@prefix rdf: . @prefix ns1: . rdf:type ns1:CitationPaper , ns1:RelevantPaper , ns1:ReferencePaper . @prefix rdfs: . rdfs:seeAlso , , , . @prefix bibo: . bibo:cites , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , ; ns1:cocitationWith , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , ; ns1:hasRelevantBibliographicResourceOf _:b555871580 , _:b555871581 , _:b555871582 , _:b555871583 , _:b555871588 , _:b555871589 , _:b555871590 , _:b555871591 , _:b555871584 , _:b555871585 , _:b555871586 , _:b555871587 , _:b555871596 , _:b555871597 , _:b555871598 , _:b555871599 , _:b555871592 , _:b555871593 , _:b555871594 , _:b555871595 , _:b555871604 , _:b555871605 , _:b555871606 , _:b555871607 , _:b555871600 , _:b555871601 , _:b555871602 , _:b555871603 , _:b555871612 , _:b555871613 , _:b555871614 , _:b555871615 , _:b555871608 , _:b555871609 , _:b555871610 , _:b555871611 , _:b555871620 , _:b555871621 , _:b555871622 , _:b555871623 , _:b555871616 , _:b555871617 , _:b555871618 , _:b555871619 , _:b555871628 , _:b555871629 , _:b555871630 , _:b555871631 , _:b555871624 , _:b555871625 , _:b555871626 , _:b555871627 , _:b555871636 , _:b555871637 , _:b555871638 , _:b555871639 , _:b555871632 , _:b555871633 , _:b555871634 , _:b555871635 , _:b555871644 , _:b555871645 , _:b555871646 , _:b555871647 , _:b555871640 , _:b555871641 , _:b555871642 , _:b555871643 , _:b555871652 , _:b555871653 , _:b555871654 , _:b555871655 , _:b555871648 , _:b555871649 , _:b555871650 , _:b555871651 , _:b555871660 , _:b555871661 , _:b555871662 , _:b555871663 , _:b555871656 , _:b555871657 , _:b555871658 , _:b555871659 , _:b555871668 , _:b555871669 , _:b555871670 , _:b555871671 , _:b555871664 , _:b555871665 , _:b555871666 , _:b555871667 , _:b555871676 , _:b555871677 , _:b555871678 , _:b555871679 , _:b555871672 , _:b555871673 , _:b555871674 , _:b555871675 , _:b555871684 , _:b555871685 , _:b555871686 , _:b555871687 , _:b555871680 , _:b555871681 , _:b555871682 , _:b555871683 , _:b555871692 , _:b555871693 , _:b555871694 , _:b555871695 , _:b555871688 , _:b555871689 , _:b555871690 , _:b555871691 , _:b555871700 , _:b555871701 , _:b555871702 , _:b555871703 , _:b555871696 , _:b555871697 , _:b555871698 , _:b555871699 , _:b555871708 , _:b555871709 , _:b555871710 , _:b555871711 , _:b555871704 , _:b555871705 , _:b555871706 , _:b555871707 , _:b555871716 , _:b555871717 , _:b555871718 , _:b555871719 , _:b555871712 , _:b555871713 , _:b555871714 , _:b555871715 , _:b555871724 , _:b555871725 , _:b555871726 , _:b555871727 , _:b555871720 , _:b555871721 , _:b555871722 , _:b555871723 , _:b555871732 , _:b555871733 , _:b555871734 , _:b555871735 , _:b555871728 , _:b555871729 , _:b555871730 , _:b555871731 , _:b555871740 , _:b555871741 , _:b555871742 , _:b555871743 , _:b555871736 , _:b555871737 , _:b555871738 , _:b555871739 , _:b555871748 , _:b555871749 , _:b555871750 , _:b555871751 , _:b555871744 , _:b555871745 , _:b555871746 , _:b555871747 , _:b555871756 , _:b555871757 , _:b555871752 , _:b555871753 , _:b555871754 , _:b555871755 ; ns1:pmcid "PMC0" ; bibo:doi "10.1186%2F1746-1596-9-131" . @prefix ns4: . ns4:contains _:b32410448 , _:b32410389 , _:b32410385 , _:b32410387 . _:b32410385 rdf:type ns4:Section . @prefix dc: . _:b32410385 dc:title "results" ; ns4:contains _:b32410386 . _:b32410386 rdf:type ns1:Context ; rdf:value "According to the criteria by Deyrup et al. [>>4<<], 12 tumors were classified as high risk with a tumor size >3\u00A0cm in 9 lesions and a high mitotic rate (>3/50 HPF) in 4 instances including one tumor which showed both." ; ns1:mentions . _:b32410387 rdf:type ns4:Section ; dc:title "methods" ; ns4:contains _:b32410388 . _:b32410388 rdf:type ns1:Context ; rdf:value "Case 8 