure turbine and 300 MW subcritical turbine, the subcritical parameters were introduced in the 220 MW turbine.By Optimizing blade type, reducing seal leakage and improving the efficiency of the turbine cylinder, the heat consumption rate decreased to 8 026 kJ/kWh.After the tur-bine operating successfully, each unit can save standard coal equivalent 10 161.5 ton per year.Therefore, it will improve the econo-my of power plant and create a greater economic benefits.Furthermore, it can provide a good reference for choosing high efficient turbine in the same class power unit.%以越南某2×220 MW火力发电厂工程的设计、采购、施工( EPC )合同要求为出发点,从技术、能耗方面进行分析,根据国内200 MW等级超高压汽轮机和300 MW等级亚临界汽轮机的特点,通过分析比较两者的差别,提出了220 MW等级汽轮机可采用亚临界参数方案,通过优化叶片型式,减少轴封漏气,提高汽轮机汽缸效率,汽轮机热耗降低至8026 kJ/kWh,并最终在工程中顺利实施。机组投运后,每年可节约10161.5 t标煤,提高了机组的经济性,创造了较大的经济效益,可为后续同类项目的汽轮机选型提供参考。

  1. Vectors and malaria transmission in deforested, rural communities in north-central Vietnam

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    Do Manh Cuong


    Full Text Available Abstract Background Malaria is still prevalent in rural communities of central Vietnam even though, due to deforestation, the primary vector Anopheles dirus is uncommon. In these situations little is known about the secondary vectors which are responsible for maintaining transmission. Basic information on the identification of the species in these rural communities is required so that transmission parameters, such as ecology, behaviour and vectorial status can be assigned to the appropriate species. Methods In two rural villages - Khe Ngang and Hang Chuon - in Truong Xuan Commune, Quang Binh Province, north central Vietnam, a series of longitudinal entomological surveys were conducted during the wet and dry seasons from 2003 - 2007. In these surveys anopheline mosquitoes were collected in human landing catches, paired human and animal bait collections, and from larval surveys. Specimens belonging to species complexes were identified by PCR and sequence analysis, incrimination of vectors was by detection of circumsporozoite protein using an enzyme-linked immunosorbent assay. Results Over 80% of the anopheline fauna was made up of Anopheles sinensis, Anopheles aconitus, Anopheles harrisoni, Anopheles maculatus, Anopheles sawadwongporni, and Anopheles philippinensis. PCR and sequence analysis resolved identification issues in the Funestus Group, Maculatus Group, Hyrcanus Group and Dirus Complex. Most species were zoophilic and while all species could be collected biting humans significantly higher densities were attracted to cattle and buffalo. Anopheles dirus was the most anthropophilic species but was uncommon making up only 1.24% of all anophelines collected. Anopheles sinensis, An. aconitus, An. harrisoni, An. maculatus, An. sawadwongporni, Anopheles peditaeniatus and An. philippinensis were all found positive for circumsporozoite protein. Heterogeneity in oviposition site preference between species enabled vector densities to be high in both

  2. Primary Thyroid Sarcoma: A Systematic Review. (United States)

    Surov, Alexey; Gottschling, Sebastian; Wienke, Andreas; Meyer, Hans Jonas; Spielmann, Rolf Peter; Dralle, Henning


    Different types of malignant tumors can occur within the thyroid. Primary cancer is the most common type of thyroid malignancy. Non-epithelial malignancies can also arise within the thyroid. The aim of the present study was to analyze clinical and radiological characteristics of reported primary thyroid sarcomas (PTS), based on a large sample of cases. The PubMed database was screened for articles from between 1990 and 2014. Overall, 86 articles with 142 patients were identified. Ultrasound evaluation was reported for 36 patients. Data regarding computed tomography of the neck were available for 29 cases. Magnetic resonance imaging was performed for eight patients. The following data were retrieved for the identified sarcomas: localization, size, homogeneity, internal texture, and margin characteristics. In most cases, PTS occurred in patients over 40 years of age, with a peak incidence for the group aged 60-79 years. Angiosarcoma was diagnosed in 29 cases (20.4%), followed by malignant hemangioendothelioma (n=23, 16.3%), malignant fibrous histiocytoma (n=20, 14.1%), leiomyosarcoma (n=16, 11.3%), and fibrosarcoma (n=13, 9.2%). In most patients (n=113, 79.6%), PTS manifested clinically as a painless goiter. On ultrasound, PTS were predominantly mixed hypo-to-hyperechoic in comparison to the normal thyroid tissue. On non-contrast computed tomography, most sarcomas were inhomogeneous hypo-to-hyperdense. On post-contrast magnetic resonance images, most sarcomas showed marked non-homogenous enhancement. In 26.8%, infiltration of the adjacent organs was seen. The trachea or esophagus was affected more frequently in patients with malignant histiocytoma and liposarcoma. Different strategies were used in the treatment of PTS. Our analysis provides clinical and radiological characteristics of PTS. The described features should be taken into consideration in the differential diagnosis of thyroid tumors.