was already included in the series by Antonescu et al. [>>10<<]. The study was performed in accordance with the Code of Conduct of the Federation of Medical Scientific Societies in the Netherlands and Germany." ; ns1:mentions . _:b32410389 rdf:type ns4:Section ; dc:title "discussion" ; ns4:contains _:b32410432 , _:b32410433 , _:b32410434 , _:b32410435 , _:b32410436 , _:b32410437 , _:b32410438 , _:b32410439 , _:b32410440 , _:b32410441 , _:b32410442 , _:b32410443 , _:b32410444 , _:b32410445 , _:b32410446 , _:b32410447 , _:b32410416 , _:b32410417 , _:b32410418 , _:b32410419 , _:b32410420 , _:b32410421 , _:b32410422 , _:b32410423 , _:b32410424 , _:b32410425 , _:b32410426 , _:b32410427 , _:b32410428 , _:b32410429 , _:b32410430 , _:b32410431 , _:b32410400 , _:b32410401 , _:b32410402 , _:b32410403 , _:b32410404 , _:b32410405 , _:b32410406 , _:b32410407 , _:b32410408 , _:b32410409 , _:b32410410 , _:b32410411 , _:b32410412 , _:b32410413 , _:b32410414 , _:b32410415 , _:b32410390 , _:b32410391 , _:b32410392 , _:b32410393 , _:b32410394 , _:b32410395 , _:b32410396 , _:b32410397 , _:b32410398 , _:b32410399 . _:b32410390 rdf:type ns1:Context ; rdf:value "Similar to other translocation associated tumors, the t (1;3)(p36;q23-25) or t(11;X)(q13;p11) translocations are seemingly early causative events in EHE oncogenesis [5,>>11<<] which initiate a novel transcription program in cells with endothelial properties [5]." ; ns1:mentions . _:b32410391 rdf:type ns1:Context ; rdf:value "The corresponding fusion proteins, WWTR1-CAMTA1 and YAP1-TFE3, may serve as chimeric transcription factors, which manifest their oncogenic function via a promoter switch [5,>>12<<]. Another possible oncogenic effect could be due to the loss of regulatory domains of the C-terminus of WWTR1 or YAP1 and the N-terminus of CAMTA1 or TFE3[5]." ; ns1:mentions . _:b32410392 rdf:type ns1:Context ; rdf:value "WWTR1 on 3q23-24, encodes a transcriptional co-activator involved in mesenchymal stem cell differentiation and is highly expressed in endothelial cells [5,>>12<<], while CAMTA1, a calmodulin-binding transcription activator, located on 1p36.23, has been proposed as an oncogene under the control of the WWTR1 promotor." ; ns1:mentions . _:b32410393 rdf:type ns1:Context ; rdf:value "Alternatively, the YAP1-TFE3 in-frame fusion was recently found in a small and distinct subset of EHE [>>10<<]." ; ns1:mentions . _:b32410394 rdf:type ns1:Context ; rdf:value "YAP1, located on 11q13, is a member of the FAT-family genes similar to WWTR1, and encodes for another WW-domain containing transcriptional co-activator [>>10<<]. The protein of TFE3, located on Xp11.22, is a member of the microphtalmia transcription factor family with oncogenic properties in several tumor types [10,13]." ; ns1:mentions . _:b32410395 rdf:type ns1:Context ; rdf:value "The protein of TFE3, located on Xp11.22, is a member of the microphtalmia transcription factor family with oncogenic properties in several tumor types [>>10<<,13]." ; ns1:mentions . _:b32410396 rdf:type ns1:Context ; rdf:value "The protein of TFE3, located on Xp11.22, is a member of the microphtalmia transcription factor family with oncogenic properties in several tumor types [10,>>13<<]." ; ns1:mentions . _:b32410397 rdf:type ns1:Context ; rdf:value "Although these gene fusions have not been identified in other neoplasms, in particular other epithelioid vascular tumors, it is not clear yet whether they are unique to this entity [5,>>9<<]." ; ns1:mentions . _:b32410398 rdf:type ns1:Context ; rdf:value "The known WWTR1 breakpoints are in intron 3 and 4, and the CAMTA1 breakpoints are located in intron 7 and 8, and at different sites within