  3. Tumors of the liver; a ten year study in Children Medical Center

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    Farahmand F


    Full Text Available Background: The aim of this study was to review the frequency, histopathology and outcome in children with tumors of the liver. Methods: Included in this retrospective/descriptive study were 30 children treated for liver tumors from 1375-1384 (ca. 1996-2005, at Children’s Hospital Medical Center, Tehran, Iran. We included the clinical, radiologic, and pathologic data of our patients, focusing on the frequency, etiology and outcome. Results: Patient ages ranged from three months to 12 years (median 3.8 years, with 18 males (60% and 12 females (40%. Of these, 17 patients had hepatoblastoma (55.66%, including 13 males and four females, with an age range of six months to five years. Four cases (13.33% had neuroblastoma. Hepatocellular carcinoma (HCC was found in three cases (10%, all of whom were carriers of hepatitis B. Two cases (6.66% were diagnosed with mesenchymal hamartoma, two cases (6.66% with hemangioendothelioma and two cases (6.66% with rhabdomyosarcoma and leiomyosarcoma of the biliary tract. Abdominal swelling and hepatomegaly were seen in all of patients. Jaundice was observed in two cases. Serum alpha-fetoprotein levels greater than 500 ng/ml were seen in 17 cases (56.66%. All patients were receiving specific treatment. The three-year survival rate was 65% for hepatoblastoma and 2% for HCC Conclusion: With the introduction of specific treatment, the survival rate for children with tumors of the liver has significantly increased. Further improvement can be achieved using diagnostic biopsy for hepatoblastoma, although it may result in complications, and preoperative chemotherapy followed by complete surgical excision (per International Society of Pediatric Oncology guidelines, yielding an outstanding survival rate of 80%.

  4. Uncommon liver tumors (United States)

    Wu, Chia-Hung; Chiu, Nai-Chi; Yeh, Yi-Chen; Kuo, Yu; Yu, Sz-Shian; Weng, Ching-Yao; Liu, Chien-An; Chou, Yi-Hong; Chiou, Yi-You


    hemangioendothelioma and inflammatory pseudotumor-like follicular dendritic cell sarcoma, are also discussed. Hepatoblastoma and mesenchymal hamartoma, mostly in children, are also briefly reviewed as well. Conclusion: It is important for radiologists to be familiar with the typical imaging features of the uncommon hepatic neoplasms. If imaging findings are not typical or diagnostic, further biopsy is required. PMID:27684838

  5. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis. (United States)

    Kollár, A; Jones, R L; Stacchiotti, S; Gelderblom, H; Guida, M; Grignani, G; Steeghs, N; Safwat, A; Katz, D; Duffaud, F; Sleijfer, S; van der Graaf, W T; Touati, N; Litière, S; Marreaud, S; Gronchi, A; Kasper, B


    Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited. The main objective of this study was to investigate the activity of pazopanib in vascular sarcomas. A retrospective study of patients with advanced vascular sarcomas, including angiosarcoma (AS), epithelioid hemangioendothelioma (HE) and intimal sarcoma (IS) treated with pazopanib in real life practice at EORTC centers as well as patients treated within the EORTC phase II and III clinical trials (62043/62072) was performed. Patient and tumor characteristics were collected. Response was assessed according to RECIST 1.1. and survival analysis was performed. Fifty-two patients were identified, 40 (76.9%), 10 (19.2%) and two (3.8%) with AS, HE and IS, respectively. The response rate was eight (20%), two (20%) and two (100%) in the AS, HE and IS subtypes, respectively. There was no significant difference in response rate between cutaneous and non-cutaneous AS and similarly between radiation-associated and non-radiation-associated AS. Median progression-free survival (PFS) and median overall survival (OS; from commencing pazopanib) were three months (95% CI 2.1-4.4) and 9.9 months (95% CI 6.5-11.3) in AS, respectively. The activity of pazopanib in AS is comparable to its reported activity in other STS subtypes. In this study, the activity of pazopanib was similar in cutaneous/non-cutaneous and in radiation/non-radiation-associated AS. In addition, pazopanib showed promising activity in HE and IS, worthy of further evaluation.

  6. 脾脏硬化性血管瘤样结节性转化%Sclerosing angiomatoid nodular transformation of spleen

    Institute of Scientific and Technical Information of China (English)

    付琼; 滕晓东


    Sclerosing angiomatoid nodular transformation (SANT) is a rarely encountered benign spleen lesion which has recently been gaining recognition. However,the actual pathogenesis of this disease remains unclear.SANT's remarkably characteristic appearance consists grossly of multiple well-circumscribed vascular/angiomatoid nodules of various sizes in a fibrosclerotic stroma. Immunohistochemical studies display a heterogeneous staining pattern of endothelial phenotypes in the angiomatoid nodules,with some cells resembling splenic sinusoids (CD34 -/CD31 +/CD8 + ),capillaries (CD34 +/CD31 +/CD8 - ) and small veins (CD34 -/CD31+/CD8-).Due to the rarity of this disease,it is often mistaken for splenic hamartoma,inflammatory pseudotumor,littoral cell angima or hemangioendothelioma. So far,SANT exhibits a benign clinical course because splenectomy can be curative.In this review,we outline the clinical and pathologic features of SANT and discuss its probable disease mechanism to provide a holistic overview of the disease at this time.%脾脏硬化性血管瘤样结节性转化( sclerosing angiomatoid nodular transformation,SANT)是一种少见、发病原因尚不清楚的脾脏良性病变.SANT具有独特的病理形态特点:境界清楚的实性肿块,多个血管瘤样结节为纤维硬化间质所包绕;血管瘤样区域对血管免疫标记显示出异质性;手术切除即可治愈.临床易将其误诊为其他类型肿瘤.通过复习SANT现有中、英文文献,从命名、临床及病理形态学特征、鉴别诊断、发病机制以及治疗与预后等方面总结现阶段对该病变的认识.

  7. Hepatectomia parcial em cirurgia pediátrica Partial hepatectomy in pediatric surgery

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    Roberto Oliveira Cardoso dos Santos


    . METHODS: A retrospective review of clinical and surgical data of 12 patients submitted to hepatic lobectomy, at Pediatric Surgery Service of Hospital das Clinicas da Faculdade de São Paulo and Hospital Santa Lydia, Ribeirão Preto (Brazil, between 1985 and 1995. RESULTS: There were eight left lobectomies and four right lobectomies. The indication of surgery were neoplastic diseases in 10 children, and complicated hepatic trauma in two. The age range was six days to 16 months in children with hepatic tumors, and the mean age was 3,8 months. Histopathological diagnosis were hemangioendothelioma in five (50%, hepatic adenoma in two (20%, hepatoblastoma in two (20%, and hepatic carcinoma in one (10%. Mean tumor weight was 7,1 % of body weight. Mean surgical time was 2h 58m. Postoperative follow-up was up to 141 months, and mean follow-up was 76,5 months. Seven patients received intraoperative blood transfusion, corresponding to 23,3 % of volemy. Only one patient had a late reoperation for resection of a new tumor. There were no postoperative complications. CONCLUSION: Although hepatic lobectomy is a major surgery, and undoubtedly technically difficult, it can be safely performed without expensive devices and sophisticated methods (some of them not always available in common hospitals if the surgeon is skilled and well prepared for such a procedure.

  8. Hemangioendotelioma Epitelioide Pulmonar: reporte de un caso

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    Victoria Monterroso-Azofeifa


    granulomas to clearly developed sarcomas. Eventually the tumor was found to be of vascular origin, and was called epithelioid hemangio endothelioma (EHE, later on it was also reported in other anatomical regions. We describe here, as far as we could find, the first costarican case of this particular neoplasia. A 42 year old male was referred to us with disseminated metastatic disease. He had been seen 16 years prior with asymptomatic, bilateral, small lung nodules. At that time he underwent a lung biopsy and the diagnosis of epitheliod hemangioendothelioma was made. He had remained stable, on no therapy, until his current and final presentation. We elected to biopsy his lung again to verify the histology and to see weather the tumor had undergone differentiation. He did not respond to chemotherapy and died shortly thereafter. The original lung biopsy material was compared to the recent one, finding much more cellularity and atypiae in the latter one. Immuno-histochemistry studies were carried out on the tissue samples and the endothelial origin of the tumor was demonstrated. EHE is an unusual type of neoplasia, it grows very slowly and has low metastatic potential in spite of its vascular origin, interestingly some authors have shown a hormonal dependency and this needs to be explored to see if therapeutic applications can be developed.