exon 9 [5,>>9<<]. The resulting fusion-transcript variants which we found in our study were exon 4-exon 8 (n\u2009=\u200913), exon 4-exon 9 (n\u2009=\u20097) and exon 3-exon 9 (n\u2009=\u20093) (listed in decreasing frequency)." ; ns1:mentions . _:b32410399 rdf:type ns1:Context ; rdf:value "One of them (Case 8) was included in the study by Antonescu et al. [>>10<<], in which the gene fusion was demonstrated by FISH." ; ns1:mentions . _:b32410400 rdf:type ns1:Context ; rdf:value "This case was not only remarkable because of the histomorphological characteristics as reported in the mentioned paper [>>10<<] but also because it occured in a lymph node as the primary site." ; ns1:mentions . _:b32410401 rdf:type ns1:Context ; rdf:value "A more frequently occurring metastasis should be excluded in such cases [>>2<<,14]. In contrast, the other case with a YAP1-TFE3 fusion showed a classical morphology of a multifocal lung EHE (Case 31)." ; ns1:mentions . _:b32410402 rdf:type ns1:Context ; rdf:value "A more frequently occurring metastasis should be excluded in such cases [2,>>14<<]. In contrast, the other case with a YAP1-TFE3 fusion showed a classical morphology of a multifocal lung EHE (Case 31)." ; ns1:mentions . _:b32410403 rdf:type ns1:Context ; rdf:value "Interestingly, these 2 cases involved adolescents/young adults in concert with the finding of young age reported by Antonescu et al. [>>10<<]." ; ns1:mentions . _:b32410404 rdf:type ns1:Context ; rdf:value "TFE3 rearrangements are also present in alveolar soft part sarcoma, certain pediatric renal cancers and a subset of PEComas [>>15<<-18]. The use of an antibody against the C-terminal portion of TFE3 seems to be a useful diagnostic tool in all rearranged tumors [10,19], but one has to be aware of an unspecific staining pattern [17] as we found in a proportion of cases" ; ns1:mentions , , , . _:b32410405 rdf:type ns1:Context ; rdf:value "The use of an antibody against the C-terminal portion of TFE3 seems to be a useful diagnostic tool in all rearranged tumors [>>10<<,19], but one has to be aware of an unspecific staining pattern [17] as we found in a proportion of cases with WWTR1-CAMTA1 fusion." ; ns1:mentions . _:b32410406 rdf:type ns1:Context ; rdf:value "The use of an antibody against the C-terminal portion of TFE3 seems to be a useful diagnostic tool in all rearranged tumors [10,>>19<<], but one has to be aware of an unspecific staining pattern [17] as we found in a proportion of cases with WWTR1-CAMTA1 fusion." ; ns1:mentions . _:b32410407 rdf:type ns1:Context ; rdf:value "The use of an antibody against the C-terminal portion of TFE3 seems to be a useful diagnostic tool in all rearranged tumors [10,19], but one has to be aware of an unspecific staining pattern [>>17<<] as we found in a proportion of cases with WWTR1-CAMTA1 fusion." ; ns1:mentions . _:b32410408 rdf:type ns1:Context ; rdf:value "In addition to CD31, these 2 markers are helpful in highlighting the vascular nature of EHE [>>10<<,20,21] and showed expression in all cases examined." ; ns1:mentions . _:b32410409 rdf:type ns1:Context ; rdf:value "In addition to CD31, these 2 markers are helpful in highlighting the vascular nature of EHE [10,>>20<<,21] and showed expression in all cases examined." ; ns1:mentions . _:b32410410 rdf:type ns1:Context ; rdf:value "In addition to CD31, these 2 markers are helpful in highlighting the vascular nature of EHE [10,20,>>21<<] and showed expression in all cases examined." ; ns1:mentions . _:b32410411 rdf:type ns1:Context ; rdf:value "These markers are of course expressed in all other vascular lesions [>>8<<,20,21], therefore, detection of the aforementioned fusion