  9. Total sacrectomy via posterior approach for malignant sacral tumors%单纯后路全骶骨切除治疗骶骨恶性肿瘤

    Institute of Scientific and Technical Information of China (English)

    孙伟; 陈泉池; 马小军; 华莹奇; 蔡郑东


    high level (S1,S2).Methods 5 cases of primary malignant sacral tumors treated by total sacrectomy via posterior approach and iliolumbar reconstruction from March 2010 to March 2011 were analyzed retrospectively.There were 3 males and 2 females.The mean age was 41,ranging from 32 to 55.The imageology examination showed osteolytic destruction,among which there were 4 cases of obvious soft tissue mass,1 case of obvious sacral foramina expansion with bone damage.MRI showed 1 case of the tumors in S1-S5,1 case of the tumors in S1 、S2,1 case of the tumors in S1-S3.The smallest tumor was 9.2 cm×7.6 cm×4.1 cm,while the largest was 22.0 cm× 19.0 cm× 16.0 cm.Preoperative TTNB were done on the 5 patients.Pathological diagnosis:2 cases of chordoma,1 case of malignant neurilemmoma,1 case of chondrosarcoma and 1 case of malignant hemangioendothelioma.5 patients had sacrococcygeal pain or lumbocrural pain before the surgery.Visual analogue scale (VAS):2 cases of 2,2 cases of 6 and 1 case of 8.Ilium stability reconstructions were all performed on 5 patients with spine pedicle screw-rod system.Results The mean operation time was 6.5 hours (range,4.5-11 hours),with the mean intraoperative blood loss of 3 700 ml (range,2 000-7 200 ml).There was no perioperative death.The mean follow-up time was 17 months (range,9-23 months).There were 2 cases of wound complications 2 weeks after surgery and healed by second intention with washing and drainage after debridement.There was no deep infection.1 case of rectal injury,which was performed with colostomy during the operation,and stoma returned 12 weeks after surgery.4 cases of sciatic nerve symptom of lower limbs and plantar flexion dyskinesia after bilateral S1 nerve roots resection.The patients walked with ankle brace fixed after the surgery.There was 1 case of implant breakage and no obvious spine down.5 patients had functional disability in sphincter after surgery.1 case of hemangioendothelioma recurred locally 9 months later

  10. EDITORIAL: From reciprocal space to real space in surface science From reciprocal space to real space in surface science (United States)

    Bartels, Ludwig; Ernst, Karl-Heinz


    cover image of this issue honors this achievement. It shows 27 chiral heptahelicene molecules arranged by one of the guest editors (KHE) with the very same STM at IBM Almaden Research Center, San José that was used for moving xenon atoms more than 20 years ago. Karl-Heinz Rieder's career, as briefly outlined in the biography by G Benedek in this issue, reflected this paradigm shift in surface science from reciprocal to real space investigations: initially focusing on helium scattering as a superior technique for the investigation of delicate surfaces, his group at the Freie University in Berlin became the second in the world to rearrange atoms and molecules at surfaces in a controlled way and they generated much of the foundational work of low temperature STM investigations. Besides his saxophone skills and many other hobbies, Karl-Heinz is a real poet and loves composing funny limericks and spoonerisms. He wrote the following poem (passed on to us by W-D Schneider) at the International School of Solid State Physics in 1998 in Erice, Italy. It is a real masterpiece, beautifully summarizing us all, the 'Surface Science Society'. The top atom sheetSome become real slaves is important and neat.looking at standing waves. Therefore there is a strong raceThe producers of coatings in the science of surface.get really good quotings. To clean it you needStill others use new ways ions and heat.with electrons and x-rays. Then the atoms arrangeAnd all has to be in some ways that are strange,in good UHV—except theory, they relax, reconstructwhich thinks on extensions as a matter of still lower dimensions, And reacting with gasesand with lots of computing they form quite new phasestries to get solid footing. with large unit cellsSo everybody with joy as diffraction tells.plays his own special toy. Some people use lightAnd all think they are better from a synchrotron brightshould get published a letter. and claim it is bestBut before there's more cluster to do this in

  11. 骨血管源性恶性肿瘤的临床分析%Clinical analysis of malignant bone vascular tumors

    Institute of Scientific and Technical Information of China (English)

    燕太强; 李晓; 沈丹华; 董森; 杨荣利; 郭卫


    Objective To retrospectively analyze the diagnosis, treatment and clinical outcomes of malignant tumors of blood vessels in bone. Methods From October 1998 to November 2008, 7 patients were diagnosed as malignant vascular tumors of bone, which include 4 hemangioendothehomas, and 2 an-giosarcomas. Their data was retrospectively reviewed. There were 4 males and 3 females, with the average age of 42.6 years, range from 19 to 65 years old. The lesions were located in the vertebral body (1 case) and extremities (6 cases). One patient was multiple lesions and the others were solitary. All patients suffered with local pain, and showed osteolytic lesions in X-ray, two of them associated with sofe tissue tumor. The patient, whose vertebral body was involved, underwent marginal resection and postoperative radiation. Among the ex-tremity patients, one patient with multiple lesions had not accepted any treatment, and the other 5 patients received wide resection or amputation. The diagnosis of all patients was confirmed by pathology. All patients were followed up 1-89 months (average 40 months). Results Among the patients, two died during the fol-low-up, one died due to lung or liver metastases at the 23rd month after the operation, and the other unrelat-ed to the disease three years after the operation. One recurred in the soft tissue six years after operation and got local resection. The untreated patient was alive with the tumor and lung metastases. The other patients remained disease free during follow-up. Conclusion The key of diagnosis is histological examination, there are some specific radiographic appearances help diagnose malignant vascular tumor of bone. It is essential to perform wide resection for malignant tumor of blood vessels, adjuvant radiotherapy could be considered for spinal patients with currottagc. The hemangioendothelioma seems to have better chnical outcome than an-giosarcoma.%目的 回顾性分析临床上少见的血管源性恶性骨肿瘤的诊断

  12. 12例肿瘤性骨软化症的临床诊治及术后随访%Clinical management and postoperative follow up of 12 patients with tumor-induced osteomalacia

    Institute of Scientific and Technical Information of China (English)

    巴建明; 杜锦; 郭清华; 谷伟军; 金楠; 桑艳红; 陆菊明; 母义明; 窦京涛; 吕朝晖; 王先令; 杨国庆; 欧阳金枝


    .8±9.6) years (20 to 56 years). The course of disease was from 2 to 14 years ( median course 4.0 years). They all presented with bone pain, gait disturbance, muscle pain, and muscle weakness. Serum phosphate( Pi)levels were low in 12 cases with a range from 0.30 to 0.56 mmol/L. 99mTc-Oct was performed in 9 cases and it showed that the lesions were located in head of femur, fibula, retrocalcaneal area, foot, humerus,metacarpal, posterior chest wall or near nasal bone (apex partis petrosae ossis temporalis). Subcutaneous soft tissue mass was found in another 3 cases at loin, thigh, and foot by physical examination. The tumors were confirmed by CT, MRI or ultrasonography. Twelve patients underwent operation to remove the tumors and histopathology showed hemangioendothelioma or fibrous angioma (6 cases), giant cell tumor or fibroma of tendon sheath(4 cases), liposarcoma(1case), and phosphaturic mesenchymal tumor(1case). Serum Pi levels returned to normal in 10 patients after resection of tumor. During 2 to 64 months follow up, symptoms of bone pain and muscle weakness were improved obviously. Conclusions Patients with hypophosphatemic osteomalacia should be thoroughly investigated for TIO. 99mTc-Oct and other imaging examinations can effectively locate the tumors. Once the hidden tumor is found and excised, the patient will recover and enjoy normal life with normalized Pi concentrations and marked improvement of symptoms.