genes may be a valuable discriminatory tool in diagnosing EHE, especially in difficult cases." ; ns1:mentions . _:b32410412 rdf:type ns1:Context ; rdf:value "These markers are of course expressed in all other vascular lesions [8,>>20<<,21], therefore, detection of the aforementioned fusion genes may be a valuable discriminatory tool in diagnosing EHE, especially in difficult cases." ; ns1:mentions . _:b32410413 rdf:type ns1:Context ; rdf:value "These markers are of course expressed in all other vascular lesions [8,20,>>21<<], therefore, detection of the aforementioned fusion genes may be a valuable discriminatory tool in diagnosing EHE, especially in difficult cases." ; ns1:mentions . _:b32410414 rdf:type ns1:Context ; rdf:value "It seems that higher mitotic activity (>3/50 HPF) and tumor size exceeding 3\u00A0cm are associated with higher mortality, irrespective of anatomic site, presence of cytological atypia, tumor cell spindling, or necrosis [>>4<<]. Due to the small number of our cases with available clinical data, we were able to show only tendencies for the low-risk and high-risk category. Furthermore, in skin lesions, a favorable outcome is well known similar to all other" ; ns1:mentions . _:b32410415 rdf:type ns1:Context ; rdf:value "Furthermore, in skin lesions, a favorable outcome is well known similar to all other sarcomas at this site [>>2<<]. Whether it is useful to include lung-, liver- and bone lesions in this risk stratification scheme remains uncertain so far, especially with respect to multifocal occurrence (or early metastases) [9]." ; ns1:mentions . _:b32410416 rdf:type ns1:Context ; rdf:value "Whether it is useful to include lung-, liver- and bone lesions in this risk stratification scheme remains uncertain so far, especially with respect to multifocal occurrence (or early metastases) [>>9<<]." ; ns1:mentions . _:b32410417 rdf:type ns1:Context ; rdf:value "such as epithelioid hemangioma, cutaneous epithelioid angiomatous nodule, epithelioid angiosarcoma and pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma, PM-H) can enter the differential diagnoses [>>1<<,2,8,22-24]. In bone, chondrosarcoma can also be a potential pitfall." ; ns1:mentions . _:b32410418 rdf:type ns1:Context ; rdf:value "such as epithelioid hemangioma, cutaneous epithelioid angiomatous nodule, epithelioid angiosarcoma and pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma, PM-H) can enter the differential diagnoses [1,>>2<<,8,22-24]. In bone, chondrosarcoma can also be a potential pitfall." ; ns1:mentions . _:b32410419 rdf:type ns1:Context ; rdf:value "such as epithelioid hemangioma, cutaneous epithelioid angiomatous nodule, epithelioid angiosarcoma and pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma, PM-H) can enter the differential diagnoses [1,2,>>8<<,22-24]. In bone, chondrosarcoma can also be a potential pitfall." ; ns1:mentions . _:b32410420 rdf:type ns1:Context ; rdf:value "such as epithelioid hemangioma, cutaneous epithelioid angiomatous nodule, epithelioid angiosarcoma and pseudomyogenic hemangioendothelioma (epithelioid sarcoma-like hemangioendothelioma, PM-H) can enter the differential diagnoses [1,2,8,>>22<<-24]. In bone, chondrosarcoma can also be a potential pitfall." ; ns1:mentions , , . _:b32410421 rdf:type ns1:Context ; rdf:value "Epithelioid hemangioma occurring in skin, soft tissue and bone is distinguished by a lobular architecture of well-formed vessels with a pericytic cuff, highlighted by ASMA/MSA (muscle specific actin) immunohistochemical reaction [>>1<<,22,25,26]. In the center of the lesion, the plump epithelioid cells are sometimes rather tightly packed and arranged in solid