  13. An analysis of therapeutic effects of one-stage posterior total en bloc spondylectomy for primary and metastatic tumors of the thoracolumbar spine%后路I期全脊椎整块切除治疗胸腰椎原发和转移性肿瘤的疗效分析

    Institute of Scientific and Technical Information of China (English)

    栗向东; 王臻; 郭征; 李靖; 范宏斌; 付军; 陈国景; 石磊


    Objective To evaluate the clinical outcomes of one-stage posterior total en bloc spondylectomy ( TES ) in the treatment of primary and metastatic tumors of the thoracolumbar spine. Methods From January 2007 to July 2012, 21 patients with primary and metastatic tumors of the thoracolumbar spine underwent TES, whose clinical data were retrospectively analyzed. There were 11 males and 10 females, whose average age was 47.8 years old ( range;21-66 years ). There were 8 cases of primary tumors and 13 cases of solitary metastatic tumors. Among the 8 cases of primary tumors, there were 3 cases of giant cell tumors of bone, 2 cases of plasmacytoma, 1 case of osteosarcoma, 1 case of chondrosarcoma and 1 case of epithelioid hemangioendothelioma. Among the 13 cases of metastatic tumors, there were 4 cases of breast cancer, 3 cases of lung cancer, 2 cases of renal cancer, 1 case of thyroid cancer, 1 case of prostate cancer, 1 case of bladder cancer and 1 case of unknown adenocarcinoma. The tumors were located from T3 to L3, including the thoracic vertebra ( n=12 ) and the lumbar vertebra ( n=9 ). The tumors were detected in the single vertebral body in 19 cases and multiple vertebral bodies ( 3 adjacent vertebral bodies ) in 2 cases. According to Tomita surgical classiifcation system, there were 5 cases of type I, 10 cases of type II, 1 case of type III, 2 cases of type V and 3 cases of type VI. All the patients had intractable back pain or neurologic disturbances before the operation. Vertebral bone defects were repaired, and internal ifxation and reconstruction were performed with titanium mesh, allograft or autograft and pedical screws. The operation was completed in all patients through the posterior approach at the ifrst stage. Results The mean operation time was 6.4 hr ( range;4.0-8.5 hr ). The average amount of blood loss was 4500 ml ( range;1300-11 600 ml ). Perioperative complications occurred in 2 cases, including 1 case of pleural effusion and pulmonary infection

  14. Application of preoperative CT-guided Hook-wire localization of pulmonary nodules in thoracoscopic lobectomy of lung%CT引导下带钩钢丝定位行肺部磨玻璃影结节全腔镜下肺切除术28例分析

    Institute of Scientific and Technical Information of China (English)

    束余声; 赵伟刚; 石维平; 陆世春


    Objective To evaluate the application of preoperative CT-guided Hook-wire localization of pulmonary nodules in thoracoscopic lobectomy of lung.Method Twenty eight patients with 31 pulmonary nodules of ground-glass opacity (GGO) detected by CT scan were admitted in our hospital from December 2008 to December 2013,including 17 males and 11 females with an average age of 55.The lesions were located by CT-guided Hook-wire technique,and lobectomy plus lymphadenectomy or pulmonary wedge resection were performed according to the results of fast frozen pathology.The characteristics of pulmonary nodules and general condition of the patients were analyzed.Results Among 31 nodules,19 were malignant and 12 benign with an average size of (1.8 ± 0.6) cm and thickness of (1.2-± 0.4) cm.The vertical distance from pleural to nodules was (1.5 ± 0.7) cm and the insert depth of needle was (2.5 ± 0.9) cm.The time for CT-guided Hook-wire localization was (16.5 ± 5.2) min.The operation time for pulmonary wedge resection and pulmonary lobectomy was (18.5 ± 5.5) min and (54.7 ± 12.5) min,respectively.Intraoperative frozen section showed malignant tumors in 18 patients (19 GGO nodules),lobectomy plus lymphadenectomy were performed in 16 cases; 2 patients did not undergo pulmonary lobectomy because of poor physical condition or 2 GGO nodules in different lobes.There were no operation complications in this series.Postoperative pathologic findings of 31 nodules showed 9 cases of adenocarcinoma,10 bronchioloalveolar carcinoma,5 atypical adenomatous hyperplasia,2 epithelioid hemangioendothelioma,2 chronic inflammation,1 hamartoma,1 inflammatory pseudotumor and 1 sarcoidosis.The average postoperative hospital stay was (6.6 ± 1.9) days.Conclusions Endoscopic resection of pulmonary GGO nodules with CT-guided Hook-wire localization technique is feasible and safe,with less complications and less time consuming.%目的 探讨术前CT引导下Hook-wire定位在全腔镜下肺部磨玻璃影(GGO