sheets simulating a more aggressive tumor." ; ns1:mentions . _:b32410422 rdf:type ns1:Context ; rdf:value "Epithelioid hemangioma occurring in skin, soft tissue and bone is distinguished by a lobular architecture of well-formed vessels with a pericytic cuff, highlighted by ASMA/MSA (muscle specific actin) immunohistochemical reaction [1,>>22<<,25,26]. In the center of the lesion, the plump epithelioid cells are sometimes rather tightly packed and arranged in solid sheets simulating a more aggressive tumor." ; ns1:mentions . _:b32410423 rdf:type ns1:Context ; rdf:value "Epithelioid hemangioma occurring in skin, soft tissue and bone is distinguished by a lobular architecture of well-formed vessels with a pericytic cuff, highlighted by ASMA/MSA (muscle specific actin) immunohistochemical reaction [1,22,>>25<<,26]. In the center of the lesion, the plump epithelioid cells are sometimes rather tightly packed and arranged in solid sheets simulating a more aggressive tumor." ; ns1:mentions . _:b32410424 rdf:type ns1:Context ; rdf:value "The distinctive zonation pattern with peripheral maturation is a helpful finding [>>25<<,26]. A myxohyaline stroma is not a feature of epithelioid hemangioma. As in EHE, vascular invasion can occur. Multifocality of epithelioid hemangioma with involvement of soft tissue and/or bone, skin and lymph nodes should not be confused" ; ns1:mentions . _:b32410425 rdf:type ns1:Context ; rdf:value "Multifocality of epithelioid hemangioma with involvement of soft tissue and/or bone, skin and lymph nodes should not be confused with metastases of EHE [26,>>27<<]." ; ns1:mentions . _:b32410426 rdf:type ns1:Context ; rdf:value "This lesion lacks cords and strands of tumor cells in a myxohyaline or fibrous stroma which is seen in EHE [>>22<<]." ; ns1:mentions . _:b32410427 rdf:type ns1:Context ; rdf:value "Epithelioid cells with eosinophilic cytoplasm are present to a variable extent in approximately 70% of angiosarcomas (AS) of soft tissue [>>23<<] and, of course, they can be prominent in AS at other sites as bone, skin and parenchymatous organs." ; ns1:mentions . _:b32410428 rdf:type ns1:Context ; rdf:value "Keratins can be expressed in both EHE and angiosarcomas (in ca. 30% of the cases, respectively) [>>23<<]." ; ns1:mentions . _:b32410429 rdf:type ns1:Context ; rdf:value "Expression of keratins is an overlapping feature, although CD34 and pan-keratin MNF116 are lacking in PM-H [>>8<<,24]. Recently, SERPINE1-FOSB, has been identified as a specific fusion gene in PM-H [28]." ; ns1:mentions . _:b32410430 rdf:type ns1:Context ; rdf:value "Expression of keratins is an overlapping feature, although CD34 and pan-keratin MNF116 are lacking in PM-H [8,>>24<<]. Recently, SERPINE1-FOSB, has been identified as a specific fusion gene in PM-H [28]." ; ns1:mentions . _:b32410431 rdf:type ns1:Context ; rdf:value "Recently, SERPINE1-FOSB, has been identified as a specific fusion gene in PM-H [>>28<<]." ; ns1:mentions . _:b32410432 rdf:type ns1:Context ; rdf:value "Carcinomas tend to manifest as more pleomorphic, mitotically active tumors [>>1<<] evoking a desmoplastic response and usually exhibit stronger keratin expression whereas endothelial markers are absent." ; ns1:mentions . _:b32410433 rdf:type ns1:Context ; rdf:value "However, cords and strands embedded in a (chondro) myxoid/hyaline matrix is a shared feature and cytoplasmic vacuolation can occur [>>29<<]. Endothelial markers are absent in myoepithelial tumors. S-100 and keratins, usually expressed in myoepithelial tumors [29], can also be occasionally positive in EHE as demonstrated in two of our cases [2]. EWSR1 and PLAG rearrangements" ; ns1:mentions . _:b32410434 rdf:type ns1:Context ; rdf:value "S-100 and keratins, usually expressed in myoepithelial tumors [>>29<<], can also be occasionally positive in EHE as demonstrated in two of our cases [2]." ; ns1:mentions . _:b32410435 rdf:type ns1:Context ; rdf:value "S-100 and keratins, usually expressed in myoepithelial tumors [29], can also be occasionally positive in EHE as demonstrated in two of our cases [>>2<<]. EWSR1 and PLAG rearrangements are characteristic genetic changes in myoepithelial tumors [30-33]." ; ns1:mentions . _:b32410436 rdf:type ns1:Context ; rdf:value "EWSR1 and PLAG rearrangements are characteristic genetic changes in myoepithelial tumors [>>30<<-33]." ; ns1:mentions , , , . _:b32410437 rdf:type ns1:Context ; rdf:value "Although some vacuolated cells can be observed, the cytoplasm is more eosinophilic [>>1<<,34] and vascular markers are not expressed." ; ns1:mentions . _:b32410438 rdf:type ns1:Context ; rdf:value "Although some vacuolated cells can be observed, the cytoplasm is more eosinophilic [1,>>34<<] and vascular markers are not expressed." ; ns1:mentions . _:b32410439 rdf:type ns1:Context ; rdf:value "Although some vacuolated cells can be observed, the cytoplasm is more eosinophilic [1,34] and vascular markers are not expressed. NR4A3 rearrangement is a pathognomonic genetic aberration [>>35<<]." ; ns1:mentions . _:b32410440 rdf:type ns1:Context ; rdf:value "Vacuolated cells can possibly be confused with intracytoplasmic lumina of EHE [>>36<<]. When the matrix is myxoid, there are often still prominent collagen bundles [37], a feature which is not seen in the myxohyaline stroma of EHE. Immunohistochemically, this tumor type typically shows keratin- and EMA-expression, loss of" ; ns1:mentions . _:b32410441 rdf:type ns1:Context ; rdf:value "When the matrix is myxoid, there are often still prominent collagen bundles [>>37<<], a feature which is not seen in the myxohyaline stroma of EHE." ; ns1:mentions . _:b32410442 rdf:type ns1:Context ; rdf:value "Immunohistochemically, this tumor type typically shows keratin- and EMA-expression, loss of INI1 [>>38<<] and occasionally a membranous CD31 staining [39]." ; ns1:mentions . _:b32410443 rdf:type ns1:Context ; rdf:value "CD34 and D2-40 can be either positive or negative in both tumor types [>>40<<-42]. Interestingly, ERG and FLI1 (antibody to the N-terminus) expression was recently shown in up to 70% and more than 90% of ES, respectively [40,41]." ; ns1:mentions , . _:b32410444 rdf:type ns1:Context ; rdf:value "Interestingly, ERG and FLI1 (antibody to the N-terminus) expression was recently shown in up to 70% and more than 90% of ES, respectively [>>40<<,41]. Therefore, these markers can only be interpreted in the right context together with CD31 and INI1. The latter is retained in EHE [38] as shown in some of our cases." ; ns1:mentions . _:b32410445 rdf:type ns1:Context ; rdf:value "Interestingly, ERG and FLI1 (antibody to the N-terminus) expression was recently shown in up to 70% and more than 90% of ES, respectively [40,>>41<<]. Therefore, these markers can only be interpreted in the right context together with CD31 and INI1. The latter is retained in EHE [38] as shown in some of our cases." ; ns1:mentions . _:b32410446 rdf:type ns1:Context ; rdf:value "Therefore, these markers can only be interpreted in the right context together with CD31 and INI1. The latter is retained in EHE [>>38<<] as shown in some of our cases." ; ns1:mentions . _:b32410447 rdf:type ns1:Context ; rdf:value "Although pankeratin and D2-40 can be positive in both malignancies, as also demonstrated in a number of our cases, calretinin, keratin 5/6, keratin 7, and WT1 are typically distinguishing mesotheliomas [>>43<<]." ; ns1:mentions . _:b32410448 rdf:type ns4:Section ; dc:title "background" ; ns4:contains _:b32410452 , _:b32410453 , _:b32410454 , _:b32410455 , _:b32410449 , _:b32410450 , _:b32410451 , _:b32410456 , _:b32410457 . _:b32410449 rdf:type ns1:Context ; rdf:value "Epithelioid hemangioendothelioma (EHE), first described by Weiss and Enzinger in 1982, is a malignant vascular neoplasm with indolent behavior in the majority of cases [>>1<<]. Although, a progressive clinical course with tumor-related fatality has been documented in some instances, this lesion does not behave as aggressively as a conventional angiosarcoma [1-4]." ; ns1:mentions . _:b32410450 rdf:type ns1:Context ; rdf:value "Although, a progressive clinical course with tumor-related fatality has been documented in some instances, this lesion does not behave as aggressively as a conventional angiosarcoma [>>1<<-4]." ; ns1:mentions , , , . _:b32410451 rdf:type ns1:Context ; rdf:value "EHEs arise in soft tissue, bone, skin and various parenchymatous organs [>>1<<-7]." ; ns1:mentions , , , , , . _:b32410452 rdf:type ns1:Context ; rdf:value "This is complicated by the bland epithelioid cytomorphology and the presence of subtle, mostly intracytoplasmic, vascular lumina [>>1<<,2]. Classically, the vacuolated endothelial cells are arranged in short cords and strands recapitulating the primitive angiogenic cords of the yolk sac [8]." ; ns1:mentions . _:b32410453 rdf:type ns1:Context ; rdf:value "This is complicated by the bland epithelioid cytomorphology and the presence of subtle, mostly intracytoplasmic, vascular lumina [1,>>2<<]. Classically, the vacuolated endothelial cells are arranged in short cords and strands recapitulating the primitive angiogenic cords of the yolk sac [8]." ; ns1:mentions . _:b32410454 rdf:type ns1:Context ; rdf:value "Classically, the vacuolated endothelial cells are arranged in short cords and strands recapitulating the primitive angiogenic cords of the yolk sac [>>8<<]." ; ns1:mentions . _:b32410455 rdf:type ns1:Context ; rdf:value "After discovering the nonrandom reciprocal t (1;3)(p36;q25) translocation [>>3<<], the corresponding fusion gene WWTR1-CAMTA1 was detected [5,9]. More recently, an alternative gene fusion, YAP1-TFE3, was found in a small subset of lesions with distinct morphology and arising mainly in young patients [10]." ; ns1:mentions . _:b32410456 rdf:type ns1:Context ; rdf:value "After discovering the nonrandom reciprocal t (1;3)(p36;q25) translocation [3], the corresponding fusion gene WWTR1-CAMTA1 was detected [5,>>9<<]. More recently, an alternative gene fusion, YAP1-TFE3, was found in a small subset of lesions with distinct morphology and arising mainly in young patients [10]." ; ns1:mentions . _:b32410457 rdf:type ns1:Context ; rdf:value "More recently, an alternative gene fusion, YAP1-TFE3, was found in a small subset of lesions with distinct morphology and arising mainly in young patients [>>10<<]." ; ns1:mentions . _:b555871580 rdf:type ns1:RelevantBibliographicResource . @prefix xsd: . _:b555871580 ns1:RelevantScore "16"^^xsd:nonNegativeInteger ; ns1:hasRelevantPaperId . _:b555871581 rdf:type ns1:RelevantBibliographicResource ; ns1:RelevantScore "12"^^xsd:nonNegativeInteger ; ns1:hasRelevantPaperId . _:b555871582 rdf:type ns1:RelevantBibliographicResource ; ns1:RelevantScore "11"^^xsd:nonNegativeInteger ; ns1:hasRelevantPaperId . _:b555871583 rdf:type ns1:RelevantBibliographicResource ; ns1:RelevantScore "11"^^xsd:nonNegativeInteger ; ns1:hasRelevantPaperId . _:b555871584 rdf:type ns1:RelevantBibliographicResource ; 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